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Heart Failure with Preserved Ejection Fraction and Cardiomyopathy: an Under-recognized Complication of Systemic Sclerosis

  • Heart Failure (W Tang, Section Editor)
  • Published:
Current Treatment Options in Cardiovascular Medicine Aims and scope Submit manuscript

Abstract

Purpose of Review

Heart failure with preserved ejection fraction (HFpEF) and diastolic dysfunction are major sequelae of primary cardiac disease in patients with systemic sclerosis (SSc) and contribute to increased mortality in this population.

Recent Findings

Studies of animal models of SSc and human tissue samples indicate that the pathophysiology underlying these conditions involves microvascular dysfunction, cardiomyocyte apoptosis, and replacement fibrosis. Diagnostic recommendations for HFpEF in SSc include clinical examination and screening with laboratory studies and cardiac imaging to detect diastolic dysfunction early in the disease course. Management of HFpEF in this population does not significantly differ from general HFpEF guidelines. It is focused on symptomatic management of volume status and treatment of underlying SSc with immune-modulating therapies.

Summary

Although cardiac disease is a major cause of mortality in patients with SSc, current treatments are lacking. There is a great need for novel therapeutics targeting the underlying pathophysiology in SSc to inhibit or reverse myocardial fibrosis, thus preventing cardiac remodeling and dysfunction in these patients.

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Funding

Dr. Tang is partially supported by grants from the National Institutes of Health (R01HL126827).

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  1. Cleveland Clinic Lerner College of Medicine of Case Western Reserve University School of Medicine, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Alexia A. Zagouras BA, Soumya Chatterjee MD MS FRCP & W. H. Wilson Tang MD

  2. Department of Rheumatic and Immunologic Diseases, Orthopaedic & Rheumatologic Institute, Cleveland Clinic, Cleveland, OH, USA

    Soumya Chatterjee MD MS FRCP

  3. Kaufman Center for Heart Failure Treatment and Recovery, Desk J3-4, Heart Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH, USA

    W. H. Wilson Tang MD

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Correspondence to W. H. Wilson Tang MD.

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Dr. Tang is a consultant for Sequana Medical A.G., Owkin Inc., PreCARDIA Inc., Relypsa Inc., Genomics plc, and Cardiol Therapeutics Inc. and has received honorarium from Springer Nature for authorship/editorship and American Board of Internal Medicine for exam writing committee participation, all unrelated to the contents of this paper. All other authors have no relationships to disclose.

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Zagouras, A.A., Chatterjee, S. & Tang, W.H.W. Heart Failure with Preserved Ejection Fraction and Cardiomyopathy: an Under-recognized Complication of Systemic Sclerosis. Curr Treat Options Cardio Med 23, 71 (2021). https://doi.org/10.1007/s11936-021-00947-w

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  • DOI: https://doi.org/10.1007/s11936-021-00947-w

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