Background: We describe outcomes and management strategies for single ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus.
Methods: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995-2023, excluding those with biventricular repair.
Results: Median age at centralization was 9 days (range 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n=20, 91%; 2 following bilateral ductal stents) or bidirectional cavopulmonary connection (n=2, 9%) via pericardial roll (n=14, 64%), patch-augmented direct anastomosis (n=7, 32%), and interposition graft (n=1, 5%) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n=11, 50%) was associated with significantly inferior survival (P=.01). Five patients (23%) died at a median of 59 (6-257) days post-centralization, all with non-cardiac TAPVC. At latest follow-up for 17 survivors (median 13.5 [0.5-25.1] years post-centralization), 12 completed Fontan, 4 completed second stage palliation, and 1 was transplanted prior to second stage palliation. Fourteen patients (64%) required PA reintervention, including 3 with reoperations independent of staged palliation. Baseline to pre-second stage echocardiography demonstrated branch PA growth with significantly increased diameters (left P=.0006, right P=.0002); z-scores significantly increased for right (P=.004) but not left (P=.11).
Conclusions: Successful single ventricle palliation is possible, though high-risk, for patients with bilateral discontinuous ductal PAs. Early post-centralization mortality remains substantial, particularly with associated non-cardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.
Copyright © 2024. Published by Elsevier Inc.