Myocardial and Pericardial Disease

Question 1

What is cardiomyopathy?

Answer 1

A group of diseases affecting the heart muscle (myocardium), leading to mechanical and/or electrical dysfunction.

Cardiomyopathy can lead to heart failure, arrhythmias, thromboembolism, or sudden cardiac death.

Question 2

How is cardiomyopathy typically classified?

Answer 2

Based on structural and functional abnormalities.

It may be classified as primary (genetic, mixed, or acquired) or secondary to systemic diseases.

Question 3

What is dilated cardiomyopathy (DCM)?

Answer 3

A subtype of cardiomyopathy characterized by dilation of the left or both ventricles with systolic dysfunction in the absence of abnormal loading conditions.

This includes conditions like hypertension or valve disease.

Question 4

What are the key epidemiological factors for dilated cardiomyopathy?

Answer 4

Prevalence, age, sex, ethnicity, and family history.

Approximately 1 in 250 individuals are affected, most commonly diagnosed between 20 and 60 years.

Question 5

What is the prevalence of dilated cardiomyopathy?

Answer 5

Approximately 1 in 250 individuals.

It is one of the most common forms of cardiomyopathy.

Question 6

At what age is dilated cardiomyopathy most often diagnosed?

Answer 6

Between 20 and 60 years of age.

This age range indicates a significant impact on young to middle-aged adults.

Question 7

Is dilated cardiomyopathy more common in males or females?

Answer 7

More common in males than females.

Question 8

Which ethnic group has a higher incidence of dilated cardiomyopathy?

Answer 8

Black populations compared to White populations.

This group also experiences more severe progression of the disease.

Question 9

What percentage of dilated cardiomyopathy cases have a familial or genetic basis?

Answer 9

Up to 30–50% of cases.

This includes genetic mutations such as titin gene mutations.

Question 10

What are some causes or risk factors for dilated cardiomyopathy?

Answer 10

Genetic mutations, viral myocarditis, alcohol abuse, chemotherapy, peripartum cardiomyopathy, autoimmune and metabolic disorders.

Examples of genetic mutations include TTN and LMNA.

Question 11

Fill in the blank: Dilated cardiomyopathy is characterized by dilation of the left or both ventricles with _______.

Answer 11

systolic dysfunction (reduced ejection fraction).

Question 12

True or False: Dilated cardiomyopathy can occur in the presence of significant coronary artery disease.

Answer 12

False.

DCM is characterized by systolic dysfunction in the absence of abnormal loading conditions.

Question 13

What is the key feature of Dilated Cardiomyopathy (DCM)?

Answer 13

Dilation of the ventricles with systolic dysfunction (reduced ejection fraction)

DCM is characterized by an enlarged heart and reduced ability to pump blood effectively.

Question 14

What is the effect of Dilated Cardiomyopathy (DCM)?

Answer 14

Impaired contraction and decreased cardiac output

This leads to symptoms of heart failure.

Question 15

What are common causes of Dilated Cardiomyopathy (DCM)?

Answer 15

• Genetic mutations
• Alcohol
• Viral myocarditis
• Peripartum
• Toxins

These factors can contribute to the development of DCM.

Question 16

What are the complications associated with Dilated Cardiomyopathy (DCM)?

Answer 16

• Heart failure with reduced ejection fraction (HFrEF)
• Arrhythmias
• Thromboembolism

These complications can significantly impact patient health.

Question 17

What is the key feature of Hypertrophic Cardiomyopathy (HCM)?

Answer 17

Unexplained left ventricular hypertrophy (often asymmetric), usually with preserved systolic function

HCM leads to thickening of the heart muscle.

Question 18

What is the effect of Hypertrophic Cardiomyopathy (HCM)?

Answer 18

Impaired relaxation and diastolic dysfunction, often with dynamic outflow tract obstruction

This can cause issues with blood flow out of the heart.

Question 19

What are common causes of Hypertrophic Cardiomyopathy (HCM)?

Answer 19

• Genetic mutations in sarcomeric proteins (e.g., β-myosin heavy chain)

These genetic factors are critical in HCM development.

Question 20

What are the complications of Hypertrophic Cardiomyopathy (HCM)?

Answer 20

• Sudden cardiac death (especially in young athletes)
• Syncope
• Angina

These complications highlight the risks associated with HCM.

Question 21

What is the key feature of Restrictive Cardiomyopathy (RCM)?

Answer 21

Normal ventricular size with markedly reduced compliance (stiff ventricular walls)

RCM leads to restrictions in heart filling.

Question 22

What is the effect of Restrictive Cardiomyopathy (RCM)?

Answer 22

Severe diastolic dysfunction with relatively preserved systolic function

Patients may experience symptoms of heart failure despite normal ejection fraction.

Question 23

What are common causes of Restrictive Cardiomyopathy (RCM)?

Answer 23

• Amyloidosis
• Hemochromatosis
• Radiation
• Endomyocardial fibrosis

These conditions can lead to the development of RCM.

Question 24

What are the complications associated with Restrictive Cardiomyopathy (RCM)?

Answer 24

• Right-sided heart failure
• Atrial enlargement
• Arrhythmias

These complications can severely affect patient outcomes.

Question 25

What is the key feature of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

Answer 25

Fibrofatty replacement of right ventricular myocardium ## Footnote This leads to structural and electrical changes in the heart.

Question 26

What is the effect of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

Answer 26

Electrical instability and arrhythmias ## Footnote This can lead to life-threatening arrhythmias.

Question 27

What are common causes of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

Answer 27

* Genetic (e.g., desmosomal protein mutations) ## Footnote Genetic predisposition plays a significant role in ARVC.

Question 28

What are the complications associated with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

Answer 28

* Ventricular arrhythmias * Sudden cardiac death (especially in young adults) ## Footnote These complications highlight the serious risks associated with ARVC.

Question 29

What is the etiology of dilated cardiomyopathy (DCM)?

Answer 29

Diverse, classified into genetic, acquired, and idiopathic causes ## Footnote Up to 50% of cases have a known or suspected cause, while the remainder are idiopathic.

Question 30

What percentage of dilated cardiomyopathy cases are attributed to genetic causes?

Answer 30

30–50% ## Footnote Autosomal dominant inheritance is most common.

Question 31

Name two common genes associated with genetic causes of dilated cardiomyopathy.

Answer 31

* TTN (titin) * LMNA (lamin A/C) * MYH7 (β-myosin heavy chain) * DES (desmin) ## Footnote TTN is the most frequent gene affected.

Question 32

What are some infectious causes of dilated cardiomyopathy?

Answer 32

* Viral myocarditis (Coxsackie B virus, adenovirus, parvovirus B19, HIV) * Chagas disease (Trypanosoma cruzi) * Other infections (bacterial, fungal, parasitic) ## Footnote Chagas disease is endemic in Latin America.

Question 33

What is the most common toxic cause of dilated cardiomyopathy in developed countries?

Answer 33

Alcohol abuse ## Footnote Other toxic causes include chemotherapeutic agents, cocaine, amphetamines, and heavy metals.

Question 34

List three nutritional deficiencies that can lead to dilated cardiomyopathy.

Answer 34

* Thiamine (beriberi) * Selenium * Carnitine ## Footnote Diabetes mellitus and thyroid disease are also metabolic causes.

Question 35

What are some autoimmune and inflammatory causes of dilated cardiomyopathy?

Answer 35

* Systemic lupus erythematosus (SLE) * Sarcoidosis * Rheumatoid arthritis * Giant cell myocarditis ## Footnote These conditions can cause inflammation of the heart muscle.

Question 36

What is peripartum cardiomyopathy?

Answer 36

Occurs during the last trimester or within 5 months postpartum ## Footnote Involves inflammation, autoimmunity, or angiogenic imbalance.

Question 37

What can lead to tachycardia-induced cardiomyopathy?

Answer 37

Persistent supraventricular tachycardia or atrial fibrillation ## Footnote This condition can lead to reversible DCM.

Question 38

What characterizes idiopathic dilated cardiomyopathy?

Answer 38

No identifiable cause despite thorough evaluation ## Footnote Often presumed genetic.

Question 39

True or False: The majority of dilated cardiomyopathy cases have a known cause.

Answer 39

False ## Footnote Up to 50% of cases are idiopathic.

Question 40

Fill in the blank: The most common acquired cause of dilated cardiomyopathy is _______.

Answer 40

[alcohol abuse]

Question 41

What leads to systolic heart failure in DCM?

Answer 41

Impaired contractility and ventricular dilation

Question 42

What are the symptoms of left-sided heart failure?

Answer 42

* Dyspnea on exertion * Orthopnea * Paroxysmal nocturnal dyspnea (PND) * Fatigue and weakness * Cough

Question 43

What are the symptoms of right-sided heart failure?

Answer 43

* Peripheral edema * Abdominal discomfort * Ascites * Jugular venous distention

Question 44

What are some other symptoms associated with heart failure?

Answer 44

* Palpitations * Syncope or presyncope * Chest pain

Question 45

What does a displaced apex beat indicate?

Answer 45

Ventricular enlargement

Question 46

What does an S3 gallop indicate?

Answer 46

Volume overload and systolic dysfunction

Question 47

What physical exam sign indicates pulmonary congestion?

Answer 47

Crackles/rales

Question 48

What does elevated JVP indicate?

Answer 48

Right heart failure

Question 49

What is hepatomegaly related to in heart failure?

Answer 49

Congestive hepatopathy

Question 50

What complications can arise from DCM?

Answer 50

* Arrhythmias * Sudden cardiac death * Thromboembolism * Progressive heart failure * Cardiogenic shock

Question 51

True or False: Peripheral edema is a symptom of left-sided heart failure.

Answer 51

False

Question 52

Fill in the blank: Dyspnea on exertion is an _______ symptom of left-sided heart failure.

Answer 52

early

Question 53

What may chest pain in heart failure mimic?

Answer 53

Angina

Question 54

What is a common arrhythmia associated with DCM?

Answer 54

Atrial fibrillation

Question 55

What type of shock can occur in advanced cases of heart failure?

Answer 55

Cardiogenic shock

Question 56

What is the primary method for diagnosing DCM?

Answer 56

Clinical evaluation supported by imaging, ECG, labs, and exclusion of secondary causes.

Question 57

What are common symptoms in the clinical evaluation of DCM?

Answer 57

History of progressive heart failure symptoms.

Question 58

What are some risk factors for DCM?

Answer 58

* Family history * Alcohol * Chemotherapy * Viral illness * Postpartum state

Question 59

What are the key findings on an ECG for DCM?

Answer 59

Non-specific: sinus tachycardia, LBBB, ST-T changes, Q waves, arrhythmias.

Question 60

What does a chest X-ray typically show in DCM?

Answer 60

Cardiomegaly and pulmonary congestion.

Question 61

What are the diagnostic cornerstones of an echocardiogram in DCM?

Answer 61

* Dilated LV (± RV) * Globally reduced LV systolic function (↓ EF) * Functional MR/TR * Thin ventricular walls

Question 62

What does cardiac MRI confirm in DCM?

Answer 62

Ventricular dilation and fibrosis (late gadolinium enhancement).

Question 63

What do elevated BNP / NT-proBNP levels indicate?

Answer 63

Marker of heart failure.

Question 64

Why is cardiac catheterization performed in DCM?

Answer 64

To rule out ischemic heart disease (normal coronaries in DCM).

Question 65

When is endomyocardial biopsy used in DCM?

Answer 65

Rarely used; reserved for suspected myocarditis or infiltrative disease.

