This Article Full Text Full Text (PDF) All Versions of this Article: 112/5/707    most recent CIRCULATIONAHA.104.500207v1 Alert me when this article is cited Alert me if a correction is posted Citation Map 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 Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by McElhinney, D. B. Articles by Lock, J. E. Search for Related Content PubMed PubMed Citation Articles by McElhinney, D. B. Articles by Lock, J. E. Related Collections Pediatric and congenital heart disease, including cardiovascular surgery

(Circulation. 2005;112:707-714.)
© 2005 American Heart Association, Inc.

Pediatric Cardiology

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; 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.

Correspondence to James E. Lock, MD, Department of Cardiology, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail james.lock{at}cardio.chboston.org

Received September 8, 2004; revision received April 1, 2005; accepted April 15, 2005.

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

This article has been cited by other articles:

				M. Nobuyoshi, T. Arita, S.-i. Shirai, N. Hamasaki, H. Yokoi, M. Iwabuchi, H. Yasumoto, and H. Nosaka Percutaneous Balloon Mitral Valvuloplasty: A Review Circulation, March 3, 2009; 119(8): e211 - e219. [Full Text] [PDF]

				A. Toscano, L. Pasquini, R. Iacobelli, R. M. Di Donato, F. Raimondi, A. Carotti, V. Di Ciommo, and S. P. Sanders Congenital supravalvar mitral ring: an underestimated anomaly. J. Thorac. Cardiovasc. Surg., March 1, 2009; 137(3): 538 - 542. [Abstract] [Full Text] [PDF]

				E. S. Selamet Tierney, F. A. Pigula, C. I. Berul, J. E. Lock, P. J. del Nido, and D. B. McElhinney Mitral valve replacement in infants and children 5 years of age or younger: Evolution in practice and outcome over three decades with a focus on supra-annular prosthesis implantation J. Thorac. Cardiovasc. Surg., October 1, 2008; 136(4): 954 - 961. [Abstract] [Full Text] [PDF]

				A. J. Mittnacht, M. Fanshawe, and S. Konstadt Anesthetic Considerations in the Patient With Valvular Heart Disease Undergoing Noncardiac Surgery Seminars in Cardiothoracic and Vascular Anesthesia, March 1, 2008; 12(1): 33 - 59. [Abstract] [PDF]

				J. D. St. Louis, M. M. Bannan, W. A. Lutin, and H. B. Wiles Surgical Strategies and Outcomes in Patients With Shone Complex: A Retrospective Review Ann. Thorac. Surg., October 1, 2007; 84(4): 1357 - 1363. [Abstract] [Full Text] [PDF]

				T. P. Graham Jr The Year in Congenital Heart Disease J. Am. Coll. Cardiol., June 20, 2006; 47(12): 2545 - 2553. [Full Text] [PDF]