Supravalvular Ring Mitral Stenosis Follow-up

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

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 CHF.


Inpatient & Outpatient Medications

Continue treatment with diuretics and digoxin in patients with supravalvar mitral ring and CHF.

Recommend use of a potassium supplement, especially in children receiving furosemide therapy.

Antibiotics are necessary for intercurrent bacterial infections and for prophylaxis of infective endocarditis during dental or surgical procedures.



Among patients with congenital mitral stenosis, those with supravalvar mitral ring have a relatively good prognosis. Complete surgical excision is feasible and usually provides lasting relief. The presence of a normal underlying mitral valve and absence of other major cardiac lesions are associated with a better surgical outcome.

The prognosis is poor in patients who require resection at an early age. The mortality rate is high. Recurrent supravalvar mitral stenosis is a risk in survivors, probably because of continuing turbulence across the small left ventricular (LV) inflow tract.

In patients with Shone complex, thickened mitral valve leaflets, shortened chordae, subvalvar abnormalities, left ventricular outflow obstruction, and aortic incompetence were associated with rapid progression of hemodynamic abnormalities and poor prognosis. [13]


Possible complications of supravalvar mitral ring include pulmonary edema, pulmonary arterial hypertension, atrial arrhythmia, left atrial thrombus, embolic episodes, recurrent pulmonary infections, and infective endocarditis.

Cerebral venous thrombosis has been described in infants with supravalvar mitral ring.


Patient Education

Educate the patient and family about the importance of regular medical treatment, of periodic medical review, of restricting heavy physical exertion, of the need for antibiotic prophylaxis during dental and surgical procedures, and of the need to promptly attend to all infections.