Managing bilateral discontinuous pulmonary arteries of ductal origin in single ventricle anatomy

Abstract

We describe outcomes and management strategies for single ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995-2023, excluding those with biventricular repair. 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). 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.