Question 66

When is genetic testing considered in DCM?

Answer 66

When familial DCM is suspected.

Question 67

What is a gross pathology feature of DCM related to chamber dilation?

Answer 67

Especially of the left ventricle; all four chambers may be dilated.

Question 68

What does 'flabby myocardium' indicate in DCM?

Answer 68

Poor contractility.

Question 69

What can the wall thickness appear as in DCM?

Answer 69

May appear normal or thin despite large chamber size.

Question 70

Why are mural thrombi common in DCM?

Answer 70

Due to stasis in dilated ventricles.

Question 71

What leads to functional regurgitation in DCM?

Answer 71

Mitral/tricuspid annular dilation.

Question 72

What is a microscopic feature of DCM related to myocytes?

Answer 72

Myocyte hypertrophy with enlarged, irregular nuclei due to increased workload.

Question 73

What does myofiber atrophy or dropout indicate in DCM?

Answer 73

Loss of myocytes in advanced stages.

Question 74

What type of fibrosis is observed in DCM?

Answer 74

Interstitial and endocardial fibrosis, replacement of necrotic myocytes with fibrous tissue.

Question 75

What is seen in the histological analysis of DCM regarding inflammation?

Answer 75

Scattered inflammatory cells indicating mild chronic inflammation, especially in post-viral cases.

Question 76

Fill in the blank: Summary mnemonic for DCM is 'Dilated = ______, ______, ______.'

Answer 76

Deflated, Droopy, Dysfunctional.

Question 77

What are the general measures for managing heart failure?

Answer 77

Lifestyle changes: * Sodium restriction * Fluid restriction (in advanced cases) * Smoking and alcohol cessation * Regular, monitored exercise (cardiac rehab) * Treat underlying cause if known (e.g., alcohol-induced, viral myocarditis) ## Footnote General measures aim to improve heart function and overall health.

Question 78

What is the pharmacological treatment for Heart Failure with Reduced Ejection Fraction (HFrEF)?

Answer 78

Drug Class and Examples: * ACE inhibitors / ARBs: Enalapril, Losartan * ARNI: Sacubitril/valsartan * Beta-blockers: Bisoprolol, Carvedilol, Metoprolol succinate * Mineralocorticoid antagonists: Spironolactone, Eplerenone * Loop diuretics: Furosemide * SGLT2 inhibitors: Dapagliflozin, Empagliflozin * Digoxin: Consider in persistent symptoms or AF ## Footnote Each drug class has specific benefits, including mortality reduction and symptom relief.

Question 79

What is the benefit of ACE inhibitors and ARBs in HFrEF?

Answer 79

↓ Mortality, ↓ afterload ## Footnote ACE inhibitors and ARBs are vital in reducing the burden on the heart.

Question 80

What is the indication for ICD (implantable cardioverter defibrillator) device therapy?

Answer 80

EF < 35% and high risk of sudden cardiac death ## Footnote ICDs are crucial for preventing sudden cardiac death in high-risk patients.

Question 81

What are the indications for anticoagulation in heart failure?

Answer 81

For patients with: * Atrial fibrillation * Previous thromboembolism * LV thrombus * Severe LV dysfunction (sometimes considered prophylactically) ## Footnote Anticoagulation helps prevent thromboembolic events.

Question 82

What advanced therapies are available for heart failure?

Answer 82

Advanced Therapies: * Left ventricular assist device (LVAD): Bridge to transplant or destination therapy * Heart transplantation: For end-stage refractory heart failure ## Footnote These therapies are often considered for patients with severe heart failure.

Question 83

What is the median survival after diagnosis of DCM without transplant?

Answer 83

Median survival ~5–10 years ## Footnote Prognosis can vary significantly based on individual circumstances.

Question 84

What are poor prognostic indicators in DCM?

Answer 84

Poor prognostic indicators: * EF < 25% * NYHA Class III/IV * Persistent arrhythmias * Elevated BNP / NT-proBNP * Non-response to therapy * RV dysfunction ## Footnote These indicators are associated with higher mortality and poorer outcomes.

Question 85

True or False: Patients with DCM can experience recovery with therapy if the etiology is treatable.

Answer 85

True ## Footnote Treatable causes, such as peripartum or alcohol-related DCM, can lead to recovery.

Question 86

Fill in the blank: The new standard therapy for heart failure is __________.

Answer 86

[SGLT2 inhibitors] ## Footnote SGLT2 inhibitors have emerged as a crucial option in treating heart failure.

Question 87

What is hypertrophic cardiomyopathy (HCM)?

Answer 87

A genetic heart muscle disorder characterized by unexplained left ventricular hypertrophy, often involving the interventricular septum ## Footnote Occurs in the absence of secondary causes such as hypertension or aortic stenosis.

Question 88

What are common associations with HCM?

Answer 88

• Myofiber disarray • Diastolic dysfunction • ± Left ventricular outflow tract (LVOT) obstruction • Increased risk of sudden cardiac death (SCD) ## Footnote These associations highlight the complications and risks associated with HCM.

Question 89

What is the prevalence of HCM in the general population?

Answer 89

Affects ~1 in 500 people (0.2% of general population) ## Footnote This indicates that HCM is relatively uncommon but significant in its impact.

Question 90

At what age is HCM typically diagnosed?

Answer 90

Typically diagnosed between teens and mid-30s, but can present at any age ## Footnote This variability in onset age underscores the need for awareness across all age groups.

Question 91

What is the sex distribution for HCM?

Answer 91

Slight male predominance ## Footnote This suggests that males may be more frequently affected by the condition.

Question 92

What type of genetic inheritance is associated with HCM?

Answer 92

Autosomal dominant inheritance with variable penetrance ## Footnote This means that a single copy of the mutated gene can cause the disorder, but not all individuals with the mutation will express the disease.

Question 93

Name common mutations associated with HCM.

Answer 93

• MYH7 (β-myosin heavy chain) • MYBPC3 (myosin-binding protein C) • TNNT2 (troponin T) ## Footnote These mutations are linked to the structural proteins of the heart muscle.

Question 94

What percentage of HCM cases have a positive family history?

Answer 94

Positive in ~50–60% of cases ## Footnote This highlights the hereditary nature of the condition.

Question 95

Which populations are considered high-risk for HCM?

Answer 95

Athletes, particularly with undiagnosed HCM ## Footnote This group is at increased risk of sudden cardiac death.

Question 96

What is the most common cause of sudden cardiac death in young athletes?

Answer 96

Hypertrophic cardiomyopathy (HCM) ## Footnote Awareness of HCM is crucial in the context of sports and athletic participation.

Question 97

Can HCM be asymptomatic?

Answer 97

Yes, HCM may remain asymptomatic for years ## Footnote This can delay diagnosis and treatment.

Question 98

What are the two forms of HCM?

Answer 98

• Non-obstructive • Obstructive (HOCM: hypertrophic obstructive cardiomyopathy) ## Footnote The obstructive form can lead to more severe symptoms and complications.

Question 99

What is the primary cause of hypertrophic cardiomyopathy?

Answer 99

Genetic disorder caused by mutations in sarcomeric proteins ## Footnote Hypertrophic cardiomyopathy is primarily caused by genetic mutations affecting heart muscle proteins.

Question 100

What inheritance pattern does hypertrophic cardiomyopathy follow?

Answer 100

Autosomal dominant ## Footnote This means that only one copy of the mutated gene is sufficient for the disease to manifest.

Question 101

What does variable expression and incomplete penetrance mean in the context of hypertrophic cardiomyopathy?

Answer 101

Not all individuals with a mutation will show symptoms or the same severity of disease ## Footnote This indicates that the manifestation of the disease can vary widely among affected individuals.

Question 102

Name a common gene involved in hypertrophic cardiomyopathy.

Answer 102

MYH7 – β-myosin heavy chain ## Footnote Other common genes include MYBPC3, TNNT2, TNNI3, ACTC1, and TPM1.

Question 103

What are the mechanisms leading to hypertrophic cardiomyopathy?

Answer 103

Abnormal contractile proteins → inefficient contraction → compensatory hypertrophy → disorganized myocyte architecture and fibrosis ## Footnote This sequence of events describes how the disease progresses at a cellular level.

Question 104

What percentage of patients with hypertrophic cardiomyopathy have no identifiable family history?

Answer 104

30–40% ## Footnote These sporadic cases may arise from de novo mutations or unrecognized family involvement.

Question 105

What is Fabry disease and how does it relate to hypertrophic cardiomyopathy?

Answer 105

X-linked lysosomal storage disorder; LVH + neuropathy, renal involvement ## Footnote Fabry disease can mimic the symptoms of hypertrophic cardiomyopathy.

Question 106

What condition is characterized by neurological symptoms and cardiomyopathy that can mimic hypertrophic cardiomyopathy?

Answer 106

Friedreich ataxia ## Footnote This is an autosomal recessive disorder that can present similarly to HCM.

Question 107

Which infiltrative disease results in concentric left ventricular thickening and may mimic hypertrophic cardiomyopathy?

Answer 107

Amyloidosis ## Footnote It is important to differentiate this condition from true hypertrophic cardiomyopathy.

Question 108

What is Noonan syndrome and how does it relate to hypertrophic cardiomyopathy?

Answer 108

Genetic syndrome with short stature, facial dysmorphism, congenital heart disease ## Footnote It can present with features that mimic those of hypertrophic cardiomyopathy.

Question 109

What is athlete’s heart and how does it differ from hypertrophic cardiomyopathy?

Answer 109

Physiological hypertrophy; reversible, symmetrical, no fibrosis ## Footnote Athlete's heart is a normal adaptation to exercise, unlike the pathological changes in HCM.

Question 110

What is a mnemonic to remember key aspects of hypertrophic cardiomyopathy?

Answer 110

HCM is a Hereditary Cardiac Mutation ## Footnote This mnemonic captures the genetic nature and structural changes associated with the condition.

Question 111

Fill in the blank: Hypertrophic cardiomyopathy is primarily caused by mutations in _______.

Answer 111

sarcomeric proteins ## Footnote These mutations lead to the characteristic features of the disease.

Question 112

What is the clinical presentation of Hypertrophic Cardiomyopathy (HCM)?

Answer 112

Highly variable; ranges from asymptomatic individuals to sudden cardiac death (SCD), particularly in young athletes.

Question 113

What is the most common symptom of HCM?

Answer 113

Dyspnea on exertion ## Footnote Due to diastolic dysfunction and elevated filling pressures.

Question 114

What causes angina in patients with HCM?

Answer 114

Increased oxygen demand of hypertrophied myocardium and reduced coronary perfusion.

Question 115

What are the possible causes of syncope or presyncope in HCM?

Answer 115

LVOT obstruction, arrhythmias, or abnormal baroreceptor response.

Question 116

What are palpitations in the context of HCM due to?

Answer 116

Atrial fibrillation or ventricular tachyarrhythmias.

Question 117

What leads to fatigue in patients with HCM?

Answer 117

Reduced cardiac output, especially with exertion.

Question 118

What may be the first presentation of HCM in young athletes?

Answer 118

Sudden cardiac death ## Footnote Usually from ventricular arrhythmia.

Question 119

What is a harsh crescendo–decrescendo systolic murmur indicative of in HCM?

Answer 119

Heard best at left lower sternal border; increases with Valsalva or standing.

Question 120

What does a bifid carotid pulse indicate in HCM?

Answer 120

Dynamic obstruction.

Question 121

What causes the S4 heart sound in HCM?

Answer 121

Forceful atrial contraction against a stiff ventricle.

Question 122

What is a double apical impulse associated with in HCM?

