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Dr. Luigi Sansone

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1. MEBIC Consortium, San Raffaele University, 00166 Rome, Italy
2. Cellular and Molecular Pathology, IRCCS San Raffaele Roma, Via di Val Cannuta 247, 00166 Rome, Italy
Interests: molecular pathology; autophagy; heart diseas; cellular pathology; sirtuins; cell death; electron microscopy

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Dear Colleagues,

Cardiomyopathies are primary heart disorders that occur in the absence of underlying causes such as coronary artery disease, hypertension, and valvular or congenital heart disease. Based on the predominant clinical abnormalities in cardiac structure and function, cardiomyopathies are classified into three major subtypes: hypertrophic, dilated, and arrhythmogenic. Discoveries made over the past several decades have defined the precise genetic etiology in many patients with these disorders, which have propelled insights into the molecular mechanisms by which pathogenic variants cause cardiomyopathies. These advances raise the prospect for new treatments that directly target gene variants or the proximal downstream pathways that mediate disease. The aim of the current Special Issue is to collect articles and reviews aiming to provide updates on clinical aspects of the main cardiomyopathies.

Dr. Luigi Sansone
Guest Editor

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Keywords

cardiomyopathy
heart failure
cardiomyopathy hypertrophic
cardiomyopathy dilated
heart disease
Fabry disease

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17 pages, 1443 KiB

Open AccessSystematic Review

Cardiomyopathy in Celiac Disease: A Systematic Review

by Stefan Milutinovic, Predrag Jancic, Adam Adam, Milan Radovanovic, Charles W. Nordstrom, Marshall Ward, Marija Petrovic, Dorde Jevtic, Maja Delibasic, Magdalena Kotseva, Milan Nikolajevic and Igor Dumic

J. Clin. Med. 2024, 13(4), 1045; https://doi.org/10.3390/jcm13041045 - 12 Feb 2024

Viewed by 1153

Abstract

(1) Background: Cardiomyopathy in celiac disease or celiac cardiomyopathy (CCM) is a serious and potentially life-threatening disease that can occur in both adults and children. However, data supporting the causal relationship between celiac disease (CD) and cardiomyopathy (CMP) are still inconsistent. The aim of this study was to review and synthesize data from the literature on this topic and potentially reveal a more evidence-based causal relationship. (2) Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to search Medline, Embase, and Scopus databases from database inception until September 2023. A total of 1187 original articles were identified. (3) Results: We identified 28 CCM patients (19 adult and 9 pediatric) with a mean age of 27.4 ± 18.01 years. Adult patients with CCM were predominantly male (84.2%) while pediatric patients were predominantly female (75%). The most common comorbidities associated with CCM were anemia (75%) and pulmonary hemosiderosis (20%). In 35% of patients, CCM occurred before the diagnosis of CD, while in 48% of patients, CCM and CD were diagnosed at the same time. Diagnosis of CD preceded diagnosis of CCM in only 18% of patients. Diagnosis of CCM is often delayed with an average, from the onset of symptoms to diagnosis, of 16 months. All patients were treated with a gluten-free diet in addition to guideline-directed medical therapy. At 11-month follow-up, cardiovascular improvement was seen in 60.7% of patients. Pediatric mortality was 33.3%, while adult mortality was 5.3%. (4) Conclusions: Clinicians should be aware of the possible association between CD and CMP, and we recommend CD work-up in all patients with CMP who have concomitant anemia. While we identified only 28 cases in the literature, many cases might go unreported due to a lack of awareness regarding CCM. A high degree of clinical suspicion and a prompt diagnosis of CCM are essential to minimizing the risks of morbidity and mortality, as the combination of a gluten-free diet and guideline-directed medical therapy can improve clinical outcomes. Full article

(This article belongs to the Special Issue Cardiomyopathy: A Comprehensive Review)

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