358 Clinical characteristic and natural history of chemotherapy induced dilated cardiomyopathy

Abstract Chemotherapy can lead to chemotherapy-induced dilated cardiomyopathy (CI-DCM), recognized as one of the Non-ischaemic Dilated Cardiomyopathy (DCM) phenotypes characterized by worse outcome. Evidences on a direct comparison between idiopathic-DCM (iDCM) and CI-DCM still lack. We included all the consecutive patients enrolled in the Trieste Muscle Heart Disease Registry. C-DCM was defined according to current recommendations. Uni- and multivariable analysis and Kaplan-Meier were performed. The primary outcome was all-cause death and the secondary outcomes were cardiac death and a composite of heart failure hospitalization, heart transplantation, ventricular assist-device implantation and major ventricular arrhythmias. The study included 511 patients (499 patients affected by iDCM and 52 patients affected by CI-DCM). Compared to iDCM, CI-DCM patients were older (51 ± 14 years vs. 58 ± 3 years respectively, P < 0.001) and had a higher LVEF (35%±10 vs. 32%±9, P = 0.03). CI-DCM patients had a higher incidence of all-cause of death compared to iDCM (36.5% vs. 8.4%, P < 0.001), while the incidence of cardiac death (7% in the CI-DCM group vs. 4% in the iDCM group, P = 0. 232) and of the composite secondary outcome was comparable amongst the two groups. At multivariable analysis, the diagnosis of CI-DCM was an independent predictor of primary outcome incidence (HR: 5.79, 95% CI: 1.83–18.27), P = 0.003, together atrial fibrillation. In a well-selected DCM cohort, patients with a chemotherapic etiology had a higher incidence of all-cause mortality compared to iDCM, while the incidence of cardiac adverse events was comparable among CI-DCM and iDCM

Sudden Cardiac Death in Athletes: Facts and Fallacies

The benefits of exercise for cardiovascular and general health are many. However, sudden cardiac death (SCD) may occur in apparently healthy athletes who perform at the highest levels. A diverse spectrum of diseases is implicated in SCD in athletes, and while atherosclerotic coronary artery disease predominates in individuals of >35 years of age, primary cardiomyopathies and ion channelopathies are prevalent in young individuals. Prevention of SCD in athletes relies on the implementation of health policies aimed at the early identification of arrhythmogenic diseases (such as cardiac screening) and successful resuscitation (such as widespread utilization of automatic external defibrillators and training members of the public on cardiopulmonary resuscitation). This review will focus on the epidemiology and aetiologies of SCD in athletes, and examine fallacies in the approach to this controversial field. Furthermore, potential strategies to prevent these tragic events will be discussed, analysing current practice, gaps in knowledge and future directions

Re-Definition of the Epidemiology of Cardiac Amyloidosis

The epidemiology of cardiac amyloidosis (CA), traditionally considered a rare and incurable disease, has changed drastically over the last ten years, particularly due to the advances in diagnostic methods and therapeutic options in the field of transthyretin CA (ATTR-CA). On the one hand, the possibility of employing cardiac scintigraphy with bone tracers to diagnose ATTR-CA without a biopsy has unveiled the real prevalence of the disease; on the other, the emergence of effective treatments, such as tafamidis, has rendered an early and accurate diagnosis critical. Interestingly, the following subgroups of patients have been found to have a higher prevalence of CA: elderly subjects > 75 years, patients with cardiac hypertrophy hospitalized for heart failure with preserved ejection fraction, subjects operated on for bilateral carpal tunnel syndrome, patients with cardiac hypertrophy not explained by concomitant factors and individuals with aortic valve stenosis. Many studies investigating the prevalence of CA in these particular populations have contributed to rewriting the epidemiology of the disease, increasing the awareness of the medical community for a previously underappreciated condition. In this review, we summarized the latest evidence on the epidemiology of CA according to the different clinical settings typically associated with the disease

Clinical characterization and natural history of chemotherapy-induced dilated cardiomyopathy

AIMS: Chemotherapy‐induced dilated cardiomyopathy (CI‐DCM) is a well‐recognized phenotype of non‐ischemic dilated cardiomyopathy (DCM), characterized by poor outcomes. However, a detailed comparison between idiopathic DCM (iDCM) and CI‐DCM is still lacking. METHODS AND RESULTS: All consecutive DCM patients enrolled in the Trieste Muscle Heart Disease Registry were analysed. CI‐DCM and iDCM were defined according to current recommendations. The primary study outcome measure was all‐mortality death and secondary outcomes were a) a composite of cardiovascular death/heart‐transplantation/ventricular‐assist‐device implantation, and b) major ventricular arrhythmias. The study included 551 patients (499 iDCM and 52 CI‐DCM). At enrolment, compared with iDCM, CI‐DCM patients were older (51 ± 14 years vs. 58 ± 3 years, respectively, P < 0.001) and had a higher left ventricular ejection fraction (32% ± 9 vs. 35% ± 10, respectively, P = 0.03). Over a median follow‐up of 90 months (IQR 54–140 months), CI‐DCM patients had a higher incidence of all‐cause mortality compared with iDCM (36.5% vs. 8.4% in CI‐DCM and iDCM respectively, P < 0.001), while the incidence of major ventricular arrhythmias was higher in the iDCM group compared with CI‐DCM (4% vs. 0%, in CI‐DCM and iDCM respectively, P = 0.03). The risk of the composite outcome was comparable between the two groups (P = 0.91). At Cox multivariable analysis, the diagnosis of CI‐DCM emerged as independently associated to primary outcome (HR 6.42, 95% C.I. 2.52–16.31, P < 0.001). CONCLUSIONS: In a well‐selected DCM cohort, patients with a chemotherapy‐induced aetiology had a higher incidence of all‐cause mortality compared with iDCM. Conversely, the incidence of life‐threatening ventricular arrhythmic events was higher among patients with iDCM

Data_Sheet_1_Evolving trends in epidemiology and natural history of cardiac amyloidosis: 30-year experience from a tertiary referral center for cardiomyopathies.docx

ObjectiveNatural history of cardiac amyloidosis (CA) is poorly understood. We aimed to examine the changing mortality of different types of CA over a 30-year period.Patients and methodsConsecutive patients included in the “Trieste CA Registry” from January 1, 1990 through December 31, 2021 were divided into a historical cohort (diagnosed before 2016) and a contemporary cohort (diagnosed after 2016). Light chain (AL), transthyretin (ATTR) and other forms of CA were defined according to international recommendations. The primary and secondary outcome measures were all-cause mortality and cardiac death, respectively.ResultsWe enrolled 182 patients: 47.3% AL-CA, 44.5% ATTR-CA, 8.2% other etiologies. The number of patients diagnosed with AL and ATTR-CA progressively increased over time, mostly ATTR-CA patients (from 21% before 2016 to 67% after 2016) diagnosed non-invasively. The more consistent increase in event-rate was observed in the long-term (after 50 months) in ATTR-CA compared to the early increase in mortality in AL-CA. In the contemporary cohort, during a median follow up of 16 [4–30] months, ATTR-CA was associated with improved overall and cardiac survival compared to AL-CA. At multivariable analysis, ATTR-CA (HR 0.42, p = 0.03), eGFR (HR 0.98, p = 0.033) and ACE-inhibitor therapy (HR 0.24, p ConclusionIncidence and prevalence rates of ATTR-CA and, to a less extent, of AL-CA have been increasing over time, with significant improvements in 2-year survival of ATTR-CA patients from the contemporary cohort. Reaching an early diagnosis and starting disease-modifying treatments will improve long-term survival in CA.</p