Answer 122

Forceful atrial kick and hyperdynamic contraction.

Question 123

What is a common complication of HCM related to left atrial dilation?

Answer 123

Atrial fibrillation ## Footnote Increases stroke risk.

Question 124

What is the leading cause of death in young athletes with HCM?

Answer 124

Sudden cardiac death (SCD) due to ventricular arrhythmias.

Question 125

What type of heart failure is primarily associated with HCM?

Answer 125

Diastolic heart failure ## Footnote May progress to systolic dysfunction in late stages.

Question 126

What can stroke or systemic embolism in HCM often be related to?

Answer 126

Atrial fibrillation and left atrial thrombus.

Question 127

Is infective endocarditis common in HCM?

Answer 127

No, it is rare but can affect abnormal mitral valve in HCM.

Question 128

True or False: Sudden cardiac death is a red flag in HCM that may occur during exercise.

Answer 128

True.

Question 129

What is the main basis for the diagnosis of HCM?

Answer 129

Clinical suspicion, family history, and imaging, particularly echocardiography or cardiac MRI.

Question 130

What symptoms should be evaluated in the clinical assessment of HCM?

Answer 130

History of exertional dyspnea, syncope, palpitations, or family history of sudden cardiac death.

Question 131

What physical exam findings are associated with HCM?

Answer 131

Murmur that increases with Valsalva, double apical impulse, S4 sound.

Question 132

What does LVH stand for in the context of ECG findings?

Answer 132

Left ventricular hypertrophy.

Question 133

Which ECG finding is especially common in inferior and lateral leads?

Answer 133

Deep Q waves.

Question 134

What does atrial enlargement indicate in HCM?

Answer 134

Due to impaired ventricular filling.

Question 135

What types of arrhythmias may be seen in HCM?

Answer 135

Atrial fibrillation (AF), ventricular tachycardia (VT).

Question 136

What is the main diagnostic tool for HCM?

Answer 136

Echocardiogram.

Question 137

What is a key finding of asymmetric septal hypertrophy in HCM?

Answer 137

Septal wall >15 mm, often ≥1.3–1.5× thicker than posterior wall.

Question 138

What leads to LVOT obstruction in HCM?

Answer 138

Systolic anterior motion (SAM) of mitral valve.

Question 139

What characterizes the left ventricle (LV) cavity in HCM?

Answer 139

Small LV cavity with preserved or hyperdynamic systolic function.

Question 140

What causes mitral regurgitation in HCM?

Answer 140

Due to systolic anterior motion (SAM).

Question 141

What type of dysfunction is observed in HCM?

Answer 141

Diastolic dysfunction with impaired relaxation and compliance.

Question 142

What is the role of cardiac MRI in diagnosing HCM?

Answer 142

More sensitive than echo for detecting focal hypertrophy and myocardial fibrosis.

Question 143

When is genetic testing used in HCM?

Answer 143

For confirmation and family screening.

Question 144

Which gene mutations are commonly involved in genetic testing for HCM?

Answer 144

MYH7, MYBPC3.

Question 145

What does exercise stress testing assess in HCM patients?

Answer 145

Functional capacity and provoked LVOT gradient.

Question 146

What morphological feature is the most common pattern seen in gross pathology of HCM?

Answer 146

Asymmetric septal hypertrophy.

Question 147

What is a characteristic shape of the LV cavity due to HCM?

Answer 147

Banana-shaped LV cavity.

Question 148

What happens to the LV chamber in HCM?

Answer 148

Small LV chamber with thick walls and reduced compliance.

Question 149

What can be seen at autopsy or surgical specimens in HCM?

Answer 149

Systolic anterior motion (SAM) of mitral valve.

Question 150

What may form in the left atrium or ventricle due to stasis in dilated atria?

Answer 150

Mural thrombi.

Question 151

What is a classic microscopic feature of HCM?

Answer 151

Myofiber disarray.

Question 152

What does myocyte hypertrophy involve in HCM?

Answer 152

Enlarged nuclei and thickened fibers.

Question 153

What can contribute to arrhythmogenic potential in HCM?

Answer 153

Interstitial fibrosis.

Question 154

What does thickened intramural arteries in HCM lead to?

Answer 154

Small vessel ischemia.

Question 155

Fill in the blank: The mnemonic for morphology in HCM is '_______'.

Answer 155

SHaMiF.

Question 156

What does 'SHaMiF' stand for in HCM morphology?

Answer 156

* Septal hypertrophy (asymmetric) * Hypercontractile LV * anterior mitral valve motion (SAM) * Myocyte disarray * Fibrosis (interstitial)

Question 157

What is the primary focus of HCM management?

Answer 157

Symptom control, prevention of sudden cardiac death (SCD), and lifestyle modification

Question 158

What factors determine the management approach for HCM?

Answer 158

Whether the patient has obstructive (HOCM) or non-obstructive disease and presence of high-risk features

Question 159

What lifestyle measure prevents exacerbation of LVOT obstruction?

Answer 159

Avoid dehydration and excessive exertion

Question 160

Why should vasodilators and diuretics be avoided in obstructive HCM?

Answer 160

They reduce preload and worsen gradient

Question 161

What screening is recommended for first-degree relatives of HCM patients?

Answer 161

ECG and echocardiography

Question 162

What activity restriction is advised for high-risk HCM patients?

Answer 162

Advised against competitive sports due to SCD risk

Question 163

What is the first-line medication class for HCM management?

Answer 163

Beta-blockers

Question 164

List two examples of beta-blockers used in HCM treatment.

Answer 164

* Metoprolol * Atenolol

Question 165

What alternative medication class can be used instead of beta-blockers?

Answer 165

Non-dihydropyridine CCBs

Question 166

Name two examples of non-dihydropyridine CCBs.

Answer 166

* Verapamil * Diltiazem

Question 167

What is the role of disopyramide in HCM treatment?

Answer 167

Negative inotrope for HOCM, often used with β-blocker or CCB

Question 168

What are antiarrhythmics used for in HCM?

Answer 168

For atrial fibrillation

Question 169

Name two examples of antiarrhythmics.

Answer 169

* Amiodarone * Sotalol

Question 170

What is the purpose of anticoagulation in HCM?

Answer 170

For AF or left atrial thrombus

Question 171

What are two examples of anticoagulants used in HCM?

Answer 171

* DOACs * Warfarin

Question 172

What is the gold standard procedure for refractory HOCM?

Answer 172

Surgical septal myectomy

Question 173

What is alcohol septal ablation?

Answer 173

Less invasive option; infarcts basal septum

Question 174

What is dual-chamber pacing (DDD) used for?

Answer 174

Rarely used; reduces gradient in some patients

Question 175

What are the indications for an Implantable Cardioverter-Defibrillator (ICD)?

Answer 175

* Previous cardiac arrest or sustained VT * Family history of SCD * Unexplained syncope * Massive LVH (≥30 mm) * Non-sustained VT on Holter * Abnormal exercise BP response

Question 176

What is the prognosis for HCM with treatment?

Answer 176

Generally good; normal life expectancy for many

Question 177

What is the risk of SCD in the general population with HCM?

Answer 177

0.5–1% per year

Question 178

What factors lead to better outcomes in HCM?

Answer 178

* No obstruction * Good functional capacity * Absence of arrhythmias

Question 179

What factors indicate a poorer prognosis in HCM?

Answer 179

* Multiple high-risk features (e.g., VT, family history of SCD, EF <50%)

Question 180

How does AF development affect HCM prognosis?

Answer 180

Increases risk of stroke and heart failure

Question 181

What may progressive symptoms in HCM lead to?

Answer 181

Heart failure with preserved or reduced EF (burned-out HCM)

Question 182

What is a key prognosis pearl regarding patients with HCM?

Answer 182

Many patients live normal lives with medical therapy

Question 183

Why is early identification of high-risk features important in HCM?

Answer 183

Allows for life-saving ICD placement

Question 184

What is the general outcome for most deaths in HCM?

Answer 184

Most deaths are preventable with appropriate monitoring and treatment

Question 185

What is restrictive cardiomyopathy (RCM)?

Answer 185

A form of heart muscle disease characterized by impaired ventricular filling due to abnormally stiff ventricular walls with normal or near-normal systolic function and wall thickness.

Question 186

What is a key consequence of RCM on the heart's function?

Answer 186

Diastolic dysfunction, atrial enlargement, and eventually heart failure.

Question 187

How does RCM differ from hypertrophic or dilated cardiomyopathy?

Answer 187

Ventricular size and wall thickness are usually preserved in RCM unless infiltrated.

Question 188

What age group is primarily affected by RCM?

Answer 188

Adults over 40 years, although some forms occur in children.

Question 189

What is the rarity of RCM compared to other major cardiomyopathies?

Answer 189

Least common of the three major cardiomyopathies (DCM, HCM, RCM).

Question 190

Is there a strong sex predilection for RCM?

Answer 190

No strong sex predilection; it varies by underlying cause.

Question 191

What is the most common cause of RCM worldwide?

Answer 191

Endomyocardial fibrosis, especially in tropical regions (Africa, India, South America).

Question 192

What is the most common cause of RCM in developed countries?

Answer 192

Amyloidosis.

Question 193

Which condition is more common in men regarding RCM?

Answer 193

Hemochromatosis.

Question 194

What factors affect the prevalence of sarcoidosis related to RCM?

Answer 194

Ethnicity and region.

Question 195

Why is the incidence of RCM difficult to estimate?

Answer 195

Due to underdiagnosis and misclassification as constrictive pericarditis or HFpEF.

Question 196

What are common infiltrative causes of RCM?

Answer 196

Amyloidosis, sarcoidosis.

Question 197

Name two storage diseases associated with RCM.

Answer 197

Hemochromatosis, Fabry disease.

Question 198

What are endomyocardial causes of RCM?

Answer 198

Endomyocardial fibrosis, Loeffler endocarditis (eosinophilic), radiation fibrosis.

Question 199

What is an idiopathic cause of RCM?

Answer 199

No identifiable cause in some cases.

Question 200

What is the primary effect of restrictive cardiomyopathy on the heart?

Answer 200

Impairment of diastolic filling while usually preserving systolic function

Question 201

How are the causes of restrictive cardiomyopathy broadly classified?

Answer 201

Infiltrative, storage, endomyocardial, and other systemic or idiopathic categories

Question 202

What is the most common cause of RCM in developed countries?

Answer 202

Infiltrative diseases

Question 203

What mechanism causes amyloidosis to lead to RCM?

Answer 203

Extracellular deposition of amyloid fibrils stiffens the myocardium

Question 204

What is the effect of sarcoidosis on the myocardium?

Answer 204

Granulomatous infiltration causes fibrosis and stiffness

Question 205

Name a malignancy that can cause restrictive cardiomyopathy.

Answer 205

Metastatic cancer or leukemia

Question 206

What characterizes storage disorders in the context of RCM?

Answer 206

Inherited metabolic disorders causing deposition of abnormal substances in myocardial cells

Question 207

What condition is associated with iron overload leading to myocardium damage?

Answer 207

Hemochromatosis

Question 208

What is Fabry disease?

Answer 208

An X-linked lysosomal storage disorder characterized by buildup of globotriaosylceramide

Question 209

Give an example of a glycogen storage disease that can cause RCM.

Answer 209

Pompe disease (type II)

Question 210

What is endomyocardial fibrosis (EMF)?

Answer 210

Most common cause of RCM worldwide; prevalent in tropical regions, often related to malnutrition, infection, or eosinophilia

Question 211

What leads to fibrosis in Loeffler endocarditis?

