title: Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy.
abstract: The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983-2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at follow-up. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (P=0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results.
authors.affiliation: Department of Congenital Heart Defects, Bakoulev Center for Cardiovascular Surgery, Moscow, Russia.
authors.forename: Ivan A
authors.initials: IA
authors.lastname: Yurlov
authors.forename: Vladimir P
authors.initials: VP
authors.lastname: Podzolkov
authors.forename: Mikhail M
authors.initials: MM
authors.lastname: Zelenikin
authors.forename: Dmitry V
authors.initials: DV
authors.lastname: Kovalev
authors.forename: Guvandg K
authors.initials: GK
authors.lastname: Babaev
authors.forename: Neele A
authors.initials: NA
authors.lastname: Putiato
authors.forename: Sergey B
authors.initials: SB
authors.lastname: Zaets
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D003914:Dextrocardia / Q000150:complications / Q000401:mortality / Q000503:physiopathology; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality; D040181:Genetic Diseases, X-Linked / Q000150:complications / Q000401:mortality / Q000503:physiopathology; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D059446:Heterotaxy Syndrome; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D016017:Odds Ratio; D020127:Recovery of Function; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012426:Russia; D012857:Situs Inversus / Q000150:complications / Q000401:mortality / Q000503:physiopathology; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult