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Supravalvular Ring Mitral Stenosis Clinical Presentation

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

History

Supravalvar mitral ring can be diagnosed in one of the following ways:

  • Supravalvar mitral ring is most commonly diagnosed as an associated finding in other congenital heart disease (CHD).
  • Supravalvar mitral ring is occasionally the cause of congenital mitral stenosis in symptomatic children with dyspnea or pulmonary hypertension. The severity of symptoms depends on the level of left atrial and pulmonary venous hypertension.
  • Most patients become symptomatic by age 2 years.
  • In rare cases, this condition may be detected as an incidental finding in asymptomatic patients undergoing echocardiography for some unrelated reason.

Symptoms of supravalvar mitral ring with mitral stenosis include one or more of the following:

  • Dyspnea, nocturnal cough, and tachypnea from pulmonary venous congestion and increased lung stiffness
  • Frequent respiratory infections and wheezing from pulmonary congestion, increased fluid exudation, and airway narrowing
  • Poor feeding, failure to thrive, fatigue, and sweating from heart failure and reduced cardiac output
  • Occasionally acute pulmonary edema or generalized edema
  • Hemoptysis and syncope in older patients
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Physical

Physical signs in supravalvar mitral ring are usually related either to the associated CHD or to pulmonary arterial hypertension. Children with clinically significant mitral obstruction are often sick, with tachypnea and respiratory distress. Diminished cardiac output and poor perfusion lead to a low volume pulse and peripheral cyanosis. Systemic venous pressure may be elevated with the development of congestive heart failure (CHF). A prominent parasternal heave indicates right ventricular hypertrophy from pulmonary hypertension.

The pulmonary component of the second heart sound is accentuated. Unlike acquired mitral-valve stenosis, an opening snap of the mitral valve is not common in supravalvar mitral ring. An apical middiastolic murmur of mitral stenosis may be audible at the apex, especially in the left lateral decubitus, and it may exhibit presystolic accentuation. The murmur is prominent when supravalvar mitral ring is associated with ventricular septal defect (VSD) or patent ductus arteriosus (PDA), causing a large mitral inflow.[1]

Patients with chronic mitral obstruction develop signs of tricuspid regurgitation and CHF, such as hepatomegaly, engorged neck veins, large expansile CV waves in the jugular venous pulse, and a systolic murmur that accentuates in inspiration at the lower left sternal border.

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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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