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Association of tetralogy of Fallot and complete atrioventricular canal: a single-centre 40-year experience

医学 法洛四联症 心室流出道 房室管 房室间隔缺损 三体 外科 心脏病学 内科学 心脏病 遗传学 生物
作者
Alejandro Fernández‐Cisneros,Steven J. Staffa,Sitaram M. Emani,Mariana Chávez,Kevin G. Friedman,David M. Hoganson,Aditya K. Kaza,Pedro J. del Nido,Christopher W. Baird
出处
期刊:European Journal of Cardio-Thoracic Surgery [Oxford University Press]
卷期号:65 (2)
标识
DOI:10.1093/ejcts/ezae037
摘要

Abstract OBJECTIVES Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population. METHODS Single-centre, retrospective analysis of patients who underwent surgical repair with the diagnosis of ToF-CAVC from 1979 to 2022, divided into 2 different periods and compared. RESULTS A total of 116 patients were included: 1979–2007 (n = 61) and 2008–2021 (n = 55). Balanced CAVC (80%) and Rastelli type C CAVC (81%) were most common. Patients in the later era were younger (4 vs 14 months, P < 0.001), fewer had trisomy 21 (60% vs 80%, P = 0.019) and fewer had prior palliative prior procedures (31% vs 43%, P < 0.001). In the earlier era, single-patch technique was more common (62% vs 16%, P < 0.001), and in recent era, double-patch technique was more common (84% vs 33%, P < 0.001). In the earlier era, right ventricular outflow tract was most commonly reconstructed with transannular patch (51%), while in more recent era, valve-sparing repairs were more common (69%) (P < 0.001). In-hospital mortality was 4.3%. The median follow-up was 217 and 74 months for the first and second eras. Survival for earlier and later eras at 2-, 5- and 10-year follow-up was (85.1%, 81.5%, 79.6% vs 94.2%, 94.2%, 94.2% respectively, log-rank test P = 0.03). CONCLUSIONS The surgical approach to ToF-CAVC has evolved over time. More recently, patients tended to receive primary repair at younger ages and had fewer palliative procedures. Improved surgical techniques allowing for earlier and complete repair have shown a decrease in mortality, more valve-sparing procedures without an increase in total reoperations. Presented at the 37th EACTS Annual Meeting, Vienna, Austria.

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