Robbins - Ch 12

Question 1

The overall weight and size of the heart matters when determining hypertrophy and dilation. What is the normal weight and size of the LV?

Answer 1

Size in gm: 250-320 male; 300-360 female. anything bigger is hypertrophy
LV width: 1.3-1.5 cm. anything bigger is dilation

Question 2

the LV myocytes arrangement helps to generate a coordinated wave of contraction that spreads from apex to the base of the heart. As such, how are the myocytes arranged?

Answer 2

Circumferentially in a spinal orientation.

Question 3

In congestive heart failure, explain how the interactions of the actin and myosin are changed.

Answer 3

With excessive ventricular myocytes dilation as seen in CHF, the overlap of the actin and myosin filament is reduced and the force of contraction decreases sharply.

Question 4

Atrial natriuretic peptides are located in _ and they act as a hormone to stimulate _

Answer 4

Located in atria.

Stimulates renal salt and water elimination (natriuresis and diuresis)

Question 5

The coordinated beating of cardiac myocytes depends on 1, which are specialized intercellular junctions that facilitate cell to cell mechanical and electrical coupling. Within the intercalated discs 2 facilitate synchronized waves of myocytes contraction by permitting rapid movement of ion between adjoining cells. Abnormalities in the spatial distribution of gap junctions can cause electromechanical dysfunction such as 3 and/or heart failure.

Answer 5

1. Intercalated discs.
2. gap junctions
3. arrhythmia

Question 6

Cardiac valves are lined by endothelium and share what three trilayered architecture

Answer 6

1. dense collagenous core (fibrosa) at the outflow surface
2. Central core of loose connective tissue (spongiosa)
3. Ventricularis or atrialis - elastin rich layer faces inflow surface

Question 7

The mechanical integrity of a valve is large due to what layer?

Answer 7

Layer 1: fibrosa

Question 8

The rapid recoil to achieve prompt valve closure is large due to mainly what layer?

Answer 8

Layer 3: elastin rich layer of ventricularis/atrialis

Question 9

The most abundant cell type int he heart valves are 1 and they are mainly responsible for synthesizing _ 2_

Answer 9

1. Valvular interstitial cells

2. extracellular matrix and express matrix degrading enzymes.

Question 10

The function of the semilunar valves depends on integrity and coordinated movements of the cuspal attachments and so dilation of the aortic root can hinder coaptation of the aortic valve cusps during closure and result in _

Answer 10

valvular regurgitation

Question 11

LV dilation, a ruptured tendinous cord, or papillary muscle dysfunction can all interfere with valve closure causing _

Answer 11

valvular insufficiency

Question 12

Pathologic changes of valves are largely of three types. Namely _

Answer 12

1. damage to collagen that weakens the leaflets (mitral valve prolapse
2. nodular calcification beginning in interstitial cells (calcific aortic stenosis
3. fibrotic thickening as seen in rheumatic heart disease

Question 13

What are the components of the conduction system and where are they located?

Answer 13

1. SV node (junction of RA appendage and SVC)
2. AV node (RA along the atrial septum
3. Bundle of Hiss (connects RA to ventricular septum)
4. Purkinje network (ventricles)

Question 14

Cardiac myocytes rely almost exclusively on what for their energy need?

Answer 14

oxidative phosphorylation

Question 15

Myocardium are extremely vulnerable to ischemia because they require supply of_

Answer 15

oxygenated blood

Question 16

The three major epicardial coronary arteries are:

Answer 16

1. left anterior descending (LAD) –> diagonal branches
2. Left circumflex arteries –> marginal branches
   (both 1 and 2 arise from Left (main) coronary artery)
3. Right coronary artery

Question 17

Blood flow to the myocardium occurs during which phase of the cardiac cycle?

Answer 17

ventricular diastole

Question 18

From an anatomical perspective, explain how tachycardia compromises cardiac perfusion.

Answer 18

Since myocardium perfusion occurs during diastole following the closure of the aortic valve when the microcirculation is not compressed by cardiac contraction, and 2/3 of cardiac cycle is in diastole, with tachycardia the duration of diastole is shortened and thus blood flow to the coronary is compromised.

Question 19

Explain what changes in a aging heart is seen in the chambers of the heart.

Answer 19

1. increased LA cavity size
2. decreased LV cavity size
3. sigmoid-shaped ventricular septum

Question 20

Explain what valvular changes are seen in the aging heart

Answer 20

1. aortic valve calcific deposits
2. mitral velave anular calcific deposits
3. fibrous thickening of leaflets
4. Buckling of mitral leaflets toward the left atrium
5. Lambl excrescenes (small filiform processes on closures lines of aortic and mitral valves probably form small thrombi)

Question 21

Explain what changes with age are seen in epicardial coronary arteries

Answer 21

1. tortuosity
2. diminished compliance
3. Calcific deposits
4. Atherosclerotic plaque

Question 22

Explain what changes with age are seen in the myocardium

Answer 22

1. decreased mass
2. increased subepicardial fat
3. brown atrophy
4. lipofuscin deposits
5. basophlic degeneraton
6. amyloid deposits

Question 23

explain what changes with age are seen in aorta

Answer 23

1. dilated ascending aorta with rightward shift
2. elonaged thoracic aorta
3. sinotubular junction calcific deposits
4. Elastic fragmentation and collagen accumulation
5. atherosclerotic plaque

Question 24

The hallmark of cardiac pathophysiology stems from what six principal mechanisms and provide an example of pathology for each?

Answer 24

1. Pump failure (weak contraction–>diminished CO during systole, or insufficient relaxation during diastole)
2. Flow obstruction (atherosclerotic plaque, aortic valvular stenosis, HTN, aortic coarctation)
3. Regurgitant flow (incompetent valve–>LV in aortic regurgitation)
4. Shunted flow (congenital or acquired as post MI)
5. Disorders of cardiac conduction (a or v-fi)
6. Rupture of heart or a major vessel ( trauma)

Question 25

_ results when the heart is unable to pump blood at a rate sufficient to meet the metabolic demands of the tissues or can do so only at an elevated filling pressure.

Answer 25

Congestive heart failure

Question 26

CHF is the common end stage for what chronic conditions?

Answer 26

1. chronic workload as seen in valve disease or HTN, and 2) ischemic heart disease as following an MI

Question 27

when cardiac workload increase or cardiac function is compromised list or briefly explain the physiologic mechanism that maintain arterial pressure and organ perfusion

Answer 27

1. Frank starling mechanism (increase filling and dilate heart to increase actin-myosin cross bridge to enhance contractility and SV) 2. Myocardial adaptations, including hypertrophy with or without cardiac chamber dilation. --> ventricular remodeling to increase mechanical work 3. Activation of neurohumoralsystems: release norepi --> increase HR and contractility; activate RAAS; release ANP

Question 28

Systolic dysfunction is defined as _1_ and is measured as a percentage of _2_ where normal is _3_.

Answer 28

1. deterioration of myocardial contractility function. 2. Ejection fraction 3. 45-65% according to robbin (everywhere else it's 50-70%)

Question 29

Diastolic dysfunction is defined as_1_ and can be due to _2_

Answer 29

1. inability of the heart chamber to expand and fill sufficiently 2. LVH, myocardial fibrosis, constrictive pericarditis, or amyloid deposition

Question 30

sustained increase in mechanical work due to pressure or volume overload, or trophic signals causes _

Answer 30

hypertrophy (myocytes increase in size and thus the heart increase in size and weight)

Question 31

at a cellular level, explain how the heart increases in size

Answer 31

Increase protein synthesis, increased number of mitochondria. Myocytes have larger nuclei due to increased DNA ploidy resulting from DNA replication in the absence of cell division.

Question 32

The pattern of cardiac hypertrophy reflects the nature of the stimulus. Explain the pattern of hypertrohpy seen in pressure-overload vs volume overload.

Answer 32

Pressure overload (e.g. due to HTN or aortic stenosis), new sarcomeres are assembled in parallel to the long axes of cells --> expand cross-sectional area of myocytes in ventricles and thus cause a concentric increase in wall thickness. In volume-overload new sarcomeres are in series within existing ones --> ventricular dilation.

Question 33

What is the best mode of measure to determine hypertrophy in dilated hearts?

Answer 33

heart weight. Not wall thickness as we'd do in pressure overload hypertrophy.

Question 34

Explain the changes in blood supply and O2 demand to a hypertrophic heart esp one seen in volume overload vs a normal heart, and what those changes mean at a functional level.

Answer 34

Myocyte hypertrophy is not accompanied by a proportional increase in capillary numbers. As a result the supply of oxygen and nutrients to the hypertrophied heart particularly one undergoing pressure overload hypertrophy is more tenous than in normal heart. In addition hypertrophied heart needs more O2. With heightened metabolic demand due to increase in heart rate, and contractility, makes the heart vulnerable to ischemia-related decompensation which can evolve to cardiac failure and eventually death.

Question 35

Explain the hypertrophy seen in long distance runner

Answer 35

They undergo volume load hypertrophy with increase in capillary density and decreases in resting heart rate and blood pressure --effects that are all beneficial. static exercise like weight lifting has the opposite effect.

Question 36

What is the consequence of CHF?

Answer 36

- decreased CO and tissue perfusion as well as pooling of blood in venous capacitance system --> pulmonary edema, peripheral edema or both. - Clinical features and changes are secondary to injuries induced by hypoxia and congestion in tissues due to the poor perfusion.

Question 37

What are some common causes of Left-sided heart failure?

Answer 37

- Ischemic heart disease - HTN - Aortic and mitral valvular disease - primary myocardial diseases

Question 38

What is the clinical and morphologic effects of left sided CHF

Answer 38

Consequence of passive congestion (blood backing up int he pulmonary circulation), stasis of blood in left-sided chambers, and inadequate perfusion of down stream tissues leading to organ dysfunction

Question 39

what are the signs of early CHF?

Answer 39

Early signs are related to pulmonary congestion and edema: cough, dyspnea only with exertion.

Question 40

What are signs of progressive CHF?

