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Ann Thorac Surg 2002;74:786-791
© 2002 The Society of Thoracic Surgeons

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Original article: cardiovascular

Management and outcome of patients with abnormal ventriculo-arterial connections and mitral valve cleft

^a Service de Cardiologie Pédiatrique, Hôpital Necker Enfants Malades, Paris, France
^b Service de Chirurgie Cardiaque, Hôpital Necker Enfants Malades, Paris, France
^c Service de Chirurgie Thoracique et Cardiovasculaire, Hôpital d’Enfants de la Timone, Marseille, France
^d Laboratoire de Biomathématiques, Université de la Méditerranée, Marseille, France

Accepted for publication April 30, 2002.

* Address reprint requests to Dr Fraisse, Service de Cardiologie A, Hôpital de la Timone, 13005 Marseille, France
e-mail: afraisse{at}mail.ap-hm.fr

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.

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