Published Online
on July 25, 2005

A more recent version of this article appeared on August 2, 2005

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Submitted on September 8, 2004
Revised on April 1, 2005
Accepted on April 15, 2005

Current Management of Severe Congenital Mitral Stenosis. Outcomes of Transcatheter and Surgical Therapy in 108 Infants and Children

Doff B. McElhinney MD, Megan C. Sherwood MBBS, FRACP, John F. Keane MD, Pedro J. del Nido MD, Christopher S.D. Almond MD, MPH, and James E. Lock MD^*

From the Departments of Cardiology (D.B.M., M.C.S., J.F.K., C.S.D.A., J.E.L.) and Cardiac Surgery (P.J.d.N.), Children’s Hospital, and Departments of Pediatrics (D.B.M., M.C.S., J.F.K., C.S.D.A., J.E.L.) and Surgery (P.J.d.N.), Harvard Medical School, Boston, Mass.

^* To whom correspondence should be addressed. E-mail: james.lock{at}cardio.chboston.org.

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 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.

Key words: mitral valve • catheterization • heart defects, congenital • pediatrics • balloon

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