Mitral Valve Disease, Congenital

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

Surgery

Indications

• • Severe symptoms

Treatment Monitoring

• • Replacement commits patient to anticoagulation therapy and repeat replacement

  • Repair may need reintervention in 25-50%

Prognosis

• • Good for cor triatriatum

References

Mitral Valve Disease, Congenital