Answer 211

Toxic eosinophilic infiltration

Question 212

What can cause radiation-induced fibrosis in the myocardium?

Answer 212

Thoracic radiotherapy

Question 213

Name a type of drug that can cause myocardial fibrosis.

Answer 213

Chemotherapeutic agents like anthracyclines

Question 214

What systemic condition is known to cause myocardial fibrosis?

Answer 214

Scleroderma

Question 215

What can lead to myocardial fibrosis post-chemotherapy?

Answer 215

Direct myocardial injury

Question 216

What is graft-versus-host disease?

Answer 216

A rare cause of myocardial fibrosis that occurs after bone marrow transplant

Question 217

What is meant by idiopathic in the context of RCM?

Answer 217

No identifiable cause despite extensive work-up

Question 218

What mnemonic can be used to remember the major categories of RCM causes?

Answer 218

"I SEE My Heart Restrict"

Question 219

What does 'I' represent in the mnemonic for RCM causes?

Answer 219

Infiltrative (e.g., Amyloidosis, Sarcoidosis)

Question 220

What does 'S' represent in the mnemonic for RCM causes?

Answer 220

Storage (e.g., Hemochromatosis, Fabry)

Question 221

What does 'E' represent in the mnemonic for RCM causes?

Answer 221

Endomyocardial (e.g., EMF, Loeffler)

Question 222

What does 'M' represent in the mnemonic for RCM causes?

Answer 222

My (Myeloproliferative disorders)

Question 223

What does 'H' represent in the mnemonic for RCM causes?

Answer 223

Heart (Systemic autoimmune diseases)

Question 224

Fill in the blank: RCM stands for _______.

Answer 224

Restrictive cardiomyopathy

Question 225

What is the primary characteristic of restrictive cardiomyopathy?

Answer 225

Impaired diastolic filling due to stiff, noncompliant ventricular walls.

Question 226

What function is typically preserved until late in restrictive cardiomyopathy?

Answer 226

Systolic function.

Question 227

What do clinical features of restrictive cardiomyopathy primarily reflect?

Answer 227

Diastolic heart failure and systemic congestion.

Question 228

Name two common complications associated with restrictive cardiomyopathy.

Answer 228

* Arrhythmias * Thromboembolic complications

Question 229

What is the most common initial symptom of restrictive cardiomyopathy?

Answer 229

Exertional dyspnea.

Question 230

What causes fatigue and weakness in patients with restrictive cardiomyopathy?

Answer 230

Low cardiac output and poor perfusion.

Question 231

What symptom is indicative of right-sided congestion in restrictive cardiomyopathy?

Answer 231

Peripheral edema.

Question 232

What is a common cause of abdominal discomfort/fullness in restrictive cardiomyopathy?

Answer 232

Hepatic congestion or ascites.

Question 233

What symptom may occur due to pulmonary venous congestion in restrictive cardiomyopathy?

Answer 233

Orthopnea and paroxysmal nocturnal dyspnea (PND).

Question 234

Palpitations in restrictive cardiomyopathy are often due to what condition?

Answer 234

Atrial fibrillation.

Question 235

What less common symptom may indicate arrhythmias or low stroke volume?

Answer 235

Syncope or presyncope.

Question 236

What sign on physical examination is characterized by elevated jugular venous pressure (JVP)?

Answer 236

Prominent v waves due to impaired right ventricular filling.

Question 237

What is the Kussmaul sign and its significance in restrictive cardiomyopathy?

Answer 237

↑ JVP on inspiration, a classic RCM finding.

Question 238

What condition might cause hepatomegaly in restrictive cardiomyopathy?

Answer 238

Congestive hepatopathy.

Question 239

What causes peripheral edema in restrictive cardiomyopathy?

Answer 239

Right-sided heart failure.

Question 240

What advanced symptom may occur in restrictive cardiomyopathy?

Answer 240

Ascites.

Question 241

What do S3 or S4 gallops indicate in the context of restrictive cardiomyopathy?

Answer 241

* Stiff ventricles (S4) * Rapid early filling (S3)

Question 242

What is pulsus paradoxus and when might it be present in restrictive cardiomyopathy?

Answer 242

Sometimes present if pericardial involvement coexists.

Question 243

What complication is common due to biatrial enlargement in restrictive cardiomyopathy?

Answer 243

Atrial fibrillation.

Question 244

What causes thromboembolism in restrictive cardiomyopathy?

Answer 244

Stasis in enlarged atria.

Question 245

What complication can arise from backward transmission of elevated LV filling pressures?

Answer 245

Pulmonary hypertension.

Question 246

What is a possible severe outcome of restrictive cardiomyopathy, especially in infiltrative causes?

Answer 246

Sudden cardiac death.

Question 247

What condition is dominant in the advanced stages of restrictive cardiomyopathy?

Answer 247

Progressive right heart failure.

Question 248

True or False: Restrictive cardiomyopathy is a pericardial disease.

Answer 248

False.

Question 249

What is critical for management differentiation in restrictive cardiomyopathy?

Answer 249

Distinguishing it from constrictive pericarditis.

Question 250

What is the main challenge in the diagnosis of RCM?

Answer 250

It shares features with other causes of diastolic heart failure, especially constrictive pericarditis.

Question 251

What combination of methods is required to confirm the diagnosis of RCM?

Answer 251

Clinical evaluation, imaging, biomarkers, and sometimes biopsy.

Question 252

What are common clinical signs of RCM?

Answer 252

Progressive dyspnea, fatigue, right-sided heart failure, and possible arrhythmias.

Question 253

Which underlying etiology might be indicated by neuropathy?

Answer 253

Amyloidosis or Fabry disease.

Question 254

What condition is indicated by iron overload?

Answer 254

Hemochromatosis.

Question 255

Name two systemic illnesses that may be associated with RCM.

Answer 255

* Sarcoidosis * Scleroderma

Question 256

What is the interpretation of low voltage QRS on an ECG?

Answer 256

Especially in amyloidosis, despite thickened walls.

Question 257

What does atrial fibrillation indicate in the context of RCM?

Answer 257

Due to biatrial enlargement.

Question 258

What is a key finding on chest X-ray for RCM?

Answer 258

Normal heart size or mild cardiomegaly.

Question 259

What does pulmonary congestion indicate in RCM?

Answer 259

Due to diastolic dysfunction and high filling pressures.

Question 260

What is the hallmark feature of RCM observed in echocardiography?

Answer 260

Biatrial enlargement.

Question 261

What does impaired diastolic filling show in echocardiography?

Answer 261

Abnormal mitral inflow Doppler (↓ E′, ↑ E/E′).

Question 262

What is indicated by a normal pericardium in echocardiography?

Answer 262

Helps distinguish from constrictive pericarditis.

Question 263

What does Tissue Doppler Imaging reveal in RCM?

Answer 263

Markedly reduced mitral annular motion (E′ velocity), confirming diastolic dysfunction.

Question 264

What is the gold standard for tissue characterization in diagnosing RCM?

Answer 264

Cardiac MRI.

Question 265

What does late gadolinium enhancement indicate in cardiac MRI?

Answer 265

Diffuse subendocardial enhancement in amyloidosis; patchy in sarcoidosis.

Question 266

What is the purpose of serum and urine electrophoresis (SPEP/UPEP)?

Answer 266

To detect light chains (AL amyloidosis).

Question 267

What is the significance of elevated troponins and BNP in RCM?

Answer 267

Elevated in myocardial strain (e.g., amyloidosis).

Question 268

What is the primary use of an endomyocardial biopsy?

Answer 268

Confirms infiltrative or inflammatory diseases (e.g., amyloidosis, sarcoidosis, eosinophilic myocarditis).

Question 269

What staining technique is used for confirming amyloidosis?

Answer 269

Congo red stain (apple-green birefringence for amyloid).

Question 270

What does cardiac catheterization help differentiate?

Answer 270

RCM from constrictive pericarditis.

Question 271

Fill in the blank: RCM shows _______ of diastolic pressures without respiratory variation.

Answer 271

Equalization

Question 272

Fill in the blank: Constrictive pericarditis shows prominent discordance of LV and RV pressures with _______.

Answer 272

Respiration

Question 273

What is the primary issue in restrictive cardiomyopathy?

Answer 273

Diastolic dysfunction from stiff ventricles

Question 274

What is the main focus of management in restrictive cardiomyopathy?

Answer 274

Symptomatic relief, treatment of the underlying cause, and prevention of complications

Question 275

What is the mainstay treatment for reducing congestion in restrictive cardiomyopathy?

Answer 275

Diuretics

Question 276

What should be avoided to maintain preload in restrictive cardiomyopathy management?

Answer 276

Over-diuresis

Question 277

What is the strategy for managing arrhythmias in restrictive cardiomyopathy?

Answer 277

Rate/rhythm control in atrial fibrillation

Question 278

When should anticoagulation be considered in restrictive cardiomyopathy?

Answer 278

If atrial fibrillation or intracardiac thrombus is present

Question 279

What caution should be taken regarding negative inotropes in restrictive cardiomyopathy?

Answer 279

Caution with beta-blockers and calcium channel blockers in some patients

Question 280

What is the specific therapy for AL type amyloidosis?

Answer 280

Chemotherapy (e.g., bortezomib), stem cell transplant

Question 281

What treatment is indicated for transthyretin amyloidosis?

Answer 281

Tafamidis (stabilizes transthyretin), supportive care

Question 282

What is the treatment approach for hemochromatosis?

Answer 282

Phlebotomy, iron chelators (e.g., deferoxamine)

Question 283

What therapy is used for Fabry disease?

Answer 283

Enzyme replacement therapy (agalsidase)

Question 284

What is the treatment for Loeffler endocarditis?

Answer 284

Corticosteroids, cytotoxic agents if hypereosinophilic

Question 285

What is the recommended treatment for sarcoidosis in restrictive cardiomyopathy?

Answer 285

Corticosteroids, immunosuppressants

Question 286

What is the management for radiation-induced restrictive cardiomyopathy?

Answer 286

Supportive care; no specific reversal therapy

Question 287

What advanced therapy is indicated for significant conduction disease?

Answer 287

Pacemaker or ICD

Question 288

When is heart transplant considered in restrictive cardiomyopathy?

Answer 288

End-stage disease unresponsive to medical therapy; must rule out systemic forms

Question 289

What factors heavily influence the prognosis of restrictive cardiomyopathy?

Answer 289

Underlying etiology, severity of diastolic dysfunction, presence of complications

Question 290

What is the prognosis of untreated AL amyloidosis?

Answer 290

Poor prognosis (median survival ~6–12 months)

Question 291

How does the prognosis of transthyretin amyloidosis compare to AL?

Answer 291

Better than AL; improved with tafamidis

Question 292

What is the prognosis for hemochromatosis if diagnosed early?

Answer 292

Good if diagnosed early and treated

Question 293

What is the prognosis of endomyocardial fibrosis?

Answer 293

Often progressive; poor in advanced cases

Question 294

What conditions are associated with worsening outcomes in restrictive cardiomyopathy?

Answer 294

Atrial fibrillation and pulmonary hypertension

Question 295

What is the impact of heart failure symptoms in restrictive cardiomyopathy?

Answer 295

Progressively disabling; right-sided failure often dominant

Question 296

What improves prognosis in restrictive cardiomyopathy?

Answer 296

If reversible cause is treated (e.g., iron overload)

Question 297

Is there a cure for most forms of restrictive cardiomyopathy?

Answer 297

No cure; supportive and cause-specific therapy is essential

Question 298

What improves outcomes in restrictive cardiomyopathy?

