Early Outcomes for Management of Atrioventricular Septal Defect—Tetralogy of Fallot in the Last Decade: A Congenital Heart Surgeons’ Society Study

医学 法洛四联症 房室间隔缺损 房室瓣 外科 房室管 心室流出道梗阻 队列 心脏病学 内科学 心脏病 二尖瓣 心室
作者
Connor P. Callahan,Madison B. Argo,Brian W. McCrindle,David J. Barron,Anusha Jegatheeswaran,Osami Honjo,Anastasios C. Polimenakos,Joseph W. Turek,Robert J. Dabal,James K. Kirklin,William M. DeCampli,Pirooz Eghtesady,David M. Overman
出处
期刊:World Journal for Pediatric and Congenital Heart Surgery [SAGE Publishing]
标识
DOI:10.1177/21501351241293158
摘要

Background We sought to determine the management and early outcomes of complete atrioventricular septal defect—tetralogy of Fallot (AVSD-TOF) for a contemporary multicenter cohort. Methods Of 739 participants in the Congenital Heart Surgeons' Society AVSD cohort (January 2012-May 2021), 40 had AVSD-TOF. We first compared survival differences for patients with AVSD-TOF versus those with isolated AVSD using propensity matching. Secondly, for patients with AVSD-TOF, we compared staged (n = 16) versus primary (n = 24) repair by assessing the following: patient characteristics, progression of atrioventricular valve (AVV) regurgitation, and time-related reoperation and survival. Results Five-year survival was similar between matched AVSD-TOF and isolated AVSD groups (80% vs 81%, P = .9). Compared with primary repair patients, staged patients had smaller pulmonary valve annulus Z-score measured at first presentation (−2.2 vs −2.9, P = .006). All staged patients (12 Blalock-Thomas-Taussig shunts, 3 right-ventricular-outflow-tract stents, 1 ductal stent) survived to complete repair. Freedom from AVSD-related reoperation five years post-AVSD-TOF repair was 57% after staged versus 90% after primary repair ( P < .05) and left AVV reoperations were the most frequent reintervention. Survival five years after AVSD-TOF repair was 80% (63% after staged vs 90% after primary repair; P = .08). Conclusions Patients undergoing AVSD-TOF repair have similar survival compared with matched isolated AVSD patients. Although approximately half of AVSD-TOF patients had initial palliation and all survived to complete repair, staged repair patients had lower survival and a higher reintervention rate compared with primary repair patients. The decision to pursue staged versus primary repair for future babies with AVSD-TOF remains challenging and should be chosen based on individual circumstances.
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