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

Abnormal conduction-induced cardiomyopathy: a poorly explored entity

A dyssynchronous biventricular activation, which can be determined by left bundle branch block, chronic right ventricular pacing, frequent premature ventricular complexes, or pre-excitation, can cause a global abnormal contractility, thus leading to systolic dysfunction and left ventricular remodelling in a unique nosological entities: abnormal conduction-induced cardiomyopathies. In this clinical scenario, the mainstay therapy is eliminating or improving LV dyssynchrony, removing the trigger. This usually ensures the improvement and even recovery of cardiac geometry and left ventricular function, especially in the absence of genetic background. A multidisciplinary approach, integrating advanced multimodal imaging, is essential for the systematic aetiological definition and the subsequent evaluation and aetiology-guided therapies of patients and their families. This review aims to describe mechanisms, prevalence, risk factors, and diagnostic and therapeutic approach to the various abnormal conduction-induced cardiomyopathies, starting from reasonable certainties and then analysing the grey areas requiring further studies

Arrhythmic risk stratification in non-ischaemic dilated cardiomyopathy

Dilated cardiomyopathy is a primary disease of the heart muscle, which affects relatively young patients with a low comorbidity profile. It is characterized by structural and/or functional abnormalities leading to systolic dysfunction of the left ventricle or of both ventricles, often associated with dilatation, in the absence of an ischaemic, valvular, or pressure overload cause sufficient to explain the phenotype. Although the prognosis of the disease has greatly improved over the last few decades, prognostic stratification remains a fundamental objective, especially about the prediction of potentially life-threatening arrhythmic events. An accurate diagnostic work-up and an appropriate aetiopathogenetic characterization affect the patients' outcome and represent the essential basis of an adequate prognostic stratification. It is necessary to adopt a multiparametric approach, especially when the aim is the prediction of arrhythmic risk; it includes an integration of medical history and physical examination with cardiac imaging and genetic testing, in order to obtain a personalized diagnosis and therapeutic strategies. Furthermore, the evaluation should be repeated at every clinical check-up, considering the dynamic trend of the pathology and the arrhythmic risk changes over time. This article aims to illustrate how, starting from an exhaustive aetiological and clinical-instrumental characterization, including all diagnostic methods available at present time, it is possible to obtain a tailored diagnostic evaluation and stratification of the arrhythmic risk as accurate as possible

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

Conoscere e Curare il Cuore 2024 - Le cardiomiopatie da disturbo di conduzione

Capitolo dedicato alle cardiomiopatie da disturbo di conduzion

Tafamidis in the Treatment of ATTR-related Cardiomyopathy

: Transthyretin amyloid cardiomyopathy (ATTR-CM) is caused by the myocardial extracellular deposition of amyloid fibrils formed from the dissociation of TTR tetramer into monomers. The rate-limiting step in TTR amyloidogenesis is the dissociation of the TTR tetramer into monomers: Tafamidis is an effective TTR-stabilizer in its native homotetrameric structure. Tafamidis is a safe and effective drug in reducing symptoms, hospitalization and mortality in accurately selected patients affected by hereditary and wild-type transthyretin amyloid cardiomyopathy

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