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Congenital Mitral Stenosis Differential Diagnoses

  • Author: M Silvana Horenstein, MD; Chief Editor: Stuart Berger, MD  more...
Updated: Apr 29, 2014

Diagnostic Considerations

Important considerations

It is important that clinicians diagnose not only the primary problem but also any additional congenital cardiovascular abnormalities.

Refer patients who demonstrate increasing symptoms, especially those of congestive heart failure.

Special concerns

Pregnant women

Patients who are pregnant have a higher risk of developing pulmonary edema because of the increased intravascular volume. For this reason, they should be closely monitored.

Asymptomatic or minimally symptomatic patients may only require close observation, but severely symptomatic patients may require urgent surgical intervention.

Pregnant women who require anticoagulation because of a prosthetic mitral valve should receive heparin, which does not cross the placental barrier. They should not receive warfarin because of its teratogenic effects and fetal wastage.

Pregnant women with underlying heart disease require antibiotic prophylaxis with ampicillin and gentamicin or with amoxicillin if they undergo potentially bacteremic procedures.

Of note, cesarean delivery or uncomplicated abdominal delivery are not indications for antibiotic prophylaxis.

Differential Diagnoses

Contributor Information and Disclosures

M Silvana Horenstein, MD Assistant Professor, Department of Pediatrics, University of Texas Medical School at Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Medical Association

Disclosure: Nothing to disclose.


Henry Walters, III, MD Associate Professor of Surgery, Wayne State University School of Medicine; Chief, Department of Surgery, Division of Cardiovascular Surgery, Children's Hospital of Michigan

Henry Walters, III, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Thoracic Surgery, American Medical Association, International Society for Heart and Lung Transplantation, Phi Beta Kappa, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

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

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

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.

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Hemodynamic changes in severe congenital mitral valve stenosis (MS). MS causes an obstruction (in diastole) to blood flow from the left atrium (LA) to the left ventricle (LV). Increased LA pressures are transmitted retrograde to pulmonary veins and pulmonary capillaries, resulting in capillary leak with subsequent development of pulmonary edema. To overcome pulmonary edema, the arterioles constrict, increasing pulmonary pressures. With time, capillaries develop intimal thickening, causing fixed (permanent) pulmonary hypertension. The right ventricle (RV) hypertrophies to generate enough pressure to overcome the increased afterload. Eventually, the RV fails, which manifests as hepatomegaly and/or ascites, edema of the extremities, and cardiomegaly on radiography.
Two-dimensional echocardiograph, parasternal long axis view of a 5-month-old boy with congenital mitral valve stenosis. A small mitral valve annulus (star) is appreciated when compared with the normal-sized tricuspid valve annulus. Mitral valve stenosis has caused left atrial (LA) enlargement. AoV = Aorta; LA = Left atrium; LV = Left ventricle; RA = Right atrium; RV = Right ventricle.
Two-dimensional echocardiograph, parasternal long axis view of a patient who required mitral valve replacement with a St. Jude's prosthetic mitral valve (star). He developed a stroke one month after mitral valve replacement despite anticoagulation with warfarin and required re-replacement of the prosthetic mitral valve. He will eventually outgrow this new prosthetic mitral valve and require subsequent mitral valve replacements with a larger mitral valve prosthesis. AoV = Aorta; LA = Left atrium; LV = Left ventricle; RA = Right atrium; RV = Right ventricle.
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