Complex pulmonary atresia with congenital systemic collaterals. Classification and management

Area of Science:

• Cardiology
• Pediatric Surgery
• Medical Imaging

Background:

• Pulmonary atresia with subaortic ventricular septal defect presents complex anatomical challenges.
• Accurate delineation of central pulmonary arteries and systemic lung supply is crucial for effective management.
• Congenital systemic collateral arteries are a frequent finding in this patient cohort.

Purpose of the Study:

• To characterize the anatomy of central pulmonary arteries and systemic collateral circulation in patients with pulmonary atresia and subaortic ventricular septal defect.
• To evaluate a novel two-stage surgical strategy for managing patients with hypoplastic central pulmonary arteries.

Main Methods:

• Complete angiography, including right ventricular, aortic, and selective arterial angiograms, was performed on 80 patients.
• A two-stage surgical procedure was implemented for patients with hypoplastic central pulmonary arteries, involving initial conduit placement and delayed ventricular septal defect closure.

Main Results:

• Eighty-five percent of patients (67/80) exhibited 1-5 large congenital systemic collateral arteries.
• Variable central pulmonary artery development was observed, with complete absence in 17 patients and hypoplasia in 33 patients (49%).
• Initial results in 6 patients undergoing the two-stage procedure were encouraging.

Conclusions:

• The presence and anatomy of systemic collateral arteries significantly influence management strategies.
• A staged surgical approach, focusing on pulmonary artery development before ventricular septal defect closure, offers a promising therapeutic option for complex pulmonary atresia.
• Further evaluation of this two-stage procedure is warranted to confirm long-term outcomes.