Salvage Heart Transplantation for Severe Biventricular Failure Associated with Systemic Sclerosis-Rheumatoid Arthritis Overlap Syndrome

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Severe biventricular failure associated with systemic sclerosis-rheumatoid arthritis (SSc/RA) overlap syndrome (n=1). Intervention: Urgent heart transplantation bridged with mechanical circulatory support and bidirectional Glenn procedure. Primary outcome: Survival and freedom from graft rejection, allograft dysfunction, or relapse of systemic sclerosis/rheumatoid arthritis.

Ayano Hara; Toru Hashimoto; Yuki Kodono; Komei Mizokami; Takafumi Sakamoto; Keimei Yoshida; Shouji Matsushima; Kayo Misumi; Takeo Fujino; Shunsuke Katsuki; Hirohide Matsuura; Naoya Nishimura; Takeshi Iwasaki; Tomoki Ushijima; Hiromichi Sonoda; Akira Shiose; Kohtaro Abe

doi:10.1536/ihj.26-381

January 1, 2026International Heart JournalOpen Access

Salvage Heart Transplantation for Severe Biventricular Failure Associated with Systemic Sclerosis-Rheumatoid Arthritis Overlap Syndrome

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Key result

Urgent heart transplantation after mechanical circulatory support and a bidirectional Glenn shunt resulted in 1.5-year survival without rejection or autoimmune relapse in a patient with SSc/RA.

Key Points

To describe the clinical management and outcome of catastrophic biventricular failure in a patient with systemic sclerosis and rheumatoid arthritis overlap syndrome.
Clinical case evaluation of a patient with systemic sclerosis-rheumatoid arthritis overlap syndrome presenting with biventricular failure mimicking arrhythmogenic cardiomyopathy
Implementation of central extracorporeal membrane oxygenation as a bridge to urgent salvage heart transplantation
Urgent salvage heart transplantation was performed successfully following central extracorporeal membrane oxygenation stabilization
Post-transplant course showed no graft rejection, no allograft dysfunction, and no recurrence of systemic sclerosis or rheumatoid arthritis

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P

Population

1 male patient in his 30s with systemic sclerosis and seronegative rheumatoid arthritis overlap syndrome complicated by severe biventricular failure and cardiogenic shock, followed for 1.5 years post-transplant.

I

Intervention

Central extracorporeal membrane oxygenation (ECMO) and urgent salvage heart transplantation (HTx)

O

Outcome

Survival post-transplanthard clinical

Limitations

Single-patient case report
The long-term risk of recurrent connective tissue disease-associated myocardial involvement after heart transplantation remains undetermined

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Implication

This case report demonstrates that urgent heart transplantation following central ECMO can be a life-saving intervention for severe biventricular failure associated with systemic sclerosis-rheumatoid arthritis overlap syndrome.

Abstract

Systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by vasculopathy and fibrosis, and it often affects the heart, leading to ventricular dysfunction, conduction abnormalities, and arrhythmias. Rheumatoid arthritis (RA) is also complicated by pericarditis, valvular heart diseases, and diastolic dysfunction. SSc-associated right ventricular (RV) dysfunction is a rare but life-threatening condition. Herein, we present a case of SSc/RA overlap syndrome associated with catastrophic biventricular failure mimicking arrhythmogenic cardiomyopathy/arrhythmogenic right ventricular cardiomyopathy (ACM/ARVC), requiring central extracorporeal membrane oxygenation (ECMO). Urgent heart transplantation (HTx) successfully saved the patient's life, and he experienced no rejection, allograft dysfunction, or relapse of SSc/RA post-transplant.

Discussion

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Cite This Study

Hara et al. (2026) conducted a case report in Severe biventricular failure associated with systemic sclerosis-rheumatoid arthritis (SSc/RA) overlap syndrome (n=1). Urgent heart transplantation bridged with mechanical circulatory support and bidirectional Glenn procedure was evaluated on Survival and freedom from graft rejection, allograft dysfunction, or relapse of systemic sclerosis/rheumatoid arthritis. Urgent heart transplantation after mechanical circulatory support and a bidirectional Glenn shunt resulted in 1.5-year survival without rejection or autoimmune relapse in a patient with SSc/RA.

synapsesocial.com/papers/6af88c410e4d8f4b089c1ab2 https://doi.org/10.1536/ihj.26-381

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Salvage Heart Transplantation for Severe Biventricular Failure Associated with Systemic Sclerosis-Rheumatoid Arthritis Overlap Syndrome

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

Salvage Heart Transplantation for Severe Biventricular Failure Associated with Systemic Sclerosis-Rheumatoid Arthritis Overlap Syndrome

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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