Clinical Presentation of Congenital Heart Disease in the First Week of Life: Cyanosis
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Question 1 of 9
1. Question
You are called to evaluate a newborn male patient who appears “blue”. His O2 saturation is 70% on room air. An x-ray of his chest demonstrates increased pulmonary vasculature. You perform an echocardiogram and note that the right ventricle pumps blood directly into the aorta in the absence of a VSD. Which of the following conditions does the patient have?
Hint
This child has hypoxemia in the context of a congenital heart lesion. Most children with cyanotic congenital heart disease have diminished pulmonary blood flow. This is not true for children with D-transposition of the great vessels.
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Question 2 of 9
2. Question
A 2-day-old infant is noted to have persistent cyanosis. The pregnancy was unremarkable, but the mother has a history of bipolar disorder and has been taking lithium throughout her pregnancy. The birth was uncomplicated. On exam, his O2 saturation is 65%. You obtain a chest x-ray which demonstrates an unusually large heart. Which of the following is the most likely diagnosis?
Hint
The use of lithium in pregnancy has been associated with an increased risk of developing Ebstein’s anomaly. The pathognomonic finding on chest x-ray is a very large heart with decreased pulmonary blood flow.
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Question 3 of 9
3. Question
You are called to the neonatal intensive care unit to evaluate a 2-day old baby born at 28 weeks gestation via emergent C-section for preterm labor and fetal distress for respiratory distress. The baby has a respiratory rate of 80 breaths per minute with moderate subcostal and intercostal retractions. He is requiring CPAP to maintain adequate ventilation and oxygenation. A chest x-ray shows a diffuse pattern consistent with fluid-filled alveoli. Which of the following is the MOST likely diagnosis of this baby’s condition?
Hint
This baby was born prematurely with underdeveloped lungs based on gestational age. Although congenital heart disease is a consideration in the cyanotic newborn, based on gestational age, it is much more likely that his respiratory status is related to lung underdevelopment.
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Question 4 of 9
4. Question
You are evaluating a 2-day-old term neonate in the newborn nursery who is hypoxemic. The chest x-ray is unrevealing. The ECG reveals normal sinus rhythm with a superior axis and left ventricular hypertrophy. Which of the following congenital heart disease lesions is MOST consistent with these ECG findings?
Hint
Complete atrio-ventricular canal and tricuspid atresia affect electrical conduction through the heart and will result in a superior axis on ECG due to displacement of the tricuspid valve.
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Question 5 of 9
5. Question
You are evaluating a newborn infant with a prenatal diagnosis of Tetralogy of Fallot with pulmonary stenosis. His ECG confirms right ventricular hypertrophy and his chest x-ray shows decreased pulmonary blood flow. What type of murmur would you expect to hear on cardiac auscultation?
Hint
Tetralogy of Fallot presents with right ventricular hypertrophy and decreased pulmonary flow. Patients with pulmonary stenosis will have a systolic ejection murmur turbulent blood flows through the stenosed pulmonary valve during systole.
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Question 6 of 9
6. Question
You perform an echocardiogram on a hypoxemic newborn infant and diagnose right-to-left shunting at the atrial level. Chest radiograph shows normal heart size. Which of the following diagnoses is MOST likely?
Hint
Think about how when blood cannot get out through a stenotic pulmonary valve it backs up in the right ventricle and right atrium, and from there it either backs up into the body or can shunt across an intracardiac connection.
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Question 7 of 9
7. Question
You are caring for a hypoxemic neonate who has an echocardiogram which reveals that blood flow to the pulmonary arteries largely is supplied by the ductus arteriosus as opposed to from the right ventricle. The right ventricle appears hypertrophied and the aortic inlet is overriding the ventricular septum. There is a ventricular septal defect present. Which of the following congenital cardiac lesions are these echocardiogram findings consistent with?
Hint
The lack of blood flow out the right ventricle and into the pulmonary arteries raises concern for an atretic valve. In this case there are several other findings that raise concern for a congenital cardiac lesion involving more than just the pulmonary valve.
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Question 8 of 9
8. Question
Which of the following BEST distinguishes between pulmonary stenosis and pulmonary atresia with intact ventricular septum?
Hint
Pulmonary atresia with intact ventricular septum and pulmonary stenosis both have decreased pulmonary blood flow on chest x-ray, left ventricular hypertrophy on ECG, and an inferior axis on ECG.
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Question 9 of 9
9. Question
Which of the following congenital heart lesions will have a normal oxygen saturation?
Hint
The common forms of cyanotic congential heart lesions include d-transposition of the great vessels with intact ventricular septum, total anomalous pulmonary venous connection, Ebstein’s anomaly, tricuspid atresia, pulmonary atresia with intact ventricular septum, severe pulmonary stenosis, Tetralogy of Fallot with pulmonary atresia, and Tetralogy of Fallot with pulmonary stenosis.