Current outcomes of the Glenn bidirectional cavopulmonary connection for single ventricle palliation

ObjectivesThe Glenn bidirectional cavopulmonary connection (BCPC) is an established procedure in multi-stage palliation of various single ventricle anomalies. We aimed to report current outcomes following BCPC and to examine risk factors affecting survival and progression to next palliation stage.Methods227 consecutive children with variable single ventricle pathologies underwent BCPC from 2002 to 2007. Competing risks analyses were performed to model events after BCPC (death and transition to Fontan) and subsequently after Fontan (death and cardiac reoperation); and to examine associated risk factors for poor outcomes.ResultsThere were 139 males (61%) with median age of 7.6months (interquartile range (IQR) 6.0–10.8) and median weight of 6.2kg (IQR 5.2–7.4). Forty-three patients (19%) had primary BCPC and 184 (81%) had prior palliation: aortopulmonary shunt (n=83), Norwood (n=55), pulmonary artery band (n=48), atrial septectomy (n=25), pulmonary artery reconstruction (n=14), anomalous pulmonary venous connection repair (n=7), other (n=8). Predominant ventricle was left morphology (n=122, 54%), right morphology (n=95, 42%), two equally developed ventricles (n=10, 4%). Twenty-six patients (12%) had bilateral SVC. Concomitant surgery included atrioventricular valve repair (n=18), pulmonary artery augmentation (n=80), percutaneous Fontan preparation (n=34), other (n=24). Competing risks analysis showed that 5-years following BCPC, approximately 17% have died, 76% have undergone Fontan and 7% were alive awaiting or not qualifying for Fontan. On multivariable analysis, risk factors for death prior to Fontan were PVRI>3 WU/M2 (HR 3.9, p=0.001), dominant right ventricle (HR 2.1, p=0.03), prior palliation other than aortopulmonary shunt (HR 0.4, p=0.03). Competing risks analysis showed that 3-years following 172 Fontan operations, approximately 10% have died, 6% have undergone further cardiac surgery and 84% were alive and free from reoperation. Overall, 8-year survival following BCPC was only 74%.ConclusionsDespite established selection criteria and improved surgical technique and medical management, there is a continuous failure and attrition risk following BCPC. Outcomes are influenced by underlying cardiac anomaly; patients with dominant left ventricle (i.e. tricuspid atresia, DILV) having the best survival while those with dominant right ventricle (i.e. HLHS, DORV with heterotaxy) having the worst survival. Increased pulmonary vascular resistance remains a significant factor affecting mortality