Anomalous Origin of One Pulmonary Artery From the Ascending Aorta: From Diagnosis to Treatment in Angola

World J Pediatr Congenit Heart Surg. 2015 Oct;6(4):521-5. doi: 10.1177/2150135115601832.

Abstract

Background: Anomalous origin of one pulmonary artery is a rare congenital heart disease in which one pulmonary artery branch originates from the ascending aorta.

Objective: To describe the experience of a cardiothoracic center in an African country to repair anomalous origin of one pulmonary artery in the context of Portugal-Angola collaboration.

Methods: Between March 2011 and March 2015, four consecutive patients with anomalous origin of pulmonary artery branch underwent surgical correction. The mean age was 1.6 months. The mean weight was 4 kg. All had right pulmonary artery branch originating from the ascending aorta. All patients underwent direct implantation of right pulmonary branch to main pulmonary artery. Two patents had patent ductus arteriosus and one had atrial septal defect. Two patients had pulmonary hypertension.

Results: There was no registration of death. The mean cardiopulmonary bypass time was 75.5 ± 4.5 minutes, mean aortic cross-clamping time was 40 ± 5.6 minutes, and mean duration of the postoperative intensive care unit stay was 6.8 ± 5.7 days. At discharge, one patient had residual gradient of 25 mm Hg, the remainder had no significant gradient. The mean follow-up time was 11 months (5-28 months). One week after discharge, one patient presented operative wound dehiscence. At the last follow-up, all patients were alive, and no significant residual gradient or stenosis at site of anastomosis was observed. No reintervention was required.

Conclusion: Anomalous origin of one pulmonary artery is a rare but potentially treatable lesion if operated early in life. Direct implantation was a good technique with good short-term results.

Keywords: aorta; congenital heart disease; congenital heart surgery; hemitruncus; pulmonary artery; pulmonary hypertension.

MeSH terms

  • Angiography
  • Angola / epidemiology
  • Aorta / abnormalities*
  • Aorta / surgery
  • Diagnosis, Differential
  • Echocardiography
  • Female
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Male
  • Middle Aged
  • Pulmonary Artery / abnormalities*
  • Pulmonary Artery / surgery
  • Survival Rate / trends
  • Tomography, X-Ray Computed
  • Vascular Malformations / diagnosis*
  • Vascular Malformations / surgery
  • Vascular Surgical Procedures / methods*