Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ 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 ++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 Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth