PMID- 12238840 OWN - NLM STAT- MEDLINE DCOM- 20021001 LR - 20190628 IS - 0003-4975 (Print) IS - 0003-4975 (Linking) VI - 74 IP - 3 DP - 2002 Sep TI - Management and outcome of patients with abnormal ventriculo-arterial connections and mitral valve cleft. PG - 786-91 AB - BACKGROUND: In patients with abnormal ventriculo-arterial connections, a mitral valve cleft different from an atrioventricular canal is occasionally associated. It may cause outflow obstruction, mitral regurgitation, and complicate biventricular repair. METHODS: A retrospective review identified 21 patients operated upon with mitral valve cleft, abnormal ventriculo-arterial connections, and two well-developed ventricles. Eight patients had a ventricular outflow obstruction due to the mitral valve, whereas 2 had more than mild mitral regurgitation. One patient required initial mitral valve surgery. Eleven patients underwent biventricular repair, associated with mitral valve repair in 2 cases: arterial switch operation (n = 4), Senning operation (n = 3) associated with an arterial switch operation in one case, intraventricular repair (n = 3), and Rastelli-type extracardiac conduit repair (n = 1). Single-ventricle palliation was preferred in 10 patients with major mitral valve straddling (n = 5), outflow tract obstruction (n = 2), and noncommitted or multiple VSDs (n = 3). RESULTS: There were three hospital deaths, two of which occurred after biventricular repair and one after an early reoperation after a bidirectional cavopulmonary anastomosis. Postoperatively after biventricular repair, 1 patient required permanent pacemaker implantation and 3 patients were reoperated on for subaortic stenosis (n = 1) and mitral regurgitation (n = 2), with one late death. By multivariate analysis, patients with a double-outlet right ventricle were at greater risk of death (p = 0.04). After a mean follow-up period of 60.7 months (+/- 68.6 months), 16 patients are in New York Heart Association (NYHA) class I. One patient with a moderate mitral regurgitation on Doppler study is in NYHA class II. CONCLUSIONS: The surgical management remains controversial in patients with abnormal ventriculo-arterial connections and mitral valve cleft. Biventricular repair may not always be feasible, especially in cases of complex intracardiac anatomy associated with mitral valve straddling. Single-ventricle palliation can be achieved in these patients, although it is unknown whether the long-term results are as good as those obtained with biventricular repair. FAU - Fraisse, Alain AU - Fraisse A AD - Service de Cardiologie Pediatrique, Hopital Necker Enfants Malades, Paris, France. afraisse@mail.ap-hm.fr FAU - Massih, Tony Abdel AU - Massih TA FAU - Vouhe, Pascal AU - Vouhe P FAU - Kreitmann, Bernard AU - Kreitmann B FAU - Gaudart, Jean AU - Gaudart J FAU - Sidi, Daniel AU - Sidi D FAU - Bonnet, Damien AU - Bonnet D LA - eng PT - Journal Article PL - Netherlands TA - Ann Thorac Surg JT - The Annals of thoracic surgery JID - 15030100R SB - IM MH - Adolescent MH - Cause of Death MH - Child MH - Child, Preschool MH - Female MH - Heart Defects, Congenital/mortality/*surgery MH - Hospital Mortality MH - Humans MH - Infant MH - Infant, Newborn MH - Male MH - Mitral Valve/*abnormalities/surgery MH - Postoperative Complications/mortality MH - Reoperation/mortality MH - Retrospective Studies MH - Ventricular Outflow Obstruction/*congenital/mortality/surgery EDAT- 2002/09/20 10:00 MHDA- 2002/10/03 04:00 CRDT- 2002/09/20 10:00 PHST- 2002/09/20 10:00 [pubmed] PHST- 2002/10/03 04:00 [medline] PHST- 2002/09/20 10:00 [entrez] AID - S0003-4975(02)03743-8 [pii] AID - 10.1016/s0003-4975(02)03743-8 [doi] PST - ppublish SO - Ann Thorac Surg. 2002 Sep;74(3):786-91. doi: 10.1016/s0003-4975(02)03743-8.