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Published on: June 14, 2016
Prognostic value of the collagen volume fraction in hypertrophic cardiomyopathy
Edmundo Arteaga1, Aloir Queiroz de Araújo, Mauricio Bernstein
1Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil. edmundo.arteaga@incor.usp.br
Insights
Interstitial myocardial fibrosis in hypertrophic cardiomyopathy (HCM) is linked to worse outcomes. Quantifying this fibrosis may help predict sudden death risk and guide treatment for HCM patients.
Area of Science:
- Cardiology
- Pathology
- Medical Research
Background:
- Interstitial myocardial fibrosis is a key feature in hypertrophic cardiomyopathy (HCM).
- This fibrosis is associated with increased risk of sudden cardiac death and disease progression.
- Accurate prognostic markers are crucial for managing HCM patients.
Purpose of the Study:
- To prospectively assess the prognostic significance of collagen volume fraction (CVF) in patients with HCM.
- To determine if CVF can predict adverse outcomes in HCM.
- To establish a link between myocardial fibrosis and prognosis in HCM.
Main Methods:
- Endomyocardial biopsies were obtained from 21 symptomatic HCM patients.
- Myocardial collagen volume fraction (CVF) was quantified histologically.
- Patients were stratified by median CVF, and outcomes were compared over a median follow-up of 110 months.
Main Results:
- HCM patients exhibited significantly higher CVF (median 6.19%) compared to controls (median 0.61%).
- No baseline clinical or echocardiographic differences were found between high and low CVF groups.
- Increased CVF was associated with a significantly higher incidence of death (sudden cardiac death and heart failure) during follow-up (p=0.02).
Conclusions:
- Myocardial fibrosis, quantified by CVF, is prospectively associated with a worse prognosis in HCM.
- Quantifying myocardial fibrosis is essential for risk stratification in HCM.
- This quantification may aid in decisions regarding defibrillator implantation and pharmacologic therapy for myocardial repair.
Background:
In hypertrophic cardiomyopathy (HCM), interstitial myocardial fibrosis is an important histological modification that has been associated with sudden death and evolution toward myocardial dilation.
Objective:
To prospectively evaluate the prognostic value of the collagen volume fraction in HCM.
Methods:
An endomyocardial biopsy of the right ventricle was successfully performed in 21 symptomatic patients with HCM. The myocardial collagen volume fraction (CVF) was determined by histology. The CVF was also determined in fragments of nine normal hearts from subjects deceased from non-cardiac causes. The patients were divided into above-median CVF and below-median CVF groups, and their clinical and echocardiographic characteristics and survival curves were compared.
Results:
Among the patients, the CVF ranged from 1.86% to 29.9%, median 6.19%; in normal hearts, from 0.13% to 1.46%, median 0.61% (p <0.0001 between HCM and control). There were no significant correlations between CVF and baseline echocardiographic measures. Patients with CVF < or =6.19% and CVF> 6.19% were compared and no baseline differences were observed. However, after an average follow-up period of 110 months, four deaths occurred (two sudden, two due to heart failure) in the group with increased CVF, whereas the patients of the group with lower CVF were all alive at the end of the period (p = 0.02).
Conclusion:
For the first time, myocardial fibrosis was prospectively associated with a worse prognosis in patients with HCM. Efforts should be directed to the quantification of myocardial fibrosis in HCM, on the premise that its association with the prognosis can aid in the stratification of risk for defibrillator implantation, and in the prescription of drugs that potentially promote myocardial repair.
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