Heart Transplantation in Systemic Sclerosis: New Impulses for Conventional Scleroderma Transplantation Regimen and Scleroderma Diagnostic Monitoring: 2 Case Reports
| Autor: | R. Kandolf, Buntaro Fujita, Isabel Faust, Tanja Vollmer, Doris Hendig, Joachim Kuhn, Cornelius Knabbe, U. Kellner, Jan Weile, A.-C. Stan, Jan Gummert |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Adult
medicine.medical_specialty medicine.medical_treatment Transplants Antiviral Agents Scleroderma medicine.artery Internal medicine medicine Humans Heart transplantation Heart Failure Transplantation Scleroderma Systemic business.industry Hepatitis C Hepatitis C Chronic medicine.disease Tissue Donors Regimen Heart failure Pulmonary artery Cardiology Biomarker (medicine) Heart Transplantation Surgery Female business |
| Zdroj: | Transplantation proceedings. 51(3) |
| ISSN: | 1873-2623 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect