Herin Patel, Pulkit Malhotra, Kartik Patel, Mrinal Patel, Trushar Gajjar, Amit Mishra, Megha Sheth
U. N. Mehta Institute of Cardiology and Research Center.
India
World Journal of Pediatric and Congenital Heart Surgery
World J Pediatr Congenit Heart Surg 2024; 15: 365-370
DOI: 10.1177/21501351231221467
Abstract
Background: Anomalous origin of pulmonary artery (AOPA) is a rare congenital cardiac anomaly. It requires early surgical intervention (<6 months) to prevent irreversible pulmonary vaso-occlusive disease. This study was conducted to determine the surgical outcomes of this rare and intriguing anomaly.
Methods: From January 2015 to 2022, we have studied, 20 patients who underwent surgical correction for this anomaly with a mean age of 6.25 ± 3.7 months. There were 12 patients of <6 months of age. Their preoperative, intraoperative, and postoperative data were collected. They were followed up at every three months for the first year, every six months for two years, and then annually.
Results: Among three early mortalities, two were operated before six months of age and one was older than six months (ten months). Those patients presented after six months had longer ventilation time (P = .001). There was no difference in their pulmonary artery pressure (P = .06), right ventricle systolic pressure (RVSP) (P = .85), postoperative saturation (P = .51), inotropic score (P = .06), hospital and intensive care unit stay (P > .05), or mortality (P = .79). There was no late mortality at mean follow-up of 51.31 ± 20.27 months with Kaplan-Meier survival of 85% at 1, 5, and ten years. All patients were asymptomatic, with normal biventricular function and RVSP. One patient required balloon dilatation of the anastomotic site, with Kaplan-Meier event-free survival of 100% at one year, 92% at five and ten years.
Conclusion: Surgical correction of AOPA in patients beyond six months is still feasible with a higher early morbidity and comparable mortality with good clinical and echocardiographical outcomes at mid-term follow-up.
Category
Segmental Pulmonary Arterial Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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