Answer 40

Worsening pulmonary edema and cause orthopnea (dyspnea when supine, relieved by sitting of standing) or paroxysmal nocturnal dyspnea. - dyspnea even with rest may follow - Afib exacerbates it owing to loss of atrial kick - reduced renal perfusion --> RAAS activation --> expansion of interstitial and intravascular fluid volumes --> exacerbate pulmonary edema; and reduced excretion of nitrogenous wastes --> azotemia - Far advanced CHF: hypoxic encephalopathy (irirtability, loss of attention span, restlessness --> stupor and coma with ischemic cerebral injury

Question 41

Left-sided heart failure can be divided into two categories?

Answer 41

Systolic failure - insufficient ejection fraction diastolic failure - failure to relax. predominantly in patients older than 65 yr with HTN, DM, obesity and bilateral renal artery being common etiologies.

Question 42

The most common cause of right-sided heart (aka cor pulmonale) failure is _

Answer 42

left -sided heart failure. Clinical findings for right and left are pretty much the same.

Question 43

what is the major morphologic and clinical effects of primary right-sided heart failure as it differ from those o left-sided heart fialure?

Answer 43

with right sided heart failure pulmonary congestion is minimal whie engorgement of the systemic and portal venous systems is pronounced

Question 44

what is the standard therapy for CHF?

Answer 44

Mainly pharmacologic: drugs that relieve fluid overload (diuretic), that block RAAS (ACEI), and that lower adrenergic tone (beta blockers).

Question 45

what are the signs and symptoms of Right sided heart failure?

Answer 45

1. Liver and spleen: passive congestion (nutmeg liver); congestive splenomegaly; ascites 2. Kidneys - azotemia 3. Pleural/pericardium: pleural and pericardial effusions; transudates 4. Peripheral tissues congestion and hypoperfusion Pts appear fatigued, JVD elevated, cynotic, increased peripheral venous pressure

Question 46

what is the most common congenital defect of the CV?

Answer 46

Ventricular septal defect (42%)

Question 47

List the congenital malformation that results in a Left to Right shunt (shunting from systemic to pulmonary)

Answer 47

- Ventricular septal (D)efect - Atrial septal (D)efect - Patient (D)uctus arteriosus - Atrioventricular septal (D)efect (The parenthetical D is a way to remember that anything that has a D in it it's gonna result in a Left to right shunt)

Question 48

What congenital malformation results in a right to left shunt and babies appear cynotic?

