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Clinical features of idiopathic restrictive cardiomyopathy: A retrospective multicenter cohort study over 2 decades

Jung Ae Hong1, Min-Seok Kim, Min-Su Cho

  • 1Department of Cardiology, Asan Medical Center, University of Ulsan College of Medicine Department of Internal Medicine, Seoul National University Hospital Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul Department of Cardiology, Translational Research Center on Aging, Chonnam National University Hospital, Gwangju Department of Internal Medicine, Wonju College of Medicine,Yonsei University, Wonju Department of Internal Medicine, Kangbuk Samsung Hospital, Seoul Department of Internal Medicine, CHA Bundang Medical Center, CHA University school of Medicine, Bundang Department of Cardiology, Sejong General Hospital Department of Cardiovascular Medicine, Gachon University Gil Medical Center, Incheon Department of Internal Medicine, Chungbuk National University School of Medicine, Cheongju, Korea.

Medicine
|September 9, 2017
PubMed

Insights

Idiopathic restrictive cardiomyopathy (RCMP) has a poor prognosis. Advanced tricuspid regurgitation and smaller left ventricular end-diastolic diameter predict mortality in these patients.

Area of Science:

  • Cardiology
  • Internal Medicine
  • Clinical Research

Background:

  • Idiopathic restrictive cardiomyopathy (RCMP) diagnosis is challenging.
  • Limited understanding of RCMP clinical profile and outcomes exists.

Purpose of the Study:

  • To assess the clinical profile and outcomes of idiopathic RCMP.
  • To identify prognostic factors for mortality in idiopathic RCMP patients.

Main Methods:

  • Retrospective multicenter cohort study (10 Korean centers, 1990-2010).
  • Included 53 patients with idiopathic RCMP.
  • Multivariate Cox proportional hazards regression analyses for mortality predictors.

Main Results:

  • Median follow-up: 1.7 years; 32.1% mortality, 9.4% heart transplant.
  • 5-year survival rate: 64.4% ± 7.8%.
  • Predictors of mortality: moderate or severe tricuspid regurgitation (TR) (HR 32.55) and lower left ventricular end-diastolic diameter (LVEDD) (HR 0.85).

Conclusions:

  • Idiopathic RCMP demonstrates an unfavorable prognosis.
  • Advanced TR and reduced LVEDD are independent adverse predictors of mortality.
  • Further research into RCMP management and treatment is warranted.

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