Arrival and survival of a 3-week-old boy from Pakistan with an arterial oxygen saturation of 17%

Abstract

In newborn infants, acute perinatal hypoxic/ischaemic events and associated hyperoxia/reperfusion injury frequently lead to devastating neonatal brain damage. The present report concerns a 3-week-old boy from Pakistan with d-transposition of the great arteries (d-TGA), prolonged and severe hypoxaemia, and multiresistant bacterial sepsis. The term newborn infant underwent public airline transportation to Europe and presented on the airport's runway with severe hypoxaemia (pulsoximetric oxygen saturations (SpO2) 17%) and systemic hypotension. The patient eventually underwent late balloon atrial septostomy, followed by a successful two-stage arterial switch operation. A clinical follow-up 3-5 years later revealed lack of cerebral dysfunction, adequate neurodevelopment, good biventricular function, regular coronary flow, as well as normal ECG, blood pressure and SpO(2). The findings may indicate the neonatal brain adjusts better to chronic, slowly worsening hypoxia than to acute hypoxia (eg, "birth asphyxia"), and also suggests a greater tolerance for chronic hypoxia in neonates vs adults.

Figure 1

A. Term infant (10 days old) born in Pakistan with central cyanosis and postductal pulsoximetric oxygen saturation (SpO₂) of 70% to 72% at day of diagnosis. For echocardiographic findings and cardiovascular diagnosis see text and fig 2. B,C. The same patient 3 years later (B) and 4 years later (C) in excellent clinical condition with SpO₂ of 98%, mild remaining right pulmonary artery stenosis, good cardiac function, normal ECG and adequate neurodevelopment.

Figure 2

Colour Doppler echocardiography showing left-to-right atrial shunting via a patent formamen ovale (PFO) in a newborn infant with d-transposition of the great arteries (d-TGA). A. Restricted interatrial blood flow from the left atrium (LA) through the PFO into the right atrium (RA) as indicated by the narrowing and turbulence of the red signal across the atrial septum. Systemic venous return via the inferior vena cava (IVC) into the RA is indicated by blue colour. B. Aimilar image as (A) after successful balloon atrial septostomy (BAS; Rashkind procedure). Note the large left-to-right atrial shunt after the successful procedure.