Neonatal Cyanosis: Diagnostic and Management Challenges

A Izraelit,G Krishnamurthy,V Ten,V Ratner

doi:10.5402/2011/175931

Abstract

Neonatal central cyanosis is always a sign of serious pathological processes and may involve diverse organs and impose a significant diagnostic and therapeutic challenge. Here, we report an unusual presentation of Ebstein's anomaly, a rare congenital heart malformation, as the cause of central cyanosis in a one-week-old full-term infant. Initiation of PEG1 therapy in neonates with Ebstein's anomaly always needs a very careful consideration because of a high risk for the development of a “circular shunt” leading to severe deterioration of multiple organs perfusion.

Highlights

Cyanosis is a blue discoloration of the skin and mucus membranes caused by an increased concentration of reduced hemoglobin (>1.9–3.1 mmol/L) in the blood
Transthoracic ECHO revealed an unobstructed antegrade flow into the hypoplastic right ventricle and outflow without evident restriction or insufficiency (Figure 3(a)); a significantly echogenic tricuspid valve with inferior displacement of anterior and septal leaflets into the ventricle and absent tricuspid valve regurgitation (Figure 3(a)); right atrium moderately dilated with right-to-left
The presence of central cyanosis in the newborn without respiratory distress, with boot-shaped cardiomegaly and failed hyperoxia test favored a diagnosis of congenital heart disease

Summary

Introduction

Cyanosis is a blue discoloration of the skin and mucus membranes caused by an increased concentration of reduced hemoglobin (>1.9–3.1 mmol/L) in the blood. Central cyanosis is a serious pathological sign and involves discoloration of lips and tongue. It can be a formidable task to reach the right diagnosis in a neonate with central cyanosis. On the 8th day of life, the infant exhibited central cyanosis during crying. Vital signs at that time were as follows: body temperature of 36.8◦C, heart rate 193 beats/min, respiratory rate 45 breaths/min, pre and postductal oxygen saturations 77% and 81%, respectively, and blood pressure 93/45 mmHg without gradient between upper and lower extremities. Chest X-ray illustrated a mildly enlarged boot-shaped cardiac silhouette with absent pulmonary artery shadows, oligemic lung fields, and no focal pathology (Figure 2). Transthoracic ECHO revealed an unobstructed antegrade flow into the hypoplastic right ventricle and outflow without evident restriction or insufficiency (Figure 3(a)); a significantly echogenic tricuspid valve with inferior displacement of anterior and septal leaflets into the ventricle and absent tricuspid valve regurgitation (Figure 3(a)); right atrium moderately dilated with right-to-left

ISRN Pediatrics

Abnormal pattern of pulmonary blood flow

Discussion

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