FE Congenital Heart Disease Recognition 2 — Questions and Answers
Question 1: Which echocardiographic finding is most characteristic of hypoplastic left heart syndrome (HLHS)?
- Dilated left ventricle with poor contractility
- Severely diminutive left ventricle with aortic and mitral atresia or stenosis (Correct answer)
- Absent right ventricle with pulmonary atresia
- Large ventricular septal defect with overriding aorta
Correct answer: Severely diminutive left ventricle with aortic and mitral atresia or stenosis
HLHS is characterized by a severely underdeveloped left ventricle associated with aortic and/or mitral atresia or severe stenosis.
Question 2: In fetal echocardiography, which view best demonstrates the relationship between the great arteries in transposition of the great arteries (TGA)?
- Four-chamber view
- Three-vessel view
- Long-axis view of the outflow tracts showing parallel great vessels (Correct answer)
- Ductal arch view
Correct answer: Long-axis view of the outflow tracts showing parallel great vessels
In TGA, the long-axis outflow tract view demonstrates the aorta and pulmonary artery running parallel rather than crossing each other normally.
Question 3: A fetus is found to have a single large vessel overriding the ventricular septum with no separate pulmonary artery identified. This is most consistent with:
- Tetralogy of Fallot
- Truncus arteriosus (Correct answer)
- Double outlet right ventricle
- Pulmonary atresia with intact ventricular septum
Correct answer: Truncus arteriosus
Truncus arteriosus features a single arterial trunk overriding the ventricular septum that gives rise to both systemic and pulmonary circulations.
Question 4: Which cardiac defect is most commonly associated with Turner syndrome (45,X) on fetal echocardiography?
- Tetralogy of Fallot
- Transposition of the great arteries
- Coarctation of the aorta and bicuspid aortic valve (Correct answer)
- Atrioventricular septal defect
Correct answer: Coarctation of the aorta and bicuspid aortic valve
Turner syndrome is strongly associated with left-sided obstructive lesions, particularly coarctation of the aorta and bicuspid aortic valve.
Question 5: On fetal echocardiography, the 'levocardia with situs inversus' configuration most commonly occurs with:
- Normal intracardiac anatomy
- Congenitally corrected transposition
- Complex congenital heart disease in up to 95% of cases (Correct answer)
- Isolated ventricular septal defect
Correct answer: Complex congenital heart disease in up to 95% of cases
Isolated levocardia with situs inversus is strongly associated with complex congenital heart disease in the majority of affected fetuses.
Question 6: A fetal echocardiogram shows both the aorta and pulmonary artery arising exclusively from the morphologic right ventricle. This defines:
- Tetralogy of Fallot
- Double outlet right ventricle (DORV) (Correct answer)
- Transposition of the great arteries
- Truncus arteriosus
Correct answer: Double outlet right ventricle (DORV)
DORV is defined by both great arteries arising entirely or predominantly from the morphologic right ventricle.
Question 7: Which finding on the four-chamber view suggests total anomalous pulmonary venous return (TAPVR)?
- Enlarged right atrium and ventricle with no pulmonary veins draining to the left atrium (Correct answer)
- Dilated coronary sinus only
- Absent foramen ovale
- Hydrops with pericardial effusion alone
Correct answer: Enlarged right atrium and ventricle with no pulmonary veins draining to the left atrium
TAPVR may appear as right heart enlargement with absence of normal pulmonary venous connections to the left atrium on the four-chamber view.
FE Congenital Heart Disease Recognition 2
Which echocardiographic finding is most characteristic of hypoplastic left heart syndrome (HLHS)?