Noncompaction cardiomyopathy: A new mechanism for mitral regurgitation with distinct clinical, echocardiographic features and pathological correlations
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An Image Guided Transapical Mitral Valve Leaflet Puncture Model of Controlled Volume Overload from Mitral Regurgitation in the Rat
Published on: May 19, 2020
Sulafa K M Ali1, Riyadh Abu-Sulaiman2, Rihab Beshir Agouba2
1Sudan Heart Institute, Khartoum, Sudan.
Insights
Noncompaction cardiomyopathy (NCCM) can cause mitral regurgitation (MR) through unique mechanisms. This study identified specific clinical and echocardiographic features associated with MR in NCCM patients.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Noncompaction cardiomyopathy (NCCM) is a primary genetic heart muscle disease.
- Mitral regurgitation (MR) is a common clinical manifestation of NCCM.
Purpose of the Study:
- To describe a novel mechanism of MR in patients with NCCM.
- To identify associated clinical, echocardiographic, and pathological features.
Main Methods:
- Retrospective analysis of 19 patients with NCCM and MR from two cardiac centers (2002-2013).
- Review of clinical, echocardiographic, and histopathological findings.
Main Results:
- 85% of identified patients were female.
- Echocardiography revealed leaflet retraction (100%), malcoaptation, and zigzag deformity (57%).
- Ruptured chordae were observed in 15% of patients; one case showed myxomatous degeneration.
Conclusions:
- A new mechanism for MR in NCCM is proposed.
- Distinct clinical and echocardiographic features correlate with pathological findings in MR due to NCCM.
Unlabelled:
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.
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