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Published on: November 2, 2020
Heart Failure in Hypertrophic Cardiomyopathy
Olives Nguyen1, Daniel Kamna1, Ahmad Masri1
1Knight Cardiovascular Institute, Oregon Health & Science University Portland, OR, US.
Insights
End-stage hypertrophic cardiomyopathy (ES-HCM) exists on a spectrum, impacting prognosis and arrhythmia risk. Advanced therapies improve outcomes, but early recognition and targeted treatments are crucial for managing this condition.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- End-stage hypertrophic cardiomyopathy (ES-HCM) presents a spectrum from systolic dysfunction to a restrictive phenotype.
- Patients face poor prognoses due to low cardiac output and high arrhythmic risk.
Purpose of the Study:
- To review the spectrum of ES-HCM, current therapeutic outcomes, and future directions.
- To emphasize the importance of timely recognition and intervention in ES-HCM management.
Main Methods:
- Literature review of contemporary guideline-directed therapies for ES-HCM.
- Analysis of emerging treatments like cardiac myosin inhibitors and anti-fibrotic strategies.
Main Results:
- Guideline-directed therapies (ICDs, CRT, transplantation) have improved ES-HCM outcomes.
- Neurohormonal modulation and LVAD roles require further definition.
- Early recognition and intervention are vital for reducing morbidity and mortality.
Conclusions:
- Phenotype-specific approaches and equitable advanced therapy application are critical for addressing ES-HCM.
- Advancing targeted treatments may reduce the incidence of ES-HCM by modifying its natural history.
Abstract:
End-stage hypertrophic cardiomyopathy (ES-HCM) presents on a spectrum between the classic left ventricular systolic dysfunction and a less-recognised, restrictive phenotype with small-to-normal-sized left ventricular cavity and preserved ejection fraction. Relatively poor prognoses follow those on this morpho-functional spectrum, marked by low cardiac output and high arrhythmic risk. Contemporary guideline-directed therapies, such as ICDs, CRT and cardiac transplantation, have significantly improved outcomes, reshaping the natural history of ES-HCM. Meanwhile, the roles of neurohormonal modulation and left ventricular assist devices remain less well defined. Still, timely recognition and intervention remain essential in patients with ES-HCM, given its considerable morbidity and mortality. As cardiac myosin inhibitors and anti-fibrotic strategies advance, early targeted treatment may modify the natural history of hypertrophic cardiomyopathy, leading to a reduction in the incidence of ES-HCM. Moving forward, broader adoption of phenotype-specific approaches and equitable application of advanced therapies are critical to address the rare but clinically significant burden of ES-HCM.
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