Supravalvular Ring Mitral Stenosis Treatment & Management

Updated: Dec 15, 2020
  • Author: Michael D Pettersen, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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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 hemodynamic assessment, 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).


Consult a cardiologist and a cardiothoracic surgeon.


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. [8]

  • 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. [9, 10, 11] 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. [12, 13]

  • 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. 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. [14] In a more recent series, Brown at al reported a 20-year survival of 82% and 20-year freedom from reoperation of 88%. [9] 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.


Diet and Activity


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).


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.


Long-Term Monitoring

Provide follow-up care on an outpatient basis for monitoring symptoms, compliance with treatment, dose requirements, and early recognition of adverse drug effects.

Periodically check serum electrolyte levels and renal function in patients taking diuretics.

Promptly treat any intercurrent infections, arrhythmia, or other complications helps to reduce morbidity and prevent worsening of congestive heart failure.