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Congenital Mitral Valve Disease

  • • Mitral valve disease uncommon in children

    • Can result in stenosis or insufficiency

    • Wide spectrum of disease, most have associated pathology

    • Associated with ventricular septal defect, atrial septal defect, coarctation

    Insufficiency: Dilated annulus, shortened chordae, leaflets restricted

    Stenosis: Supravalvular ring, parachute valve, commissural fusion, decreased interpapillary distance

    Shone syndrome: Supravalvular ring, parachute mitral valve, subaortic stenosis, aortic coarctation

Cor Triatriatum

  • • Rare anomaly

    • -Pulmonary veins enter accessory venous chamber demarcated from true LA by a diaphragm

    • Obstructive orifice connecting chamber to LA

    • -Less commonly, chamber connects to RA

    • Left-sided superior vena cava (SVC) common

    • Results in pulmonary venous hypertension, pulmonary congestion, elevated pulmonary artery pressures

    • Respiratory compromise soon ensues

Symptoms and Signs

  • • Obstructive symptoms including heart failure, ventricular hypertrophy

  • • Evaluate for other cardiac or extracardiac anomalies

  • • Physical exam

    • Echocardiography

Congenital Mitral Valve Disease

  • • Surgical treatment: Repair preferred over replacement if possible

    • Ensure adequate heart function prior to leaving operating room

Cor Triatriatum

  • • Surgical excision of membrane corrects abnormality



  • • Severe symptoms

Treatment Monitoring

  • • Replacement commits patient to anticoagulation therapy and repeat replacement

    • Repair may need reintervention in 25-50%


  • • Good for cor triatriatum


Yoshimura N et al. Surgery for mitral valve disease in the pediatric age group. J Thorac Cardiovasc Surg. 1999;118:99.  [PubMed: 10384192]

Mitral Valve Disease, Congenital

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