Spectrum of Clinicopathologic Manifestations of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Multicenter Study
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Objectives The aim of the present investigation was to redefine the clinicopathologic profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC), with special reference to disease progression and left ventricular (LV) involvement.Background Long-term follow-up data from clinical studies indicate that ARVC is a progressive heart muscle disease that with time may lead to more diffuse right ventricular (RV) involvement and LV abnormalities and culminate in heart failure.Methods Forty-two patients (27 male, 15 female; 9 to 65 years old, mean [+/-SD] age 29.6 +/- 18) from six collaborative medical centers, with a pathologic diagnosis of ARVC at autopsy or heart transplantation, and with the whole heart available, were studied according to a specific clinicomorphologic protocol.Results Thirty-four patients died suddenly (16 during effort); 4 underwent heart transplantation; 2 died as a result of advanced heart failure; and 2 died of other causes. Sudden death was the first sign of disease in 12 patients; the other 30 had palpitations, with syncope in 11, heart failure in 8 and stroke in 3. Twenty-seven patients experienced ventricular arrhythmias (ventricular tachycardia in 17), and 5 received a pacemaker. Ten patients had isolated RV involvement (group A); the remaining 32 (76%) also had fibrofatty LV involvement that was observed histologically only in 15 (group B) and histologically and macroscopically in 17 (group C). Patients in group C were significantly older than those in groups A and B (39 +/- 15 years vs. 20 +/- 8.8 and 25 +/- 9.7 years, respectively), had significantly longer clinical follow-up (9.3 +/- 7.3 years vs. 1.2 +/- 2.1 and 3.4 +/- 2.2 years, respectively) and developed heart failure significantly more often (47% vs. 0 and 0, respectively). Patients in groups B and C had warning symptoms (80% and 87%, respectively, vs. 30%) and clinical ventricular arrhythmias (73% and 82%, respectively, vs. 20%) significantly more often than patients in group A. Hearts from patients in group C weighed significantly more than those from patients in groups A and B (500 +/- 150 g vs. 328 +/- 40 and 380 +/- 95 g, respectively), whereas hearts from both group B and C patients had severe RV thinning (87% and 71%, respectively, vs. 20%) and inflammatory infiltrates (73% and 88%, respectively, vs. 30%) significantly more often than those from group A patients.Conclusions LV involvement was found in 76% of hearts with ARVC, was age dependent and was associated with clinical arrhythmic events, more severe cardiomegaly, inflammatory infiltrates and heart failure. ARVC can no longer be regarded as an isolated disease of the right ventricle.
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Inflammatory infiltrates consistent with T lymphocytes and monocytes are frequently reported in pathological samples from both ventricles of patients with ACM, often fulfilling Dallas and ESC criteria for acute myocarditis, with or without detection of cardiac viral genomes.
“Inflammatory infiltrates, consistent with T lymphocytes and monocytes, are frequently reported in pathological samples from both ventricles of patients with ACM [15,17,42,43], often fulfilling both the Dallas and European Society of Cardiology (ESC) criteria for acute myocarditis [44,45], with or without the detection of cardiac viral genomes.”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [17]
Split into 2 atoms — 0 confirmed, 1 partially supported, 0 disputed, 0 misattributed, 1 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measuredInflammatory infiltrates are observed in 60-88% of patients with ACM and are especially common in patients who died suddenly.
“considering inflammatory infiltrates are observed in 60–88% of patients with ACM and are especially common in patients who died suddenly [16,17]”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [17]
The Histologic findings section reports inflammatory infiltrates in 29 of 42 hearts (69%), and the Abstract reports rates of 73% and 88% in two groups with left-ventricular involvement. These findings support a high prevalence in this autopsy/transplant ARVC series, but not the full 60–88% range across patients with ACM. The Isolated RV involvement section describes patients who more often died suddenly without warning yet infrequently had inflammatory infiltrates; it does not support the claim that infiltrates were especially common among those who died suddenly. Page numbers were unavailable in our copy. (LLM, conf=0.87)
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- Authors
- Corrado, D., Basso, C., Thiene, G., McKenna, W. J., Davies, M. J., Fontaliran, F., Nava, A., Silvestri, F., Blomstrom-Lundqvist, C., Wlodarska, E. K., Fontaine, G., & Camerini, F.
- Affiliations
- University of Padua (IT)
- University of London (GB)
- University of Trieste (IT)
- Corresponding author
- Corrado, Domenico
- ORCID
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 1997-11
- Volume
- 30
- Issue
- 6
- Pages
- 1512-1520
- ISSN
- 0735-1097 (ISSN-L)
- OpenAlex
- W1969774384
- Cited by
- 978
- References
- 46
- Citations by year
- 2026: 5 · 2025: 24 · 2024: 40 · 2023: 29 · 2022: 26 · 2021: 44 · 2020: 33 · 2019: 23 · 2018: 33 · 2017: 47 · 2016: 46 · 2015: 35 · 2014: 48 · 2013: 49 · 2012: 36
- FWCI
- 12.41
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- ArTS Archivio della ricerca di Trieste (University of Trieste https://www.units.it/) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Keywords
- arrhythmogenic right ventricular cardiomyopathy, left ventricular involvement, disease progression, sudden cardiac death, ventricular tachycardia, heart failure, clinicopathological correlation, autopsy findings, heart transplantation, cardiac magnetic resonance imaging, cardiac magnetic resonance spectroscopy, myocardial fibrosis, myocardial edema, myocarditis
- MeSH terms
- Adolescent, Adult, Aged, Arrhythmias, Cardiac, Child, Electrocardiography, Female, Humans, Male, Middle Aged, Myocardium, Death, Sudden, Cardiac, Disease Progression, Arrhythmogenic Right Ventricular Dysplasia
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