A shunt decision-making protocol in the surgical palliation of hypoplastic left heart syndrome from 2004 to 2016

Abstract

OBJECTIVES The aim of this study was to study the impact of a decision-making protocol for shunt type in the Norwood procedure for hypoplastic left heart syndrome. Our cohort extends from 2004 to 2016. In era 1 (pre-2008), there was no policy for the choice of Norwood shunt. In era 2 (post-2008), a standard protocol was implemented. The right ventricle (RV)-to-pulmonary artery conduit was utilized for low-birth weight patients (<2.5 kg). The right modified Blalock–Taussig Shunt (RBTS) was constructed for normal birth weight patients. METHODS The records of 133 consecutive operative patients with hypoplastic left heart syndrome anatomy between 2004 and 2016 were retrospectively reviewed. Survival risk factors were analysed using the Cox proportional hazards risk model. RESULTS The Norwood procedure was performed at a mean age of 2.9 ± 1.9 days. Bidirectional cavopulmonary shunt was performed at a median age of 99 days (interquartile range 91–107). In era 1, 38.6% (22/57) of patients received the RBTS and 61.4% (35/57) of patients received the RV-to-pulmonary artery conduit. In era 2, 86.8% (66/76) of patients received the RBTS and 13.2% (10/76) of patients received the RV-to-pulmonary artery conduit. The actuarial survival to Fontan was 72.2% (96/133). Era 1 patients were more likely to die within the 1st year (hazard ratio = 2.310, P = 0.025). CONCLUSIONS The shunt protocol may improve outcomes in high-risk patients, and we have demonstrated the reliability of the RBTS in low-risk patients. The short- and mid-term outcomes of our Norwood population justify the continued efforts to improve surgical and perioperative management.

Exploration and validation of hub genes and pathways in the progression of hypoplastic left heart syndrome via weighted gene co-expression network analysis

“…Hypoplastic left heart syndrome (HLHS) is a group of complex congenital heart malformations characterized by severe stenosis or atresia of the aortic valve and mitral valve, ascending aorta, and left ventricular dysplasia [ 7 , 12 , 19 ]. It was first proposed by Noonan and Nadas [ 29 ].…”

Exploration and Validation of Hub Genes and Pathways in the Progression of Hypoplastic Left Heart Syndrome via Weighted Gene Co-Expression Network Analysis

“…Hypoplastic left heart syndrome (HLHS) is a group of complex congenital heart malformations characterized by severe stenosis or atresia of the aortic valve and mitral valve, ascending aorta, and left ventricular dysplasia [1][2][3]. It was rst proposed by Noonan and Nadas in 1958 [4].…”

Commentary: Introducing positive discrimination

“…In Melbourne, we have demonstrated the benefits of reducing the volume loading in patients with right ventricular dominance with the bidirectional cavopulmonary connection, 2 and we do this at 3 months of age. 3 In addition, we have described the detrimental role of the common atrioventricular valve anatomy and its propensity to evolve toward moderate or severe regurgitation, even when the Fontan operation is completed. 4 This has led to the pursuit of a policy in which patients are divided into 2 categories.…”