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com Volume 193

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50 Years Ago in The Journal of Pediatrics

Differential Cyanosis in Congenital Heart Disease
Hipona FA, Sanyal SK. J Pediatr 1968;72:194-200

T his 1968 article by Hipona and Sanyal published in The Journal reported 3 children with congenital heart disease
with specific patterns of differential cyanosis in different extremities.
The first child was an 8-month-old infant with transposition of the great arteries, patent ductus arteriosus (PDA),
and pulmonary hypertension with reversed differential cyanosis (cyanosis was found more in the face and right hand
than in the legs) because of the reversal of ducal flow owing to increased pulmonary vascular resistance. A high pulse
volume recording and significant preductal aortic coarctation cause reversed flow through the descending aorta, re-
sulting in differential cyanosis in children with transposition of the great arteries.
The second case was a neonate with critical aortic stenosis and hypoplastic aortic arch causing reversed blood flow
from pulmonary hypertension through the PDA into the descending aorta perfusing the left upper limb and lower
part of the body (pink acyanotic right arm and differential cyanosis of left arm and lower legs). The third case was a
17-day-old neonate with fatal endocardial sclerosis with PDA and persistent fetal circulation who had differential cya-
nosis in legs.
The observational and clinical examination skills and interpretational abilities of clinicians in the 1950s and 1960s
allowed them to come to a reasonably precise clinical diagnosis, which was later confirmed by invasive diagnostic cardiac
angiographic procedures in the absence of noninvasive modalities like pulse oximetry and echocardiography. Differ-
ential cyanosis in a neonate indicates persistent pulmonary hypertension and critical coarctation of the aorta or in-
terrupted aortic arch with PDA. In an older child, it might indicate PDA with Eisenmenger syndrome. Reverse differential
cyanosis is diagnostic of transposition of the great arteries or Taussig Bing anomaly with ductal flow reversal owing to
high pulmonary vascular resistance or preductal coarctation of the aorta and rarely supracardiac total anomalous pul-
monary venous return with severe pulmonary hypertension.

Sadagopan Srinivasan, MD
Department of Pediatrics
JIPMER
Pondicherry

Kothandam Sivakumar, MD, DM

Pediatric Cardiology
Madras Medical Mission
Chennai, India

Reference

1. Yap SH, Anania N, Alboliras ET, Lilien LD. Reversed differential cyanosis in the newborn: a clinical finding in the supracardiac total anomalous
pulmonary venous connection. Pediatr Cardiol 2009;30:359-62.

210 Barrière et al