Dilated pulmonary arteries in TOF with absent pulmonary valve

Dilated pulmonary arteries in TOF with absent pulmonary valve

In Tetralogy of Fallot with absent pulmonary valve, the main pulmonary artery and intramediastinal portion of right and left pulmonary arteries are dilated. Hence the dilatation of right and left pulmonary arteries may not be evident on chest x-ray and visualised only on pulmonary angiogram. The reason for dilatation of the intramediastinal portion alone is thought to be because this region is not surrounded by lungs.

Absent pulmonary valve syndrome can occur in 3 to 6% cases of tetralogy of Fallot. Detection at 27 weeks of gestation by fetal echocardiography was reported by Ashok N. Bhupali and associates [1]. They concluded that when a paracardiac cystic and pulsatile lesion with dilated pulmonary arteries are noted in fetal ultrasound, other associations like tetralogy of Fallot and ductus arteriosus have to be looked for. There was no ductus arteriosus in their case.

The aneurysmal dilatation of the pulmonary artery may result in compression of the bronchial tree and esophagus. Bronchomalacia and polyhydramnios may result due to these compressions [1].

Reference

1. Ashok N Bhupali, Kiran B Patankar, Sayi Prasad, Jeetendra K Patil, Ajitey Tamhane. Absent Pulmonary Valve Syndrome With Tetralogy of Fallot Detected at an Early Gestational Age of 27 Weeks – A Case Report. Indian Heart J. Mar-Apr 2013;65(2):191-3.