My curious case: Infant with rare lung disorder

TAPVC, a condition where the blood vessels that transport pure blood from the lung to the left side of the heart are attached elsewhere.

Written by Anuradha Mascarenhas | Published:February 6, 2016 12:25 am

My unusual case was that of a 16-day-old boy with mixed total anomalous pulmonary venous connection (TAPVC), a condition where the blood vessels that transport pure blood from the lung to the left side of the heart are attached elsewhere. The infant weighed just 2,500 grams at birth and had to be wheeled in for an emergency paediatric cardiac surgery.

The baby was born full term and was suspected of having meconium aspiration, a condition when the meconium, the first stool of an infant, is present in the lungs during or before delivery. This meconium is composed of materials a newborn ingests when in the uterus.
The baby was immediately shifted to the neonatal intensive care unit and was kept on ventilator for conservative management. There was no improvement and hence the possibility of TAPVC was considered. This is a birth defect of the heart in which the pulmonary veins don’t connect to the left atrium like they should. Instead they go to the heart by way of an abnormal connection. The baby had a mixed variety of TAPVC — one of the complex congenital heart diseases. Also it is not uncommon for a heart murmur to be absent right at birth, so many a times this condition can be missed.

We had to hence use an echocardiogram and doppler colour flow mapping to initially diagnose the problem and locate the presence of pulmonary venous obstructions. The baby though had very low oxygen saturation and it was a medical challenge to bring out the little one from the ventilator. A median sternotomy approach, procedure where a vertical incision is made along the sternum, as then done. It provides access to the heart and lungs. After the surgery, the baby could maintain 96 per cent saturation in room air. Post operative recovery was uneventful. The patient underwent a major and tricky surgery just 16 days after being born but has had a good post operative back up.

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