Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology

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Abstract

<p class="first" id="d934665e849">Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. In DCM, HF with reduced ejection fraction (HFrEF) has high incidence and prevalence and represents the most frequent cause of death, despite improvements in treatment. In addition, advanced HF in DCM is one of the leading indications for heart transplantation. In HCM, HF with preserved ejection (HFpEF) affects most patients with obstructive, and ∼10% of patients with non-obstructive HCM. A timely treatment is important, since development of advanced HF, although rare in HCM, portends a poor prognosis. In RCM, HFpEF is common, while HFrEF occurs later and more frequently in amyloidosis or iron overload/haemochromatosis. Irrespective of RCM aetiology, HF is a harbinger of a poor outcome. Recent advances in our understanding of the mechanisms underlying the development of HF in cardiomyopathies have significant implications for therapeutic decision-making. In addition, new aetiology-specific treatment options (e.g. enzyme replacement therapy, transthyretin stabilizers, immunoadsorption, immunotherapy, etc.) have shown a potential to improve outcomes. Still, causative therapies of many cardiomyopathies are lacking, highlighting the need for the development of effective strategies to prevent and treat HF in cardiomyopathies. </p>

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The role of cardiac glycosides in treating patients with chronic heart failure and normal sinus rhythm remains controversial. We studied the effect of digoxin on mortality and hospitalization in a randomized, double-blind clinical trial. In the main trial, patients with a left ventricular ejection fraction of 0.45 or less were randomly assigned to digoxin (3397 patients) or placebo (3403 patients) in addition to diuretics and angiotensin-converting-enzyme inhibitors (median dose of digoxin, 0.25 mg per day; average follow-up, 37 months). In an ancillary trial of patients with ejection fractions greater than 0.45, 492 patients were randomly assigned to digoxin and 496 to placebo. In the main trial, mortality was unaffected. There were 1181 deaths (34.8 percent) with digoxin and 1194 deaths (35.1 percent) with placebo (risk ratio when digoxin was compared with placebo, 0.99; 95 percent confidence interval, 0.91 to 1.07; P=0.80). In the digoxin group, there was a trend toward a decrease in the risk of death attributed to worsening heart failure (risk ratio, 0.88; 95 percent confidence interval, 0.77 to 1.01; P=0.06). There were 6 percent fewer hospitalizations overall in that group than in the placebo group, and fewer patients were hospitalized for worsening heart failure (26.8 percent vs. 34.7 percent; risk ratio, 0.72; 95 percent confidence interval, 0.66 to 0.79; P<0.001). In the ancillary trial, the findings regarding the primary combined outcome of death or hospitalization due to worsening heart failure were consistent with the results of the main trial. Digoxin did not reduce overall mortality, but it reduced the rate of hospitalization both overall and for worsening heart failure. These findings define more precisely the role of digoxin in the management of chronic heart failure.

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Mitral-valve repair can be accomplished with an investigational procedure that involves the percutaneous implantation of a clip that grasps and approximates the edges of the mitral leaflets at the origin of the regurgitant jet. We randomly assigned 279 patients with moderately severe or severe (grade 3+ or 4+) mitral regurgitation in a 2:1 ratio to undergo either percutaneous repair or conventional surgery for repair or replacement of the mitral valve. The primary composite end point for efficacy was freedom from death, from surgery for mitral-valve dysfunction, and from grade 3+ or 4+ mitral regurgitation at 12 months. The primary safety end point was a composite of major adverse events within 30 days. At 12 months, the rates of the primary end point for efficacy were 55% in the percutaneous-repair group and 73% in the surgery group (P=0.007). The respective rates of the components of the primary end point were as follows: death, 6% in each group; surgery for mitral-valve dysfunction, 20% versus 2%; and grade 3+ or 4+ mitral regurgitation, 21% versus 20%. Major adverse events occurred in 15% of patients in the percutaneous-repair group and 48% of patients in the surgery group at 30 days (P<0.001). At 12 months, both groups had improved left ventricular size, New York Heart Association functional class, and quality-of-life measures, as compared with baseline. Although percutaneous repair was less effective at reducing mitral regurgitation than conventional surgery, the procedure was associated with superior safety and similar improvements in clinical outcomes. (Funded by Abbott Vascular; EVEREST II ClinicalTrials.gov number, NCT00209274.).

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Association of fibrosis with mortality and sudden cardiac death in patients with nonischemic dilated cardiomyopathy.

