Document Detail

Current management of severe congenital mitral stenosis: outcomes of transcatheter and surgical therapy in 108 infants and children.
MedLine Citation:
PMID:  16043648     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Severe congenital mitral stenosis (MS) is a rare anomaly that is frequently associated with additional left heart obstructions. Anatomic treatments for congenital MS include balloon mitral valvuloplasty (BMVP), surgical mitral valvuloplasty (SMVP), and mitral valve replacement (MVR), although the optimal therapeutic strategy is unclear. METHODS AND RESULTS: Between 1985 and 2003, 108 patients with severe congenital MS underwent BMVP or surgical intervention at a median age of 18 months (range 1 month to 17.9 years). Anatomic subtypes of MS were "typical" congenital MS in 78 patients, supravalvar mitral ring in 46, parachute mitral valve in 28, and double-orifice mitral valve in 11, with multiple types in approximately 50% of patients. Additional left heart anomalies were present in 82 patients (76%). The first MS intervention was BMVP in 64 patients, SMVP in 33, and MVR in 11. BMVP decreased peak and mean MS gradients by a median of 33% and 38%, respectively (P<0.001), but was complicated by significant mitral regurgitation in 28%. Cross-sectional follow-up was obtained at 4.8+/-4.2 years. Overall, Kaplan-Meier survival was 92% at 1 month, 84% at 1 year, and 77% at 5 years, with 69% 5-year survival during the first decade of our experience and 87% since (P=0.09). Initial MVR and younger age were associated with worse survival. Survival free from failure of biventricular repair or mitral valve reintervention was 55% at 1 year among patients who underwent BMVP and 69% among patients who underwent supravalvar mitral ring resection initially. Among patients who underwent BMVP, survival free from failure of biventricular repair or MVR was 79% at 1 month and 55% at 5 years, with worse outcome in younger patients and those who developed significant postdilation mitral regurgitation. CONCLUSIONS: BMVP effectively relieves left ventricular inflow obstruction in most infants and children with severe congenital MS who require intervention. However, surgical resection is preferable in patients with MS due to a supravalvar mitral ring. Five-year survival is relatively poor in patients with severe congenital MS, with worse outcomes in infants and patients undergoing MVR, but has improved in our more recent experience. Many patients have undergone second procedures for either recurrent/residual MS or mitral regurgitation resulting from dilation-related disruption of the mitral valve apparatus.
Doff B McElhinney; Megan C Sherwood; John F Keane; Pedro J del Nido; Christopher S D Almond; James E Lock
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Publication Detail:
Type:  Journal Article     Date:  2005-07-25
Journal Detail:
Title:  Circulation     Volume:  112     ISSN:  1524-4539     ISO Abbreviation:  Circulation     Publication Date:  2005 Aug 
Date Detail:
Created Date:  2005-08-02     Completed Date:  2006-02-14     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  United States    
Other Details:
Languages:  eng     Pagination:  707-14     Citation Subset:  AIM; IM    
Department of Cardiology, Children's Hospital, 300 Longwood Ave, Boston, MA 02115, USA.
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MeSH Terms
Child, Preschool
Heart Valve Prosthesis Implantation
Mitral Valve
Mitral Valve Insufficiency / epidemiology
Mitral Valve Stenosis / congenital,  surgery,  therapy*
Retrospective Studies
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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