Term boy was delivered with birth weight of 1.8 kg. Bedside 2D echocardiography done for an incidental murmur showed truncus arteriosus (TA) type II with interrupted aortic arch (IAA) type B and a large patent ductus arteriosus (PDA) and hence, was started on intravenous prostaglandin. He had multiple other co-morbidities such as congenital pneumonia, anorectal malformations, and glanular hypospadias. FISH test was negative for DiGeorge syndrome.
In view of low birth weight and multiple co-morbidities, staged repair was planned and bilateral PA banding was done at 11 days of life. A 3-mm polytetrafluorethylene (PTFE) tube was used to create a band and placed on both PAs. The systemic partial pressure of oxygen after PA banding was 44 mm of mercury with FiO2 of 21%. The patient had good post-operative recovery and was sent to the primary hospital on the fourth post-operative day. Figure 1 demonstrates the computerized tomography (CT) scan of the pulmonary artery after PA banding.
However, the corrective surgery was delayed due to multiple episodes of Klebsiella pneumoniae bacteremia. Immunodeficiency study done revealed deficiency in T cell line. The PDA was kept patent by intravenous prostin at 5 ng/kg/min. Following optimization, with a weight of 7 kg, the baby was rendered suitable for definitive surgery after 13 months of PA banding and underwent truncus repair with IAA repair. Cardiopulmonary bypass (CPB) was established using bi-arterial (aortic and PDA) and bicaval cannulation and left heart venting via right superior pulmonary vein. PDA was ligated, and branch PAs were debanded and checked for distensibility. We occluded the branch PAs distal to the debanded site with neurosurgical clips. Ventilation was resumed while partially coming off CPB to allow the heart to pump against the distally clipped branched pulmonary arteries and allowed them to distend. The branch PAs distended completely without narrowing. CPB was resumed and pulmonary button was harvested. The arch was reconstructed using autologous pericardium under antegrade cerebral perfusion. Right ventricle to PA continuity was established using a 12-mm Contegra graft. Termination of CPB was eventful. The chest was closed after 24 h. The patient developed chylothorax, which responded to conservative management and was discharged on the seventh POD. The echocardiography done at the 4th post-operative day showed the gradient across the right PA to be 15 mmHg and through left PA as 20 mmHg. On 1 year follow-up, the child was noted to be doing well. The follow-up CT scan showed good branch PAs with no residual stenosis (Fig. 2).