Emerging Trends in TransplantationThoracic transplantationHeart Transplantation in Systemic Sclerosis: New Impulses for Conventional Scleroderma Transplantation Regimen and Scleroderma Diagnostic Monitoring: 2 Case Reports
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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microRNA-145 mediates xylosyltransferase-I induction in myofibroblasts via suppression of transcription factor KLF4
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