Answer 48

``` (T)etralogy of allot (T)ransposition of great arteries (T)runcus arteriosus (T)otal anomalous pulmonary venous connection (T)ricuspid atresia ``` (the parenthetical T's helps to remember that malformations with T in its name will cause R-->L shunt

Question 49

Which trisomies are associated with congenital heart defect?

Answer 49

21 (most common), 13, 15, 18, XO

Question 50

Mutations of which genes are associated with congenital heart defect?

Answer 50

TBX5, TBX1, GATA4 --> ASD and/or VSD | NKX2.5 --> ASD, or conduction defects

Question 51

Region of chromosome 22 is important in heart development. Particularly 22q11.2 deletion can lead to _

Answer 51

conotruncus, branchial arch, face defect.

Question 52

in Left to right shunting what is the most feared irreversible consequnce?

Answer 52

Pulmonary hypertension--> causes irreversible to the lungs and babies most likely die.

Question 53

What gene is implicated in Tetralogy of Fallot?

Answer 53

1. transcription factors ZFPM2 or NKX2.5 (nonsyndromic) | 2. JAG1 or NOTCH2 (syndromeic)

Question 54

Which shunts is the most common congenital heart disease?

Answer 54

Left to right shunts caused by ASD, VSD and PDA

Question 55

_ are abnormal fixed openings in the atrial septum caused by incomplete tissue formation that allows communications of blood between the left and right atria and are asymptomatic until _

Answer 55

atrial septal defect (ASD) | adulthood

Question 56

Is ASD due to patent foramen ovale?

Answer 56

No, they're separate entities.

Question 57

ASDs are classified according to their location. 90% of all ASD is a result from a deficient formation of what?

Answer 57

Septum secundum fomration near the center of the atrial septum. 5% is due to primum anomalies. another 5% is due to sinus venosus defect located near entrance of the SVC

Question 58

VSD is commonly seen with what other CHD?

Answer 58

tetralogy of fallot

Question 59

90% of VSD involves what structure?

Answer 59

membranous septum

Question 60

A VSD with multiple holes is likely to involve what structure?

Answer 60

Muscular septum

Question 61

While small VSD often close spontaneously, large ones progresses to _

Answer 61

pulmonary hypertension

Question 62

_ arises from pulmonary artery and joins the aorta just distal to the origin of the left subclavian artery.

Answer 62

patent ductus arteriosus (PDA). Normally it closes within 1-2 days after birth and within a month becomes the ligamentum arteriosum.

Question 63

Do most PDA occur alone or with other congenital heart disease?

Answer 63

90% occur as isolated defects.

Question 64

Upon auscultation, what are you likely to hear with someone with a patent ductus arteriosus?

Answer 64

HARSH, machinery-like murmur.

Question 65

It was argued that both keeping the patent ductus arteriosus open, and closing it are life saving. Explain each case.

Answer 65

Keeping it open mixes oxygenated blood with deoxygenated blood, but in babies with other malformation that obstruct the pulmonary or systemic outflow, keeping it open via prostaglandin E may be beneficial.

Question 66

atrial ventricular septal defect (AVSD) are associated with defective inadequate AV valves which can be partial or complete. If it's complete which chambers freely communicate?

Answer 66

ALL 4 chambers freely communicate

Question 67

Of all the Right to Left shunts defects, which one is the MOST common?

Answer 67

Tetralogy of Fallot (TOF)

Question 68

what are the four cardinal features of tetralogy of fallot?

Answer 68

1. VSD, large 2. Obstruction to RV flow 3. an Aorta that OVERRIDEs the VSD 4. RVH

Question 69

All the cardinal features of TOF results embryologically from anterosuperior displacement of what structure?

Answer 69

infundibular septum

Question 70

A 2-week-old newborn is brought to the physician because his lips have turned blue on three occasions during feeding. His BP is 75/45, Pulse is 170, and RR is 44. A grade 3/6, harsh, systolic ejection murmur is heard at the left upper sternal border. A CXR shows a small boot-shaped heart and decreased pulmonary vascular markings. what is the likely diagnosis?

Answer 70

Tetrology of fallot

Question 71

The most important determinant for prognosis of tetralogy of fallot is _

Answer 71

pulmonary stenosis ( obstruction of RV flow) - cyanosis may or may not be present depending on degree of stenosis. With more severe RV obstruction, right-sdied prssures approach or exceed left sided pressure and right to left shunting develops producing cyanosis.

Question 72

what are the risk factors associated with TOF?

Answer 72

Down syndrome Cri-du-chat syndrome trisomy 13/18

Question 73

A 4-month-old is noted to have a grade 3/6, harsh, systolic ejection murmur heard at the left upper sternal border. The mother reports that the child's lips occasionally turn blue during feeding. A cardiologist recommends surgery. Later, the physician remarks that the infant's congenital abnormality was related to a failure of neural crest cell migration. Prior to surgery, which of the following was a likely finding? 1. Atrial septal defect 2. Pulmonic stenosis 3. Triscuspid atresia 4. Coarctation of the aorta 5. Transposition of the great vessels

Answer 73

2. Pulmonic stenosis

Question 74

A 4-year-old Caucasian male suffers from cyanosis and dyspnea relieved by squatting. Which of the following abnormalities is most likely present? 1. Left ventricular hypertrophy 2. Atrial septal defect 3. Ventricular septal defect 4. Coarctation of the aorta 5. Patent ductus arteriosus

Answer 74

3. Ventricular septal defect. The patient described is experiencing a "tet spell", characteristic of Tetrology of Fallot (TOF). By definition, TOF consists of 4 components: pulmonic stenosis, ventricular septal defect, right ventricular hypertrophy, and overriding aorta (straddling the VSD).

Question 75

A 7-year-old boy is being evaluated in pediatric cardiology clinic. He appears grossly normal, but suddenly becomes tachypneic and cyanotic when his mom takes a toy away from him. These symptoms resolve somewhat when he drops into a squatting position. Transthoracic echocardiography reveals pulmonic stenosis, a ventricular septal defect, right ventricular hypertrophy, and an overriding aorta. Which of the following best predicts the degree of cyanosis and other hypoxemic symptoms in this patient? 1. Degree of pulmonic stenosis 2. Size of ventricular septal defect (VSD) 3. Degree of right ventricular hypertrophy (RVH) 4. Degree to which aorta overrides right ventricle 5. Presence of S3

Answer 75

1. Degree of pulmonic stenosis

Question 76

A 24-hour-old newborn appears blue in all extremities, is cyanotic, and is transferred immediately to the NICU, where he is found to have a single, loud S2 murmur. what is the most likely diagnosis?

Answer 76

Transposition of great vessels

Question 77

what is the anatomic anomaly in transposition of great vessels?

Answer 77

Aorta arises from the right ventricle and pulmonary artery arises from the left ventricle.

Question 78

patients with Transposition of great vessels usually also have what other defects in order to be compatible with life?

Answer 78

AV communication such as patent ductus arteriosus or patent foramen ovale

Question 79

what are some risk factors for developing transposition of great vessels?

Answer 79

- diabetic mom - down's syndrome - Apert syndrome - cri-du chat - trisomy 13/18

Question 80

How do babies with transposition of great arteries usually present?

Answer 80

newborns are extremely cyanotic and are critically ill. on PE, cyanosis, tachypnea and progressive respiratory failure is seen

Question 81

what is the charcteristic finding on Xray of a baby with transposition of great arteries?

Answer 81

Enlarged egg-shaped heart, often referred to as an "egg on a string"

Question 82

what is the treatment for long term survival for transposition of great vessel ?

Answer 82

surgery

Question 83

A new born baby appears cyanotic. Further work up shows an unequal division of the atrioventricular canal with mitral valve being larger than normal and right ventricular hypoplasia. A. what is the defect? B. How can survival be attained ?

Answer 83

A. Tricuspid atresia B. R--> L shunt through an interatrial communication (ASD or patent foramen ovale, in addition to a VSD that affords communication between the left ventricle and the pulmonary artery from the hypoplastic right ventricle.

Question 84

A 7-year-old-boy is seen for the first time by a primary care physician. His parents report that he tires easily and often complains of weakness in his legs. Physical exam shows a healthy boy with a blood pressure of 141/91 mm Hg. His lower extremities are slightly atrophic with a mottling appearance. what is the likely diagnosis?

Answer 84

Coartation of the aorta

Question 85

what are the two forms of coarctation of the aorta?

Answer 85

1. Infantile forme - often symptomatic in early childhoood with tubular hypoplasia of the aortic arch proximal to a PDA 2. adult form with a discrete ridgelike infolding of the aorta just opposite the closed ductus arteriosus distal to the arch vessels

Question 86

what is the defect seen in coarctation of aorta?

Answer 86

Narrowing of the lumen of the aorta. Infantile occurs distal to the subclavian artery and proximal to the ductus arteriosus. Adult/postductal type: occurs distal to the ductus arteriosus/ligamentum arteriosum.

Question 87

what are the risk factors associated with coarctation of the aorta?

Answer 87

Turner syndrome affects males > females - associated with bicuspid aortic valve (70%)

Question 88

how do patients with coarctation of the aorta present?

Answer 88

patient presents in childhood with asymptomatic HTN, dyspnea on exertion, systemic hypoperfusion(shock state) and without cyanosis. ON PE systolic BP in the upper extremeies greater than int he lower extremity; continuous murmur over collateral vessels in the back and lower extremity skin mottling; and leg claudication

Question 89

A 5-year-old girl presents to the physician with increased muscle cramping in her lower extremities after walking extended distances. The young girl is in the 10th percentile for height. Her past medical history is notable only for a cystic hygroma detected shortly after birth. Which of the following findings is most likely in this patient? 1. Decreased blood pressure in the upper and lower extremities 2. Barr bodies on buccal smear 3. Endocardial cushion defect 4. Inferior erosion of the ribs 5. Apparent hypertrophy of the calves

Answer 89

4. inferior erosion of the ribs

Question 90

what are the three common obstructive congential heart defect?

Answer 90

1. Coarctation of aorta 2. Pulmonary steosis/atresia 3. aortic stenosis/atresia

Question 91

Pulmonary stenosis or atresia is commonly at the level of the _

Answer 91

pulmonary valve

Question 92

100% pulmonic atresia is associated with hypoplastic _ 1_with ASD and blood reaches lungs through _2_

Answer 92

1. RV | 2. patent ductus arteriosus

Question 93

Congential narrowing and obstruction of the aortic valve can occur at what three location?

Answer 93

Aortic stenosis/atresia can occur in: 1. valvular 2. subvalvular 3. supravalvular

Question 94

What are the findings in hypoplastic left heart syndrome?

Answer 94

A severe congenital aortic stenosis or atresia where obstruction of the LV ouflow tract leads to hypoplasia of the LV and ascending aorta, some times with porcelain-like LV endocardial fibroelastosis and ductus must be oepn to allow blood flow to aorta and coronary arteries.

Question 95

Subaortic stenosis is caued by _

Answer 95

thickened ring or collar of dense endocardial fibrous tissue below the level of cusps.

Question 96

Supravalvular aortic stenosis is caused by _

Answer 96

congenital aortic dysplasia with thickening of ascending aortic walla nd consequent luminal constriction. Sometimes its due to deletions on chromosome 7 that include gene for elastin, where other feautures include hypercalcemia, cognitive abnormalities and facial anomalies (Williams-Beuren syndrome).

Question 97

Subaortic stenosis is usualy associated with prominent _ murmur and sometimes thrill

Answer 97

systolic

Question 98

what is Eisenmenger syndrome?

Answer 98

Process in which a long standing left to right cardiac shunt caused by a congenital heart defect (typically VSD, ASD) causes pulmonary hypertension and eventual reversal of the shunt inot a cyanotic R--> L shunt.

Question 99

an imbalance between myocardial supply (perfusion) and cardiac demand for oxygenated blood is referred to as _

Answer 99

myocardial ischemia

Question 100

In more than 90% of cases myocardial ischemia is from reduced blood flow due to _

Answer 100

atherosclerotic lesions in the epicardial coronary arteries; consequently, ischemic heart disease (IHD) is commonly known as coronary artery disease (CAD)

Question 101

IHD can present as _

Answer 101

1. myocardial infarction (MI) - result of acute plaque change that induces an abrupt thrombotic occlusion ---> necrosis 2. angina pectoris (stable and unstable) (stable =increased O2 demand that outstrip ability to stenosed cornary arteries to increase O2 delivery; unstable plaque disruption -->thrombosis and vasoconstriction) 3. Chronic IDH with heart failure 4. Sudden cardiac death (SCD - caused by regional ischemia that induces a fatal V-arrhythmia

Question 102

how is stable angina described as?

Answer 102

deep, poorly localized pressure, squeezing, or burning sensation (like indigestion) but unusually as pain, and is usually relieved by rest or administering vasodilators, such as nitroglycerin and calcium channel blockers (increase perfusion)

Question 103

Prinzzemetal varient angina is caused by _

Answer 103

coronary artery spasm, unrelated to activity, HR or BP. responds well to vasodilators

Question 104

About 50 of patients with this form of angina have evidence of myocardial necrosis; and for some MI may be imminent.

Answer 104

unstable or cresendo angina

Question 105

A 67-year-old construction foreman is brought to the emergency department by ambulance after complaining to his coworkers of sudden onset chest tightness and shortness of breath. He has a 45 pack-year smoking history, takes aspirin and simvastatin, and has a BMI of 33. what is the most likely daignosis?

Answer 105

Ischemic heart disease and probably presenting with angina.

Question 106

A 53-year-old man with a past medical history significant for hyperlipidemia, hypertension, and hyperhomocysteinemia presents to the emergency department complaining of 10/10 crushing, left-sided chest pain radiating down his left arm and up his neck into the left side of his jaw. His ECG shows ST-segment elevation in leads V2-V4. He is taken to the cardiac catheterization laboratory for successful balloon angioplasty and stenting of a complete blockage in his left anterior descending coronary artery. Echocardiogram the following day shows decreased left ventricular function and regional wall motion abnormalities. A follow-up echocardiogram 14 days later shows a normal ejection fraction and no regional wall motion abnormalities. This post-infarct course illustrates which of the following concepts? 1. Reperfusion injury 2. Ventricular remodeling 3. Myocardial hibernation 4. Myocardial stunning 5. Coronary collateral circulation

Answer 106

4. Myocardial stunning This patient exhibits transient myocardial dysfunction following an episode of acute ischemia. Such reversible dysfunction is known as myocardial stunning. Myocardial stunning occurs in the setting of short-term total reduction of coronary blood flow with subsequent reestablishment of coronary blood flow. Stunned myocardium exhibits LV dysfunction for a limited duration that subsequently resolves.

Question 107

Explain the pathogenesis of MI

Answer 107

Coronary artery occlusion. the following events likely underlies most MI: coronary artery atheromatous plaque undergoes an acute change consisting of intraplque hemorrhage, erosion or rupture; when exposed to subendothelial collagen and necrotic plaque contents, platelet adheres and activated and release granules and form microthrombi; vasopasm mediator released from platelets; tissue factor activates coagulation pathway; within minutes the thrombus can expand to completely occlude the vessel

Question 108

The precise location, size and specific morphologic features of an AMI depend on what factors?

Answer 108

1. the location, severity and rate of development of coronary obstruction 2. size of vascular bed perfused by obstructed vessels 3. duration of the occlusion 4. metabolic and o2 need of the myocardial at risk 5. extent of collateral blood vessels 6. presence, site, and severity of coronary arterial spasm 7. other factors such as HR, cardiac rhythm, and blood oxygenation

Question 109

within 2-3 hours of the onset of severe myocardiac ischemia, necrosis involves _; within 6 necrosis it's usually transmural

Answer 109

half of the thickness of the myocardium

Question 110

Transmural infarction is usually associated with a combination of _

Answer 110

coronary atherosclerosis, acute plaque change, and superimposed thrombosis

Question 111

subendocardial infarction occur as a global hypotension, and myocardial damage are in what pattern?

Answer 111

circumfrerential rather than being limited to the distribution of a single major artery

Question 112

Multifocal microinfarction is seen when there is a pathology involving _

Answer 112

only smaller intramural vessels

Question 113

Acute coronary syndrome is defined by what four major factors?

Answer 113

1. Acute plaque change (MOST important) 2. inflammation 3. thrombus 4. vasoconstriction

Question 114

what is defined as acute plaque change?

Answer 114

1. rupture/refissuring 2. erosion/ulceration, exposing ECM 3. acute hemorrhage

Question 115

Which angina is also known as Q wave angina?

Answer 115

Unstable angina

Question 116

Which form of angina pectoris is consider PRE-infarction?

Answer 116

Unstable angina

Question 117

Disruption of plaque is MOST often seen which form of angina?

Answer 117

Unstable angina

Question 118

Which Angina is predictable, presents with consistent exertion and relief with rest or nitroglycerin?

Answer 118

Stable angina

Question 119

what is the most definitive way to diagnose ischemic heart disease?

Answer 119

Cardiac catheterization

Question 120

In consideration of ischemic heart disease, what lab values are usually elevated?

Answer 120

- troponin, | - CK, and/or CK-MB

Question 121

WHat changes are you likely to see on EKG of a patient with ischemic heart disease?

Answer 121

- ST elevation or depression depending on severity of ischemia - Q waves

Question 122

What is the treatment for acute coronary syndrome?

Answer 122

``` MONA-B - Morphine -Oxygen -Nitroglycerin -aspirin -ACEI (may also use beta blockers, GPIIb/IIIa antagonists and angioplasty) ```

Question 123

What drugs are used post MI to improve mortality rates?

Answer 123

- Aspirin -B-blocker -ACEI -ARB -HMG-CoA reductase inhbitors (statins) NOT CCB

Question 124

what is the triad of symptoms for a stable angina?

Answer 124

1. chest pain 2. precipitated by exertion 3. relieved by rest or nitrates

Question 125

Classic symptoms of angina do not always present in what patient populations?