Ankur Gulati, Andrew Jabbour, Tevfik Ismail … (2013)

Risk stratification of patients with nonischemic dilated cardiomyopathy is primarily based on left ventricular ejection fraction (LVEF). Superior prognostic factors may improve patient selection for implantable cardioverter-defibrillators (ICDs) and other management decisions. To determine whether myocardial fibrosis (detected by late gadolinium enhancement cardiovascular magnetic resonance [LGE-CMR] imaging) is an independent and incremental predictor of mortality and sudden cardiac death (SCD) in dilated cardiomyopathy. Prospective, longitudinal study of 472 patients with dilated cardiomyopathy referred to a UK center for CMR imaging between November 2000 and December 2008 after presence and extent of midwall replacement fibrosis were determined. Patients were followed up through December 2011. Primary end point was all-cause mortality. Secondary end points included cardiovascular mortality or cardiac transplantation; an arrhythmic composite of SCD or aborted SCD (appropriate ICD shock, nonfatal ventricular fibrillation, or sustained ventricular tachycardia); and a composite of HF death, HF hospitalization, or cardiac transplantation. Among the 142 patients with midwall fibrosis, there were 38 deaths (26.8%) vs 35 deaths (10.6%) among the 330 patients without fibrosis (hazard ratio [HR], 2.96 [95% CI, 1.87-4.69]; absolute risk difference, 16.2% [95% CI, 8.2%-24.2%]; P < .001) during a median follow-up of 5.3 years (2557 patient-years of follow-up). The arrhythmic composite was reached by 42 patients with fibrosis (29.6%) and 23 patients without fibrosis (7.0%) (HR, 5.24 [95% CI, 3.15-8.72]; absolute risk difference, 22.6% [95% CI, 14.6%-30.6%]; P < .001). After adjustment for LVEF and other conventional prognostic factors, both the presence of fibrosis (HR, 2.43 [95% CI, 1.50-3.92]; P < .001) and the extent (HR, 1.11 [95% CI, 1.06-1.16]; P < .001) were independently and incrementally associated with all-cause mortality. Fibrosis was also independently associated with cardiovascular mortality or cardiac transplantation (by fibrosis presence: HR, 3.22 [95% CI, 1.95-5.31], P < .001; and by fibrosis extent: HR, 1.15 [95% CI, 1.10-1.20], P < .001), SCD or aborted SCD (by fibrosis presence: HR, 4.61 [95% CI, 2.75-7.74], P < .001; and by fibrosis extent: HR, 1.10 [95% CI, 1.05-1.16], P < .001), and the HF composite (by fibrosis presence: HR, 1.62 [95% CI, 1.00-2.61], P = .049; and by fibrosis extent: HR, 1.08 [95% CI, 1.04-1.13], P < .001). Addition of fibrosis to LVEF significantly improved risk reclassification for all-cause mortality and the SCD composite (net reclassification improvement: 0.26 [95% CI, 0.11-0.41]; P = .001 and 0.29 [95% CI, 0.11-0.48]; P = .002, respectively). Assessment of midwall fibrosis with LGE-CMR imaging provided independent prognostic information beyond LVEF in patients with nonischemic dilated cardiomyopathy. The role of LGE-CMR in the risk stratification of dilated cardiomyopathy requires further investigation.

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Author and article information

Journal

Title: European Journal of Heart Failure

Abbreviated Title: Eur J Heart Fail

Publisher: Wiley

ISSN (Print): 1388-9842

ISSN (Electronic): 1879-0844

Publication date Created: May 2019

Publication date (Electronic): April 16 2019

Publication date (Print): May 2019

Volume: 21

Issue: 5

Pages: 553-576

Affiliations

[1 ]University of Belgrade Faculty of Medicine Belgrade Serbia

[2 ]Serbian Academy of Sciences and Arts Belgrade Serbia

[3 ]Department of CardiologyClinical Center of Serbia Belgrade Serbia

[4 ]Department of Cardiology and AngiologyMedical School Hannover Hannover Germany

[5 ]Cardiomyopathy Clinic and Heart Failure Institute, Leviev Heart Center, Sheba Medical Center and Sackler School of Medicine, Tel Aviv University Tel Aviv Israel

[6 ]Department of CardiologyRabin Medical Center, Sackler Faculty of Medicine, Tel Aviv University Tel Aviv Israel

[7 ]Department of MedicineKarolinska Institutet, and Heart and Vascular Theme, Karolinska University Hospital Stockholm Sweden

[8 ]Department of Internal Medicine BUniversity Medicine Greifswald Greifswald Germany

[9 ]Centre for Inherited Cardiovascular Diseases, IRCCS Foundation, University Hospital Policlinico San Matteo Pavia Italy

[10 ]Division of Cardiology, Department of Cardiological, Thoracic and Vascular SciencesUniversity of Padua Padua Italy

[11 ]University of Cyprus Medical School, Nicosia, Cyprus; Heart Failure Unit, Department of CardiologyAthens University Hospital Attikon, National and Kapodistrian University of Athens Athens Greece

[12 ]Second Department of CardiologyHeart Failure and Preventive Cardiology Section, Henry Dunant Hospital Athens Greece

