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Background: Transplant-free survival for single right ventricle (RV) lesions remains less than 70% at 3 years. Arrhythmia burden, influence of shunt type at Norwood procedure (RV-to-pulmonary artery shunt [RVPAS] versus Blalock-Taussig shunt [BTS]), and implications for mortality risk are not well defined., Methods: The authors performed a single-center retrospective analysis of patients with single RV lesions enrolled in a prospective study of arrhythmias after congenital heart surgery., Results: Fifty-eight patients received a RVPAS and 62 received a BTS, with a median follow-up of 773 days. Overall arrhythmia incidence was 78%, two-thirds of which prompted intervention. Among all types of arrhythmias, only ventricular arrhythmias (VAs) differed by shunt type, which were more common in patients receiving an RVPAS (29% RVPAS versus 14% BTS; p = 0.049). The majority of VAs were transient (69% less than 1 minute), and typically occurred early post-Norwood procedure (median 12 days). No additional variables were associated with development of VAs. Shunt type did not influence transplant-free survival. Within the entire cohort, there was a trend toward increased mortality with prior history of VA (odds ratio, 2.90; 95% confidence interval, 0.99 to 8.90; p = 0.052). For interstage survivors to Glenn palliation, any VA associated with a 14-fold increased risk of death or transplant (hazard ratio, 14.00; 95% confidence interval, 3.66 to 53.40; p < .001). No other tachyarrhythmia or bradyarrhythmia was associated with mortality., Conclusions: In this cohort with single RV lesions and prospective rhythm surveillance, patients receiving an RVPAS at Norwood surgery had an increased incidence of VAs compared with patients with a BTS. VAs correlated with late mortality in patients who survived the interstage period., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.) more...

We report a novel method of constructing an extracardiac bidirectional inferior cavopulmonary anastomosis. Our patient was a 4-month-old girl with complex single-ventricle anatomy and a small-caliber superior vena cava. A direct anastomosis from the inferior vena cava to the main pulmonary artery was constructed by using all autologous tissue. The resulting pathway remained patent and exhibited growth with age. Furthermore, a pulmonary arteriovenous malformation did not develop. This case demonstrates how a bidirectional inferior cavopulmonary anastomosis is feasible in the human and can provide similar hemodynamic results as the bidirectional superior cavopulmonary anastomosis., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.) more...

Background: Late left pulmonary artery (LPA) stenosis occurs commonly after the Norwood procedure, and complicates subsequent stages. Compression by the neoaorta and ductal stump may favor flow into the right pulmonary artery, resulting in LPA hypoplasia. We hypothesize that an early compromise of LPA flow contributes to late LPA stenosis, and have modified our shunt to compensate., Methods: We reviewed 34 consecutive neonates undergoing the Norwood procedure between 1999 and 2002, and morphometric data from angiograms obtained before the bidirectional cavopulmonary anastomosis (BDCPA). The Norwood technique included an autologous arch reconstruction with or without augmentation, and a polytetrafluoroethylene Blalock-Taussig shunt (BTS). Starting February 2001, the distal shunt was modified from an end-to-side construction to an oblique anastomosis directed into the retroaortic LPA., Results: Norwood survival was 82%. LPA stenosis required plasty in 10 of 13 (77%) premodification survivors, and in 2 of 9 (22%) postmodification (p = 0.027). Bypass time was 151 +/- 65 minutes with LPA plasty versus 95 +/- 50 minutes without. Mortality (15% vs 0%), hospital stay (25 +/- 35 vs 9 +/- 6 days), and incidence of subsequent interventions were correspondingly higher with LPA stenosis. Ten of 13 patients (77%) with a BTS insertion point outside the central region of the pulmonary artery required LPA plasty, versus 2 of 9 (22%) with an insertion nearer to the center (p = 0.027)., Conclusions: An oblique distal BTS anastomosis directed leftward onto the retroaortic pulmonary artery at the time of the Norwood procedure may prevent late LPA stenosis and its attendant morbidity. more...