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Supravalvular Ring Mitral Stenosis Treatment & Management

  • Author: Michael D Pettersen, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Feb 11, 2014
 

Medical Care

Evaluation of patients with supravalvar mitral ring is generally performed on an outpatient basis.

Hospital admission may be indicated in order to perform cardiac catheterization, for treatment of severe heart failure or pulmonary edema, and for surgery. Therefore, transfer patients to a tertiary cardiac center for further diagnostic evaluation and surgical correction. Adjunctive therapeutic measures may be needed.

The goals of medical treatment include the following:

  • To relieve symptoms caused by pulmonary venous congestion and congestive heart failure (CHF)
  • To stabilize the patient's condition before undertaking detailed assessment and surgical repair
  • To serve as an adjunct to surgical repair in the postoperative period
  • To control heart failure in small infants, in whom it may be the only option (Controlling CHF may temporarily defer surgery.)

Note the following:

  • Administer potassium supplements to all patients receiving furosemide or thiazide diuretics.
  • Restrict physical activity of symptomatic patients.
  • Place patients with severe pulmonary venous congestion in the sitting or propped-up position.
  • Administer parenteral morphine in patients with pulmonary edema to help relieve anxiety and reduce pulmonary congestion.
  • Administer oxygen by a nasal catheter or mask to improve oxygenation in acute pulmonary edema.
  • Vigorously treat concurrent infections or other aggravating factors.
  • Correct anemia if present. Increase the oxygen carrying capacity by a packed-cell transfusion to give considerable relief in patients with severe symptoms of congestive heart failure (CHF).

Consultations

Consult a cardiologist and a cardiothoracic surgeon.

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Surgical Care

Indications for and goals of surgical therapy

Note the following:

  • Surgical repair should be considered in all symptomatic patients with supravalvar mitral stenosis to relieve the obstruction.
  • Early operation to resect the supravalvar mitral ring should be considered in the presence of severe heart failure, pulmonary edema, or pulmonary arterial hypertension.[5]
  • The type of type of surgical intervention depends on the anatomy of the supravalvar ring and mitral valve apparatus, as well as any associated congenital heart defects. Every effort should be taken to define the anatomy in detail before undertaking surgery. In many patients, the supravalvar ring can be completely excised, while any associated mitral valve abnormality is simultaneously repaired.[6, 7, 8] If the supravalvar ring is strongly adherent to the mitral valve leaflet or if the mitral valve apparatus is grossly abnormal, replacement of the mitral valve may be necessary.[9, 10]
  • All associated defects should be repaired at the same time. In fact, surgery is often necessary for the associated heart defects even if the supravalvar mitral ring is not causing major hemodynamic disturbance.
  • The presence of a normal underlying mitral valve is associated with a surgical outcome better than that obtained with an abnormal valve apparatus, which may need replacement.
  • In patients who require surgery at an early age, the prognosis is poor. The mortality rate is high. Recurrent supravalvar mitral stenosis is a risk in as many as 15% of survivors, probably because of continuing turbulence across the small LV inflow tract.
  • Patients with Shone complex have a wide spectrum of anatomic abnormalities. Staged repair is usually necessary for coarctation treatment, the relief of left ventricular outflow tract obstruction, and reconstruction of the mitral valve. The results are encouraging. Bolling et al reported an actuarial survival rate of 89% 15 years after repair.[11] In a more recent series, Brown at al reported a 20-year survival of 82% and 20-year freedom from reoperation of 88%.[6] Operative mortality is increased by earlier age of repair, severe mitral valve disease, left ventricular hypoplasia, and the need for multiple operative procedures.

Percutaneous transcatheter balloon dilation

Percutaneous transcatheter balloon dilation has been used in selected cases of supravalvar mitral ring, but the results are less satisfactory than surgical outcomes. Surgical resection is considered the treatment of choice.

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Diet

No special diet is required in asymptomatic patients with supravalvar mitral ring.

Advise patients with heart failure to avoid excess intake of salt or to reduce their salt intake. Prescribe salt restriction cautiously in infants.

Restrict fluid intake to approximately 60-80 mL/kg/d in infants with congestive heart failure (CHF).

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Activity

Advise patients with pulmonary venous congestion or CHF to avoid strenuous exertion.

Symptomatic patients with supravalvar mitral ring should avoid sports and other strenuous activity that could aggravate pulmonary congestion and CHF.

Asymptomatic children without pulmonary hypertension may participate in normal activities.

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Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Julian M Stewart, MD, PhD Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College

Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Autonomic Society, American Physiological Society

Disclosure: Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous author Raghavan Subramanyan, MD, DM, to the original writing and development of this article.

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Parasternal long axis echocardiographic image showing a supra mitral variant of supravalvular mitral stenosis. A discrete membrane is visualized superior to but distinct from the mitral valve. The mitral valve appears otherwise normal.
Parasternal long axis color flow image showing a supra mitral variant of supravalvular mitral stenosis. Turbulence is noted at the level of the supra mitral membrane. In this case, Doppler interrogation revealed only mild obstruction.
Apical 4-chamber echocardiographic image showing an intramitral variant of supravalvular mitral stenosis. A membrane is visualized that is closely adherent to the mitral valve leaflets, restricting leaflet mobility.
Apical 4-chamber color flow echocardiographic image showing an intramitral variant of supravalvular mitral stenosis. Color flow imaging demonstrates severe mitral valve stenosis.
Continuous wave Doppler interrogation of the mitral valve in a patient with supravalvular mitral stenosis demonstrates severe stenosis with a mean gradient of 25 mm Hg.
Simultaneous recording of pressures in the pulmonary artery wedge position (PAW) and the left ventricle (LV) shows a large gradient in diastole across the mitral valve. PAW pressure is markedly elevated.
 
 
 
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