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.