Background There is no specific treatment for primary cardiac involvement in systemic sclerosis (SSc). Even if heart transplantation is an option, only 1 case has been reported1.
Objectives Analyse data of patients with SSc and primary cardiac involvement resulting in heart transplantation.
Methods Retrospective review of charts from patients with SSc and primary cardiac involvement requiring a heart transplant in Strasbourg University Hospitals.
Results Patient 1: 37 year-old woman was diagnosed with diffuse SSc in 1988 when she was 12. She had cutaneous sclerosis, digital ulcers (DU) and oesophageal dysmotility coupled with positive ANA without specificity and a pathologic capillaroscopy. In 1999, she had 2 episodes of junctional tachycardia with ventricular ectopic beats and a complete right bundle branch block (RBBB) with left anterior fascicular block on EKG. Transthoracic echocardiogram (TTE) was normal.
In 2000, an asymptomatic left ventricular dysfunction and segmental akinesia were noticeable, with ejection fraction (EF) of 45%. The EKG showed a progressive LBBB dictating a need for a defibrillator. The installation of the device was made in 2000 and the patient experienced 11 internal shocks afterwards. There was a progressive left-side heart dysfunction on serial TEE with dilatation of heart chambers. On right heart catheterisation (RHC) prior to transplantation, there was a slight post-capillary pulmonary hypertension and cardiac index (CI) was 2.6L/min/m2. The transplantation was realised on August 31st 2011 and the pathology of the explanted specimen showed myocardial fibrosis. The intervention was followed by multiple complications: right hand amputation for acute ischemia, post-operative renal insufficiency resulting in severe chronic renal insufficiency and 4 episodes of serious infections. There was no transplant rejection during 29 months of follow-up.
Patient 2: 26 year-old female was diagnosed with diffuse SSc and anti-topo I antibodies in 2003 when she was 16. The mRSS score was 30/51 and she experienced DU, polyarthritis and gastro-intestinal involvement with malabsorption. Right ventricle dysfunction was discovered on TTE screening in 2008 while the patient was asymptomatic. The cardiac MRI detected myocardial fibrosis of the right ventricle with reduced EF=23% and normal left side function. Despite intensive immunosuppressive treatment with steroids, cyclophosphamide followed by mycophenolate mofetil and IVIg, there was a noticeable progression of myocardial fibrosis on serial MRI.
In 2010, the patient was hospitalised for ventricular tachycardia which was an indication for a defibrillator. The recurrence of arrhythmic episodes afterwards justified a heart transplantation on October 12th 2013. The CI was 1.72L/min/m2 on pre-intervention RHC, without pulmonary hypertension. The pathology specimen demonstrated myocardial fibrosis without inflammation nor necrosis. There was no infectious, ischemic or graft rejection complications 3 months after the procedure.
Conclusions Heart transplantation is probably a life-saving procedure in SSc patients with primary cardiac involvement manifesting with progressive dysfunction and/or arrhythmic complications.
Martens E. et al. Transplantation 2012; 94 (2)
Disclosure of Interest None declared