[13 ]Department of Cardiothoracic Sciences, Università della Campania ‘Luigi VanvitellI’Monaldi Hospital, AORN Colli, Centro di Ricerca Cardiovascolare, Ospedale Monaldi, AORN Colli, Naples, Italy, and UCL Institute of Cardiovascular Science London UK

[14 ]Second Department of Medicine, Department of Cardiovascular MedicineGeneral University Hospital, Charles University in Prague Prague Czech Republic

[15 ]National Heart and Lung Institute, Imperial College London and Royal Brompton Hospital London UK

[16 ]Centre for Radiology and Magnetic Resonance Imaging, Clinical Centre of Serbia Belgrade Serbia

[17 ]Department of Cardiovascular DiseasesUniversity Hospital Center Zagreb, University of Zagreb Zagreb Croatia

[18 ]Mid‐German Heart Center, Department of Internal Medicine III, Division of CardiologyAngiology and Intensive Medical Care, University Hospital Halle, Martin‐Luther‐University Halle Halle Germany

[19 ]Department of CardiologyHacettepe University Faculty of Medicine Ankara Turkey

[20 ]Department of Cardiovascular SurgeryDokuz Eylül University Faculty of Medicine İzmir Turkey

[21 ]Pacemaker Center, Clinical Center of Serbia Belgrade Serbia

[22 ]Heart Failure Unit, CardiologyG. da Saliceto Hospital Piacenza Italy

[23 ]Centre for Clinical and Basic Research, Department of Medical SciencesIRCCS San Raffaele Pisana Rome Italy

[24 ]Medizinische Klinik, Kardiologie & Internistische Intensivmedizin, Klinikum Würzburg‐Mitte Würzburg Germany

[25 ]Cardiovascular DivisionBrigham and Women's Hospital, Harvard Medical School Boston MA USA

[26 ]Clinic for Endocrinology, Diabetes and Metabolic Disorders, Clinical Center Serbia and Faculty of MedicineUniversity of Belgrade Belgrade Serbia

[27 ]Department of CardiologyUniversity Heart Center Zürich Switzerland

[28 ]Clinic of Cardiac and Vascular Diseases, Institute of Clinical Medicine, Faculty of MedicineVilnius University Vilnius Lithuania

[29 ]State Research Institute Centre for Innovative Medicine Vilnius Lithuania

[30 ]Department of Social and Welfare Studies, Faculty of Health ScienceLinköping University Linköping Sweden

[31 ]Cardiovascular Research Institute Basel (CRIB) and Department of CardiologyUniversity Hospital Basel, University of Basel Basel Switzerland

[32 ]Cardiology DepartmentCentro Hospitalar São João Porto Portugal

[33 ]School of Nursing and Midwifery, Queen's University Belfast Belfast UK

[34 ]Department of CardiologyIRCCS San Raffaele Pisana Rome Italy

[35 ]Volgograd State Medical University, Regional Cardiology Centre Volgograd Volgograd Russia

[36 ]Cardiology, Department of Medical and Surgical SpecialtiesRadiological Sciences, and Public Health, University of Brescia Brescia Italy

[37 ]Department of Molecular Cardiology and EpigeneticsUniversity of Heidelberg Heidelberg Germany

[38 ]DZHK (German Centre for Cardiovascular Research) partner site Heidelberg/Mannheim Heidelberg Germany

[39 ]BIOMED ‐ Biomedical Research Institute, Faculty of Medicine and Life SciencesHasselt University Diepenbeek Belgium

[40 ]Department of CardiologyZiekenhuis Oost‐Limburg Genk Belgium

[41 ]University of Medicine Carol Davila Bucharest Romania

[42 ]Emergency Institute for Cardiovascular Diseases, ‘Prof. C. C. Iliescu’ Bucharest Romania

[43 ]Department of CardiologyUniversity Medical Center Groningen, University of Groningen Groningen The Netherlands

[44 ]Division of Cardiology and Metabolism, Department of Cardiology (CVK)Charité Berlin Germany

[45 ]Berlin‐Brandenburg Center for Regenerative Therapies (BCRT) Berlin Germany

[46 ]DZHK (German Centre for Cardiovascular Research) partner site Berlin, Charité Berlin Germany

[47 ]Cardiology, Department of ExperimentalDiagnostic and Specialty Medicine, Alma Mater Studiorum University of Bologna Bologna Italy

[48 ]Monash University, Australia, and University of Warwick Coventry UK

[49 ]Pharmacology, Centre of Clinical and Experimental Medicine, IRCCS San Raffaele Pisana, Rome, Italy, and St George's University of London London UK;

[50 ]Berlin‐Brandenburg Center for Regenerative Therapies, Deutsches Zentrum für Herz‐Kreislauf‐Forschung (DZHK) Berlin, Department of CardiologyCampus Virchow Klinikum, Charite ‐ Universitaetsmedizin Berlin Berlin Germany