Cardiac transplantation in systemic sclerosis: single-centre experience of three cases

Cardiac transplantation in systemic sclerosis: single-centre experience of three cases Rheumatology key message Heart transplantation for SSc-related end-stage heart failure is feasible and can be considered in selected patients. Sir, we present our monocentric experience with three SSc patients who underwent cardiac transplantation for SSc-related end-stage heart failure. SSc is a rare, severe chronic CTD, characterized by widespread vasculopathy and fibrosis of skin and internal organs. End-stage heart failure is uncommon and carries a poor prognosis. Unlike pulmonary transplantation, published evidence on cardiac transplantation in the context of SSc is scarce [1–8]. The first male patient was diagnosed at the age of 50 years with ANA-negative lcSSc with severe cardiac involvement (New York Heart Association class IV). Transthoracic echocardiography showed biventricular dilatation and a left ventricular ejection fraction (LVEF) of 20%. Late gadolinium-enhancement cardiac magnetic resonance imaging (LGE-CMR) showed endo- and subepicardial hyperenhancement of the lateral wall and apex of the left ventricle (LV), the entire free wall and septal side of the right ventricle (RV). It was transmural (75–100% of wall thickness) at the apex, and 50% elsewhere. Despite optimal medical treatment the clinical picture deteriorated. Five months after initial presentation, he received an orthotopic cardiac transplantation. The early post-transplant period was complicated by right heart failure, acute kidney failure (no features suggestive of scleroderma renal crisis, no biopsy performed) necessitating temporary dialysis, atrial fibrillation and a right upper lobe lung bleeding, which all recovered. The second male patient was diagnosed with ANA-positive (anti-RNA polymerase III) lcSSc at the age of 49 years with active myositis and persistent atrial fibrillation. The myositis was successfully treated with MTX and low-dose methylprednisolone. Following two unsuccessful ablations, the atrial fibrillation was treated with amiodarone and warfarin. Over the following years, the symptoms worsened (New York Heart Association III–IV) with echocardiographic increase of biventricular dilatation and hypo- and akinesia (LVEF 40%). He developed liver cirrhosis and ascites secondary to right heart failure, complicated by spontaneous bacterial peritonitis and sepsis. LGE-CMR showed transmural (75–100%) hyperenhancement of the RV and subendocardial (75%) to transmural hyperenhancement of the infero-lateral wall of the LV with pericardial inflammation. Five years after SSc diagnosis, he received an orthotopic cardiac transplantation. Three months later he was hospitalized with diarrhoea due to MMF (which was discontinued) and abnormal fatigue due to tertiary adrenal insufficiency, for which hydrocortisone was associated. The diarrhoea resolved and MMF was restarted. The third male patient was diagnosed with ANA-positive (anti-PM-Scl-100) diffuse cutaneous SSc at the age of 47 years. He had been in follow-up for progressive biventricular heart failure with unidentified aetiology for 1 year prior. Transthoracic echocardiography showed biventricular dilatation and LVEF of 40%. LGE-CMR showed diffuse subendocardial (75%) to transmural (75–100%) hyperenhancement in basal and mid-wall regions of the LV and hyperenhancement in the inferior wall of RV. Because of episodes of sustained monomorphic ventricular tachycardia, an implantable cardioverter defibrillator was implanted. Despite optimal medical treatment, there was a rapid decline. Twenty-seven months after diagnosis, he received an orthotopic cardiac transplantation because of inotropic-dependent heart failure and secondary kidney failure. The post-operative period was complicated by right heart failure and acute chronic kidney failure (no features of scleroderma renal crisis, no biopsy performed) in need of temporary dialysis. None of our patients had major other SSc-related organ involvement at the time of transplant (Table 1). Extensive invasive evaluation confirmed the absence of pulmonary arterial hypertension, interstitial lung disease or significant gastrointestinal involvement (Table 1). In all patients, post-transplantation immunosuppression consisted of tacrolimus, MMF and high-dose methylprednisolone, tapered to zero