Answer 125

1. elderly 2. women 3. diabetics

Question 126

ST-segment depression is due to _1_ and seen in _2_ and _ 3_anginas

Answer 126

1. sub-endocardial ischemia 2. stable 3. unstable

Question 127

ST segment elevation is due to _1_ and is seen in _2_ angina

Answer 127

1. transmural ischemia | 2. Prinzmetal's

Question 128

an occlusion or spasm causing myocardial ischemia and subsequent myocardial tissue death is defined as

Answer 128

MI

Question 129

If an MI involves the inner 1/3 of heart wall, this is called a _ MI

Answer 129

subendocardial MI

Question 130

If an MI involves full thickeness of heart wall, it is known as a _

Answer 130

transmural MI

Question 131

If an MI involves right ventricle+ posterior 1/3 of septum, what artery is likely occluded?

Answer 131

RCA

Question 132

If an MI involves anterior left ventricle + anterior 2/3 of septum, what artery is likely occluded?

Answer 132

LAD

Question 133

In the evolution of an MI, what changes are seen within 0-24 hours?

Answer 133

- Ischemic injury to cardiomyocyte is irreversible after 30minutes - No visible changes in first 4 hours - contraction bands visible 12-24hrs - coagulation necrosis with 4-24hrs - myocytes release contents around 4 hours - neutrophils beginning to emigrate around 24 hours - risk of arrhythmia which can cause sudden death

Question 134

In the evolution of an MI what changes are seen 24-72 hours post ischemia?

Answer 134

- acute inflammation - hyperemia - neutorphils destorying dead tissue, extensive neutrohilic infiltration and continue coagulation necrosis on histology - infarcted region appear pale - risk of arrhythmia

Question 135

In the evolution of an MI what changes are seen 3-7 days post ischemia

Answer 135

- early granulation tissue encircles area of infarction - macrophages remove debris via phagocytosis as well as structural components resulting in weakness in the infarcted area - highest risk of free wall rupture (tamponade), septal rupture (ventricular septal defect), papillary muscle rupture (mitrgal regurg)

Question 136

In the evolution of an MI what changes are seen 10-14 days post ischemia

Answer 136

extensive granulation tissue present | - revascularization of infarcted territory begins

Question 137

In the evolution of an MI what changes are seen 2wks-2months post ischemia

Answer 137

- fibroblasts deposti type I collagen, resultin gin notably decreased cellularity of tissue - ongoing contraction of scar through myofibroblast actoin

Question 138

In the evolution of an MI what changes are seen after 2 months post ischemia?

Answer 138

- dense collagen scar formation | - risk for ventricular aneurysm

Question 139

In the evolution of an MI during which time frame is the patient at highest risk for tamponade, ventricular septal defect and/or mitral rgurgation?

Answer 139

3-7 day spost ischemia when the early granulation tissue begins to encircle area of infarct.

Question 140

What symptoms would a patient under going an MI present with?

Answer 140

- acute onset chest pain that may radiate to the left arm, jaw, neck, epigastrum and shoulder lasting >30mins and not relived by rest or nitroglycerin - diaphroesis - SOB - nausea/vomiting - lightheadedness/dizziness

Question 141

which patients are at greatest risk of silent MI's?

Answer 141

- elderly - post-menopausal women - diabetics

Question 142

What physical findings are present on a patient with an MI?

Answer 142

- tachycardia - new mitral regurgitation - S4 - hypotension - crackles

Question 143

what EKG changes are seen in patient undergoing an MI and what do those changes indicate?

Answer 143

1. ST elevation or ST depression (reflects transmural ischemia or subendothelial ischemia) occurs within minutes and resolves after 24-48 hrs) 2. T wave inversoin (reflects transmural infarction, occurs withinhrs, returns to upright after weeks) 3. Q waves : reflect transmural infarction, occurs within hours

Question 144

What cardiac enzymes are present in a patient under going an MI?

Answer 144

1. troponin in first 8 hrs and disappears 7-10 days 2. CK-MB in the first 24hrs and disappears around 3-4 days. (assume re infarction if CK-MB is still up after 4 days) 3. LDH1 for 2-7 days after symptoms.

Question 145

The most common cause of death post MI is _

Answer 145

cardiac arrhythmias (90%) and then LV failure and pulmonary edema (60%)

Question 146

What complications are seen in a patient post MI?

Answer 146

- Cardiac arrhythmias - LV failure and pulmonary edema - Mural thrombus - Cardiogenic shock - Fibrnous pericarditis - Dressler's syndrome

Question 147

An autopsy is being performed on an elderly man who died from a myocardial infarction. Biopsy of the heart is likely to reveal necrosis most similar to necrosis seen in which of the following scenarios? 1. The central nervous system following a stroke 2. The lung following a tuberculosis infection 3. Acute pancreatitis resulting from release of enzymatically active enzymes into the pancreas 4. A region of kidney where blood flow is obstructed 5. An abscess

Answer 147

4. A region of kidney where blood flow is obstructed In a myocardial infarction (MI), the type of necrosis that presents is coagulative necrosis. Coagulative necrosis is also seen in kidney infarcts.

Question 148

A 64-year-old male with a past medical history of obesity, diabetes, hypertension, and hyperlipidemia presents with an acute onset of nausea, vomiting, diaphoresis, and crushing substernal chest pain. Vital signs are temperature 37° C, HR 110, BP 149/87, and RR of 22 with an oxygen saturation of 99% on room air. Physical exam reveals a fourth heart sound (S4), and labs are remarkable for an elevated troponin. EKG is shows ST elevation. The pathogenesis of the condition resulting in this patient’s presentation involves: 1. Genetic inheritance of a mutation in ß-myosin or troponin expressed in cardiac myocytes 2. A fully obstructive thrombus at the site of a ruptured, ulcerated atherosclerotic plaque 3. A partially occlusive thrombus at the site of a ruptured, ulcerated atherosclerotic plaque 4. Destruction of the vasa vasorum caused by vasculitic phenomena 5. A stable atheromatous lesion without overlying thrombus

Answer 148

2. A fully obstructive thrombus at the site of a ruptured, ulcerated atherosclerotic plaque. A fully obstructive thrombus at the site of a ruptured atherosclerotic plaque has caused an ST-elevation myocardial infarction (STEMI) in this patient.

Question 149

A 60-year-old woman with history of type I diabetes currently on hormone replacement therapy is seen in your ED complaining of "bad indigestion", dizziness and nausea for the past several hours. Vitals are T98.9, HR 102, BP 130/100, RR 25 and she is mildly diaphoretic. An EKG is shows ST elevation. At what time point does the injury to her affected cells become irreversible? 1. 5 seconds 2. 60 seconds 3. 10 minutes 4. 30 minutes 5. 1 hour

Answer 149

4. 30 minutes The woman in question is having an ST-elevation myocardial infarction (STEMI) resulting from reduced blood flow to cardiomyocytes. After 30 minutes of ischemia, injury to cardiomyocytes is irreversible.

Question 150

A 62-year-old male collapses while mowing the lawn, and it is determined that he experienced sudden cardiac death. The patient's medical history is significant for a preceding myocardial infarction that was managed conservatively. Posthumous histologic evaluation of the patient's heart reveals extensive granulation tissue replacing dead myocardium as well as early evidence of neovascularization. How long prior to death did this patient most likely experience his myocardial infarction? 1. 16 hours 2. 3 days 3. 8 days 4. 2 weeks 5. 2 months

Answer 150

4. 2 weeks. Extensive granulation tissue replacing dead myocardium and early neovascularization are histologic changes expected 10-14 days after a myocardial infarction (MI).

Question 151

Loss of contractility of myocardial cells after an MI occurs in how long after the onset of the MI?

Answer 151

less 2 minutes. After 10 mins ATP is reduced to 50% and after 40mins it's reduced to 10%

Question 152

If you suddenly occlude an coronary artery, the first part of the heart to die is _

Answer 152

endocardial regions because that is the part that is least well perfused compared to the epicardium that's very well perfused.

Question 153

The zone of necrosis after an coronary artery occlusion starts _ and spreads to _

Answer 153

starts in endocardium and spreads to the epicardium

Question 154

the most sensitive and specific biomarkers of myocardial damage are cardiac-specific proteins, particularly _ and _

Answer 154

cTnT and cTnI

Question 155

Prompt reperfusion is the preeminent objective for treatment of patiets with MI. this can be accomplished by a host of coronary intervention such as _

Answer 155

1. thrombolysis 2. angioplasty 3. Stent placement 4 Coronary artery bypass graft (CABG) surgery

Question 156

When perfusion is reestablished, there is an exaggerated contraction of sarcomeres that can be seen with irreversibly injured myocytes. These form an intensely eosinophilic intracellular "stripes" composed of closely packed sarcomeres. these "stripes" are called _

Answer 156

contraction bands

Question 157

what is meant by stunned myocardium?

Answer 157

In reperfused myocytes, it is a state of prolonged cardiac failure induced by short term ischemia that usually recovers after several days

Question 158

Levels of cTnT and cTnI begin to rise within _ hrs and peaks at _ hrs

Answer 158

3-12 hrs; peaks at 12-48 with max at 24 hrs.

Question 159

Why is CK-MB considered sensitive but not specific for MI?

Answer 159

CK-MB is localized specifically to the cardiac muscle with considerably less in skeletal muscle. It's levels can also rise during skeletal muscle injury.

Question 160

When are you likely to see Ck-MB levels peak?

Answer 160

starts at about 3-12 hrs and peaks at 24 hrs.

Question 161

Indicate when each of these markers return back to normal levels in circulation A. CK-MB B. cTnI c. cTnT

Answer 161

A. 48-72 hrs B. 5-10 days C. 5-14 days

Question 162

One of the complication of an MI is development of Dressler's syndrome. What is Dressler's syndrome?

Answer 162

autoimmune disease that leads to fibrinous pericarditis several weeks post-MI

Question 163

Explain how cardiogenic shock develops post MI

Answer 163

Decreased cardiac output due to decreased contractile tissue and aneurysm. Ventricular aneurysm rupture is uncommon but may facilitate embolus formation and present as precordial bulge during systole.

