Year: 2018
Type: article
Abstract: This commentary refers to ‘Heart failure with preserved ejection fraction: from mechanisms to therapies’, by C.S.P. Lam et al., 2018;39:2780–2792. We agree with Canepa et al. that hypertrophic cardiomyopathy (HCM) and amyloid cardiomyopathy are important specific diagnoses not to be missed, since they are treatable with specific therapies. By ‘Several specific diseases (e.g. infiltrative cardiomyopathies, amyloid, hypertrophic cardiomyopathy, high output failure, valvular heart disease, and pericardial disease) may present with HF but are not considered as the ‘common garden’ heart failure with preserved ejection fraction (HFpEF) and shall not be discussed further’1 we by no means meant to diminish the importance of these alternative diagnoses. Instead, we distinguished these specific diagnoses from the typical HFpEF described in large epidemiologic series and associated with multi-morbidity, because in contrast to the specific proven therapies for HCM and amyloid cardiomyopathy, no therapy has improved survival in typical HFpEF—the focus of our review.1 ,2 Such a focus was needed given the space constraints of a review article. Nonetheless, Canepa et al. bring up the interesting discussion of whether specific cardiomyopathies should be included in the spectrum of HFpEF. We acknowledge the lack of consensus on this point: many investigators in this field would not consider infiltrative cardiomyopathies, amyloid, HCM, valvular heart disease, or pericardial disease as HFpEF but rather as differential diagnoses. As such, these specific underlying diagnoses have been largely excluded from the HFpEF outcome trials (e.g. I-PRESERVE, TOPCAT, PARAGON). On the other hand, others argue that such patients should be considered as HFpEF if they present with the clinical syndrome of typical symptoms and signs of HF, normal left ventricular ejection fraction (EF), and raised filling pressures. In fact, wild-type transthyretin amyloidosis has been shown to be present in 13% of such elderly patients with symptoms, EF ≥ 50% and left ventricular end-diastolic wall thickness ≥12 mm3—a finding all the more note-worthy given the recent evidence of benefit with tafamidis for the treatment of transthyretin amyloid cardiomyopathy.4 To take the controversy even further, should a patient with severe coronary artery disease, normal EF and ischaemia-related elevated filling pressures be considered ‘HFpEF’ (no proven therapy) or ‘symptomatic coronary artery disease’ requiring coronary revascularization? Regardless of the ongoing debate on terminology, there is uniform agreement in the field that specific diagnoses of HCM or amyloid are important considerations, and patients should receive the most appropriate therapy for his/her condition including specific therapy where available. Conflict of interest: Dr. Lam is supported by a Clinician Scientist Award from the National Medical Research Council of Singapore; has received research support from Boston Scientific, Bayer, Thermofisher, Medtronic, and Vifor Pharma; and has consulted for Bayer, Novartis, Takeda, Merck, Astra Zeneca, Janssen Research & Development, LLC, Menarini, Boehringer Ingelheim, Abbott Diagnostics, Corvia, Stealth BioTherapeutics, Roche, and Amgen. Dr. Voors received consultancy fees and/or research grants from: Amgen, AstraZeneca, Bayer, Boehringer Ingelheim, Cytokinetics, GSK, Novartis, Roche Diagnostics, Servier. Dr. de Boer is supported by the Netherlands Heart Foundation (CVON DOSIS, grant 2014-40, CVON SHE-PREDICTS-HF, grant 2017 -21, and CVON REDCVD, grant 2017 -11); and the Innovational Research Incentives Scheme program of the Netherlands Organization for Scientific Research (NWO VIDI, grant 917.13.350). The UMCG, which employs Dr. De Boer has received research grants and/or fees from AstraZeneca, Abbott, Bristol-Myers Squibb, Novartis, Roche, Trevena, and ThermoFisher GmbH. Dr. de Boer is a minority shareholder of scPharmaceuticals, Inc. Dr. de Boer received personal fees from MandalMed Inc, Novartis, and Servier. Dr. Solomon has received research grants from Alnylam, Amgen, AstraZeneca, Bellerophon, Celladon, Gilead, GlaxoSmithKlein, Ionis Pharmaceutics, Lone Star Heart, Mesoblast, MyoKardia, NIH/NHLBI, Novartis, Sanofi Pasteur, Theracos, and has consulted for Alnylam, Amgen, AstraZeneca, Bayer, BMS, Corvia, Gilead, GSK, Ironwood, Merck, Novartis, Pfizer, Takeda, Theracos. Dr. Van Veldhuisen has received board membership fees or travel expenses from Novartis, Corvia Medical, ARCA Biopharma and Johnson & Johnson.
Source: European Heart Journal
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $5,922
OpenAlex ID: W2903525535