Answer 298

Early recognition and treatment of reversible causes

Question 299

What is an option for selected patients with restrictive cardiomyopathy?

Answer 299

Heart transplant

Question 300

What is Arrhythmogenic right ventricular cardiomyopathy (ARVC)?

Answer 300

A genetic heart muscle disease characterized by the replacement of right ventricular myocardium with fibrofatty tissue ## Footnote ARVC leads to right ventricular dysfunction, ventricular arrhythmias, and increased risk of sudden cardiac death.

Question 301

What are the main consequences of ARVC?

Answer 301

* Right ventricular dysfunction * Ventricular arrhythmias * Increased risk of sudden cardiac death (SCD) ## Footnote Especially in young individuals and athletes.

Question 302

Which ventricle is primarily affected in ARVC?

Answer 302

Right ventricle ## Footnote Biventricular and left-dominant forms are also recognized.

Question 303

What is the estimated prevalence of ARVC in the general population?

Answer 303

Approximately 1 in 1,000 to 1 in 5,000 ## Footnote This indicates it is a relatively rare condition.

Question 304

At what age does ARVC typically present?

Answer 304

Adolescents or young adults, usually in the 2nd–4th decade of life ## Footnote Early presentation is common.

Question 305

Is ARVC more common in males or females?

Answer 305

More common and severe in males ## Footnote This suggests a potential sex-based difference in disease expression.

Question 306

How is ARVC inherited?

Answer 306

In an autosomal dominant pattern with variable penetrance ## Footnote This means that not all individuals carrying the mutation will express the disease.

Question 307

What are common mutations associated with ARVC?

Answer 307

* PKP2 (plakophilin-2) * DSG2 (desmoglein-2) * DSP (desmoplakin) * JUP (junction plakoglobin) * TMEM43 ## Footnote These are desmosomal genes linked to the disease.

Question 308

Where are geographic clusters of ARVC found?

Answer 308

Certain regions like Padua, Italy, and Greek islands ## Footnote These areas have higher prevalence due to founder mutations.

Question 309

Who is considered a high-risk group for ARVC?

Answer 309

Young athletes ## Footnote Exertion can accelerate disease progression and trigger fatal arrhythmias.

Question 310

Fill in the blank: ARVC is a genetic arrhythmogenic cardiomyopathy that primarily affects the _______.

Answer 310

right ventricle

Question 311

True or False: ARVC can present with syncope or sudden death.

Answer 311

True ## Footnote This is especially common in young active males.

Question 312

What is ARVC primarily classified as?

Answer 312

A genetic disorder involving desmosomes

Question 313

What do desmosomes provide between cardiomyocytes?

Answer 313

Mechanical linkage

Question 314

What is the most common pathogenic mechanism in ARVC?

Answer 314

Mutations impair desmosomal integrity

Question 315

What are the consequences of mutations in desmosomal proteins?

Answer 315

Myocyte loss, inflammation, fibrofatty replacement

Question 316

What type of inheritance pattern is seen in ARVC?

Answer 316

Autosomal dominant with variable penetrance and expression

Question 317

Which desmosomal gene mutation is the most frequent in ARVC?

Answer 317

PKP2 – plakophilin-2

Question 318

List three other common desmosomal gene mutations associated with ARVC.

Answer 318

* DSG2 – desmoglein-2 * DSP – desmoplakin * DSC2 – desmocollin-2

Question 319

Which gene is notably associated with Newfoundland families in ARVC?

Answer 319

TMEM43 – transmembrane protein 43

Question 320

What is the relationship between exercise and ARVC progression?

Answer 320

Intense activity increases mechanical wall stress, accelerating myocyte detachment

Question 321

True or False: Exercise can unmask ARVC in genetically predisposed individuals.

Answer 321

True

Question 322

What are some rare secondary causes that can mimic ARVC?

Answer 322

* Cardiac sarcoidosis * Myocarditis * Uhl’s anomaly * RV infarction/scar

Question 323

What distinguishes cardiac sarcoidosis from ARVC?

Answer 323

Granulomas and fibrosis; usually systemic involvement

Question 324

Fill in the blank: ARVC = Arrhythmia, Right ventricle, Variable genes, _______.

Answer 324

Cell adhesion defect

Question 325

What is ARVC?

Answer 325

Arrhythmogenic Right Ventricular Cardiomyopathy, primarily an arrhythmogenic disorder affecting young adults or athletes

Question 326

What are common clinical presentations of ARVC?

Answer 326

Ventricular arrhythmias, symptoms of right-sided heart failure, sudden cardiac death

Question 327

What are the four phases of disease progression in ARVC?

Answer 327

* Concealed phase * Electrical phase * Structural phase * Biventricular phase

Question 328

Describe the concealed phase of ARVC.

Answer 328

Asymptomatic; subtle ECG or imaging changes; risk of sudden death may still be present

Question 329

What occurs during the electrical phase of ARVC?

Answer 329

Symptomatic ventricular arrhythmias (e.g. palpitations, syncope); RV function preserved

Question 330

What characterizes the structural phase of ARVC?

Answer 330

Progressive RV dilation/dysfunction, fibrofatty replacement, arrhythmias, right heart failure

Question 331

What are the symptoms of ARVC?

Answer 331

* Palpitations * Syncope or presyncope * Exertional dizziness or fatigue * Atypical chest pain * Sudden cardiac death * Right-sided heart failure

Question 332

What does palpitations in ARVC indicate?

Answer 332

From ventricular tachycardia (VT) or PVCs

Question 333

What is a common cause of syncope in ARVC?

Answer 333

Often exertional; due to VT or sudden hemodynamic changes

Question 334

What is the significance of sudden cardiac death in ARVC?

Answer 334

Frequently occurs in young athletes, may be first presentation

Question 335

What signs may be present on examination of a patient with ARVC?

Answer 335

* Jugular venous distension * Peripheral edema * S3 or S4 gallop * Displaced or abnormal apex beat

Question 336

What does jugular venous distension indicate in ARVC?

Answer 336

Due to RV dysfunction

Question 337

What are common ECG findings in ARVC?

Answer 337

* T-wave inversions in V1–V3 * Epsilon wave in V1–V3 * Ventricular arrhythmias with LBBB morphology * Prolonged S-wave upstroke in V1–V3 * Frequent PVCs or sustained VT

Question 338

What is the classic ECG sign of RV involvement in ARVC?

Answer 338

T-wave inversions in V1–V3

Question 339

What are some complications associated with ARVC?

Answer 339

* Sudden cardiac death * Heart failure (right or biventricular) * Thromboembolism * Progression to biventricular failure

Question 340

What should raise suspicion for ARVC in young patients?

Answer 340

Unexplained syncope, exercise-induced VT, family history of sudden death, ECG or echo suggesting right ventricular abnormalities

Question 341

What is the primary purpose of the Revised 2010 Task Force Criteria?

Answer 341

To diagnose ARVC using a combination of clinical, electrocardiographic, imaging, histological, genetic, and family history criteria ## Footnote ARVC stands for Arrhythmogenic Right Ventricular Cardiomyopathy.

Question 342

List the categories of diagnostic criteria for ARVC as per the 2010 Task Force.

Answer 342

* Structural/functional * Tissue characterization * ECG abnormalities * Arrhythmias * Family history ## Footnote Each category includes major and minor criteria.

Question 343

What are the key elements under the Structural/functional category for ARVC diagnosis?

Answer 343

* RV dilation * Dysfunction * Aneurysms on echo/MRI/angiography

Question 344

What histological feature is associated with ARVC as per the tissue characterization category?

Answer 344

Fibrofatty replacement seen on endomyocardial biopsy

Question 345

What ECG abnormalities are indicative of ARVC?

Answer 345

* T-wave inversions in V1–V3 * Epsilon waves * Prolonged S-wave upstroke

Question 346

What arrhythmias are associated with ARVC?

Answer 346

* VT with LBBB morphology * Frequent PVCs

Question 347

What family history criteria are considered for ARVC diagnosis?

Answer 347

ARVC in first-degree relative or known pathogenic mutation

Question 348

What is the significance of the electrocardiogram (ECG) in diagnosing ARVC?

Answer 348

It reveals T-wave inversions, epsilon waves, and ventricular tachycardia with LBBB pattern

Question 349

What are the echocardiographic findings in ARVC?

Answer 349

* RV dilation * Regional wall motion abnormalities * Reduced RV fractional area change * Aneurysm formation in the RV free wall

Question 350

What is the gold standard imaging technique for ARVC?

Answer 350

Cardiac MRI

Question 351

What does a cardiac MRI visualize in ARVC?

Answer 351

* RV wall thinning * Bulging or aneurysms * Fibrofatty infiltration * Global or regional RV dysfunction

Question 352

What is the purpose of an endomyocardial biopsy in ARVC diagnosis?

Answer 352

Histological confirmation of fibrofatty replacement of myocardium

Question 353

What are the limitations of endomyocardial biopsy in ARVC?

Answer 353

Often low sensitivity due to patchy disease and risk of RV perforation

Question 354

What genetic mutations are commonly associated with ARVC?

Answer 354

* PKP2 * DSP * DSG2 * JUP

Question 355

What is the utility of genetic testing in ARVC?

Answer 355

Useful for screening at-risk family members

Question 356

What are the gross pathological features of ARVC?

Answer 356

* Right ventricular dilation * RV wall thinning * Aneurysm formation * Biventricular involvement in later stages

Question 357

What is the diagnostic hallmark of ARVC on a microscopic level?

Answer 357

Fibrofatty replacement

Question 358

What microscopic feature involves myocyte loss in ARVC?

Answer 358

Myocyte loss and disarray due to detachment from defective desmosomes

Question 359

What mnemonic can be used to remember the morphological features of ARVC?

Answer 359

FAT-RV

Question 360

Fill in the blank: The mnemonic for ARVC Morphology is _______.

Answer 360

FAT-RV

Question 361

What does the 'F' in the mnemonic FAT-RV stand for?

Answer 361

Fibrofatty infiltration

Question 362

What does the 'A' in the mnemonic FAT-RV represent?

Answer 362

Aneurysms (RV)

Question 363

What does the 'T' in the mnemonic FAT-RV indicate?

Answer 363

Thin RV wall

Question 364

What does the 'RV' in the mnemonic FAT-RV stand for?

Answer 364

Right Ventricular dilation

Question 365

What are the main goals of ARVC management?

Answer 365

* Prevent sudden cardiac death (SCD) * Control arrhythmias * Manage right heart failure * Restrict disease progression

Question 366

What lifestyle modification should be avoided to reduce arrhythmic risk in ARVC?

Answer 366

Competitive or high-intensity exercise

Question 367

What is recommended for family screening in ARVC management?

Answer 367

First-degree relatives should undergo ECG, echo, ± genetic testing

Question 368

What is the purpose of beta-blockers in ARVC management?

Answer 368

Reduce arrhythmogenic burden, blunt sympathetic activity

Question 369

Name two examples of beta-blockers used in ARVC management.

Answer 369

* Metoprolol * Bisoprolol

Question 370

What is the role of antiarrhythmics in ARVC therapy?

Answer 370

Control ventricular arrhythmias

Question 371

What are two examples of antiarrhythmics used in ARVC?

Answer 371

* Amiodarone * Sotalol (use with caution)

Question 372

When are diuretics used in ARVC management?

Answer 372

For right-sided heart failure symptoms

Question 373

What are two examples of diuretics prescribed for ARVC?

Answer 373

* Furosemide * Spironolactone

Question 374

When is anticoagulation indicated in ARVC?

