Cardiology Flashcards

(123 cards)

1
Q

What is Tetralogy of Fallot (TOF) characterized by?

A

Overriding Aorta
Pulmonary Stenosis
Right Ventricular Hypertrophy
Ventricular Septal Defect (VSD)

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2
Q

What chr. deletion is Tetralogy of Fallot (TOF) associated with?

A

Chr. 22 Deletion

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3
Q

How does Tetralogy of Fallot (TOF) present?

A

Cyanosis of the lips and extremities

Holosystolic murmur best heard at left lower sternal border

Squatting after exertive activities

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4
Q

What do patients with TOF squat after exertion?

A

Increases preload and increases SVR: decreasing Right to left shunting: leading to increased pulmonary blood flow and increased blood oxygen saturation

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5
Q

What is the best initial test to diagnose TOF?

A

CXR: showing boot-shaped heart and decreased pulmonary vascular markings

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6
Q

What is the only Definitive therapy for TOF?

A

Surgical intervention

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7
Q

What are the 3 holosystolic murmurs?

A

Mitral Regurgitation (MR)

Tricuspid Regurgitation (TR)

Ventricular Septal Defect (VSD)

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8
Q

What conditions are commonly associated with VSDs?

A

Trisomy 21: Down syndrome

Trisomy 18: Edwards syndrome

Trisomy 13: Patau syndrome

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9
Q

What is the most common Congenital heart defect in Down syndrome?

A

Endocardial cushion defect of the AV canal

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10
Q

What is the most common cyanotic condition in children after the neonatal period?

A

Tetralogy of Fallot (TOF)

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11
Q

What is the most common cyanotic lesion during the neonatal period?

A

Transposition of the Great Vessels (TOGV)

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12
Q

What is Transposition of the Great Vessels (TOGV) Characterized by?

A

Aorta originates from the Right Ventricle (RV) and Pulmonary Artery that comes from the Left ventricle

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13
Q

What is needed for oxygenation to occur in Transposition of the Great Vessels (TOGV)?

A

Patent Ductus Arteriosus (PDA), Atrial Septal Defect (ASD), or VSD

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14
Q

How does Transposition of the Great Vessels (TOGV) typically present?

A

Early and severe cyanosis with Single S2

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15
Q

What imaging can be used to help diagnose Transposition of the Great Vessels (TOGV)?

A

CXR: showing “egg on a string”

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16
Q

How do you treat Transposition of the Great Vessels (TOGV)?

A

Ensure the neonate has a PDA

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17
Q

What do you give to ensure a neonate has a PDA in TOGV?

A

Prostaglandin E1 analog

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18
Q

What drugs are contraindicated in a neonate with TOGV?

A

NSAIDS: they will cause the PDA to close and oxygenation will not occur

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19
Q

What does Pulus Alternans suggest?

A

LV systolic dysfunction

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20
Q

What is the definitive treatment for TOGV?

A

2 separate surgeries: each has a 50% mortality: only 1/4 children survive

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21
Q

What does Pulsus Bigeminus suggest?

A

Hypertrophic Obstructive Cardiomyopathy (HOCM)

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22
Q

What does Pulsus Bisferiens suggest?

A

Aortic Regurgitation (AR)

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23
Q

What does Pulsus Tardus et Parvus suggest?

A

Aortic Stenosis

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24
Q

What does Pulsus Paradoxus suggest?

