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peads Cardio - meduploader
#1
1. A cyanotic newborn is suspected of having congenital heart disease. He has an increased left ventricular impulse and a holosystolic murmur along the left sternal border. The ECG shows left axis deviation and left ventricular hypertrophy (LVH). Which of the following is the most likely diagnosis?

1. Transposition of the great arteries
2. Truncus arteriosus
3. Tricuspid atresia
4. Tetralogy of Fallot
5. Persistent fetal circulation

2. A 3-day-old infant with a single second heart sound has had progressively deepening cyanosis since birth but no respiratory distress. Chest radiography demonstrates no cardiomegaly and normal pulmonary vasculature. An electrocardiogram shows an axis of 120° and right ventricular prominence. Which of the following congenital cardiac malformations is most likely responsible for the cyanosis?

1. Tetralogy of Fallot
2. Transposition of the great vessels
3. Tricuspid atresia
4. Pulmonary atresia with intact ventricular septum
5. Total anomalous pulmonary venous return below the diaphragm

3. During a physical examination for participation in a sport, a 16-year-old girl is noted to have a late apical systolic murmur, which is preceded by a click. The rest of the cardiac examination is normal. She states that her mother also has some type of heart "murmur" but knows nothing else about it. Which of the following is the most likely diagnosis?

1. Atrial septal defect
2. Aortic stenosis
3. Tricuspid regurgitation
4. Mitral valve prolapse
5. Ventricular septal defect
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#6
1. The answer is c, Tricuspid atresia

Patients with tricuspid atresia typically have a hypoplastic right ventricle, and therefore the ECG shows left axis deviation and left ventricular hypertrophy; this translates to a left ventricular impulse on physical examination. Almost all other forms of cyanotic congenital heart disease are associated with elevated pressures in the right ventricle and increased right ventricular impulse. In those conditions, therefore, the ECG will show right axis deviation and right ventricular hypertrophy.

2. The answer is b, Transposition of the great vessels.

Transposition of the great vessels with an intact ventricular septum presents with early cyanosis, a normal-sized heart (classic "egg on a string" radiographic pattern in one-third of cases), normal or slightly increased pulmonary vascular markings, and an electrocardiogram showing right axis deviation and right ventricular hypertrophy. In tetralogy of Fallot, cyanosis is often not seen in the first few days of life. Tricuspid atresia, a cause of early cyanosis, causes diminished pulmonary arterial blood flow; the pulmonary fields on x-ray demonstrate a diminution of pulmonary vascularity, and left axis and left ventricular hypertrophy are shown by electrocardiogram. Total anomalous pulmonary venous return below the diaphragm is associated with obstruction to pulmonary venous return and a classic radiographic finding of marked, fluffy-appearing venous congestion ("snowman"). In pulmonic atresia with an intact ventricular septum, cyanosis appears early, the lung markings are normal to diminished, and the heart is large.

3. The answer is d, Mitral valve prolapse

Mitral valve prolapse occurs with the billowing into the atria of one or both mitral valve leaflets at the end of systole. It is a congenital abnormality that frequently manifests only during adolescence or later. It is more common in girls than in boys and seems to be inherited in an autosomal dominant fashion. On clinical examination, an apical murmur is noted late in systole, which can be preceded by a click. The diagnosis is confirmed with an echocardiogram that shows prolapse of the mitral leaflets during mid- to late systole. The ECG and chest x-ray are usually normal. Beta blockers and digitalis are unlikely to be required, but penicillin prophylaxis for dental procedures for patients with mitral valve prolapse, especially if a murmur is present, is indicated.
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