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Treatment of hypoplastic left heart syndrome: a systematic review and meta-analysis of randomised controlled trials

 A Y Kim  ;  W Woo  ;  A Saxena  ;  I C Tanidir  ;  A Yao  ;  Y Kurniawati  ;  V Thakur  ;  Y R Shin  ;  J I Shin  ;  J W Jung  ;  D J Barron  ;  International HLHS research network collaborators 
 CARDIOLOGY IN THE YOUNG, Vol.34(3) : 659-666, 2024-03 
Journal Title
Issue Date
Blalock-Taussig Procedure* ; Cardiopulmonary Bypass ; Databases, Factual ; Heart Ventricles / surgery ; Humans ; Hypoplastic Left Heart Syndrome* / surgery ; Randomized Controlled Trials as Topic
Hypoplastic left heart syndrome ; Norwood procedure ; modified Blalock–Taussig-Thomas shunt ; right ventricle-to-pulmonary artery shunt
Background: This meta-analysis aimed to consolidate existing data from randomised controlled trials on hypoplastic left heart syndrome.

Methods: Hypoplastic left heart syndrome specific randomised controlled trials published between January 2005 and September 2021 in MEDLINE, EMBASE, and Cochrane databases were included. Regardless of clinical outcomes, we included all randomised controlled trials about hypoplastic left heart syndrome and categorised them according to their results. Two reviewers independently assessed for eligibility, relevance, and data extraction. The primary outcome was mortality after Norwood surgery. Study quality and heterogeneity were assessed. A random-effects model was used for analysis.

Results: Of the 33 included randomised controlled trials, 21 compared right ventricle-to-pulmonary artery shunt and modified Blalock-Taussig-Thomas shunt during the Norwood procedure, and 12 regarded medication, surgical strategy, cardiopulmonary bypass tactics, and ICU management. Survival rates up to 1 year were superior in the right ventricle-to-pulmonary artery shunt group; this difference began to disappear at 3 years and remained unchanged until 6 years. The right ventricle-to-pulmonary artery shunt group had a significantly higher reintervention rate from the interstage to the 6-year follow-up period. Right ventricular function was better in the modified Blalock-Taussig-Thomas shunt group 1-3 years after the Norwood procedure, but its superiority diminished in the 6-year follow-up. Randomised controlled trials regarding medical treatment, surgical strategy during cardiopulmonary bypass, and ICU management yielded insignificant results.

Conclusions: Although right ventricle-to-pulmonary artery shunt appeared to be superior in the early period, the two shunts applied during the Norwood procedure demonstrated comparable long-term prognosis despite high reintervention rates in right ventricle-to-pulmonary artery shunt due to pulmonary artery stenosis. For medical/perioperative management of hypoplastic left heart syndrome, further randomised controlled trials are needed to deliver specific evidence-based recommendations.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Thoracic and Cardiovascular Surgery (흉부외과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Ah Young(김아영) ORCID logo https://orcid.org/0000-0002-0713-4461
Shin, Yu Rim(신유림)
Shin, Jae Il(신재일) ORCID logo https://orcid.org/0000-0003-2326-1820
Jung, Jo Won(정조원)
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