A

Cardiac tamponade and Tension Pneumothorax

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25
What does an irregularly irregular pulse suggest?
Atrial Fibrillation (A-fib)
26
What is Hypoplastic Left Heart Syndrome?
Syndrome consisting of Left Ventricular Hypoplasia, Mitral Valve Atresia and Aortic Valve Lesions
27
How does Hypoplastic Left Heart Syndrome present?
Absent Pulses with a single S2 Increased RV Impulse Gray Cyanosis Inaudible Murmurs Hyperdynamic Precordium
28
What is the best initial test for Hypoplastic Left Heart Syndrome?
CXR: showing a globular-shaped heart with pulmonary edema
29
What is the most accurate diagnostic test for Hypoplastic Left Heart Syndrome?
Echocardiogram
30
What are the treatment options for Hypoplastic Left Heart Syndrome?
3 staged surgeries or heart transplant
31
What is Truncus Arteriosus (TA)?
A single trunk emerges from both right and left ventricles and gives rise to all major circulations
32
How does Truncus Arteriosus (TA) Present?
Within the first few days of life: Severe dyspnea and frequent respiratory infections Single S2 Bounding Peripheral Pulses
33
Why is there only a single S2 in Truncus Arteriosus (TA)?
only one semilunar valve and a systolic murmur is heard because valve leaflets usually have abnormal functionality
34
What testing is used to diagnose Truncus Arteriosus (TA)?
CXR: showing cardiomegaly with increased pulmonary markings
35
How do you treat Truncus Arteriosus (TA)?
Prompt Surgery to prevent Pulmonary Hypertension -without surgery most develop within 4 months
36
What is Total Anomalous Pulmonary Venous Return (TAPVR)?
There is no venous return between pulmonary veins and the left atrium: oxygenated blood returns to the superior vena cava
37
What are the 2 forms of TAPVR?
With and Without obstruction
38
How does TAPVR with obstruction present?
Early in life with respiratory distress and severe cyanosis
39
What is the initial test for TAPVR with obstruction?
CXR: showing pulmonary edema
40
What is the definitive test for TAPVR with obstruction?
Echocardiogram
41
How do you treat TAPVR with obstruction?
Surgery
42
How does TAPVR without obstruction present?
1-2 years of age with Right heart failure and tachypnea
43
What is the initial diagnostic test for TAPVR without obstruction?
CXR: show snowman or figure 8 sign
44
What is the most accurate diagnostic test for TAPVR without obstruction?
Echocardiogram
45
How do you treat TAPVR without obstruction?
Surgical intervention
46
What is Tricuspid Valve Atresia?
Lack of communication between the Right heart chambers that results in hypoplastic right ventricular and pulmonary outflow tract and underdeveloped pulmonary valve and/or artery
47
How does Tricuspid Valve Atresia present?
Severe cyanosis in a newborn
48
What must patients with Tricuspid Atresia have?
Associated congenital PFO, ASD, or VSD to allowing mixing of oxygenated and deoxygenated blood and allow survival
49
How can you diagnose Tricuspid Atresia?
CXR: decreased pulmonary flow EKG: Left axis deviation and small or absent R waves in precordial leads and left ventricular hypertrophy
50
What are the treatment options for Tricuspid Atresia?
Prostaglandin E1: Keep PDA open until aortopulmonary shunt Possible Atrial balloon septostomy to enlarge the ASD Staged surgical correction
51
What are the Cyanotic Heart Defects (5)?
Tetralogy of Fallot (TOF) Transposition of the Great Vessels (TOGV) Hypoplastic Left Heart Syndrome Truncus Arteriosus Total Anomalous Pulmonary venous return (TAPVR)
52
Which Cyanotic Heart Defects have a R to L Shunt?
All of them
53
What Cyanotic Heart Defects are PDA dependent? (2)
TOGV and Hypoplastic Left Heart Syndrome
54
What Cyanotic Heart Defects have a VSD? (2)
Tetralogy of Fallot (TOF) and Truncus Arteriosus
55
What Cyanotic Heart Defects require surgery?
All of them
56
What is the most common Congenital heart lesion?
Ventricular Septal Defect (VSD)
57
How does a Ventricular Septal Defect (VSD) present?
Dyspnea with respiratory distress High-pitched holosystolic murmur over the lower left sternal border Loud Pulmonic S2
58
What can be seen on CXR for VSD?
Increased vascular markings
59
How do you diagnose a VSD?
Echocardiogram
60
How do you definitively diagnose a VSD?
Cardiac Catheterization
61
How do you treat Smaller VSDs?
They usually close in the first 1-2 years of life
62
How do you close larger or symptomatic VSDs?
Surgical intervention
63
What are conservative medical options for VSDs?
Diuretics and Digoxin
64
What are complications of an untreated VSD?
CHF, Endocarditis, and Pulmonary Hypertension
65
What is an Atrial Septal Defect (ASD)?
Hole in the septum between both atria; 2x more common in females
66
What are the 3 major types of ASD?
Primum Defect Secundum Defect Sinus Venosus Defct
67
What is the most common type of ASD?
Secundum defect: located in the center of the atrial septum
68
What is the least common type of ASD?
Sinus venosus defect
69
What is a Primum Defect ASD?
Concomitant mitral valve abnormalities are present
70
How do ASDs typically present?
Asymptomatic except for a fixed, wide splitting of S2
71
What is the most definitive test for an ASD?
Cardiac Catheterization
72
What is an alternative to Cardiac Cath to diagnose ASD?
Echocardiogram CXR: Showing increased vascular markings and cardiomegaly
73
