Emerging Trends in Transplantation
Thoracic transplantation
Heart Transplantation in Systemic Sclerosis: New Impulses for Conventional Scleroderma Transplantation Regimen and Scleroderma Diagnostic Monitoring: 2 Case Reports

https://doi.org/10.1016/j.transproceed.2019.01.025Get rights and content

Highlights

  • Exclusive HTx is a rare approach for the treatment of heart failure caused by SSc.

  • HTx in SSc patients suffering from cardiac involvement is a safe therapeutic option.

  • We describe the first case of an SSc patient receiving an HCV-positive donor heart.

  • DAA therapy gives us new opportunities to consider HCV-positive donor organs.

  • Monitoring of serum XT activity reflects SSc progression and organ recovery.

Abstract

Background

Although low (but increasing) rates of lung/lung-heart transplantations of scleroderma (systemic sclerosis [SSc]) patients have been reported, exclusive heart transplantation is a rare approach for treatment of heart failure due to SSc.

Cases

We report on 2 cases of SSc patients receiving a heart transplantation (HTx) due to severe and progressive right heart failure without pulmonary artery hypertension. One patient received a hepatitis C virus (HCV)-positive donor heart and recovered excellently from viral transmission after administration of a direct-acting antiviral (DAA) regimen. This is the first published case of an SSc patient who underwent HTx using an HCV-positive donor heart. The clinical course of both patients was monitored by different serum SSc biomarkers. Only xylosyltransferase activity proved to be a promising biomarker for disease stage determination and therapeutic monitoring, precisely reflecting fibrotic remodeling and successful organ recovery.

Conclusions

Successful implementation of the 2 cases described here demonstrates that HTx is a safe and effective therapeutic option for defined SSc sub-patient groups despite the progressive character of the underlying disease. In the future, xylosyltransferase activity might be conducive to simplify the identification of patients with low systemic involvement but a strong indication for single heart transplantation. Finally, we demonstrate that treatment of HCV viral transmission from HCV-positive donor to organ recipient using DAA gives us new opportunities to consider HCV-positive donor organs for HTx and might reveal new possibilities to ease the lack of donor organs.

Section snippets

Case Presentations

The author(s) received informed consent from both patients.

Case Report 1

Patient 1 was a 44-year-old woman who was diagnosed with dSSc at the age of 32. A progressive involvement of the skin, Raynaud's phenomenon, left elbow calcinosis, pitting scars, and abnormal esophagomanometry without pulmonary involvement developed over time. Cardiac impairment, including a progressive atrioventricular conduction abnormality and cardiomyopathy with right ventricular failure, was detected 5 years pre-HTx. Cardiac complications worsened with the development of functional

Case Report 2

Patient 2 was a 44-year-old woman suffering from severe dcSSc who was referred to our institution due to cardiac manifestation. Weak symptoms of Raynaud's disease had emerged 14 years pre-HTx. The subsequent clinical course was complicated by the occurrence of skin sclerosis, esophageal motility disturbances, and gastroesophageal reflux. Furthermore, a slight fibrotic involvement of lung tissue was detected by a computed tomography scan. Several therapeutic approaches were attempted

Discussion

Although cardiac manifestations in SSc are associated with 70% mortality after 5 years, there are no effective treatment options [4]. We report here on 2 patients with SSc suffering from rapid progressive right heart failure treated successfully with isolated HTx.

The first finding of this report is that HTx represents a feasible approach to treat certain SSc sub-patient groups. Only a few publications describe the approach of HTx in cases of SSc [7], [8], [9]. In contrast, LTx or HTx-LTx is

Acknowledgments

We thank Christoph Lichtenberg for his technical assistance and Philip Saunders for his linguistic advice.

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