Diagnostic Considerations

Important considerations

It is important for clinicians to diagnose the primary problem, recognize worsening symptoms, and recommend prophylaxis to prevent recurrent rheumatic heart disease.

Special concerns

Pregnancy

Acquired mitral stenosis (MS), the most common valvular disease in pregnant women in developing countries, has begun to appear in the United States as a result of immigration. Other reasons for acquired heart disease in pregnant woman in the West include delayed childbearing and the rise in age-related risk of developing complications of hypertension, diabetes, obesity, etc.^[6]

During pregnancy a 50% increase in plasma volume and a 25% increase in erythrocyte volume occur. Cardiac output increases by 40%, and heart rate also increases; therefore, as transmitral flow increases and diastolic time decreases, mean pulmonary artery pressure augments by approximately 50%. It often manifests for the first time during pregnancy with orthopnea, paroxysmal nocturnal dyspnea, pulmonary edema, and hemoptysis. Finding deterioration by 1-2 New York Health Association (NYHA) classes in these patients is not unusual.^[7]

Asymptomatic or minimally symptomatic patients who usually have mitral orifice areas larger than 1.5 cm² may only require close observation. However, patients with smaller mitral orifice areas who are severely symptomatic may require balloon valvuloplasty or surgical commissurotomy before delivery. Balloon valvuloplasty of MS has a very low complication rate in experienced centers. Pregnancy is contraindicated in women with severe MS, and preconception counseling must be offered to these patients because of the high likelihood of a bad outcome.

Pregnant women requiring anticoagulation for a prosthetic mechanical mitral valve should receive heparin. Warfarin should probably be avoided, especially during the first and third trimesters.

In the event of atrial fibrillation, beta-blockers may be used. Cardioversion can be administered if necessary because it has been proven safe during pregnancy. Echocardiography must be accomplished prior to cardioversion in order to evaluate the left atrium and its appendage for thrombi.

Vaginal delivery is the recommended method in women with NYHA class I, and cesarean delivery is seldom indicated. Cesarean delivery or uncomplicated abdominal delivery is not an indication for antibiotic prophylaxis.

Lutembacher syndrome

Lutembacher syndrome is a rare clinical entity that consists of the fortuitous association of a secundum atrial septal defect (ASD) with rheumatic MS.

Because of a stenotic mitral valve, pressures in the left atrium are elevated. Because of the ASD, blood shunts left to right. This produces pulmonary overcirculation with hepatic congestion and low cardiac output.

Differential Diagnoses

Workup

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Media Gallery

Acquired Mitral Stenosis. Hemodynamic changes in severe 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. Over 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.

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Author

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.

Coauthor(s)

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.

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.

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 research grant from: Lundbeck Pharmaceuticals<br/>Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

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.

Acquired Mitral Stenosis Differential Diagnoses

Diagnostic Considerations

Important considerations

Special concerns

Differential Diagnoses

References

Tables

Contributor Information and Disclosures

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