over the course of months. At present, respectively, 109, 23 and 23 months after transplantation, all patients are functioning well (Table 1). No SSc-related complications occurred. Table 1 Cardiac transplantation in three SSc patients: SSc-related organ involvement and cardiac function pre- and post-transplant Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 ILD: interstitial lung disease; PAH: pulmonary arterial hypertension; GIT: gastrointestinal tract; NYHA: New York Heart Association; Hs Troponin T: high sensitive troponin T (normal range: ≤0.013 µg/l); NT-proBNP: N-terminal pro-brain natriuretic peptide (normal range ≤172 ng/l); LVEF: left ventricular ejection fraction; TTE: transthoracic echocardiography; RHC: right heart catheterization; mPAP: mean pulmonary arterial pressure; PCWP: pulmonary capillary wedge pressure; AVBII: second degree atrioventricular block; NA: not available; 6MWT: 6-min walk test; StO2: oxygen saturation; AES: atrial extrasystole; VES: ventricular extrasystole. Table 1 Cardiac transplantation in three SSc patients: SSc-related organ involvement and cardiac function pre- and post-transplant Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 ILD: interstitial lung disease; PAH: pulmonary arterial hypertension; GIT: gastrointestinal tract; NYHA: New York Heart Association; Hs Troponin T: high sensitive troponin T (normal range: ≤0.013 µg/l); NT-proBNP: N-terminal pro-brain natriuretic peptide (normal range ≤172 ng/l); LVEF: left ventricular ejection fraction; TTE: transthoracic echocardiography; RHC: right heart catheterization; mPAP: mean pulmonary arterial pressure; PCWP: pulmonary capillary wedge pressure; AVBII: second degree atrioventricular block; NA: not available; 6MWT: 6-min walk test; StO2: oxygen saturation; AES: atrial extrasystole; VES: ventricular extrasystole. To date, only 15 cases of cardiac transplantation in SSc-related heart involvement have been described, of which only 5 have been published in peer-reviewed journals [1–8]. The largest group of eight patients, part of the French national registry, were reported by Epailly et al. as conference abstracts [6]. In total, nine women and four men have been described (two patients without gender specification). Mean age at time of transplantation was 29 years (range 13–57 years, age reported in 7/15 patients). Seven patients suffered from dcSSc, four from lcSSc, one from RA-SSc overlap syndrome and disease subtype was unreported in three. ANA status was positive in eight, negative in two and unreported in five. None of the patients had interstitial lung disease or pulmonary arterial hypertension (reported in 11/15 patients). Four of 12 patients died during follow-up: three patients died within 15 days after transplantation, one patient died suddenly at home after 27 months. The outcome of three remaining patients is unknown. Published evidence on the feasibility of heart transplantation in SSc patients remains limited. Our monocentric experience in three patients expands the limited published data indicating that cardiac transplantation is feasible and can be considered in selected patients. Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript. Disclosure statement: The authors have declared no conflicts of interest. References 1 Bennasar G , Carlevaris L , Secco A , Romanini F , Mamani M. Cardiac transplant in young female patient diagnosed with diffuse systemic sclerosis . Reumatol Clin 2016 ; 12 : 285 – 7 . Google Scholar CrossRef Search ADS PubMed 2 Quartier P , Bonnet D , Fournet JC et al. Severe cardiac involvement in children with systemic sclerosis and myositis . J Rheumatol 2002 ; 29 : 1767 – 73 . Google Scholar PubMed 3 Martens E , Lange P , Pohl T et al. Heart transplantation in a 36-year-old experiencing terminal heart failure caused by systemic sclerosis . Transplantation 2012 ; 94 : 13 – 5 . Google Scholar CrossRef Search ADS 4 Johnson JN , Kvistad BS , Reed AM et al. Cardiac transplantation in a pediatric patient with systemic sclerosis . Congenit Heart Dis 2013 ; 8 : 1 – 4 . Google Scholar CrossRef Search ADS PubMed 5 Yabuno J , Patel J , Kittleson M et al. Patients with auto-immune disease: not a contraindication for heart transplantation . Circulation 2015 ; 132 : 13688 . 6 Epailly E , Ikic A , Chatelus E et al. Heart transplantation in 8 patients with systemic sclerosis: results of a French national registry . Transpl Int 2015 ; 28 : 267 . 7 Goena VC , Quintas OL , Lluis SI et al. Heart involvement in systemic sclerosis: recognizing etiology and therapeutic options. A case report . Eur Heart J Acute Cardiovasc Care 2015 ; 4 : 235 . 8 Siegert E , Riemekasten G. Are we too lenient with immunosuppression in severe cases of systemic sclerosis . Rheumatology (Oxford) 2016 ; 55 : 1914 – 6 . Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Rheumatology Oxford University Press

