Background: In Hypertrophic cardiomyopathy (HCM) increasing evidence suggests left atrial (LA) remodelling plays a critical role in disease progression. Traditional static LA size measurements, while established as risk markers, do not reflect the dynamic nature of LA changes over time.
Objectives: This study aimed to define long-term LA remodelling trajectories in HCM and assess their prognostic value for predicting atrial fibrillation (AF), sudden cardiac death (SCD), and heart failure (HF) death.
Hypertrophic cardiomyopathy (HCM) is a genetic heart disorder defined by the presence of a maximal wall thickness of at least 15 mm with two main forms: obstructive (oHCM) and nonobstructive (nHCM). While oHCM is characterized by left ventricular (LV) outflow tract obstruction (LVOTO), nHCM lacks this feature and shows significant variability in its hemodynamic and anatomical traits. In nHCM, LV hypertrophy (LVH) presents diverse morphologies, including apical hypertrophy and reverse septal curvature, the latter potentially causing mid-ventricular obstruction and near-complete LV emptying.
View Article and Find Full Text PDFOrphanet J Rare Dis
December 2024
Background: Hereditary transthyretin (ATTRv) amyloidosis is rare, autosomal dominant disease with a fatal outcome if left untreated. Early stages detection is crucial for intervention. We aimed identifying early indexes of cardiac involvement and their eventual correlation with neurological indexes, in pre-symptomatic subjects with TTR gene mutation.
View Article and Find Full Text PDFIn patients with severe left ventricle hypertrophy and aortic stenosis, the presence of intraventricular gradient should always be investigated. Its prompt recognition enables a precise diagnosis and safe treatment of both conditions. We report 2 cases demonstrating a successful and novel approach to this clinical situation.
View Article and Find Full Text PDFEur Heart J Case Rep
November 2024
Background: Sex-specific differences in left ventricular (LV) geometry might help in developing tailored strategies for hypertension management.
Objectives: The purpose of the study was to evaluate sex-related differences in LV geometry at baseline and over time in hypertension.
Methods: From a prospective registry, we included hypertensives without prevalent cardiovascular disease, incident myocardial infarction, chronic kidney disease > stage III, and with normal LV ejection fraction.
Front Cardiovasc Med
August 2024
Background: In obstructive hypertrophic cardiomyopathy (HOCM), disopyramide is used in patients who remain symptomatic despite -blockers or verapamil. However, effectiveness of disopyramide therapy has not been clearly established due to inconsistent definition of responders and the insufficient length of follow-ups reported in literature. To address these shortcomings, we have conducted a retrospective analysis from detailed databases with long follow-up, from two HCM Referral Centers.
View Article and Find Full Text PDFTrends Cardiovasc Med
February 2025
Hypertrophic Cardiomyopathy (HCM) presents a complex diagnostic and prognostic challenge due to its heterogeneous phenotype and clinical course. Artificial Intelligence (AI) and Machine Learning (ML) techniques hold promise in transforming the role of Electrocardiography (ECG) in HCM diagnosis, prognosis, and management. AI, including Deep Learning (DL), enables computers to learn patterns from data, allowing for the development of models capable of analyzing ECG signals.
View Article and Find Full Text PDFHypertrophic cardiomyopathy (HCM) is a genetic disease characterized by unexplained left ventricular hypertrophy (LVH), diastolic dysfunction, and increased sudden-death risk. Early detection of the phenotypic expression of the disease in genetic carriers without LVH (Gen+/Phen-) is crucial for emerging therapies. This clinical study aims to identify echocardiographic predictors of phenotypic development in Gen+/Phen-.
View Article and Find Full Text PDFInt J Cardiol
January 2024
Background And Aim: Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM) with significant effects on outcome. We aim to compare the left atrial (LA) diameter measurement with HCM-AF Score in predicting atrial fibrillation (AF) development in HCM.
Methods: From the regional cohort of the Campania Region, Italy, 519 HCM patients (38% women, age45 ± 17 years) without history of AF, were enrolled in the study.
J Cardiovasc Transl Res
April 2024
Hypertrophic cardiomyopathy (HCM) is mainly caused by sarcomeric mutations which may affect myocardial mechano-energetic efficiency (MEE). We investigated the effects of sarcomeric mutations on MEE. A non-invasive pressure/volume (P/V) analysis was performed.
View Article and Find Full Text PDFDiagnostics (Basel)
July 2023
Aortic stenosis (AS) is a valvular heart disease that significantly contributes to cardiovascular morbidity and mortality worldwide. The condition is characterized by calcification and thickening of the aortic valve leaflets, resulting in a narrowed orifice and increased pressure gradient across the valve. AS typically progresses from a subclinical phase known as aortic sclerosis, where valve calcification occurs without a transvalvular gradient, to a more advanced stage marked by a triad of symptoms: heart failure, syncope, and angina.
View Article and Find Full Text PDFHypertrophic cardiomyopathy (HCM) is a genetic disease with heterogeneous clinical presentation and prognosis. Within the broad phenotypic expression of HCM, there is a subgroup of patients with a left ventricular (LV) apical aneurysm, which has an estimated prevalence between 2% and 5%. LV apical aneurysm is characterized by an area of apical dyskinesis or akinesis, often associated with regional scarring.
View Article and Find Full Text PDFCardiac surgery is necessary in almost 50% of patients with endocarditis. Early surgery, i.e.
View Article and Find Full Text PDFA 20-year-old man was admitted to the intensive care unit for septic shock due to Lemierre's syndrome. It is a rare syndrome that manifests as an upper respiratory infection, although systemic involvement, severe coagulopathy, and multi-organ failure can dangerously complicate the clinical picture. In this syndrome, sepsis-related neuroendocrine dysregulation and microcirculation impairment can have a rapid deleterious progression.
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