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Pediatrics · Systemic paediatrics: atrioventricular septal defect in Down syndrome
Sample itemAn infant with Down syndrome has a systolic murmur and early heart failure. ECG shows a superior (left) axis deviation with a prolonged PR interval. Which lesion is most likely?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Complete atrioventricular septal defect
Atrioventricular septal defect is the characteristic cardiac lesion of Down syndrome, from abnormal endocardial cushion development. The conduction tissue is displaced, which causes a superior axis on ECG. Early surgical repair is needed because pulmonary vascular disease develops early in these infants.
Option by option
A. Patent ductus arteriosus
A PDA gives a continuous murmur and does not cause a superior axis.
B. Secundum atrial septal defect
A secundum ASD gives right axis deviation and a right bundle branch pattern.
C. Complete atrioventricular septal defect (correct)
Correct. Superior axis with a prolonged PR in Down syndrome points to an AV septal defect.
D. Isolated perimembranous ventricular septal defect
An isolated VSD does not shift the axis superiorly.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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