Heart Transplantation in Systemic Sclerosis: A Therapeutic Option. Presentation of a Case and Literature Review

Diego-Sola, Andrea de; Dubuc, César Antonio Egües; Vives, Cristina Goena; Irazabal, Juan José Intxausti; Alonso, Olga Máiz; Belaustegi, Manuel Cobo

doi:10.1016/j.reuma.2021.04.007

Cited by 3 publications

(1 citation statement)

References 9 publications

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“…Heart disease is present in all forms of Fabry disease, with different grades of organ involvement, and the concentric left ventricular (LV) hypertrophy [116]. Systemic sclerosis that is a systemic autoimmune disease of heterogeneous pathogenesis in which vascular, cutaneous, and internal organ fibrosis are prominent [117], in the literature there are reports of successful HT [115][116][117]; however, each case should never fail to be evaluated individually, for patients with PF and specifically for patients with Failing Fontan, there is no doubt that the treatment is HT and the fact of knowing that the presence of a single fault that could be PLE, PB, HF, and arrhythmias are not associated with a greater risk of death. It is new information in the literature since failures have never been evaluated in this way and it is very useful for patient care.…”

Section: Discussionmentioning

confidence: 99%

Failures of the Fontan System in Univentricular Hearts and Mortality Risk in Heart Transplantation: A Systematic Review and Meta-Analysis

Márquez-González

Hernández-Vásquez

Valle-Lom

et al. 2021

Life

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The Fontan procedure (FP) is the standard surgical treatment for Univentricular heart diseases. Over time, the Fontan system fails, leading to pathologies such as protein-losing enteropathy (PLE), plastic bronchitis (PB), and heart failure (HF). FP should be considered as a transitional step to the final treatment: heart transplantation (HT). This systematic review and meta-analysis aims to establish the risk of death following HT according to the presence of FP complications. There was a total of 691 transplanted patients in the 18 articles, immediate survival 88% (n = 448), survival from 1 to 5 years of 78% (n = 427) and survival from 5.1 to 10 years of 69% (n = 208), >10 years 61% (n = 109). The relative risk (RR) was 1.12 for PLE (95% confidence interval [CI] = 0.89–1.40, p = 0.34), 1.03 for HF (0.7–1.51, p = 0.88), 0.70 for Arrhythmias (0.39–1.24, p= 0.22%), 0.46 for PB (0.08–2.72, p = 0.39), and 5.81 for CKD (1.70–19.88, p = 0.005). In patients with two or more failures, the RR was 1.94 (0.99–3.81, p = 0.05). After FP, the risk of death after HT is associated with CKD and with the presence of two or more failures.

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Section: Discussionmentioning

confidence: 99%

Failures of the Fontan System in Univentricular Hearts and Mortality Risk in Heart Transplantation: A Systematic Review and Meta-Analysis

Márquez-González

Hernández-Vásquez

Valle-Lom

et al. 2021

Life

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Heart involvement in patients with systemic sclerosis—what have we learned about it in the last 5 years

Nadel,

Taborska

et al. 2024

Rheumatol Int

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This review provides a detailed examination of original research and previously published reviews regarding cardiovascular involvement in systemic sclerosis (SSc). Our study aims to evaluate the current understanding of SSc-associated heart involvement (SHI), focusing on its most prevalent forms, diagnostic methods and treatment options. A comprehensive search of PUBMED, Medline, Web of science, Scopus and DOAJ databases was conducted, involving articles published between January 2019 and August 2024, available in English, both original research and reviews. Additionally, the authors examined the references cited in the selected articles, reviewed relevant literature, and included key publications dating back to 2010. Systemic Sclerosis (SSc) is an autoimmune connective tissue disease characterized by skin and internal organs fibrosis with accompanying vasculopathy. SHI encompasses both primary and secondary cardiac disease with a prevalence rate of up to 39%. It constitutes one of the leading causes of death among affected individuals. Systemic sclerosis- primary heart involvement comprises a wide range of conditions including arrhythmias, heart failure, pericardial disease, valvular abnormalities, and myocardial inflammation. However, its subclinical course, often misinterpreted as other forms of cardiomyopathy, poses true diagnostic challenges, requiring diagnostic tools like transthoracic echocardiography with tissue Doppler echocardiography and cardiac magnetic resonance imaging. The review underscores the importance of SHI and a holistic approach to managing patients with systemic sclerosis. Furthermore, it emphasizes the need for further investigation into potential pathogenetic mechanisms and biomarkers crucial for targeted treatment to fully optimize recommendations for this patient subgroup.

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Heart transplantation in juvenile-onset systemic sclerosis with primary cardiac involvement: report of two cases and comprehensive literature review

Bacich,

Tessari,

Andreis

et al. 2025

Current Problems in Cardiology

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Heart Transplantation in Systemic Sclerosis: A Therapeutic Option. Presentation of a Case and Literature Review

Cited by 3 publications

References 9 publications

Failures of the Fontan System in Univentricular Hearts and Mortality Risk in Heart Transplantation: A Systematic Review and Meta-Analysis

Failures of the Fontan System in Univentricular Hearts and Mortality Risk in Heart Transplantation: A Systematic Review and Meta-Analysis

Heart involvement in patients with systemic sclerosis—what have we learned about it in the last 5 years

Heart transplantation in juvenile-onset systemic sclerosis with primary cardiac involvement: report of two cases and comprehensive literature review

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