Intracardiac repair of lesions associated with atrioventricular discordance

• Published in: European journal of cardio-thoracic surgery Vol. 10; no. 6; pp. 443 - 448
• Main Authors: SZUFLADOWICZ, M, HORVATH, P, DE LEVAL, M, ELLIOTT, M, WYSE, R, STARK, J
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier Science B.V 1996; Elsevier Science
• Subjects: Adolescent; Adult; Biological and medical sciences; Cardiology. Vascular system; Cause of Death; Child; Child, Preschool; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Female; Heart; Heart Atria - abnormalities; Heart Atria - surgery; Heart Block - diagnosis; Heart Block - mortality; Heart Defects, Congenital - diagnosis; Heart Defects, Congenital - mortality; Heart Defects, Congenital - surgery; Heart Septal Defects - diagnosis; Heart Septal Defects - mortality; Heart Septal Defects - surgery; Heart Ventricles - abnormalities; Heart Ventricles - surgery; Humans; Infant; Male; Medical sciences; Postoperative Complications - diagnosis; Postoperative Complications - mortality; Survival Rate; Vascular disease; Human; Prognosis; Heart disease; Surgery; Cardiovascular disease; Congenital disease; Corrected transposition of the great vessels
• ISSN: 1010-7940; 1873-734X
• DOI: 10.1016/S1010-7940(96)80113-8

OBJECTIVE: Discordant atrioventricular (AV) connection is a rarecongenital cardiac anomaly. Associated cardiac defects modify thephysiology, clinical presentation, and surgical management of thesepatients. We have reviewed our overall experience with 90 patients operatedfor lesions associated with AV discordance between 1975-1990. METHODS: 90patients, consecutively operated between 1975-1990, were reviewed.Patients' charts, angiograms and echocardiograms were studied. Follow-upwas obtained from our records or was requested from referringcardiologists/paediatricians. It was completed in December 1992. For theanalysis of risk factors of early death we used P values by chi-squared andFisher's exact test. For the analysis of the triangulated events, we usedthe Kaplan-Meier method. Variables associated with P value over 0.20 wereeliminated. RESULTS: 90 patients aged 6 months-30 years (mean 9.1 year)underwent repair of cardiac anomalies associated with AV discordance. Threepatients had total cavopulmonary connection; the remainder receivedbiventricular repair. Most important associated malformations wereventricular septal defect (77), subpulmonary obstruction (68) and tricuspidvalve anomaly (21). 59 patients received extracardiac valved conduit, 10had tricuspid valve replacement. Thirteen patients died in hospital (14%).One year and 10 year actuarial survival rate was 84% and 70% respectively.The most significant factors for early death were: anatomically abnormaltricuspid valve (P < 0.0001), tricuspid valve regurgitation (P <0.002), date of operation (P < 0.012), preoperative or perioperativecomplete heart block (P < 0.015), and tricuspid valve surgery (P <0.05). Complete heart block developed in 17 (20%) out of 85 patients whodid not have it preoperatively. During the follow-up, 22 intracardiacreoperations were necessary (4 died). 63 patients of 73 survivors (86%)remain in NYHA Class 1, 6 in Class 2 and 4 in Class 3. CONCLUSIONS: Theoperative mortality was 14%. Twenty per cent of patients developed completeheart block. These results improved in the later part of the series(1985-1990); mortality decreased to 5% and incidence of heart block to 14%.Cardiac anomalies associated with AV discordance still present a surgicalchallenge. The data reported in our study should serve as a useful baselinefor evaluation of newer surgical techniques, such as the "double switch"operation.