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Supravalvular Ring Mitral Stenosis Follow-up

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

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.

Contributor Information and Disclosures

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.


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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