Noncompaction cardiomyopathy: A new mechanism for mitral regurgitation with distinct clinical,echocardiographic features and pathological correlations

Noncompaction cardiomyopathy (nccm) is a primary,genetic cardiomyopathy with variable clinical manifestations that include mitral regurgitation (mr). methods: this study comprised patients diagnosed with nccm and mr in two cardiac centers (king abdul-aziz cardiac center,riyadh,kingdom of saudi arabia and sudan heart institute,khartoum,sudan),and seen in the period between 2002 and 2013. the study describes follow up,clinical,echocardiographic,and histopathological findings. results: nineteen cases (85% females) were identified. ten percent of the cases had relapses and remissions of heart failure. echocardiographic features included leaflet retraction in all patients,characteristic malcoaptation,and a zigzag deformity of anterior leaflet in 57% of patients. ruptured chordae were found in 15% of the patients. one patient had pathological examination of the mitral valve which showed myxomatous degeneration,and sclerotic and calcific changes. conclusion: we describe and discuss a new mechanism for mr caused by nccm with identifiable clinical and echocardiographic features,and pathological correlations. © 2014 king saud university.