Answer 374

If atrial fibrillation or RV thrombus present

Question 375

What are two options for anticoagulation in ARVC?

Answer 375

* DOACs * Warfarin

Question 376

What is the mainstay of life-saving therapy in ARVC?

Answer 376

Implantable Cardioverter-Defibrillator (ICD)

Question 377

What is an indication for ICD implantation in ARVC?

Answer 377

Survivor of cardiac arrest or sustained VT

Question 378

What is a high-risk feature for ICD consideration in ARVC?

Answer 378

Syncope with VT on monitoring

Question 379

What indicates primary prevention for ICD in ARVC?

Answer 379

Extensive RV disease or family history of SCD

Question 380

What is the role of catheter ablation in ARVC management?

Answer 380

Used in refractory ventricular tachycardia

Question 381

What is a limitation of catheter ablation in ARVC?

Answer 381

Often a temporary solution, as the disease is progressive

Question 382

What is indicated when advanced therapies are needed in ARVC?

Answer 382

Heart transplant for end-stage biventricular failure or intractable arrhythmias not controlled by ICD/medications

Question 383

What factors influence the prognosis of ARVC?

Answer 383

* Extent of RV and LV involvement * Presence of arrhythmias * Response to treatment * Adherence to activity restrictions

Question 384

What is the leading cause of death in ARVC?

Answer 384

Sudden cardiac death

Question 385

How does ICD implantation affect survival in ARVC?

Answer 385

Improves survival by preventing SCD

Question 386

What is associated with worsening heart failure and poorer outcomes in ARVC?

Answer 386

Biventricular involvement

Question 387

What improves quality of life and reduces hospitalization in ARVC?

Answer 387

Good arrhythmia control

Question 388

What does a family history of SCD indicate in ARVC patients?

Answer 388

Indicates higher risk

Question 389

Fill in the blank: ARVC is progressive but manageable with early diagnosis, _______ changes, and ICD when needed.

Answer 389

[lifestyle]

Question 390

True or False: The risk of SCD in ARVC is significant and must be aggressively mitigated in high-risk individuals.

Answer 390

True

Question 391

What is the outlook for many patients with ARVC when managed appropriately?

Answer 391

Many patients have a favorable long-term outcome, especially if detected early

Question 392

What is pericardial effusion?

Answer 392

The accumulation of fluid (serous, blood, pus, or chyle) in the pericardial sac.

Question 393

What are the types of pericardial effusion?

Answer 393

Acute or chronic, small or large, hemodynamically insignificant or life-threatening (e.g., tamponade).

Question 394

Name the first category of causes for pericardial effusion.

Answer 394

Inflammatory (Infectious and Non-Infectious).

Question 395

What are examples of viral causes of pericardial effusion?

Answer 395

* Coxsackie B virus * Echovirus * HIV

Question 396

What bacterial infection is commonly associated with pericardial effusion in endemic areas?

Answer 396

Tuberculosis.

Question 397

List autoimmune diseases that can cause pericardial effusion.

Answer 397

* Systemic lupus erythematosus (SLE) * Rheumatoid arthritis * Scleroderma * Sjögren’s

Question 398

What is Dressler syndrome?

Answer 398

An immune-mediated condition that can cause pericardial effusion post-myocardial infarction (MI).

Question 399

What is the cause of uremic pericarditis?

Answer 399

End-stage renal disease.

Question 400

What types of tumors can lead to neoplastic pericardial effusion?

Answer 400

* Primary (e.g., mesothelioma, pericardial sarcoma) * Secondary (e.g., breast, lung, lymphoma, leukemia, melanoma)

Question 401

What are some trauma/iatrogenic causes of pericardial effusion?

Answer 401

* Blunt or penetrating trauma * Cardiac surgery * Invasive procedures

Question 402

Fill in the blank: _______ is a metabolic cause of pericardial effusion from chronic kidney disease.

Answer 402

Uremia

Question 403

What condition classically causes serous effusion associated with hypothyroidism?

Answer 403

Myxedema.

Question 404

What is a potential cause of hemorrhagic pericardial effusion?

Answer 404

* Aortic dissection * Myocardial rupture post-MI * Anticoagulation

Question 405

What is the most common cause of idiopathic pericardial effusion in developed countries?

Answer 405

Presumed viral or autoimmune.

Question 406

Name a miscellaneous cause of pericardial effusion.

Answer 406

* Radiation * Chylous effusion * Drugs (e.g., hydralazine, isoniazid, phenytoin, procainamide)

Question 407

What factors influence the symptoms and signs of pericardial effusion?

Answer 407

Volume of fluid, rate of accumulation, underlying cause, presence or absence of tamponade physiology

Question 408

What is the nature of chest pain associated with pericardial effusion?

Answer 408

Dull, pressure-like; less sharp than pericarditis; may radiate to the neck or shoulder

Question 409

What causes dyspnea in patients with pericardial effusion?

Answer 409

Reduced lung expansion or decreased cardiac output

Question 410

What is orthopnea and how is it related to pericardial effusion?

Answer 410

Worsens when lying flat if large effusion compresses lungs or heart

Question 411

What symptoms may arise due to compression of adjacent structures in pericardial effusion?

Answer 411

Cough or hoarseness, dysphagia

Question 412

What can cause fatigue and weakness in patients with pericardial effusion?

Answer 412

Low cardiac output

Question 413

What is a possible cause of palpitations in pericardial effusion?

Answer 413

Atrial stretch or arrhythmias

Question 414

What symptoms suggest tamponade physiology in pericardial effusion?

Answer 414

Syncope or dizziness

Question 415

What does distant/muffled heart sounds indicate during physical examination?

Answer 415

Fluid insulating the heart sounds

Question 416

What does jugular venous distension (JVD) indicate in the context of pericardial effusion?

Answer 416

Tamponade or significant compression of right heart

Question 417

What is pulsus paradoxus?

Answer 417

Drop in systolic BP >10 mmHg during inspiration; suggests tamponade

Question 418

What compensatory response is indicated by tachycardia in pericardial effusion?

Answer 418

Compensatory for low stroke volume

Question 419

What late sign may indicate tamponade in pericardial effusion?

Answer 419

Hypotension

Question 420

What is Ewart’s sign?

Answer 420

Dullness to percussion and decreased breath sounds at left scapular angle from large effusion compressing lung

Question 421

What does low voltage QRS on ECG suggest?

Answer 421

Electrical dampening by fluid

Question 422

What is electrical alternans and what does it suggest?

Answer 422

Alternating QRS amplitude; suggests tamponade

Question 423

What is a common ECG finding in response to pericardial effusion?

Answer 423

Sinus tachycardia

Question 424

What chest X-ray finding indicates a large pericardial effusion?

Answer 424

Enlarged, globular ('water bottle') heart silhouette

Question 425

What is the test of choice for diagnosing pericardial effusion?

Answer 425

Echocardiogram

Question 426

What imaging modalities can confirm size, extent, and composition of pericardial effusion?

Answer 426

CT/MRI

Question 427

True or False: Slow, small effusions are often asymptomatic.

Answer 427

True

Question 428

Rapid accumulation of even ______ mL of fluid can lead to tamponade.

Answer 428

200–300

Question 429

What should always be assessed to rule out cardiac tamponade?

Answer 429

Hemodynamic compromise

Question 430

What is the primary goal of diagnosing pericardial effusion?

Answer 430

Recognizing clinical signs, confirming fluid accumulation around the heart, and determining the underlying cause

Question 431

What are common symptoms of pericardial effusion?

Answer 431

* Chest discomfort * Dyspnea * Orthopnea * Fatigue * Cough * Hoarseness * Syncope

Question 432

What physical exam findings may indicate pericardial effusion?

Answer 432

* Muffled heart sounds * Elevated JVP * Pulsus paradoxus * Hypotension * Tachycardia

Question 433

What does low voltage QRS on an ECG suggest?

Answer 433

Electrical insulation by fluid

Question 434

What is electrical alternans on an ECG indicative of?

Answer 434

Alternating QRS amplitude due to swinging heart, suggests large effusion or tamponade

Question 435

What finding on a chest X-ray indicates chronic large pericardial effusion?

Answer 435

Enlarged, globular silhouette or 'water bottle' appearance

Question 436

What does a normal cardiac silhouette on a chest X-ray suggest?

Answer 436

Small or acute effusions

Question 437

What are the purposes of CBC, CRP, and ESR tests in diagnosing pericardial effusion?

Answer 437

To evaluate for inflammatory or infectious etiology

Question 438

What blood test is used if myocarditis or myocardial infarction is suspected?

Answer 438

Troponin

Question 439

What is the first-line diagnostic tool for pericardial effusion?

Answer 439

Echocardiography

Question 440

What does a transthoracic echo (TTE) show in pericardial effusion?

Answer 440

Fluid as an echo-free space between pericardial layers

Question 441

What signs of tamponade can be assessed by echocardiography?

Answer 441

* RA/RV diastolic collapse * Plethoric IVC * Exaggerated respiratory inflow variation

Question 442

What is the purpose of diagnostic pericardiocentesis?

Answer 442

For large or unexplained effusions, especially if fever, malignancy, or TB suspected

Question 443

What analyses are performed on fluid obtained from pericardiocentesis?

Answer 443

* Cytology * AFB stain/culture * Gram stain * LDH * Protein * Glucose

Question 444

What does a CT chest help detect in pericardial effusion?

Answer 444

* Pericardial thickening * Calcification * Hemorrhage * Loculated effusions

Question 445

What can cardiac MRI characterize in pericardial effusion?

Answer 445

Composition (e.g., exudative vs hemorrhagic) and shows pericardial inflammation or infiltration

Question 446

What is the gold standard for detecting and assessing pericardial fluid?

Answer 446

Echocardiography (TTE/TEE)

Question 447

What ECG findings are associated with large pericardial effusions?

Answer 447

* Low voltage * Electrical alternans

Question 448

What does pericardiocentesis provide, apart from being diagnostic?

Answer 448

Therapeutic benefits

Question 449

What additional roles do CT and MRI have in diagnosing pericardial disease?

Answer 449

Further characterize effusion or pericardial disease

Question 450

What factors determine the management of pericardial effusion?

Answer 450

Factors include: * Hemodynamic stability * Size and rate of fluid accumulation * Presence of cardiac tamponade * Underlying cause ## Footnote These factors guide the urgency and type of intervention needed.

Question 451

What is the management approach for small, asymptomatic effusion?

Answer 451

Monitor with serial echocardiography and treat underlying cause ## Footnote This approach emphasizes observation and addressing any causative conditions.

Question 452

What is the recommended action for moderate to large, symptomatic, or rapidly accumulating effusions?

Answer 452

More urgent evaluation ± drainage ## Footnote Urgent evaluation may include imaging and possibly drainage to relieve symptoms.

Question 453

What signs indicate cardiac tamponade?

Answer 453

Signs include: * Hypotension * Jugular venous distension (JVD) * Pulsus paradoxus ## Footnote These signs indicate a critical condition requiring immediate intervention.

Question 454

What is the emergency management for cardiac tamponade?

Answer 454

Management includes: * Hospital admission * Monitor vitals, ECG, and oxygenation * Pericardiocentesis * IV fluids * Avoid vasodilators and diuretics ## Footnote These steps are crucial to stabilize the patient and relieve pressure on the heart.

Question 455

What is the purpose of diagnostic pericardiocentesis?

Answer 455

Indications include: * Suspected infection (TB, bacterial, fungal) * Malignancy * Hemorrhagic effusion * Recurrent or unexplained effusion ## Footnote This procedure helps identify the cause of the effusion.

Question 456

What tests should fluid obtained from pericardiocentesis be sent for?

Answer 456

Tests include: * Gram stain * AFB * Cultures * Cytology * Protein * LDH * Glucose * ADA (TB) * Cell count ## Footnote These tests help diagnose the underlying cause of the effusion.

Question 457

What is the specific treatment for viral causes of pericardial effusion?

Answer 457

NSAIDs, colchicine, supportive care ## Footnote These treatments help manage symptoms and inflammation.

Question 458

What is the treatment for bacterial (purulent) pericardial effusion?

Answer 458

Antibiotics + surgical drainage ## Footnote This approach addresses the infection and relieves pressure.

Question 459

What is the primary treatment for tuberculosis-related pericardial effusion?

Answer 459

Anti-TB therapy (RIPE regimen) ## Footnote The RIPE regimen includes rifampicin, isoniazid, pyrazinamide, and ethambutol.

Question 460

What management options exist for recurrent or loculated effusion?

Answer 460

Options include: * Repeat pericardiocentesis * Pericardial window (surgical drain) * Pericardiectomy * Pericardial sclerosis with agents like doxycycline or talc ## Footnote These options vary based on the patient's condition and the nature of the effusion.

Question 461

What follow-up actions are recommended after treating pericardial effusion?

Answer 461

Actions include: * Repeat echocardiography * Monitor inflammatory markers (e.g., CRP) * Taper medications (NSAIDs/colchicine) ## Footnote Regular follow-up is important to ensure proper recovery and prevent recurrence.

Question 462

What is pericardial effusion?

Answer 462

Accumulation of fluid in the pericardial space which may be asymptomatic but can progress to severe complications.

Question 463

What is the most severe complication of pericardial effusion?

Answer 463

Cardiac Tamponade

Question 464

Define Cardiac Tamponade.

Answer 464

Compression of the heart due to elevated intrapericardial pressure, leading to impaired ventricular filling and decreased stroke volume.

Question 465

What are the clinical signs of Cardiac Tamponade?

Answer 465

Beck’s triad (hypotension, muffled heart sounds, elevated JVP), pulsus paradoxus, tachycardia.

Question 466

What is the management for Cardiac Tamponade?

Answer 466

Emergency pericardiocentesis.

Question 467

What is Constrictive Pericarditis?

Answer 467

Chronic inflammation causing the pericardium to become fibrotic and noncompliant.

Question 468

What condition can Constrictive Pericarditis lead to?

Answer 468

Diastolic heart failure.

Question 469

What are the signs of Constrictive Pericarditis?

Answer 469

Elevated JVP, ascites, peripheral edema.

Question 470

What may be required for severe cases of Constrictive Pericarditis?

Answer 470

Pericardiectomy.

Question 471

What are recurrent effusions associated with?

Answer 471

Malignancy, autoimmune disease, or tuberculosis.

Question 472

What interventions may be necessary for recurrent effusions?

Answer 472

Pericardial window, pericardiectomy, or intrapericardial therapy.

Question 473

What happens if the effusion becomes infected?

Answer 473

It can lead to sepsis, abscess formation, or pericardial fibrosis.

Question 474

What is required for purulent pericarditis?

Answer 474

Urgent drainage and IV antibiotics.

Question 475

What is Adhesive Pericarditis?

Answer 475

A rare condition where fibrous adhesions cause the pericardium to stick to the myocardium.

Question 476

What is the prognostic implication of small, idiopathic effusions?

Answer 476

Usually benign; resolve with supportive treatment.

Question 477

What is the prognosis for viral or post-pericardiotomy effusions?

Answer 477

Good prognosis if monitored and treated early.

Question 478

What is the mortality risk of untreated Cardiac Tamponade?

Answer 478

High mortality if untreated; requires urgent drainage.

Question 479

What is the prognosis for malignancy-related effusions?

Answer 479

Often recur; prognosis depends on cancer type and response to therapy.

Question 480

How can uremic pericardial effusion improve?

Answer 480

Can improve with intensified dialysis.

Question 481

What is required for tuberculous pericarditis?

Answer 481

Long-term therapy; may lead to constriction if untreated.

Question 482

What is the variable prognosis for autoimmune-related pericardial effusion?

Answer 482

Often responsive to steroids/immunosuppressants.

Question 483

Fill in the blank: Poor prognostic factors include large or rapidly accumulating effusion and _______.

Answer 483

hemodynamic compromise (tamponade).

Question 484

True or False: Recurrent or chronic effusion is a poor prognostic factor.

Answer 484

True

Question 485

What is cardiac tamponade?

Answer 485

A life-threatening condition where fluid accumulates in the pericardial sac, increasing intrapericardial pressure and compressing the heart.

Question 486

What does cardiac tamponade impair?

Answer 486

Ventricular filling, reducing stroke volume and cardiac output.

Question 487

What is the result of impaired ventricular filling due to cardiac tamponade?

Answer 487

Hemodynamic instability or shock.

Question 488

What can cause cardiac tamponade?

Answer 488

Rapid or excessive accumulation of pericardial fluid (e.g., blood, pus, exudate).

Question 489

What happens to diastolic pressures in cardiac tamponade?

Answer 489

They equalize in all heart chambers.

Question 490

What is the effect of decreased preload in cardiac tamponade?

Answer 490

Decreased cardiac output.

Question 491

Can cardiac tamponade occur with small volumes of fluid?

Answer 491

Yes, if the accumulation is rapid.

Question 492

What is the classic clinical triad of cardiac tamponade?

Answer 492

* Hypotension * Elevated jugular venous pressure (JVP) * Muffled heart sounds.

Question 493

What causes hypotension in cardiac tamponade?

Answer 493

Decreased stroke volume.

Question 494

What does elevated jugular venous pressure indicate in cardiac tamponade?

Answer 494

Impaired venous return.

Question 495

What is the significance of muffled heart sounds in cardiac tamponade?

Answer 495

Fluid insulates sound transmission.

Question 496

What is pulsus paradoxus?

Answer 496

>10 mmHg drop in systolic BP during inspiration.

Question 497

What is a common heart rate finding in cardiac tamponade?

Answer 497

Tachycardia.

Question 498

What lung condition helps differentiate cardiac tamponade from other causes of dyspnea?

Answer 498

Clear lungs.

Question 499

What ECG finding is associated with cardiac tamponade?

Answer 499

Electrical alternans (alternating QRS amplitude).

Question 500

What is the urgent treatment for cardiac tamponade?

Answer 500

Pericardiocentesis to relieve pressure on the heart.

Question 501

What is the primary hemodynamic consequence of fluid accumulation in cardiac tamponade?

Answer 501

Compression of the heart, especially during diastole ## Footnote This leads to profound hemodynamic changes.

Question 502

What happens to intrapericardial pressure in cardiac tamponade?

Answer 502

Increases and equalizes with intracardiac diastolic pressures ## Footnote This limits cardiac chamber expansion.

Question 503

Which cardiac chambers are most affected by increased intrapericardial pressure?

Answer 503

Right atrium (RA) and right ventricle (RV) ## Footnote They are affected due to their thinner walls.

Question 504

What is the effect of cardiac tamponade on right atrium during diastole?

Answer 504

Early diastolic collapse ## Footnote This prevents proper filling.

Question 505

What happens to the left ventricle in cardiac tamponade?

Answer 505

Indirectly affected due to reduced pulmonary return and septal shift ## Footnote This results in decreased stroke volume.

Question 506

What is the net result of decreased preload in cardiac tamponade?

Answer 506

↓ Stroke volume (SV) → ↓ Cardiac output (CO) ## Footnote This leads to significant hemodynamic consequences.

Question 507

What occurs to diastolic pressures in the heart during cardiac tamponade?

Answer 507

Equalization of diastolic pressures across RA, RV, LA, and LV ## Footnote This is a common finding on right heart catheterization.

Question 508

What compensatory mechanism occurs in response to low stroke volume in cardiac tamponade?

Answer 508

Tachycardia ## Footnote Heart rate increases to maintain cardiac output.

Question 509

What is pulsus paradoxus in the context of cardiac tamponade?

Answer 509

Exaggerated (>10 mmHg) drop in systolic BP during inspiration ## Footnote This occurs due to impaired filling of the left ventricle.

Question 510

What causes reduced aortic and pulmonary artery pressures in cardiac tamponade?

Answer 510

↓ Stroke volume → ↓ Systolic BP ## Footnote This is a direct consequence of decreased cardiac output.

Question 511

What happens to venous pressures in cardiac tamponade?

Answer 511

Elevated venous pressures ## Footnote This includes increased RA pressure and neck vein distension.

Question 512

What is the relationship between central venous pressure and cardiac tamponade?

Answer 512

Central venous pressure increases ## Footnote This is associated with symptoms like hepatic congestion.

Question 513

Fill in the blank: In cardiac tamponade, the heart rate ______ to compensate for low stroke volume.

Answer 513

increases

Question 514

What happens to systolic blood pressure in cardiac tamponade?

Answer 514

↓ Systolic BP, especially with pulsus paradoxus ## Footnote This indicates significant hemodynamic compromise.

Question 515

What is the effect of cardiac tamponade on pulmonary venous return?

Answer 515

Decreased pulmonary venous return ## Footnote This contributes to lower pulmonary artery pressures.

Question 516

List the changes in hemodynamic parameters during cardiac tamponade.

Answer 516

* Intrapericardial pressure: ↑↑ * RA/RV diastolic pressure: ↑ * LV preload: ↓ * Stroke volume (SV): ↓ * Cardiac output (CO): ↓ * Heart rate: ↑ (compensatory) * Systolic BP: ↓ * Central venous pressure: ↑↑ ## Footnote These changes summarize the hemodynamic impact of cardiac tamponade.

Question 517

What is cardiac tamponade?

Answer 517

Accumulation of pericardial fluid that impairs cardiac filling and output, leading to a life-threatening drop in perfusion.

Question 518

What determines the severity of symptoms in cardiac tamponade?

Answer 518

The rate of accumulation of fluid, more than the total volume.

Question 519

What are the classic findings of Beck’s Triad in cardiac tamponade?

Answer 519

* Hypotension * Elevated JVP * Muffled heart sounds

Question 520

What does hypotension in cardiac tamponade indicate?

Answer 520

Decreased stroke volume.

Question 521

What is pulsus paradoxus?

Answer 521

A decrease in systolic blood pressure greater than 10 mmHg with inspiration.

Question 522

What causes tachycardia in cardiac tamponade?

Answer 522

Compensatory response to low cardiac output.

Question 523

List other key signs and symptoms of cardiac tamponade.

Answer 523

* Tachypnea * Dyspnea * Fatigue * Weakness * Restlessness or altered mental status * Cold extremities * Oliguria

Question 524

What are the distinguishing features of rapidly accumulating tamponade versus slowly accumulating effusion?

Answer 524

* Rapidly Accumulating Tamponade: Small volume needed, abrupt onset, severe symptoms * Slowly Accumulating Effusion: Large volume needed, gradual onset, mild symptoms

Question 525

What is the volume needed for tamponade in rapidly accumulating cases?

Answer 525

Small volume (~200 mL).

Question 526

What is the volume needed for tamponade in slowly accumulating cases?

Answer 526

Large volume (>1–2 L).

Question 527

What is the typical compensatory adaptation in rapidly accumulating tamponade?

Answer 527

None — the heart can't adapt.

Question 528

What is the typical compensatory adaptation in slowly accumulating effusions?

