Case Report
Informed written consent was taken from the guardian for publication. A two year old male child was brought with the history of dyspnea and cyanosis on mild exertion since 3 months of age. Patient had cyanosis and a to and fro murmur was present at the upper left sternal border. Chest X-ray revealed mild cardiomegaly, hypoplastic left lung and prominent right hilar shadow. Echocardiography revealed TOF morphology, large subaortic ventricular septal defect (VSD), severe pulmonary stenosis (PS) and dilated main pulmonary artery (MPA) with continuation only as a dilated right pulmonary artery (RPA), suggestive of absent left pulmonary artery (LPA). Neither PDA nor any major collateral supplying the left lung could be appreciated on aortography. Pulmonary vein wedge injection also confirmed its absence. No obvious compression of airway on the right side could be appreciated on CT scan. Child underwent successful total correction. Intra-operatively, pulmonary annulus was mildly stenotic, RPA was aneurysmally dilated and pulmonary valve was rudimentary (Figure 1 and 2). After minimal infundibular resection, VSD was closed with 5/0 polypropylene, TF needle, using continuous suture technique. As the annulus was small with rudimentary leaflets, hence for RVOT reconstruction the infundibular incision was extended onto MPA to a limited extent. In view of absence of airway symptoms, no compression of airways and absence of LPA, the dilated RPA was only partially plicated. Completion of RVOT reconstruction was done by creating a bicuspid valve from 0.1-mm PTFE membrane using Graham Nunn technique4 (Figure 3) and augmentation of RVOT by a rectangular patch of treated pericardium. Trans-esophageal echocardiography (TEE) revealed mild pulmonary regurgitation (PR) with peak gradient of 22 mm Hg. Postoperative course was uneventful.