Question 164

Fibrinous pericardidits results in friction rub that can be present after _1_ days/weeks/months post MI, and pain is relieved by leaning _2_.

Answer 164

1. 3-5 days | 2. forward

Question 165

_ is progressive congestive heart failure as a consequence of accumulated ischemic myocardial damage and/or inadequate compensatory responses. In most cases, there has ben prior MI or previous coronary arterial interventions and/or bypass surgery.

Answer 165

Chronic ischemic heart disease

Question 166

Sudden cardiac death is defined as death within _ post onset of cardiac ischemic symptoms.

Answer 166

within 1 hr.

Question 167

what are some causes of sudden cardiac death?

Answer 167

- Ventricular arrhythmia secondary to ischemia caused by severe CAD, disruption of atherosclerotic plaque. - typically NOT associated with thrombus

Question 168

In patients with chronic ischemia heart disease, contractile myocytes are replaced with what kind of tissue?

Answer 168

non-contractile fibrous tissue

Question 169

what are the risk factors associated with chronic ischemic heart disease?

Answer 169

- DM - smoking - HTN - cholesterol/lipid abnormalities - family history - age >45 (men), age > 55 (women)

Question 170

A 50 yr old male presents with DM and long term HTN presents with angina massive cardiomegaly, pulmonary vascular congestion, interstitial pulmonary edema, dilated cardiomyopathy and reduced ejection fraction. These findings are consistent with what heart disease?

Answer 170

Chronic ischemic heart disease!

Question 171

Arrhythmias can manifest as _

Answer 171

tachycardia bradycardia ventricular fibrillation asystole Pt often complain of "racing hearts" palpitations, with severe arrhtymias leading to light-headedness, syncope --> sudden cardiac death.

Question 172

what is the most common cause of rhythm disorders?

Answer 172

Ischemic injury

Question 173

What are some causes of arrhythmias?

Answer 173

1. Ischemic injury 2. SA node damage (sick sinus syndrome) 3. atrial fibrillation 4. Dysfunction AV node --> heart block

Question 174

The most important primary electrical abnormalities predisposing to arrhythmias is _

Answer 174

channelopathies which are caused by mutations in genes that are required for normal ion channel function involving either components of the channel or accessory proteins needed for that channel function

Question 175

What is Long QT synndrome?

Answer 175

Mutation in KCNQ1, KCNH2, SCN5A or CAV3. Manifests as arrhythmias assocaited with excessive prolongation of cardiac repolarization; pt often presents with stress induced syncope or sudden cardiac death and some forms are associated with swimming

Question 176

What is short QT syndrome?

Answer 176

mutation in gene KCNQ1 or KCNH2. patients have arrhythmias associated with abbreviated repolarization intervals; they can present with palpitations, syncope, and SCD

Question 177

How do patients with Brugada syndrome present?

Answer 177

ECG abnormalities (ST elevation and right BBB) in the absence of structural heart disease; pts present with syncope or SCD during rest or sleep or after large meals.

Question 178

Which primary channelopathy is susceptible to malignant ventricular arrhythmias?

Answer 178

Long QT syndrome

Question 179

What is the leading cause of SCD?

Answer 179

coronary artery disease

Question 180

What are the minimal pathologic criteria for the diagnosis of systemic HHD?

Answer 180

1. LV hypertrophy (usually concentric) in the absence of other CV pathology. ( LV >2 cm; heart >500 gm) 2. clinical hx of pathologic evidence of HTN in other organs (e.g. kidney)

Question 181

Depending on the severity, duration, underlying basis of HTN and on the adequacy of therapeutic control patient with hypertensive heart disease will have the following course:

Answer 181

1. Normal longevity and die of unrelated causes 2. develop IHD 3. suffer renal damage or cerebrovascular stroke 4. progressive HF

Question 182

Distinguish between valvular stenosis vs valvular insufficiency

Answer 182

Stenosis is the failure of a valve to open completely, which impedes forward flow. Insufficiency results from failure of a valve to close completely, thereby allowing reversed flow.

Question 183

Generally, valvular stenosis leads to _1_overload, whereas valvular insufficiency leads to _2_ overload

Answer 183

1. Pressure | 2. Volume

Question 184

The most frequent causes of major functional valvular lesions are:

Answer 184

1. Aortic stenosis: calcification and slcerosis of anatomically normal or congenitally bicuspid aortic valves 2. Aortic insufficiency: dilation of the ascending aorta, often secondary to HTN or aging 3. Mitral stenosis: rheumatic heart disease 4. Mitral insufficiency: myxomatous degeneration (mitral valve prolapse

Question 185

The leading cause of valvular heart disease in the US is

Answer 185

mechanical degeneration

Question 186

In the developing world, the leading cause of valvular disease is _

Answer 186

rheumatic fever

Question 187

This type of valvular disease occurs mostly in the elderly and is due to mechanical wear and tear resulting in dystrophic calcification and can result from chronic rheumatic fever, mitral valve damage. On PE pts will have a paroxically split S2 and a classic crescendo-decreasendo murmur.

Answer 187

Aortic Stenosis

Question 188

what are the signs and symptoms of aortic stenosis?

Answer 188

- asymptomatic until very advanced and once symptomatic progress rapidly within 5 years. - angina w/exercise - syncope with exercise - concentric LV hypertrophy leading to CHF - parodixically split S2 - crescendo-decrescendo murmur - pulsus parvus et tardus

Question 189

Explain what is meant by parodoxically split S2 as heart in aortic stenosis

Answer 189

Aortic valve closes later than normal which results in a single sound during inhalation when P2 closes later and a split S2 during exhalation when P2 closes earlier.

Question 190

What is meant by pulsus parvus et tardus as seen in aortic stenosis

Answer 190

weak and late pulse due to lost pressure traversing the stenotic valve

Question 191

A 76-year-old male with a history of DM, HTN, and CAD presents to the ED w/ SOB and altered mental status. On PE, his BP is 85/40 mmHg and a V/VI crescendo-decrescendo systolic ejection murmur is heard that is immediately preceded by a click. Concerned about a cardiac pathology, the doc immediately obtains an EKG. Reading the EKG, she states that the EKG reading in combination with the cause of his murmur was most likely causing his current presentation. What did the doc most likely see on the EKG?

Answer 191

This patient has aortic stenosis (AS). On EKG the doc most likely saw atrial fibrillation which will cause hemodynamic compromise in pts with aortic stenosis due to redued LV preload. AS pts are high dependent on preload. Thus, a loss in atrial contraction, as occurs in atrial fibrillation, will cause a drop in cardiac output that results in hemodynamic compromise due to reduced LV preload. Aortic valve replacement is the treatment of choice for symptomatic patients.

Question 192

Aortic regurgitation can occur acutely (due to trauma, aortic dissection, or infection) or chronically causes of which include _

Answer 192

- birth defect - rhematic fever - Connective tissue disorder - longstanding essential HTN

Question 193

What are some signs and symptoms of aortic regurgitation?

Answer 193

- Pts may present with worsening symptoms including dyspnea on exertion, orthostatic hypotension. - on chest auscultation blowing early diastolic murmur at LSB due to blood rushing back into heart; Austin Flint murmur which is a retrograde blood from aortic valve during diastole hits anterior leaflet of the mitral valve - head-bobbing with heart beat - Corrigan's pulse: carotid pulse with rapid rise and fall - water hammer pulse - femoral bruits on compression of femoral pulse

Question 194

A 27-year-old male with a history of injection drug use has been feeling short of breath and fatigued for the past several weeks. He is having trouble climbing the stairs to his apartment and occasionally feels like his heart is racing out of control. His past medical history is most notable for a previous bout of infective endocarditis after which he was lost to follow-up. On exam, you note that his carotid pulse has rapid rise and fall. Which of the following would you also expect to find? 1. Mid-systolic click 2. Fixed, split S2 3. Venous hum 4. Widened pulse pressure 5. Systolic murmur that increases with valsalva

Answer 194

4. Widened pulse pressure. This patient is showing signs and symptoms of heart failure. The history of infective endocarditis paired with the carotid pulse findings raise suspicion for aortic regurgitation and one ought to examine for a widened pulse pressure. The most common cause of aortic regurgitation is a bicuspid aortic valve. Aortic regurgitation following infective endocarditis is a result of post inflammatory scarring. Early symptoms generally include palpitations and dyspnea on exertion. Late symptoms are those indicative of heart failure as well as syncope. On exam, one may detect a widened pulse pressure (a large difference between the systolic and diastolic blood pressure) and involuntary head bobbing.

Question 195

The most common etiology of mitral stenosis is _

Answer 195

rheumatic fever

Question 196

With mitral stenosis, dilation of which heart chamber is seen?

Answer 196

Left atrium

Question 197

What are some common signs and symptoms of mitral stenosis?

Answer 197

- dyspnea on exertion - arrhythmias - orthopnea - infective endocarditis - opening snap on chest auscultation - mid-diastolic rumble - crackles and rales on lungs - dilated atria --> a-fib - elevated pulmonary capillary wedge pressure - LV end diastolic pressure is NORMAL

Question 198

A 58-year-old woman with a history of rheumatic fever has been experiencing exertional fatigue and dyspnea. She has begun using several pillows at night to sleep and occasionally wakes up at night gasping for air. On exam, she appears dyspneic and thin. Cardiac exam reveals a loud S1, opening snap, and apical diastolic rumble. Which of the following is the strongest predictor of the severity of her cardiac problem? 1. Greater intensity of the diastolic rumble 2. Short time between A2 and the opening snap 3. Presence of a soft P2 4. Shorter duration of the diastolic rumble 5. Presence of rales

Answer 198

2. Short time between A2 and the opening snap This patient has mitral stenosis. The time between A2 and the opening snap is inversely correlated with the severity of mitral stenosis. With increasingly severe mitral stenosis, the duration of the diastolic murmur increases and the opening snap occurs earlier during diastole as a result of higher left atrial pressure. If flow across the mitral valve is reduced because of heart failure or other causes (pulmonary hypertension, or aortic stenosis), the intensity of the murmur of mitral stenosis may be reduced or may be inaudible.

