Surgical Strategies for Unilateral Pulmonary Artery Agenesis: A case series

TL;DR

This paper presents 5 cases of infants with rare right pulmonary artery absence who successfully underwent surgery to improve blood flow and reduce heart strain.

Contribution

The study contributes a case series from Oman on surgical strategies for unilateral pulmonary artery agenesis with favorable outcomes.

Findings

All patients had successful restoration of pulmonary blood flow with no perioperative deaths.

Four patients showed regression of pulmonary hypertension after timely surgical intervention.

Only one patient required repeat surgery due to graft compression.

Abstract

Unilateral pulmonary artery agenesis, typically affecting the right pulmonary artery, is a rare condition that may lead to congestive heart failure or pulmonary hypertension in infants and early surgical intervention improves outcomes. We report 5 cases from Muscat, Oman, of unilateral pulmonary artery agenesis who underwent surgical correction between November 2019 and August 2023, at an average age of 4.4 months. Most presented with cyanosis or respiratory distress and all had pulmonary hypertension confirmed by imaging. Surgical approaches included Gore-Tex conduit placement, autologous pericardial reconstruction and patent ductus arteriosus unifocalisation. All patients successfully restored pulmonary blood flow with no perioperative mortality. The median intensive unit care stay was 13 days and the median hospital stay was 20 days. Only 1 patient required repeat surgery for graft compression. Over a 27.6-month follow-up period, 4 patients showed regression of pulmonary hypertension and 2 underwent pulmonary angioplasty, reinforcing the benefits of timely intervention.

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