Hot Phases Cardiomyopathy: Pathophysiology, Diagnostic Challenges, and Emerging Therapies.

Purpose Of Review: Hot phases are a challenging clinical presentation in arrhythmogenic cardiomyopathy (ACM), marked by acute chest pain and elevated cardiac troponins in the absence of obstructive coronary disease. These episodes manifest as myocarditis and primarily affect young patients, contributing to a heightened risk of life-threatening arrhythmias and potential disease progression. This review aims to synthesize recent research on the pathophysiology, diagnostic challenges, and therapeutic management of hot phases in ACM.

'Hot phase' non-dilated left ventricular cardiomyopathy with atypical onset and recurrence: a case report.

Non-dilated left ventricular cardiomyopathy (NDLVC) is a newly categorized cardiomyopathy phenotype includingseveral aetiologies with a linking characteristic represented by the normal left ventricular volume. Inflammatory heart disease (InHD) is a heterogeneous process with variegate clinical manifestations, sometimes in overlap with NDLVC. A 26-year-old woman was admitted forcomplete heart block (CHB) and persistently raised troponin.

Clinical impact of myocardial fibrosis in severe aortic stenosis.

The pressure overload due to the progressive narrowing of the valve area determines the development of the left ventricular hypertrophy which characterizes aortic stenosis (AS). The onset of myocardial fibrosis marks the inexorable decline of an initially compensatory response towards heart failure. However, myocardial fibrosis does not yet represent a key element in the prognostic and therapeutic framework of AS.

Cardiomyopathies in children: classification, diagnosis and treatment.

Purpose Of Review: Cardiomyopathies are rare in the pediatric population, but significantly impact on morbidity and mortality. The present review aims to provide an overview of cardiomyopathies in children and some practical guidelines for their prognostic stratification and management.

Recent Findings: Pediatric cardiomyopathies may present as isolated cardiac muscle disease or in the context of complex clinical syndromes.

Genetic Risk of Arrhythmic Phenotypes in Patients With Dilated Cardiomyopathy.

Background: Genotype-phenotype correlations in dilated cardiomyopathy (DCM) and, in particular, the effects of gene variants on clinical outcomes remain poorly understood.

Objectives: The purpose of this study was to investigate the prognostic role of genetic variant carrier status in a large cohort of DCM patients.

Methods: A total of 487 DCM patients were analyzed by next-generation sequencing and categorized the disease genes into functional gene groups.

ECG in dilated cardiomyopathy: specific findings and long-term prognostic significance.

Objective: The objective was to provide an exhaustive characterization of ECG features in a large cohort of dilated cardiomyopathies (DCMs) and then investigate their possible prognostic role in the long term.

Background: ECG is an accessible, reproducible, low-cost diagnostic and prognostic tool. However, an extensive description of ECG features and their long-term prognostic role in a large cohort of DCM is lacking.

Prognostic role of non-sustained ventricular tachycardia detected with remote interrogation in a pacemaker population.

Background: Non-sustained ventricular tachycardia (NSVT) can occur asymptomatically and can be incidentally detected in the internal records of pacemakers (PM). The clinical value of NSVT in the population of PM patients is still uncertain.Our aim was to assess the prevalence of NSVT detected by remote PM control, to describe the clinical and demographic characteristics of patients with NSVT, and to assess the prognostic significance of NSVT in terms of both overall and cardiovascular mortality.

Prognostic Role of Cardiac Magnetic Resonance in Arrhythmogenic Right Ventricular Cardiomyopathy.

We sought to evaluate the prognostic role of cardiac magnetic resonance (CMR) in patients with definite, borderline and possible diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) as defined by the International Task Force (TF) in 2010. CMR was performed in 175 patients: 52 with definite, 50 with borderline and 73 possible ARVC. Abnormal-CMR was defined as the presence of ≥1 CMR abnormalities (including abnormalities of right ventricular and left ventricular wall motion, fat infiltration, late gadolinium enhancement, dilation and dysfunction of either ventricles).

Usefulness of Addition of Magnetic Resonance Imaging to Echocardiographic Imaging to Predict Left Ventricular Reverse Remodeling in Patients With Nonischemic Cardiomyopathy.

Defining short-term prognosis in nonischemic cardiomyopathy (NICM) is challenging in clinical practice. Although left ventricular reverse remodeling (LVRR) is a key prognostic marker in NICM there are few parameters able to predict it. We investigated whether a complete structural and functional cardiac magnetic resonance imaging (cMRI) evaluation was incremental to the classic clinical-echocardiographic approach in predicting LVRR in a large cohort of NICM patients receiving evidence-based treatment.

Association between mutation status and left ventricular reverse remodelling in dilated cardiomyopathy.

Objective: To explore the genetic landscape of a well selected dilated cardiomyopathy (DCM) cohort, assessing the possible relation between different genotypes and left ventricular reverse remodelling (LVRR).

Methods: A cohort of 152 patients with DCM from the Heart Muscle Disease Registry of Trieste has been studied by next-generation sequencing (NGS). Patients were grouped into different 'gene-clusters' with functionally homogeneous genetic backgrounds.