Question

Difficulty: MediumPediatric Cardiovascular Disorders

Match each pediatric clinical presentation and diagnostic finding to the most likely congenital cardiovascular disorder.

  • A neonate presents with cyanosis within hours of birth; chest radiography demonstrates a narrow mediastinum and an 'egg-on-a-string' cardiac silhouette.Transposition of the great arteries
  • A cyanotic infant is evaluated; electrocardiography reveals left axis deviation, tall P waves, and absent R waves in the right precordial leads.Tricuspid atresia
  • An older child presents with recurrent epistaxis; physical examination reveals bounding brachial pulses and significantly delayed, weak femoral pulses.Coarctation of the aorta
  • An asymptomatic toddler is noted to have a widely split, fixed S2 and a grade 2/6 systolic ejection murmur at the left upper sternal border.Atrial septal defect

Answer

The presentations match as follows: The 'egg-on-a-string' radiograph matches with Transposition of the great arteries. The cyanotic infant with left axis deviation matches with Tricuspid atresia. The child with epistaxis and delayed femoral pulses matches with Coarctation of the aorta. The toddler with a widely split, fixed S2 matches with Atrial septal defect.
Each clinical presentation is carefully matched to its pathognomonic physical examination, radiographic, or electrocardiographic findings. Recognizing these distinct diagnostic associations is essential for accurately identifying specific congenital heart defects.

Step-by-Step Solution

1
Analyze the first presentation: early cyanosis with an 'egg-on-a-string' chest radiograph.
Identified as Transposition of the great arteries.
The narrow mediastinum (string) and globular heart (egg) are the classic radiographic signs for Transposition of the great arteries.
2
Evaluate the second presentation: cyanosis combined with left axis deviation on an electrocardiogram.
Identified as Tricuspid atresia.
Most cyanotic congenital heart diseases feature right axis deviation and right ventricular hypertrophy. Left axis deviation in a cyanotic infant strongly points to Tricuspid atresia due to an underdeveloped right ventricle.
3
Assess the third presentation: upper body hypertension symptoms (epistaxis) and delayed lower body pulses.
Identified as Coarctation of the aorta.
A narrowing in the descending aorta causes high pressure proximal to the defect (head and arms) and low pressure distally (legs and lower body).
4
Review the fourth presentation: widely split, fixed S2 with a systolic ejection murmur.
Identified as Atrial septal defect.
The left-to-right shunt across the ASD leads to constant volume overload of the right ventricle, causing delayed pulmonic valve closure that does not vary with the respiratory cycle.

Key Concept

Classic diagnostic features and physical examination findings of pediatric congenital heart defects
Estimated Time:1m 30s
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