Myocardial Fibrosis as an Early Manifestation of Hypertrophic Cardiomyopathy
Population
107 subjects: 38 with pathogenic sarcomere mutations and overt hypertrophic cardiomyopathy, 39 with…
Design
Cross-sectional
Authors
Discussion
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Implication
May flag preclinical disease in sarcomere carriers; leaves open prospective validation before clinical use.
Key Points
- To determine whether profibrotic alterations in collagen metabolism and myocardial fibrosis occur in sarcomere-mutation carriers before the development of left ventricular hypertrophy.
- Evaluated 38 subjects with pathogenic sarcomere mutations and overt hypertrophic cardiomyopathy, 39 mutation carriers without left ventricular hypertrophy, and 30 mutation-negative controls.
- Assessed cardiac structure and fibrosis using echocardiography, cardiac MRI with late gadolinium enhancement, and serum biomarkers of collagen turnover (PICP and CITP).
- Serum PICP levels were 31% higher in mutation carriers without left ventricular hypertrophy and 69% higher in overt hypertrophic cardiomyopathy compared with controls (P<0.001).
- The ratio of PICP to C-terminal telopeptide of type I collagen was significantly increased only in subjects with overt hypertrophic cardiomyopathy.
- Late gadolinium enhancement on cardiac MRI was present in 71% of subjects with overt hypertrophic cardiomyopathy but was absent (0%) in mutation carriers without left ventricular hypertrophy.
Structured PICO
P
Population
107 subjects: 38 with pathogenic sarcomere mutations and overt hypertrophic cardiomyopathy, 39 with mutations but no left ventricular hypertrophy, and 30 controls without mutations.
C
Comparator
Controls without mutations
O
Outcome
Levels of serum C-terminal propeptide of type I procollagen (PICP) and presence of late gadolinium enhancement on cardiac MRIsurrogate
Increased myocardial collagen synthesis, indicated by elevated serum PICP, precedes the development of left ventricular hypertrophy or visible MRI fibrosis in sarcomere-mutation carriers.
Cite This Study
Ho et al. (2010) studied this question.