Cardiac transplantation in systemic sclerosis: single-centre experience of three cases

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© The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com
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Abstract

Rheumatology key message Heart transplantation for SSc-related end-stage heart failure is feasible and can be considered in selected patients. Sir, we present our monocentric experience with three SSc patients who underwent cardiac transplantation for SSc-related end-stage heart failure. SSc is a rare, severe chronic CTD, characterized by widespread vasculopathy and fibrosis of skin and internal organs. End-stage heart failure is uncommon and carries a poor prognosis. Unlike pulmonary transplantation, published evidence on cardiac transplantation in the context of SSc is scarce [1–8]. The first male patient was diagnosed at the age of 50 years with ANA-negative lcSSc with severe cardiac involvement (New York Heart Association class IV). Transthoracic echocardiography showed biventricular dilatation and a left ventricular ejection fraction (LVEF) of 20%. Late gadolinium-enhancement cardiac magnetic resonance imaging (LGE-CMR) showed endo- and subepicardial hyperenhancement of the lateral wall and apex of the left ventricle (LV), the entire free wall and septal side of the right ventricle (RV). It was transmural (75–100% of wall thickness) at the apex, and 50% elsewhere. Despite optimal medical treatment the clinical picture deteriorated. Five months after initial presentation, he received an orthotopic cardiac transplantation. The early post-transplant period was complicated by right heart failure, acute kidney failure (no features suggestive of scleroderma renal crisis, no biopsy performed) necessitating temporary dialysis, atrial fibrillation and a right upper lobe lung bleeding, which all recovered. The second male patient was diagnosed with ANA-positive (anti-RNA polymerase III) lcSSc at the age of 49 years with active myositis and persistent atrial fibrillation. The myositis was successfully treated with MTX and low-dose methylprednisolone. Following two unsuccessful ablations, the atrial fibrillation was treated with amiodarone and warfarin. Over the following years, the symptoms worsened (New York Heart Association III–IV) with echocardiographic increase of biventricular dilatation and hypo- and akinesia (LVEF 40%). He developed liver cirrhosis and ascites secondary to right heart failure, complicated by spontaneous bacterial peritonitis and sepsis. LGE-CMR showed transmural (75–100%) hyperenhancement of the RV and subendocardial (75%) to transmural hyperenhancement of the infero-lateral wall of the LV with pericardial inflammation. Five years after SSc diagnosis, he received an orthotopic cardiac transplantation. Three months later he was hospitalized with diarrhoea due to MMF (which was discontinued) and abnormal fatigue due to tertiary adrenal insufficiency, for which hydrocortisone was associated. The diarrhoea resolved and MMF was restarted. The third male patient was diagnosed with ANA-positive (anti-PM-Scl-100) diffuse cutaneous SSc at the age of 47 years. He had been in follow-up for progressive biventricular heart failure with unidentified aetiology for 1 year prior. Transthoracic echocardiography showed biventricular dilatation and LVEF of 40%. LGE-CMR showed diffuse subendocardial (75%) to transmural (75–100%) hyperenhancement in basal and mid-wall regions of the LV and hyperenhancement in the inferior wall of RV. Because of episodes of sustained monomorphic ventricular tachycardia, an implantable cardioverter defibrillator was implanted. Despite optimal medical treatment, there was a rapid decline. Twenty-seven months after diagnosis, he received an orthotopic cardiac transplantation because of inotropic-dependent heart failure and secondary kidney failure. The post-operative period was complicated