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Clinical features and management of isolated cleft mitral valve in childhood
M Tamura1, S Menahem, C Brizard
1Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
Insights
Isolated cleft mitral valve (ICMV) is a correctable cause of mitral regurgitation in children. Echocardiography aids diagnosis, and surgical repair offers good outcomes, especially when performed early for moderate to severe cases.
Area of Science:
- Pediatric cardiology
- Congenital heart disease
- Valvular heart disease
Background:
- Isolated cleft mitral valve (ICMV) is a rare congenital anomaly.
- Previous studies focused on anatomical and echocardiographic features, with limited data on clinical outcomes.
- This study reviews institutional experience with ICMV in children.
Purpose of the Study:
- To describe the clinical features and management of isolated cleft mitral valve (ICMV) in a pediatric population.
- To evaluate the outcomes of surgical and non-surgical management of ICMV.
- To assess the diagnostic accuracy of echocardiography for ICMV.
Main Methods:
- Retrospective review of pediatric patients diagnosed with ICMV.
- Exclusion of patients with atrioventricular septal defects.
- Analysis of clinical presentation, echocardiographic findings, surgical interventions, and follow-up data.
Main Results:
- Twenty pediatric patients diagnosed with ICMV, with 7 having associated cardiac lesions.
- Echocardiography with color Doppler accurately diagnosed ICMV in all patients, though pre-surgical diagnosis was incomplete in 4.
- Mitral regurgitation (MR) severity ranged from mild to severe; 10 patients underwent mitral valve repair with no more than mild MR post-operatively. Unoperated patients showed stable MR severity.
Conclusions:
- Isolated cleft mitral valve (ICMV) is a correctable cause of mitral regurgitation in children.
- Echocardiography is effective for diagnosing ICMV.
- Surgical repair of the mitral valve is indicated for moderate to severe MR and should be considered early for optimal outcomes.
Objectives:
We reviewed an institutional experience of isolated cleft mitral valve (ICMV), its clinical features, and management in a pediatric population.
Background:
As ICMV is relatively uncommon, earlier reports highlighted its anatomical and echocardiographic features. Few studies have collated their clinical features with their outcome.
Methods:
All patients with ICMV were retrospectively reviewed. Patients who were considered to have an atrioventricular septal defect or variant were excluded.
Results:
Twenty patients (9 male, 11 female) were diagnosed with ICMV. Seven patients had associated cardiac lesions. The median age of diagnosis was 5.2 years (range 0.4 to 13.6 years). Echocardiography aided by color Doppler demonstrated the ICMV in all patients. However, an incomplete diagnosis was made in 4 of 20 patients before surgery. The severity of the mitral regurgitation (MR) at presentation was mild in 11, moderate in 8, and severe in 1 patient. In the 13 patients without associated cardiac lesions, 5 underwent mitral valve (MV) repair at median age of 5.2 years (range 1.2 to 7.7 years) for moderate to severe MR, 4 being symptomatic. The severity of the MR in seven of the eight unoperated patients has remained unchanged over the follow-up period (median 8.3 years, range 0.7 to 14.4 years). In total, 10 patients underwent MV repair (median 6.4, range 0.4 to 13.8 years). No patient required MV replacement. None of the 10 patients had more than mild MR over the follow-up period (median 0.6, range 0.2 to 11.0 years).
Conclusions:
Now readily diagnosable by echocardiography, ICMV is a correctable cause of MR with a good outcome. Surgery is indicated in those patients with moderate to severe MR and probably should be done early following diagnosis.