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Congenital Mitral Stenosis Treatment & Management

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

Medical Care

Asymptomatic patients with mild mitral stenosis (MS) require no significant therapy. They should undergo yearly follow-up care with physical examination, chest radiography, and ECG with echocardiography as indicated by this assessment. These patients may remain stable for decades before MS progresses and the patient requires surgical intervention.

Note the following:

  • More significant stenosis producing mild symptoms can be managed with diuretics alone. Direct careful attention to proper diet and to early intervention for pulmonary disease.
  • For the patient with congestive heart failure, administer loop diuretics plus potassium-sparing diuretics. Digoxin may improve right ventricular function in the setting of pulmonary hypertension.
  • Address cardiac rhythm abnormalities with appropriate medications.
  • Patients with chronic uncontrolled atrial tachyarrhythmias should be on anticoagulant therapy.

Critically ill patients or patients unable to take oral medication may receive intravenous medications. Admission to the ICU and endotracheal intubation may be required because of ineffective breathing caused by pulmonary edema.

Monitor the patient's anticoagulation therapy to prevent thrombus formation and to decrease the risk of embolization in case of a mechanical mitral valve. Remember that embolization is to the systemic circulation, because these emboli come from the left atrium and many emboli reach the brain.

Consultations

Consult a cardiologist and cardiothoracic surgeon.

Transfer

Transfer the patient to an intensive care unit when general status is unstable because of low cardiac output or pulmonary edema.

Diet and activity

Restrict salt and avoid excessive fluids. Maintain proper nutrition. Caloric supplementation may be necessary in the symptomatic infant.

Patients should avoid strenuous exercise, because an increased heart rate decreases diastolic filling time. If atrial flutter and atrial fibrillation are present and atrial kick is lost, a further decrease in LV stroke volume occurs. This may result in syncope from decreased cerebral perfusion.

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Surgical Care

Unlike what occurs in acquired MS, commissural fusion of the mitral leaflets is not a predominant mechanism for stenosis in patients with congenital MS (see Background). Therefore, balloon dilation of congenital MS, although performed in some centers, is not always successful. According to a recent study, younger patients and those who develop significant mitral regurgitation after balloon-dilation have a worse outcome.[2] However, because the 5-year survival is still relatively poor in those with severe congenital MS, regardless of treatment modality, the optimal therapeutic strategy remains unclear. Surgical options depend on specific mitral valve pathology.[3]

Mitral valve repair

Commissurotomy consists of an incision of fused mitral valve commissures and shaving of thickened mitral valve leaflets. Open surgical commissurotomy is preferable.

Divide fused chordae tendineae and papillary muscles to relieve subvalvular stenosis.

Resect any supravalvular tissue contributing to the MS.

Mitral valve replacement with mechanical valve or bioprosthesis

Note the following:

  • This is reserved for patients with severe MS in whom mitral valve repair is not possible. In older children for whom warfarin (Coumadin) therapy may be contraindicated, mitral valve replacement can be performed using a bioprosthesis, although the durability of tissue valves is less than mechanical protheses.
  • The risk of warfarin therapy should be weighed against the disadvantage of progressive bioprosthetic valve deterioration resulting in the certain need for reoperation.
  • Mitral valve replacement is best avoided in infants and small children because of frequent size mismatch between the smallest mechanical valves and the hypoplastic mitral valve annulus. In addition, somatic growth in children leads to the need for subsequent mitral prosthesis replacements.
  • Warfarin therapy is also more difficult to administer and to monitor in children. A less-than-perfect mitral valve repair is frequently preferable to mitral valve replacement in this group of patients.
  • Complications after mitral valve replacement include the risks of anticoagulation, valve thrombosis, valve dehiscence, infective endocarditis, valve malfunction, and embolic events.
  • However, in complex anatomy, replacement is the only solution to achieve an acceptable result. The Ross II operation, which uses a pulmonary autograft, is a difficult technique that may be useful in the youngest patient group when prosthetic devices cannot be used. This technique is still under clinical evaluation.

Correction of associated lesions

Pediatric patients must sometimes undergo correction of associated LV obstructive lesions such as subaortic stenosis, aortic valve stenosis, coarctation of the aorta, and hypoplastic aortic arch.[4]

Cardiac anomalies

Patients with associated congenital cardiac anomalies have a higher risk of early death after mitral valve surgery.

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Contributor Information and Disclosures
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)

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.

References
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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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