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September 15, 2026Journal of the American College of CardiologyOpen Access

Spectrum of Clinicopathologic Manifestations of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Multicenter Study

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Key result

LV involvement affects ~76% of ARVC hearts and is linked to arrhythmias and heart failure.

  • n=42

Population

42 patients with a pathologic diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia at…

Design

Case_series

Authors

DCDomenico CorradoCBCristina BassoGTGaetano Thiene

Discussion

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Overview

ARVC frequently involves the left ventricle (76% of cases), which is associated with older age, arrhythmias, and heart failure, indicating it is a progressive disease not isolated to the right ventricle.

Key Points

  • To characterize the broad clinical and histopathological spectrum of arrhythmogenic right ventricular cardiomyopathy/dysplasia across multiple medical centers.
  • Multicenter clinicopathologic analysis assessing clinical presentation, cardiac electrophysiology, and gross and microscopic myocardial pathology.
  • Reveals a spectrum ranging from localized, concealed fibrofatty lesions in the right ventricle to extensive biventricular myocardial replacement.
  • Links progressive structural myocardial atrophy and electrical instability to life-threatening ventricular arrhythmias and sudden cardiac death.

Study Design

Type

Observational (n=42)

Multicenter

Yes

Structured PICO

P
Population
42 patients (mean age 29.6 years) with a pathologic diagnosis of ARVC at autopsy or heart transplantation, evaluated for clinicopathologic manifestations and disease progression.
O
Outcome
Clinicopathologic profile of ARVC, with special reference to disease progression and left ventricular (LV) involvement

Main Result

Absolute Event Rate: 47% vs 0%

ARVC frequently involves the left ventricle (76% of cases), which is associated with older age, arrhythmias, and heart failure, indicating it is a progressive disease not isolated to the right ventricle.

Cite This Study

Corrado et al. (1997) conducted an observational in Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) (n=42). Macroscopic and histologic left ventricular (LV) involvement vs. Isolated right ventricular (RV) involvement was evaluated on Heart failure. Left ventricular involvement was found in 76% of ARVC hearts and was associated with clinical arrhythmic events, inflammatory infiltrates, and a 47% rate of heart failure in severe cases.

synapsesocial.com/papers/6aa9b59083705aff8b6d8ebehttps://doi.org/10.1016/s0735-1097(97)00332-x
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Also Consider

Synapse has enriched 3 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy. An analysis using 123I-meta-iodobenzylguanidine scintigraphy.1994 · 223 citations
  2. 2Right ventricular abnormalities in ventricular tachycardia of right ventricular origin: relation to electrophysiological abnormalities.1986 · 51 citations
  3. 3Arrhythmogenic Right Ventricular Cardiomyopathy1996 · 897 citations