Impact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation.

Heinisch, Paul Philipp; Kido, Takashi; Burri, Melchior; Kornyeva, Anastasiya; Mertin, Jannik; Vodiskar, Janez; Strbad, Martina; Cleuziou, Julie; Hager, Alfred; Ewert, Peter; Hörer, Jürgen; Ono, Masamichi

doi:10.1016/j.athoracsur.2022.07.021

journal article

Title:: Impact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation.
Document type:: Journal Article
Author(s):: Heinisch, Paul Philipp; Kido, Takashi; Burri, Melchior; Kornyeva, Anastasiya; Mertin, Jannik; Vodiskar, Janez; Strbad, Martina; Cleuziou, Julie; Hager, Alfred; Ewert, Peter; Hörer, Jürgen; Ono, Masamichi
Abstract:: BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC's impact on staged palliation outcomes. METHODS: In a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (interquartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model. RESULTS: Primary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syndromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was significantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P < .001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an independent risk factor for mortality in patients with TAPVC (P < .001). CONCLUSIONS: Overall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients.
Journal title abbreviation:: Ann Thorac Surg
Year:: 2023
Journal volume:: 115
Journal issue:: 5
Pages contribution:: 1213-1221
Fulltext / DOI:: doi:10.1016/j.athoracsur.2022.07.021
Pubmed ID:: http://view.ncbi.nlm.nih.gov/pubmed/35944702
Print-ISSN:: 0003-4975
TUM Institution:: Klinik für Chirurgie angeborener Herzfehler und Kinderherzchirurgie (Prof. Hörer); Klinik für Herz- und Gefäßchirurgie (Prof. Lange)
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mediaTUM Gesamtbestand Einrichtungen Schools TUM School of Medicine and Health Departments Clinical Medicine Lehr- und Forschungskooperationen mit den Kliniken und Instituten am Deutschen Herzzentrum Klinik für Herz- und Gefäßchirurgie (DHM) (Prof. Krane)2023

mediaTUM Gesamtbestand Einrichtungen Schools TUM School of Medicine and Health Departments Clinical Medicine Lehr- und Forschungskooperationen mit den Kliniken und Instituten am Deutschen Herzzentrum Klinik für Kinderkardiologie und angeborene Herzfehler (DHM) (Prof. Ewert)2023

mediaTUM Gesamtbestand Einrichtungen Schools TUM School of Medicine and Health Departments Clinical Medicine Lehr- und Forschungskooperationen mit den Kliniken und Instituten am Deutschen Herzzentrum Klinik für Chirurgie angeborener Herzfehler und Kinderherzchirurgie (DHM) (Prof. Hörer)2023