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Difficulty: HardPediatric Cardiovascular Disorders

Match each clinical presentation and combination of diagnostic findings to the most likely congenital heart defect.

  • Neonate presenting with profound cyanosis unresponsive to supplemental oxygen, a single S2, and a narrow mediastinum on chest radiograph.Transposition of the great arteries
  • Neonate presenting with cyanosis, a holosystolic murmur, and an electrocardiogram demonstrating left axis deviation and left ventricular hypertrophy.Tricuspid atresia
  • Infant with trisomy 21 presenting with failure to thrive, a holosystolic murmur, and a superior QRS axis on electrocardiogram.Complete atrioventricular septal defect
  • Adolescent presenting with cyanosis and clubbing isolated to the lower extremities, normal oxygen saturation in the right upper extremity, and a loud P2.Patent ductus arteriosus with Eisenmenger syndrome
  • Adolescent presenting with lower extremity claudication, a notable blood pressure discrepancy between the arms and legs, and erosions of the inferior margins of the posterior ribs on chest radiograph.Coarctation of the aorta

Answer

Left 1 matches Transposition of the great arteries; Left 2 matches Tricuspid atresia; Left 3 matches Complete atrioventricular septal defect; Left 4 matches Patent ductus arteriosus with Eisenmenger syndrome; Left 5 matches Coarctation of the aorta.
Each correct matching pair relies on recognizing pathognomonic clinical associations: the 'egg-on-a-string' sign in Transposition of the great arteries, LVH/LAD in cyanotic Tricuspid Atresia, superior QRS axis and Trisomy 21 in Complete atrioventricular septal defect, differential cyanosis in PDA with Eisenmenger syndrome, and rib notching with blood pressure discrepancies in Coarctation of the aorta.

Step-by-Step Solution

1
Analyze the first presentation: unresponsive cyanosis, single S2, narrow mediastinum.
Identified as Transposition of the great arteries.
The narrow mediastinum creates the classic 'egg-on-a-string' radiographic appearance, and the single S2 is due to the anterior-posterior relationship of the great vessels.
2
Analyze the second presentation: cyanosis with left axis deviation and left ventricular hypertrophy on ECG.
Identified as Tricuspid atresia.
Most cyanotic defects cause right ventricular hypertrophy; Tricuspid atresia is a notable exception due to right ventricular hypoplasia and left ventricular volume overload.
3
Analyze the third presentation: Trisomy 21, holosystolic murmur, superior QRS axis.
Identified as Complete atrioventricular septal defect.
This is the classic presentation of an endocardial cushion defect, which is strongly associated with Down syndrome and classically demonstrates a superior QRS axis.
4
Analyze the fourth presentation: differential cyanosis (clubbing in lower extremities only).
Identified as Patent ductus arteriosus with Eisenmenger syndrome.
The ductus arteriosus connects to the descending aorta, so right-to-left shunting secondary to pulmonary hypertension delivers deoxygenated blood only post-ductally, sparing the right arm.
5
Analyze the fifth presentation: upper/lower extremity blood pressure discrepancy and rib notching.
Identified as Coarctation of the aorta.
Rib notching is caused by dilated intercostal collateral arteries that bypass the aortic narrowing to deliver blood to the lower extremities.

Key Concept

Clinical, radiographic, and electrocardiographic hallmark associations of congenital heart defects
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Topics

Pediatric Cardiovascular Disorders

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