This Article Figures Only Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Fraisse, A. Articles by Geva, T. Search for Related Content PubMed PubMed Citation Articles by Fraisse, A. Articles by Geva, T.

CLINICAL STUDY: VALVE DISEASE

Echocardiographic characteristics and outcome of straddling mitral valve

Alain Fraisse, MD^{* c}, Pedro J. del Nido, MD, PhD ^c, Jean Gaudart, MD and Tal Geva, MD, FACC^{* c}

^* Department of Cardiology, Children’s Hospital, Boston, Massachusetts, USA
Department of Cardiac Surgery, Children’s Hospital, Boston, Massachusetts, USA
^c Departments of Pediatrics and Surgery, Harvard Medical School, Boston, Massachusetts, USA
Laboratoire de Biomathématiques, Université de la Méditeranée, Marseille, France

Manuscript received January 22, 2001; revised manuscript received May 10, 2001, accepted May 23, 2001.

Reprint requests and correspondence: Dr. Tal Geva, Department of Cardiology, Children’s Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115
geva_t{at}a1.tch.harvard.edu

OBJECTIVES

This study sought to characterize the echocardiographic features of straddling mitral valve (SMV) and to determine its surgical implications and midterm outcome in a large clinical cohort.

BACKGROUND

Despite a relatively large body of literature on the postmortem anatomy of SMV, there is a paucity of information regarding its echocardiographic features, surgical implications and preoperative predictors of outcome.

METHODS

A retrospective review identified 46 patients with SMV between 1982 and 1999 who underwent echocardiography and surgery and had follow-up data. A detailed review of the echocardiograms, surgical reports and all pertinent records was undertaken.

RESULTS

Review of the echocardiograms revealed a widely varying anatomy among the study patients. However, four distinct groups with relatively uniform morphologic features could be distinguished on the basis of segmental analysis. Cardiac malposition associated with right ventricular hypoplasia, superior-inferior ventricles and criss-cross atrioventricular relations were common among patients with {S,D,L} (S = visceroatrial situs solitus, D = D-ventricular loop, L = L-malposition of the great arteries) (n = 6) and {S,L,D} (n = 5) segmental combinations but were rare among patients with {S,D,D} (n = 26) and {S,L,L} (n = 9) combinations. Surgical management consisted of a functional single-ventricle palliation in 38 patients (83%) and biventricular repair in 8 patients (17%). Overall mortality was 22%, but none of the seven patients who were operated on during the cohort’s last five years (1994 to 1999) has died. By multivariate analysis, noncommitted ventricular septal defect was the strongest independent predictor of death (relative risk = 10.2), followed by multiple ventricular septal defects (relative risk = 4.7).

CONCLUSIONS

This study demonstrates that echocardiography provides detailed noninvasive imaging of the complex anatomic features of SMV and its associated anomalies. Anatomic classification based on segmental analysis allows the distinction of four groups with more uniform morphologic features. Although a biventricular approach is feasible in selected patients, a functional univentricular palliation is indicated in those with major straddling and markedly hypoplastic ventricles.

Abbreviations and Acronyms   AV = atrioventricular   DORV = double-outlet right ventricle   LV = left ventricle   MV = mitral valve   RV = right ventricle   SMV = straddling mitral valve   TGA = transposition of the great arteries   TV = tricuspid valve   VSD = ventricular septal defect

This article has been cited by other articles:

				A. Fraisse, T. A. Massih, P. Vouhe, B. Kreitmann, J. Gaudart, D. Sidi, and D. Bonnet Management and outcome of patients with abnormal ventriculo-arterial connections and mitral valve cleft Ann. Thorac. Surg., September 1, 2002; 74(3): 786 - 791. [Abstract] [Full Text] [PDF]