How do you treat ASDs?
most close spontaneously Surgery for symptomatic patients
74
What complications are associated with ASDs?
Dysrhythmias and Paradoxical Emboli from DVTs
75
What is a Patent Ductus Arteriosus (PDA)?
failure of the ductus to spontaneously close: it usually closes when PO2>50mm Hg
76
How does PDA present?
Machine-like murmur wide pulse pressure Bounding pulses
77
What are the most common complications of a PDA?
CHF and Pulmonary Hypertension
78
What is the best initial test to diagnose a PDA?
Echocardiography
79
What is the most accurate test to diagnose a PDA?
Cardiac Catheterization
80
What might be seen on EKG for PDA?
LVH secondary to high systemic resistance
81
How do you treat a PDA?
Closure unless it is needed for concurrent cyanotic condition
82
How do you close a PDA?
Ibuprofen (NSAIDS inhibit Prostaglandins and cause closure) -if NSAID fails surgical ligation
83
What does a pear-shaped heart on CXR suggest?
Pericardial effusion
84
What does a boot-shaped heart on CXR suggest?
Tetralogy of Fallot (TOF)
85
What does a jug handle appearance of the heart on CXR suggest?
Primar Pulmonary Artery Hypertension
86
What does a "3-like" appearance or rib notching on CXR suggest?
Coarctation of the aorta
87
What is Coarctation of the Aorta?
Congenital narrowing of the aorta in the area of the ductus arteriosus
88
What is Coarctation of the Aorta frequently associated with?
Turner Syndrome
89
How does Coarctation of the Aorta Present?
Severe CHF and Respiratory distress in the first few months of life Differential pulses and pressures between upper and lower extremities Reduced pulses in the lower extremities and hypertension in upper extremities due to narrowing
90
What is the best initial test for Coarctation of the aorta?
Echocardiogram
91
What is the most accurate test for coarctation of the aorta?
MRA
92
What is seen on CXR in Coarctation of the aorta?
Rib notching and "3" sign
93
What is the confirmatory test for Coarctation of the Aorta?
Echocardiography
94
What test is done if a patient cannot get an MRA for coarctation of the aorta?
Cardiac Catheterization
95
How do you treat Coarctation of the Aorta?
Surgical Resection of the narrowed segment and balloon dilation if recurrent stenosis occurs
96
How does Long QT Syndrome present?
Hearing loss, Syncope, normal vitals and physical exam with family history of sudden cardiac death
97
What is the best treatment for Long QT syndrome?
Beta-Blocker such as Metoprolol -may shorten the QT interval by decreasing activation and reducing cardiac excitation
98
What is the treatment for Long QT syndrome if symptoms persist despite Beta-blocker usage?
Implantable cardioverter-defibrillator
99
What is the only beta-blocker that prolongs the QT interval?
Sotalol
100
What is Rheumatic Fever?
Autoimmune disease from untreated Pharyngeal streptococcal infection leading to cross-reactions between Strep Ag and Ag on joint and heart tissue
101
What is the most common heart valve involved in Rheumatic Heart Disease?
Mitral Stenosis
102
How do you diagnose Rheumatic Fever?
2 Major criteria or 1 Major + 2 minor
103
What are the Major criteria for Rheumatic Fever?
Migratory Polyarthritis Carditis: Myocarditis, Pericarditis Erythema Marginatum Subcutaneous Nodules Chorea
104
What are the Minor Criteria for Rheumatic Fever?
Fever Arthralgias Elevated ESR/CRP Prolonged PR Heart block on EKG
105
How do you treat Rheumatic Fever?
Antibiotics to eradicate Group A strep bacteria
106
What should be given to patients with Mitral valve disease from Rheumatic Fever?
Chronic Penicillin Therapy NSAIDs or Steroids to control inflammation
107
What is an Ebstein Anomaly?
Congenital heart defect where the tricuspid valve is downwardly displaced into the Right Ventricle
108
What is an Ebstein Anomaly Associted with?
Maternal Lithium Use in pregnancy
109
What will be found on Physical Exam for an Ebstein Anomaly?
Holosystolic murmur or TR over most of the anterior left chest
110
What does EKG show for an Ebstein Anomaly?
Tall P waves and Right axis deviation
111
What is the best test to evaluate the anatomy with suspected Ebstein Anomaly?
Echocardiography
112
What is used to diagnose Ebstein anomaly if Echo is nondiagnostic?
Cardiovascular MRI
113
What arrhythmia may patients with an Ebstein anomaly have?
Wolff-Parkinson-White-Syndrome -Delta wave and short PR interval
114
What is a Vascular Ring?
Abnormal development of the aortic arch forming a vascular ring that can compress the trachea, bronchi or esophagus
115
How does a vascular ring present?
Biphasic Stridor or dysphagia with spitting up after meals from compression
116
What are key facts to look for in the history for a vascular ring?
Respiratory symptoms that improve with neck extension and parents saying the child is a "noisy breather"
117
What are the 2 types of vascular rings?
Complete and Incomplete
118
What is a complete vascular ring?
Circumferential around the trachea and esophagus
119
What is an incomplete vascular ring?
Pulmonary artery sling
120
What is the diagnostic test of choice for a vascular ring?
CT or MRI
121
How do you treat asymptomatic, incidentally found rings?
Monitor
122
How do you treat symptomatic vascular rings?
Surgical correction
123
What genetic or malformation syndromes are associated with vascular rings?
DiGeorge and Down Syndrome