Long‐term prognostic impact of therapeutic strategies in patients with idiopathic dilated cardiomyopathy: changing mortality over the last 30 years

Abstract

Aims ACE‐inhibitors, β‐blockers, implantable cardioverter–defibrillator (ICD) and cardiac resynchronization therapy (CRT) improved prognosis of heart failure. We sought to analyse the long‐term prognostic impact of evidence‐based integrated therapeutic strategies in patients with idiopathic dilated cardiomyopathy (IDCM). Methods and results From 1978 to 2007, 853 IDCM patients (45 ± 15 years, 72% males) were enrolled and classified as follows: Group 1, 110 patients (12.8%) enrolled during 1978–1987; Group 2, 376 patients (44.1%) enrolled during 1988–1997; Group 3, 367 patients (43.1%) enrolled during 1998–2007. ACE‐inhibitors/angiotensin receptor blockers were administered in 34%, 93%, and 93% (P <0.001), and β‐blockers in 11%, 82%, and 86% (P <0.001) in Groups 1, 2, and 3, respectively; ICDs were implanted in 2%, 14%, and 13% (P = 0.005); mean time to device implantation was lower in Group 3. At 8 years, heart transplant (HTx)‐free survival rates were 55%, 71%, and 87% in Groups 1, 2, and 3, respectively (P <0.001). Similar progressive improvement was found for pump‐failure death (DHF)/HTx, while survival free from sudden death (SD) was significantly improved only in Group 3. Multivariable models considering competing risk indicated early diagnosis (i.e. a baseline less advanced disease stage) and tailored medical therapy (HR 0.44, CI 95% 0.19–0.98) as independent protectors against DHF/HTx. Concerning SD, lower left ventricular ejection fraction emerged as a predictor, while ICD was the only therapy with a protective role (HR 0.08, CI 95% 0.01–0.61). Treatment with digitalis emerged as a predictor of both DHF/HTx and SD. Conclusions An effective management and evidence‐based integrated therapeutic approach progressively and significantly improved the long‐term prognosis of IDCM during the last three decades.

Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life‐Threatening Arrhythmias

“…Exclusion criteria included arterial hypertension (>140/90 mm Hg),12 coronary artery disease (obstruction >50% of the luminal diameter in a major branch by coronary angiography), history of chronic excess alcohol consumption (>100 g/d), tachycardia‐induced cardiomyopathy, systemic diseases affecting short‐term prognosis, pericardial disease, congenital heart disease, and cor pulmonale 8, 13…”

Positron emission tomography: An additional prognostic tool in dilated cardiomyopathy?

“…1,2 The prevalence of DCM in the general population is thought to be &40 to 50 cases per 100,000 and the incidence is 6.95/100,000 new cases a year, but this is a rough estimation since there is a growing evidence that the course of the illness is asymptomatic and difficult to recognize for a long period. 3,4 Transplant-free survival has improved in the last three decades from 55% to 71% and 87% at 8 years 5 thanks to early diagnosis, effective management, and evidence-based integrated therapeutic approach including multiple drug therapy, implantable defibrillators, and cardiac resynchronization (CRT) treatments. Despite this success, DCM remains in most patients, a progressive disease with large individual variability and a poor prognosis.…”

Clinical management of dilated cardiomyopathy: current knowledge and future perspectives

“…In a large cohort of an Italian DCM population from the Heart Muscle Diseases Registry of Trieste, it has been shown that nowadays the incidence of major adverse cardiac events in patients affected by DCM is less than 2% per year, the survival free of transplantation at 8 years from diagnosis is more than 85% and the incidence of SCD is less than 0.5% per year. [2] Pharmacological treatment is the cornerstone for preventing the progression of the disease. According to the most recent guidelines, ACE-I, β-blockers and mineralocorticoid antagonists are the pillar therapeutic agents demonstrated to improve survival in patients affected by HF.…”

“…[1] Once symptoms develop, DCM usually leads to decompensated heart failure (HF) and it represents one of the most common causes of heart transplantation (HTx) in the Western world. [2] On the other hand often life-threatening arrhythmias and sudden cardiac death (SCD) can importantly characterize the course of DCM or represent the abrupt onset of the disease. [3] Management of DCM is currently particularly difficult for the clinicians.…”