Answer 528

Yes — the pericardium stretches over time.

Question 529

What common causes lead to rapidly accumulating tamponade?

Answer 529

* Trauma * Aortic rupture * MI rupture * Iatrogenic causes

Question 530

What common causes lead to slowly accumulating effusions?

Answer 530

* Malignancy * Uremia * Tuberculosis * Hypothyroidism

Question 531

What are the signs of shock in severe cases of cardiac tamponade?

Answer 531

* Cold extremities * Oliguria

Question 532

What are summary pearls regarding cardiac tamponade?

Answer 532

* Small but fast effusions are more dangerous than large but slow ones. * Always assess for tachycardia, hypotension, JVD, and pulsus paradoxus. * Tamponade is a clinical diagnosis supported by echo.

Question 533

True or False: Tamponade should be diagnosed through imaging before treatment.

Answer 533

False — do not delay treatment for imaging if unstable.

Question 534

What is often misdiagnosed as constrictive pericarditis?

Answer 534

Heart failure, cirrhosis, restrictive cardiomyopathy ## Footnote Misdiagnosis occurs due to overlapping symptoms.

Question 535

What are the key methods for diagnosing constrictive pericarditis?

Answer 535

Clinical suspicion, imaging, hemodynamic studies ## Footnote These methods help confirm pericardial constriction and rule out other causes of diastolic dysfunction.

Question 536

What are the signs of right-sided heart failure?

Answer 536

* Edema * Ascites * Elevated JVP ## Footnote These signs are crucial in clinical suspicion of constrictive pericarditis.

Question 537

What is Kussmaul’s sign?

Answer 537

Increased JVP on inspiration ## Footnote This sign indicates impaired filling of the right heart.

Question 538

What is a pericardial knock?

Answer 538

An early diastolic sound ## Footnote It is associated with constrictive pericarditis.

Question 539

What symptoms may be out of proportion to LV systolic function in constrictive pericarditis?

Answer 539

Signs of heart failure ## Footnote These symptoms can mislead the diagnosis.

Question 540

What history might suggest constrictive pericarditis?

Answer 540

* History of TB * Chest radiation * Cardiac surgery * Recurrent pericarditis ## Footnote These factors can contribute to the condition.

Question 541

What ECG findings are characteristic of constrictive pericarditis?

Answer 541

* Low voltage QRS * Non-specific ST-T changes * Atrial fibrillation ## Footnote Low voltage may result from pericardial thickening or fibrosis.

Question 542

What does pericardial calcification indicate on a chest X-ray?

Answer 542

Especially in TB or post-radiation cases ## Footnote Calcification can be a key diagnostic feature.

Question 543

What is a key finding in echocardiography for constrictive pericarditis?

Answer 543

Pericardial thickening if >3–4 mm ## Footnote This finding helps confirm the diagnosis.

Question 544

What is septal bounce in echocardiography?

Answer 544

Paradoxical septal motion due to ventricular interdependence ## Footnote It indicates constrictive physiology.

Question 545

What is the significance of respiratory variation in mitral/tricuspid inflow?

Answer 545

≥25% variation in mitral E velocity with respiration ## Footnote This finding supports the diagnosis of constrictive pericarditis.

Question 546

What does a dilated IVC with poor collapse indicate?

Answer 546

Elevated RA pressure ## Footnote This finding is associated with constrictive pericarditis.

Question 547

What laboratory tests are useful in diagnosing constrictive pericarditis?

Answer 547

* BNP/NT-proBNP * Inflammatory markers (CRP/ESR) * Autoimmune and infectious screens ## Footnote These tests help differentiate from other conditions.

Question 548

What does CT chest detect in constrictive pericarditis?

Answer 548

Pericardial thickening (>4 mm), calcification ## Footnote CT can provide detailed imaging of the pericardium.

Question 549

What does cardiac MRI show in constrictive pericarditis?

Answer 549

* Thickened pericardium * Septal bounce * Ventricular coupling * Late gadolinium enhancement if inflammation is active ## Footnote MRI is a valuable tool for assessing pericardial conditions.

Question 550

What is the gold standard for diagnosing constrictive pericarditis if uncertain?

Answer 550

Cardiac catheterization ## Footnote This method provides hemodynamic data crucial for diagnosis.

Question 551

What hemodynamic finding indicates constrictive physiology?

Answer 551

Discordant RV and LV pressures with respiration ## Footnote This confirms the diagnosis of constrictive pericarditis.

Question 552

What does equalization of diastolic pressures suggest?

Answer 552

RA, RV, and LV diastolic pressures are similar ## Footnote This finding is indicative of constrictive pericarditis.

Question 553

What is the dip-and-plateau pattern in cardiac catheterization?

Answer 553

Early rapid filling then abrupt halt ## Footnote This pattern is characteristic of constrictive physiology.

Question 554

What are the key diagnostic features summarized for constrictive pericarditis?

Answer 554

* ECG: Low voltage, possible AF * CXR: Pericardial calcification * Echo: Septal bounce, respiratory inflow variation * MRI/CT: Thickened pericardium * Cath: Equalized pressures, square root sign, discordant pressures ## Footnote These features are essential for accurate diagnosis.

Question 555

What is the primary symptom of constrictive pericarditis?

Answer 555

Impaired ventricular filling leading to systemic venous congestion and low cardiac output. ## Footnote Symptoms mimic right-sided heart failure but can be distinguished by specific clinical signs and findings.

Question 556

What causes fatigue and weakness in constrictive pericarditis?

Answer 556

Due to ↓ cardiac output and poor systemic perfusion. ## Footnote This is a direct result of impaired ventricular filling.

Question 557

What symptom is associated with elevated filling pressures in constrictive pericarditis?

Answer 557

Dyspnea on exertion. ## Footnote This occurs due to pulmonary congestion.

Question 558

What is orthopnea and when does it occur in constrictive pericarditis?

Answer 558

Difficulty breathing while lying flat; occurs if left-sided pressures rise (late). ## Footnote This symptom indicates worsening heart function.

Question 559

What causes abdominal distension in constrictive pericarditis?

Answer 559

Hepatic congestion and ascites. ## Footnote This is due to increased pressure in the systemic venous system.

Question 560

What is the mechanism behind early satiety and bloating in constrictive pericarditis?

Answer 560

GI venous congestion and bowel edema. ## Footnote These symptoms reflect systemic venous congestion affecting gastrointestinal function.

Question 561

What causes peripheral edema in constrictive pericarditis?

Answer 561

Due to ↑ systemic venous pressure. ## Footnote This is a common manifestation of right-sided heart failure.

Question 562

What is a common arrhythmia associated with constrictive pericarditis?

Answer 562

Atrial fibrillation. ## Footnote This occurs due to biatrial enlargement.

Question 563

What is a classic sign of constrictive pericarditis observed during physical examination?

Answer 563

Elevated JVP reflecting impaired right atrial filling. ## Footnote This is a key indicator of the condition.

Question 564

What does Kussmaul’s sign indicate in the context of constrictive pericarditis?

Answer 564

↑ JVP on inspiration (paradoxical) due to fixed pericardial volume. ## Footnote This sign helps differentiate constrictive pericarditis from other conditions.

Question 565

What is a pericardial knock?

Answer 565

An early diastolic sound from abrupt cessation of ventricular filling. ## Footnote This sound is characteristic of constrictive pericarditis.

Question 566

What is hepatomegaly and how is it related to constrictive pericarditis?

Answer 566

Congestive hepatopathy due to systemic venous congestion. ## Footnote This can lead to liver dysfunction if untreated.

Question 567

What is ascites and when is it commonly seen in constrictive pericarditis?

Answer 567

Fluid accumulation in the abdominal cavity; common in advanced cases. ## Footnote This is a result of hepatic congestion.

Question 568

What does peripheral edema indicate in a patient with constrictive pericarditis?

Answer 568

Often prominent sign of systemic venous congestion. ## Footnote This indicates worsening heart function.

Question 569

What is cachexia and in what context does it occur in constrictive pericarditis?

Answer 569

A state of malnutrition and weight loss; may occur in chronic untreated disease. ## Footnote This is related to poor systemic perfusion.

Question 570

What is pulsus paradoxus and how is it affected in constrictive pericarditis?

Answer 570

May be mild or absent; less prominent than in tamponade. ## Footnote This sign indicates hemodynamic changes.

Question 571

What is a common finding in patients with constrictive pericarditis regarding lung status?

Answer 571

Normal or quiet lungs; pulmonary congestion is usually minimal compared to left heart failure. ## Footnote This finding helps differentiate from other heart failure types.

Question 572

What does the 'dip-and-plateau' sign indicate in ventricular pressure tracing?

Answer 572

Reflects early rapid filling then abrupt halt. ## Footnote This is indicative of constrictive physiology.

Question 573

True or False: Symptoms of constrictive pericarditis often improve with diuretics.

Answer 573

True. ## Footnote However, pericardiectomy is the only curative treatment.

Question 574

What is the primary goal in managing constrictive pericarditis?

Answer 574

Relieve symptoms of heart failure, address the underlying cause, and consider surgical pericardiectomy in chronic cases.

Question 575

What are the management steps for reversible causes of constrictive pericarditis?

Answer 575

Identify and treat reversible causes such as inflammation, TB, autoimmune diseases, malignancy, and uremia.

Question 576

What types of therapy can be used for anti-inflammatory treatment?

Answer 576

* NSAIDs * Colchicine * Corticosteroids (if inflammation present on MRI or elevated CRP/ESR)

Question 577

What is the role of imaging in the management of constrictive pericarditis?

Answer 577

Monitor with imaging (repeat MRI/CT) to assess resolution or progression.

Question 578

What medications are used for symptom control in chronic constrictive pericarditis?

Answer 578

* Loop diuretics (e.g., furosemide) * Aldosterone antagonists (e.g., spironolactone) * β-blockers, digoxin (for AF) * Anticoagulation (for AF or prior thromboembolism)

Question 579

What is the definitive treatment for chronic constrictive pericarditis?

Answer 579

Surgical pericardiectomy.

Question 580

What are the indications for surgical management in constrictive pericarditis?

Answer 580

Persistent symptomatic constriction despite medical therapy.

Question 581

What does the surgical procedure for pericardiectomy involve?

Answer 581

Surgical removal of both parietal and visceral pericardium (ideally ≥80%).

Question 582

What is the mortality risk associated with pericardiectomy?

Answer 582

~5–10%, higher in patients with advanced heart failure or comorbidities.

Question 583

What is the prognosis for patients with reversible causes treated early?

Answer 583

Often excellent outcome.

Question 584

What is the expected outcome after a successful pericardiectomy?

Answer 584

>80% experience symptomatic relief.

Question 585

What factors are associated with worse outcomes in constrictive pericarditis?

Answer 585

Delayed diagnosis/surgery and irreversible myocardial dysfunction.

Question 586

True or False: Persistent inflammation may improve with anti-inflammatory treatment alone.

Answer 586

True.

Question 587

Fill in the blank: The first step in the management algorithm for constrictive pericarditis is to confirm diagnosis through _______.

Answer 587

[echo, CT/MRI, catheterization]

Question 588

What should be trialed if inflammation is suspected in constrictive pericarditis?

Answer 588

Trial of anti-inflammatory therapy.

Question 589

What is recommended for symptomatic relief in cases of volume overload in constrictive pericarditis?

Answer 589

Symptomatic relief with diuretics.

Question 590

What is the treatment approach for chronic constriction in constrictive pericarditis?

Answer 590

Definitive therapy = pericardiectomy.