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Recent progress in end-stage hypertrophic cardiomyopathy

Yan Xiao1, Kun-Qi Yang, Yong Jiang

  • 1Departments of Cardiology (YX, K-QY, X-LZ) and Echocardiology (YJ), State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

The American Journal of the Medical Sciences
|April 23, 2015
PubMed

Insights

End-stage hypertrophic cardiomyopathy (ES-HCM) involves left ventricular enlargement and dysfunction, posing high risks for heart failure and sudden death. Early recognition and new management strategies are crucial for improving outcomes in this challenging condition.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Hypertrophic cardiomyopathy (HCM) is a spectrum of diseases.
  • A subset, end-stage HCM (ES-HCM), presents with left ventricular enlargement and systolic dysfunction.
  • ES-HCM carries a high risk of refractory heart failure and sudden cardiac death.

Purpose of the Study:

  • To review the evolving clinical features of ES-HCM.
  • To discuss the elucidated underlying mechanisms of ES-HCM.
  • To highlight the importance of early recognition and novel management strategies for ES-HCM.

Main Methods:

  • Literature review of studies on ES-HCM over the past two decades.
  • Analysis of clinical features, underlying mechanisms, and treatment strategies.
  • Synthesis of evidence for early detection and intervention.

Main Results:

  • Clinical features of ES-HCM have expanded over 20 years.
  • Underlying mechanisms of ES-HCM are becoming clearer.
  • Evidence supports earlier identification and intervention for ES-HCM.

Conclusions:

  • ES-HCM is an underestimated entity within hypertrophic cardiomyopathy.
  • Improved understanding of ES-HCM facilitates early recognition.
  • New insights into prevention and management promise better clinical outcomes for ES-HCM patients.

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