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Alberto Cipriani

Publications and source records attributed to Alberto Cipriani.

2 recordsLinked to original sources
PubMed2026

Clinical Characteristics and Outcomes of Patients With Biventricular and Left-Dominant Arrhythmogenic Cardiomyopathy With Ring-Like Late Gadolinium Enhancement Pattern.

BACKGROUND: The ring-like pattern of late gadolinium enhancement (RL-LGE) on cardiac magnetic resonance (CMR) has been proposed as a distinctive imaging marker of arrhythmogenic cardiomyopathy (ACM) with left ventricular (LV) involvement. However, the clinical characteristics and prognostic significance remain to be further clarified. OBJECTIVES: This study sought to assess the clinical profile, genetic background, and prognostic significance of RL-LGE in ACM with LV involvement. METHODS: In this observational cohort study, we included consecutive patients with a diagnosis of biventricular or left-dominant ACM (BIV-ACM or LD-ACM). RL-LGE was defined as subepicardial or midmyocardial LGE involving ≥3 contiguous LV segments on the same short-axis slice. The primary endpoint was a composite of sudden cardiac arrest, sustained ventricular tachycardia, or implantable cardioverter-defibrillator (ICD) interventions. RESULTS: Among 149 patients (mean age 36 ± 12 years, 66% male), RL-LGE was identified in 73 (49%), most frequently in association with DSP variants, with a higher prevalence in BIV-ACM (70%) than in LD-ACM (30%). Over a median follow-up of 31 months, 24 patients experienced the primary endpoint, 67% of whom had RL-LGE. In multivariable Cox regression, RL-LGE emerged as an independent predictor of the primary endpoint (HR: 2.47; 95% CI: 1.10-6.02; P = 0.042), along with nonsustained ventricular tachycardia (HR: 2.62; 95% CI: 1.17-6.41; P = 0.033), whereas genetic status did not provide additional prognostic information. Incorporating RL-LGE into the arrhythmogenic right ventricular cardiomyopathy risk model significantly improved its predictive performance (likelihood ratio test: P = 0.006). CONCLUSIONS: RL-LGE independently predicts arrhythmic events in ACM, regardless of genotype status, and refines the prognostic performance of traditional risk models. Its detection may aid in early recognition of high-risk patients and inform primary-prevention ICD therapy.

Adult↗
PubMed2025

Clinical Phenotype and Prognosis of Asymptomatic Patients With Transthyretin Cardiac Amyloid Infiltration.

IMPORTANCE: Patients with transthyretin (ATTR) cardiac amyloid infiltration are increasingly diagnosed at earlier disease stages with no heart failure (HF) symptoms and a wide range of cardiac amyloid infiltration. OBJECTIVE: To characterize the clinical phenotype and natural history of asymptomatic patients with ATTR cardiac amyloid infiltration. DESIGN, SETTING, AND PARTICIPANTS: This cohort study analyzed data of all patients at 12 international centers for amyloidosis from January 1, 2008, through December 31, 2023. Inclusion criteria were asymptomatic ATTR cardiac amyloid infiltration, defined as an absence of HF history, HF signs and symptoms, diuretic therapy, and plasma cell dyscrasia with evidence of myocardial uptake on bone scintigraphy. If plasma cell dyscrasia was present, histologic confirmation of ATTR amyloid was required. EXPOSURE: Asymptomatic ATTR cardiac amyloid infiltration. MAIN OUTCOMES AND MEASURES: The primary outcomes were all-cause and cardiovascular (CV) mortality. The secondary outcomes were unplanned HF hospitalization, unplanned CV-related hospitalization, and a composite outcome of CV mortality and HF hospitalization. RESULTS: The study comprised 485 patients with asymptomatic ATTR cardiac amyloid infiltration (mean [SD] age, 74.9 [9.9] years, 85.8% male, 112 [23.1%] with hereditary ATTR amyloidosis), with 369 (76.1%) having grade 2 or 3 and 116 (23.9%) having grade 1 cardiac uptake at baseline. Patients with grade 2 or 3 uptake exhibited significantly more cardiac functional and structural abnormalities vs patients with grade 1 uptake. At 3 years, compared with grade 1 uptake, patients with grade 2 or 3 uptake had greater development of HF (54.3% [95% CI, 47.7%-61.3%] vs 23.1% [95% CI, 14.8%-35.1%]), greater outpatient diuretic initiation and N-terminal pro-B-type natriuretic peptide progression (35.0% [95% CI, 28.0%-43.2%] vs 12.4% [95% CI, 6.3%-23.7%]), and greater HF hospitalization (8.7% [95% CI, 5.9%-12.9%] vs 0%) and unplanned CV hospitalization (20.0% [95% CI, 15.7%-25.3%] vs 4.3% [95% CI, 1.6%-11.3%]). Over a median follow-up of 37 months (IQR, 20-64 months), the all-cause death rate was similar between patients with grade 1 vs 2 and 3 uptake; however, those with grade 2 or 3 compared with grade 1 uptake had a significantly higher risk of CV mortality (unadjusted hazard ratio, 5.30; 95% CI, 1.92-14.65). CONCLUSIONS AND RELEVANCE: This study shows that asymptomatic ATTR cardiac amyloid infiltration encompasses a wide spectrum of disease severity, with patients with grade 2 or 3 cardiac uptake experiencing an increased rate of CV events and CV mortality and patients with grade 1 uptake experiencing a lower CV event rate and predominantly non-CV mortality. These findings support the use of disease-modifying treatments in asymptomatic patients with grade 2 or 3 uptake and highlight the need of large-scale studies to assess their role in grade 1 uptake.

Humans↗