Question 199

A 37-year-old male presents to your clinic with shortness of breath and lower extremity edema. He was born in Southeast Asia and emigrated to America ten years prior. Examination demonstrates 2+ pitting edema to the level of his knees, ascites, and bibasilar crackles, as well as a late diastolic blowing murmur best heard at the cardiac apex. The patient undergoes a right heart catheterization that demonstrates a pulmonary capillary wedge pressure (PCWP) of 24mmHg. The patient is most likely to have which of the following? 1. Increased pulmonary vascular compliance 2. Decreased pulmonary artery systolic pressure (PASP) 3. Increased left ventricular end diastolic pressure (LVEDP) 4. Normal left ventricular end diastolic pressure (LVEDP) 5. Decreased transmitral gradient

Answer 199

4. Normal LVEDP This patient most likely has mitral stenosis (MS), which causes increased pressure proximal to the stenosis, sparing the left ventricle. This patient presents with signs and symptoms of heart failure, and on exam has a murmur consistent with mitral stenosis (MS). MS causes increased pressure proximal to the stenosis, and can result in increased pulmonary capillary wedge pressure, increased PA systolic pressure, loss of pulmonary vascular compliance, a dilated RV, and perhaps functional tricuspid regurgitation. However, it is important to note that the left ventricle is spared and does NOT experience increased pressure, and so PCWP is not an accurate measure of LVEDP. Indeed, the gradient between the PCWP (which should be equal to left atrial pressure) and the left ventricle is used to grade the severity of MS.

Question 200

What are the major causes of mitral regurgitation?

Answer 200

- rheumatic fever - chordae tendonae reupture - mitral valve prolapse

Question 201

Due to mitral regurgitation there is retrograde blood flow into LA, leading to increased _ volume and increased LA volume

Answer 201

LV end-diastolic

Question 202

In late manifestation of chronic mitral regurgitation dilation of which chamber(s) are seen?

Answer 202

LA and LV

Question 203

What are some signs and symptoms of mitral regurgitation?

Answer 203

- dyspnea - orthopnea - fatigue - on auscultation holosystolic murmur that radiates to the axilla

Question 204

A 61-year-old male dies in a motor vehicle accident. Autopsy of the heart reveals dilatation of the left atrium and expansion of the left ventricular cavity with associated eccentric hypertrophy. The structural changes in this patient's heart are most likely associated with which of the following? 1. Pulmonic stenosis 2. Mitral insufficiency 3. Chronic hypertension 4. Wolff-Parkinson-White syndrome 5. Congenital atrial septal defect

Answer 204

2. Mitral insufficiency Mitral insufficiency increases left ventricular (LV) end-diastolic volume (LVEDV) and left atrial volume due to regurgitant blood flow, causing dilatation in both chambers. Over time, Increased LVEDV and extra contraction needed to pump regurgitant volume results in eccentric LV hypertrophy.

Question 205

A 50-year-old female presents with a holosystolic murmur heard best over the apex, radiating to the axilla. She has no signs of pulmonary hypertension or edema. What best explains her lack of symptoms? 1. The right ventricle is compensating with decreased compliance 2. The left atrium is compensating with increased compliance 3. The aorta is compensating with increased compliance 4. As long as preload in the left ventricle is maintained there would be no symptoms 5. There is only a ballooning of the valve which would not result in any hemodynamic changes in the heart

Answer 205

2. The LA is compensating w/ increased compliance. The patient in this vignette most likely has chronic mitral regurgitation, based on her lack of symptoms and characteristic murmur. Chronic mitral regurgitation is characterized by an increased left atrial compliance.

Question 206

what the major causes of tricuspid regurgitation?

Answer 206

1. carcinoid heart disease (tumor of GI --> mets to liver | 2. IV drug use (results in right heart endocarditis possibly due to s. aureus infection)

Question 207

What are some signs and symptoms of tricuspid regurgitation?

Answer 207

- pulsating liver - chest auscultation reveals pansystolic murmur with increased intensity during inspiration due to increased venous return to right heart

Question 208

A 68-year-old male visits his primary care physician after an episode of syncope during a tennis match. He reports long-standing exertional dyspnea with mild substernal chest pain. On physical exam a systolic crescendo-decrescendo murmur is heard best at the right 2nd intercostal space. Which of the following would support a diagnosis of aortic stenosis? 1. Presence of S3 2. Murmur radiates to carotid arteries bilaterally 3. Murmur radiates to axilla 4. Asymmetric ventricular hypertrophy 5. Double pulsation of the carotid pulse

Answer 208

2. Murmur radiates to carotid arteries bilaterally. A systolic crescendo-decrescendo ("ejection") murmur that radiates to the carotid arteries is typical in aortic stenosis. Aortic stenosis is a narrowing of the aortic valve that can arise from congenital abnormalities (unicuspid or bicuspid valve), age-related atherosclerotic calcification, and rheumatic heart disease. Individuals with unicuspid valves usually present in childhood and those with bicuspid, in middle age, while those with other causes are usually > 65. Symptoms only occur in more advanced stages and include angina, fatigue, and dyspnea.

Question 209

Myxomatous degeneration of the mitral valve, associated with connective tissue disorder. Also known as floppy valve and easily seen on ECHO.

Answer 209

Mitral valve prolapse (MVP) - most common cause of mitral regurg.

Question 210

In this genetic disorder there are only two functional cusps unequal in size with the larger one having a midline raphe which is the major site of calcific deposits. These patients develop calcific stenosis. A. what is the likely diagnosis? B. loss of function mutation in what leads to it?

Answer 210

A. bicuspid aortic valve | B. NOTCH1

Question 211

Bicuspid aortic valve is usually asymptomatic in early life, but can develop what complications later in life?

Answer 211

1. Aortic stenosis 2. aortic regurg 3. infective endocarditis 4. aortic dilation and/or dissection

Question 212

The calcification see in aortic stenosis is usually on what structure?

Answer 212

on the cusps

Question 213

The calcification in mitral stenosis is usually on what structure?

Answer 213

fibrous annulus.

Question 214

Pts with mitral annular calcification have an increased risk of _ and calcific nodules can become a nidus for infective endocarditis.

Answer 214

embolic stroke

Question 215

In mitral valve prolapse, one or both mitral vale leaflets are _1_ into the LA during _2_

Answer 215

1. floppy, thus prolapse | 2. LA

Question 216

mitral valve prolapse is commonly seen in what gender/sex?

Answer 216

female (7:1 ratio)

Question 217

The key histologic change in the tissue in MVP is marked thickening of the spongiosa layer with deposition of mucoid material called _

Answer 217

myxomatous degeneration

Question 218

What are some signs and symptoms of MVP?

Answer 218

- most are asymptomatic - palpitations - chest pain (not asso. w/ CAD or MI) - exertional dyspnea - fatigue - cough - orthopnea - rarely presents are panic disorder - Mid-systolic click (due to sudden tensing of chorda tendinae - late systolic murmur may follow the click - may find palpable thrill over chest wall

Question 219

A 29-year-old nulliparous woman is found upon transthoracic echocardiography to have a dilated aorta and mitral valve prolapse. The patient has a history of joint pain, and physical examination reveals pectus excavatum and stretch marks on the skin. She does not take any medications and has no history of past drug use. The patient’s findings are most likely associated with which of the following underlying diagnoses? 1. Ehlers-Danlos syndrome 2. Turner syndrome 3. DiGeorge syndrome 4. Friedrich’s ataxia 5. Marfan syndrome

Answer 219

5. Marfan syndrome This patient's presentation with joint pain, pectus excavatum, stretch marks on the skin, and findings on echocardiography of mitral valve prolapse and a dilated aorta is consistent with an underlying diagnosis of Marfan syndrome.

Question 220

Rheumatic heart disease is a sequela of _ infection

Answer 220

pharyngeal streptococcal A infection

Question 221

_ is a valvular abnormality secondary to rheumatic fever and most often involves which valve?

Answer 221

RHD. -- > mitral stenosis due to sterile vegetation

Question 222

In RDH, the distinctive lesions that occur in the heart is called _1_, consisting of foci of T lymphocytes, occasional plasma cells and plump activated macphages called _2_

Answer 222

1. Aschoff bodies 2. Anitschkow cells (the macrophages have "caterpillar cell" appearance cuz of wavy slender ribbon like neuclei)

Question 223

During an acute RF, diffuse inflammation and aschoff bodies may be found on which layer(s) of the heart?

Answer 223

On ALL three ---> pancarditis (endo, epi and pericarditis)

Question 224

Explain the findings in the endocardium due to RF.

Answer 224

Inflammation of endocardium and the left-sided valves result in fibrinoid necrosis within the cusps or tendinous cords --> forms vegetations called verrucae

Question 225

Explain the findings in the subendothelium with acute RF

Answer 225

irregular thickening aled MacCallum plaques usually in lft atrium

Question 226

What are the cardinal anatomic changes seen in the mitral valve in chronic RHD?

Answer 226

leaflet thickening, commissural fusion and shortening and thickening and fusion of the tendinous cords

Question 227

What are the clinical features of RF?

Answer 227

1. migratory polyarthritis of large joints 2. pancarditis 3. subcutaneous nodules 4. erythema marginatum of skin 5. Sydenham chorea (disorder with involuntary rapid, purposeless movements)

Question 228

According to Jones Criteria what's needed to diagnose RF?

Answer 228

1. Preceding group A strep infeciton | 2. two of the major manifestation or one major and two minor clinical features associated with RF

Question 229

In most patients with RF, serology will be positive for even if culture for strep is negative?

Answer 229

ASO and DNase B

Question 230

What lab findings are associated with RF?

Answer 230

- Positive ASO - positive throat culture - elevated ESR and CRP - leukocytosis increased PR interval

Question 231

What's the treatment for RF?

Answer 231

Penicillin for acute RF

Question 232

Infective endocarditis is defined as _

Answer 232

microbial infection of the heart valves or mural endocardium that leads to the formation of vegetation composed of thrombotic debris and organisms often associated with destruction of the underlying cardiac tissues.

