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Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy
H G Klues1, B J Maron, A L Dollar
1Pathology Branches, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md 20892.
Insights
Hypertrophic cardiomyopathy (HCM) involves more than just heart muscle; many patients also exhibit structural mitral valve abnormalities. These include enlarged leaflets and unusual papillary muscle connections, suggesting a broader disease process.
Area of Science:
- Cardiology
- Cardiac Morphology
- Valvular Heart Disease
Background:
- Hypertrophic cardiomyopathy (HCM) is defined by left ventricular hypertrophy.
- HCM is traditionally considered a disease of cardiac muscle.
Purpose of the Study:
- To investigate potential mitral valve involvement in hypertrophic cardiomyopathy.
- To determine if structural abnormalities of the mitral valve are present in HCM patients.
Main Methods:
- Studied mitral valves from 94 patients with HCM and 45 controls.
- Measured mitral leaflet area, length, and circumference.
- Assessed for anomalous papillary muscle insertions.
Main Results:
- Mitral leaflet area was significantly increased in HCM patients (12.9 cm² vs. 8.7 cm²).
- Increased area was due to leaflet elongation, particularly the anterior leaflet.
- 58% of HCM valves showed increased leaflet area; 66% had malformations including anomalous papillary muscle insertion.
Conclusions:
- HCM involves structural mitral valve abnormalities in a majority of patients.
- These include increased leaflet size, elongation, and anomalous papillary muscle insertions.
- The disease process extends beyond cardiac muscle to affect mitral valve structure.
Background:
Hypertrophic cardiomyopathy (HCM) is characterized by an asymmetrically hypertrophied left ventricle and is regarded as a disease of cardiac muscle.
Methods And Results:
To assess the possibility that the mitral valve itself may be involved in the disease process, we studied mitral valves from 94 patients with HCM and 45 normal control subjects. The area of the mitral leaflets was increased in patients with HCM compared with control subjects (12.9 +/- 3.7 versus 8.7 +/- 2.0 cm2; p less than 0.001). For the overall group of patients, this increase was largely caused by an increase in anterior leaflet length (2.2 +/- 0.5 cm for HCM versus 1.8 +/- 0.3 cm for control subjects; p less than 0.001), because circumference did not differ between the two groups. Mitral leaflet area was increased (greater than or equal to 12.0 cm2) in 55 (58%) of the 94 valves. In 12 of these 55 valves, both the anterior and posterior leaflets were enlarged; the other 43 valves had asymmetrical or segmental enlargement of either the anterior leaflet (36 patients) or a portion of posterior leaflet (seven patients). In addition, nine patients had a congenital malformation of the mitral apparatus in which one or both papillary muscles inserted directly into anterior mitral leaflet (mitral valve area was normal in seven of the nine).
Conclusions:
Sixty-two (66%) of 94 mitral valves had a constellation of structural malformations, including increased leaflet area and elongation of the leaflets or anomalous papillary muscle insertion directly into anterior mitral leaflet. These findings expand the morphological definition of HCM by demonstrating that the disease process is not confined to cardiac muscle but rather many patients also have structural abnormalities of the mitral valve that are unlikely to be acquired or secondary to mechanical factors.
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