Question 233

A 38-year-old male presents to his primary care physician complaining of increasing shortness of breath over the past 2 months. He reports experiencing an extended illness of several weeks as a child that required him to miss school. He is unsure but believes it involved a sore throat followed by a fever and joint pains. He does not recall seeing a physician or receiving treatment for this. Today, on physical examination, cardiac auscultation reveals an opening snap after the second heart sound followed by a diastolic, decrescendo murmur. A follow-up echocardiogram is conducted. Which of the following best explains the pathophysiology of this patient's condition? 1. Annular calcification 2. Epitope homology 3. Myocardial ischemia 4. Atherosclerosis 5. Congenital heart defect

Answer 233

2. Epitope homology. This patient is suffering from mitral stenosis secondary to prior rheumatic fever. Rheumatic fever and heart disease are the result of bacterial and human epitope homology. Streptococcal proteins exhibit molecular mimicry that is recognized by the immune system. The bacterial M protein and human cardiac antigens, including myosin and valvular endothelium, are homologous. T-cells responding to streptococcal infection infiltrate the valvular tissue and initiate an inflammatory cascade that ultimately results in fibrosis and valve damage over time.

Question 234

A 10-year-old male presents with his mother with multiple complaints. A few weeks ago, he had a sore throat for several days that improved without specific therapy. Additionally, over the past several days he has experienced pain in his ankles and wrists and, more recently, his left knee. His mother also noted several bumps on both of his elbows, and he has also had some pain in his center of his chest. He thinks the pain is better when he leans forward. On physical examination, he is noted to be mildly febrile, and a pericardial friction rub is auscultated. Which of the following histopathologic findings is most likely associated with this patient's condition? 1. Atypical lymphocytes noted on peripheral blood smear with an initial positive heterophil antibody test 2. Plasmodium falciparum ring forms in red blood cells on peripheral blood smear 3. Needle-shaped, negatively birefringent crystal deposits surrounded by palisading histiocytes in the synovial fluid of an affected joint 4. Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin 5. Sterile vegetations on both the ventricular and atrial aspects of the mitral valve, a proliferative glomerulonephritis, and serum anti-dsDNA and anti-Sm positivity

Answer 234

4. Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin. This patient's presentation is consistent with a diagnosis of acute rheumatic fever and associated carditis. Histologic findings associated with this condition include Aschoff bodies, interstitial myocardial granulomas composed of surrounding Anitschkow or caterpillar cells, which are plump macrophages with abundant cytoplasm and central, ovoid nuclei with slender ribbons of chromatin.

Question 235

A 9-year-old male visited his primary care physician for a check-up three months after a throat infection. Upon examination, the patient exhibits painless subcutaneous nodules on the back of the wrist, the outside elbow, and the front of the knees, as well as inflammation in the joints of the lower extremities. Which of the following symptoms is most likely to also be present in this patient? 1. Renal failure 2. Hepatomegaly 3. Chorea 4. Pleural effusion 5. Butterfly rash

Answer 235

3. Chorea The clinical picture of polyarthritis of the lower extremities and subcutaneous nodules following a pharyngitis is consistent with rheumatic fever (RF). RF may result in Sydenham's chorea, a characteristic series of rapid, purposeless movements of the face and arms.

Question 236

Infective endocarditis is inflammation of the heart valve secondary to the infeciton and is usually _1_ sided, unless the patient has a history of IV drug use in which case it is usually _ 2_sided and involves _ 3_valve

Answer 236

1. Left 2. Right 3. Triuspid

Question 237

_ is typically caused by infection of a previously normal heart valve by a highly virulent organism such as staph aureus that rapidly produces necrotizing nd destructive lesion.

Answer 237

Acute infective endocarditis

Question 238

_ is characterized by organism with lower virulence such as viridans stretococci that cause insidious infections of deformed valves with overall less destruction. The disease may pursue a protracted course of weeks to months and cures can be achieved with abx.

Answer 238

Subacute

Question 239

Endocarditis of native but previously damaged or otherwise abnormal valves is most commonly cuase by what bacteria?

Answer 239

Streptococus viridans

Question 240

_ is the major offender in infective ndocarditis among IV drug users

Answer 240

S. Aureus

Question 241

What enterococci are known to cause endocarditis?

Answer 241

HACEK group (Haemophilus, actinobacillus, cardiobacterium, eikenella, kingella)

Question 242

What bacteria which is coagulase negative is known to cause endocarditis in pts with prosthetic valve?

Answer 242

Staphylococci epidermidis

Question 243

Morphologically, _ on heart valves are the classic hallmark of infective endocarditis; these are friable, bulky, potentially destructive lesion containing fibrin, inflammatory cells and bacteria or other organisms.

Answer 243

Vegetations

Question 244

Describe how infective endocarditis presents

Answer 244

Acute IE has a "storm onset" with rapidly developing fever, chills, weakness, and lassitude. - murmurs in 90% of pts with left-sided IE. - Flue-like symptoms - SOB - embolization of vegetations produce immune complex depositions and vasculitis ( Osler's nodes, Janeway lesion, Roth spots, subungual petechia)

Question 245

explain the Duke criteria required for diagnosis of IE.

Answer 245

Requires either pathologic or clinical criteria. If clinical criteria are used, 2 major, 1 major+3 minor, or 5 minor criteria are needed.

Question 246

According to Duke criteria, Osler's nodes, Janeway lesion, and Roth spots falls under which category for diagnosis of IE.

Answer 246

Minor

Question 247

What are the two noninfected endocarditis (vegetations?

Answer 247

1. Marantic endocarditis | 2. Libman-Sacks endocarditits

Question 248

What marantic endocarditis characterized by?

Answer 248

paraneoplastic syndrome: a sterile vegetation on the valves as a result of increased coagulability secondary to increased mucin production, same process underlying Trousseau's sign. - primary cancers in GI tract - may produce embli to periphery

Question 249

What is Libman-Sacks endocarditis characterized by?

Answer 249

Caused by SLE, usually asymptomatic but mitral regurg murmur can be heard

Question 250

_ refers to systemic disorder marked by flushing, diarrhea, dermatitis, and bronchoconstriction that is caused by bioactive compounds such as serotonin released by carcinoid tumors.

Answer 250

Carcinoid syndrome

Question 251

In carcinoid syndrome, cardiac lesion develop only after massive _ metastatic burden

Answer 251

hepatic, since liver normally catabolizes circulating mediators before the heart.

Question 252

what mediators are elaborated by carcinoid tumors?

Answer 252

- serotonin - kallikrein - bradykinin - histamine - prostaglandins - tachykinins

Question 253

What complications are implicated with prosthetic valves?

Answer 253

1. thrombosis/thromboembolism 2. anticoagulat-related hemorrhage 3. Prosthetic valve endocarditis (s epidermidis), s aureus, streptococi, and fungi 4. structural deteroriation (intrinsice, wear, facture, poppet fialure, cuspal tear etc) 5. inadequate healing (paravalvular leak, exuberant healing, hemolysis

Question 254

What are the three types of cardiomyopathies?

Answer 254

1. Dilated 2. Hypertrophic 3. Restrictive

Question 255

Correlate the mechanisms HF in each of the three types of cardiomyopathies

Answer 255

- Dilated = impaired contractility (systolic dysfunction) | - Hypertrophic and restrictive = impaired compliance (diastolic dysfunction)

Question 256

cardiomyopathies symptoms are often secondary to _

Answer 256

heart failure

Question 257

True or false: cardiomyopathies includes alterations in heart muscle that are secondary to HTN, CAD, or valvular disease

Answer 257

False

Question 258

what is the most common form of cardiomyopathies?

Answer 258

Dilated cardiomyopathies (DCM)

Question 259

In which cardiomyopathy is ejection fraction reduced?

Answer 259

Dilated cardiomyopathy

Question 260

What the etiologies of dilated cardiomyopathy (DCM)

Answer 260

- idiopathic - genetic: mutation in proteins that affects the interaction between cytoskeleton and sarcomere; dystrohin gene mutation (X-linked); - Toxins (alcohol abuse (via direct toxic damage to myocardium and associated with thiamine deficiency --> beriberi); doxorubicin toxicity; cobalt - Infectious (coxsakievirus, chagas) - Peripartum cardiomyopathy - Iron overload - supraphysiologic stress such as excess catecholamines, pheochromocytomas (takotsubo cardiomyopathy= LVD)

Question 261

What are the symptoms/physical findings of DCM?

Answer 261

- ineffective contraction --> slowly progressive CHF; S3 heart sound and mitral regurg murmur - on ECHO: dilated ventricles and decreased EF - on CXR: balloon like heart

Question 262

How is DCM treated?

Answer 262

- ACEI/ARB - beta blocker - spironolactone - digoxin for symptom improvement only

Question 263

What is arrhythmogenic right ventricular cardiomyopathy (ARVC)?

Answer 263

- an inherited disease of myocardium causing right ventricular failure and rhythm disturbanes (V-tach and V-fib) with sudden death.

Question 264

how does the heart appear morphologically in a patient with ARVC?

Answer 264

- RV wall is severely thinned due to loss of myocytes, accompanied by extensive fatty infiltration and fibrosis.

Question 265

Classically ARVC has _1_ inheritnace pattern attributed to defective cell adhesion proteins in the desmosomes that linke adjacent cardiac myocytes. _2_ syndrome is associated with ARVC and hyperkeratosis due to mutation in desmosome-associated protein plakoglobin.

Answer 265

1. autosomal dominant | 2. Naxos syndrome

Question 266

_ is characterized by myocardial hypertrophy, poorly compliant LV myocardium leading to abnormal diastolic filling and intermittent ventricular outflow obstruction. The heart is heavy and hyperconracting.

Answer 266

Hypertrophic cardiomyopathy (HCM)

Question 267

HCM is caused by mutation in any one of several genes that encode what proteins?

Answer 267

Sarcomeric proteins

Question 268

Over 70-80% of all cases mutation of which genes specific leads to HCM?

Answer 268

- beta-myosin heavy chain (b-MHC), - gens encoding cardiac TnT - a-tropomyosin - myosin-binding protein C

Question 269

Which cardiomyopathies is the most common cause of sudden death in young atheletes? Explain how

Answer 269

HCM. - -The thickened septal wall is too close to the anterior leaflet of mitral valve and - -obstructs flow to aortic valve (leaflet is drawn open by fast moving blood being ejected during systole - - there's also conduction irregularies which results in arrhythmias and is the main cause of death

Question 270

Friedreigh's ataxia is assocaited with which cardiomyopathy?

Answer 270

HCM

Question 271

What symptoms and physical findings are likely in HCM?

Answer 271

- harsh systolic ejection crescend-descresndo murmur heard best in apex and left sternal border. No radiation to carotids - increased intensity with decreased preload (best observed when asking pt to go from squatting to standing position) - On ECHO: asymetrically thick LV walls ; may show normal EF; and show concentric ventricular hypertrophy - On histology: tangled, disoriented myofibrils

Question 272

What is the treatment for HCM?

Answer 272

- beta blockers - CCB (non DHP) - disopyramide - septoplasty

Question 273

What are the causes of restrictive cardiomyopathy

Answer 273

- chronic, progressive infiltrative or meatbolic disease such as amyloidosis, sarcoidos, hemochromatosis, cancer, endocardial fibroelastosis, Loffler's endocardial fibrosis (eosinophilic infiltrate with endomyocardial fibrosis w/wo presence of parasitic infection), glycogen stoarge disease (pmope's disease)

Question 274

Restrictive cardiomyopathy diagnosis is usually confirmatory using ECHO which will show _

Answer 274

LVH.

Question 275

A 19-year-old basketball player unexpectedly collapses on the court. Several minutes later he returns to consciousness and is able to continue playing. This has happened several times before with similar outcomes. He had no significant past medical history. Which of the following is most likely to be found in this patient? 1. Atheromatous plaque rupture 2. Coagulation necrosis with loss of nuclei and striations 3. Septal hypertrophy 4. Postductal coarctation of the aorta 5. Cardiac myxoma

Answer 275

3. Septal hypertrophy This clinical picture is consistent with hypertrophic cardiomyopathy (HCM) and autopsy would reveal a hypertrophic intraventricular septum.

Question 276

A 12-year-old female with an autosomal dominant mutation in myosin-binding protein C is being evaluated by a pediatric cardiologist. The family history reveals that the patient's father died suddenly at age 33 while running a half-marathon. What was the likely finding on histological evaluation of his father's heart at autopsy? 1. Myocyte disarray 2. Amyloid deposits 3. Eosinophilic infiltration 4. Wavy myocytes 5. Viral particles

Answer 276

1. Myocyte disarray Based on the patient's mutation and family history, the patient described above likely has Hypertrophic Cardiomyopathy (HCM). About half of HCM cases are due to an autosomal dominant genetic disease that, along with myocyte disarray, has the histological hallmarks of fibrosis and myocyte hypertrophy.

Question 277

A 49-year-old man presents to his physician complaining of weakness and fatigue. On exam, you note significant peripheral edema. Transthoracic echocardiogram is performed and reveals a preserved ejection fraction with impaired diastolic relaxation. Which of the following is likely the cause of this patient's symptoms? 1. Previous treatment with doxorubicin 2. Hemochromatosis 3. Heavy, long-term alcohol consumption 4. History of myocardial infarction 5. History of a recent viral infection

Answer 277

2. Hemochromotosis The patient's presentation is consistent with heart failure and echocardiographic findings suggest restrictive cardiomyopathy. Hemochromatosis is the only answer option associated with restrictive cardiomyopathy.

Question 278

An 18-year-old African-American male presents to his family physician for a routine sports physical. He has a family history of sudden death at a young age. Upon physical examination the physician appreciates a systolic murmur. The intensity of the murmur increases when performing a valsalva maneuver. What is the most likely cause of this murmur? 1. Aortic valve stenosis 2. Mitral stenosis 3. Tricuspid stenosis 4. Hypertrophic cardiomyopathy 5. Benign systolic flow murmur

Answer 278

4. Hypertrophic cardiomyopathy This patient has hypertrophic obstructive cardiomyopathy (HOCM) and has developed a murmur due to abnormal systolic anterior motion of the mitral valve.

Question 279

A 66-year-old female with a past medical history significant for hypertension and breast cancer that is in remission after chemotherapy, presents to her primary care physician complaining of progressive dyspnea, decreased exercise tolerance, and paroxysmal nocturnal dyspnea. On chest auscultation you note an S3. Which of the following medications is likely responsible for the patient's current presentation? 1. Lisinopril 2. Digoxin 3. Cytarabine 4. Hydrochlorothiazide 5. Doxorubicin

Answer 279

5. Doxorubicin Doxorubicin is a common antineoplastic agent, used in treatment of breast, ovarian, and bladder cancer as well as other solid tumors. Cardiotoxicity may occur months to years after drug administration, resulting in a dilated cardiomyopathy and a clinical heart failure as seen in this patient.

Question 280

A 19-year-old Caucasian male collapsed from sudden cardiac arrest while playing in a college basketball game. Attempts at resuscitation were unsuccessful. Post-mortem pathologic and histologic examination found asymmetric left ventricular hypertrophy and myocardial disarray. Assuming this was an inherited condition, the relevant gene most likely affects which of the following structures? 1. Cardiac cell sarcomere proteins 2. Membrane potassium channel proteins 3. Ryanodine receptors 4. Autoimmune beta-cell antibodies 5. Membrane sodium channels

Answer 280

1. Cardiac cell sarcomere proteins This patient’s sudden cardiac arrest at a young age is suggestive of hypertrophic cardiomyopathy, an inherited autosomal dominant mutation of genes that encode for cardiac cell sarcomere proteins.

Question 281

What are some causes of the serous type pericarditis?

Answer 281

- SLE - RA - Viral infection - Uremia

Question 282

what are some causes of fibrnous type pericarditis?

Answer 282

- Uremia - MI (immediately after or several weeks later (dressler's syndrome) - Rheumatic fever

Question 283

Hemorrhagic pericarditis is usually caused by _

Answer 283

TB or cancer

Question 284

Constrictive pericarditis is usually caused by _

Answer 284

- Open heart surgery - Radiotherapy to the mediastinum - TB - Idiopathic

Question 285

How to patients with pericarditis usually present?

Answer 285

- dyspnea - cough - fever often after URI - pleuritic chest chain (positional in nature) - PE shows pericardial friction rub (heard when pt leans forward) - May hear pericardial knock - Kussmaul's signs (inceased JVP on inspiration)

Question 286

A 34-year-old Caucasian female presents at the ER with fever and sharp pain in her chest upon coughing and inhalation. Three weeks earlier she presented to her rheumatologist with a butterfly rash, joint pain and fatigue and was given a diagnosis of systemic lupus erythematosus. A friction rub is present upon physical exam. Which of the following do you most suspect in this patient? 1. Pulmonary hypertension 2. Interstitial lung disease 3. Acute myocardial infarction 4. Pericarditis 5. Pericardial tamponade

Answer 286

4. Pericarditis The clinical presentation is consistent with pericarditis with classic signs such as sharp pain in the chest, a friction rub, and a disease past medical history of SLE which often occurs with pericarditis.

Question 287

A 35-year-old African-American female presents to the emergency room complaining of chest pain. She also complains of recent onset arthritis and increased photosensitivity. Physical examination reveals bilateral facial rash. Which of the following is most likely to be observed in this patient? 1. Pain improves with inspiration 2. Pain relieved by sitting up and leaning forward 3. High-pitched diastolic murmur 4. Displaced apical impulse 5. Mid-systolic click

Answer 287

2. Pain relieved by sitting up and leaning forward Arthritis, photosensitivity, and facial rash in an African-American woman are consistent with systemic lupus erythematosus (SLE). The most common cardiovascular syndrome associated with SLE is pericarditis.

Question 288

A 45-year-old African-American woman presents with dyspnea, cough, and non-radiating chest pain. Her chest pain is relieved by leaning forward and worsens upon leaning backwards. A scratchy rub is heard best with the patient leaning forward. Physical examination did not elucidate evidence of a positive Kussmaul's sign, pulsus paradoxus, or pericardial knock. The patient most likely is suffering from which of the following? 1. Cardiac tamponade 2. Constrictive pericarditis 3. Acute pericarditis 4. Libman-Sacks endocarditis 5. Acute myocardial infarction

Answer 288

3. Acute pericarditis The clinical presentation is characteristic of acute pericarditis. Non-radiating chest pain that is relieved by leaning forward and a scratchy rub heard on cardiac auscultation are prominent features of pericarditis.

Question 289

A 35-year-old Caucasian female with a history of rheumatoid arthritis presents to your clinic with pleuritic chest pain that improves while leaning forward. Which of the following additional findings would you expect to observe in this patient? 1. Increase in jugular venous pressure on inspiration 2. Exaggerated amplitude of pulse on inspiration 3. Pulsatile abdominal mass 4. Continuous machine-like murmur 5. S3 heart sound

Answer 289

1. INcrease in JVP on inspiration The patient described in the question stem has pericarditis, a common complication of rheumatoid arthritis. Pericarditis can restrict venous return to the right heart resulting in an increase in JVP on inspiration (Kussmaul's sign).

Question 290

What is the most common primary cardiac tumor in adults?

Answer 290

Myxoma (has a mucopolysaccharide stroma that are mesenchymal in origin)

Question 291

where does myxoma usually occur?

Answer 291

Atria (90%)

Question 292

How is myxoma usually diagnosed?

Answer 292

Transesophageal ECHO

Question 293

A ball-valve obstruction in the mitral valve orifice is described as what primary cardiac neoplasm?

Answer 293

Myxoma

Question 294

How do patients with myxoma usually present?

Answer 294

Non-specific (flu-like symptoms)

Question 295

What is the most common complication of myxoma?

Answer 295

1. Syncopal episodes (due to tumor obstructing the mitral valve) 2. embolization

Question 296

What is the most common cardiac primary tumor in kids?

Answer 296

Rhabdomyoma.

Question 297

Rhabdomyoma has a high association with what multisystem genetic disease?

Answer 297

Tubermous sclerosis (TSC1 and TSC2)

Question 298

what's more common in the heart: primary tumors or secondary?

Answer 298

Secondary tumors/metastases from other sites.

Question 299

What are some common metastasis of the heart?

Answer 299

- Melanoma - lymphoma - lung cancer