by right heart failure and acute chronic kidney failure (no features of scleroderma renal crisis, no biopsy performed) in need of temporary dialysis. None of our patients had major other SSc-related organ involvement at the time of transplant (Table 1). Extensive invasive evaluation confirmed the absence of pulmonary arterial hypertension, interstitial lung disease or significant gastrointestinal involvement (Table 1). In all patients, post-transplantation immunosuppression consisted of tacrolimus, MMF and high-dose methylprednisolone, tapered to zero over the course of months. At present, respectively, 109, 23 and 23 months after transplantation, all patients are functioning well (Table 1). No SSc-related complications occurred. Table 1 Cardiac transplantation in three SSc patients: SSc-related organ involvement and cardiac function pre- and post-transplant Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 ILD: interstitial lung disease; PAH: pulmonary arterial hypertension; GIT: gastrointestinal tract; NYHA: New York Heart Association; Hs Troponin T: high sensitive troponin T (normal range: ≤0.013 µg/l); NT-proBNP: N-terminal pro-brain natriuretic peptide (normal range ≤172 ng/l); LVEF: left ventricular ejection fraction; TTE: transthoracic echocardiography; RHC: right heart catheterization; mPAP: mean pulmonary arterial pressure; PCWP: pulmonary capillary wedge pressure; AVBII: second degree atrioventricular block; NA: not available; 6MWT: 6-min walk test; StO2: oxygen saturation; AES: atrial extrasystole; VES: ventricular extrasystole. Table 1 Cardiac transplantation in three SSc patients: SSc-related organ involvement and cardiac function pre- and post-transplant Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 Case 1 Case 2 Case 3 SSc-related organ involvement     ANA status Negative Anti-RNA polymerase III (RP11) Anti-PM-Scl-100     Musculoskeletal No Myositis (not at time of transplant) No     Renal (scleroderma renal crisis) No No No     Pulmonary (ILD or PAH) No No No     GIT No major involvement Gastric antral vascular ectasia (no bleeding at time of transplant) Upper GIT dysmotility  Complications secondary to heart failure / Liver cirrhosis Acute kidney failure due to cardio-renal conflict Ascites Spontaneous bacterial peritonitis Pre-transplant     NYHA class IV III III     Hs Troponin T, µg/l 0.012 0.056 0.036     NT-proBNP, ng/l 4963 5571 2510     LVEF, assessed by TTE, % 20 40 40     RHC: mPAP, mmHg 26 20 31     RHC: PCWP, mmHg 20 14 28     Holter Atrial flutter AVBII NA     6MWT:         % predicted distance 50 77 31         StO2 before–after, % 95–82 99–NA 98–96 Post-transplant     NYHA class I I I     Hs Troponin T, µg/l 0.006 0.035 0.014     NT-proBNP, ng/l NA 55 208     LVEF, assessed by TTE 60 55 60     RHC: mPAP, mmHg 19 13 21     RHC: PCWP, mmHg 12 8 12     Holter AES/VES AES AES/VES     6MWT         % predicted distance 79 NA 81         StO2 before–after, % 97–87 98–96 ILD: interstitial lung disease; PAH: pulmonary arterial hypertension; GIT: gastrointestinal tract; NYHA: New York Heart Association; Hs Troponin T: high sensitive troponin T (normal range: ≤0.013 µg/l); NT-proBNP: N-terminal pro-brain natriuretic peptide (normal range ≤172 ng/l); LVEF: left ventricular ejection fraction; TTE: transthoracic echocardiography; RHC: right heart catheterization; mPAP: mean pulmonary arterial pressure; PCWP: pulmonary capillary wedge pressure; AVBII: second degree atrioventricular block; NA: not available; 6MWT: 6-min walk test; StO2: oxygen saturation; AES: atrial extrasystole; VES: ventricular extrasystole. To date, only 15 cases of cardiac transplantation in SSc-related heart involvement have been described, of which only 5 have been published in peer-reviewed journals [1–8]. The largest group of eight patients, part of the French national registry, were reported by Epailly et al. as conference abstracts [6]. In total, nine women and four men have been described (two patients without gender specification). Mean age at time of transplantation was 29 years (range 13–57 years, age reported in 7/15 patients). Seven patients suffered from dcSSc, four from lcSSc, one from RA-SSc overlap syndrome and disease subtype was unreported in three. ANA status was positive in eight, negative in two and unreported in five. None of the patients had interstitial lung disease or pulmonary arterial hypertension (reported in 11/15 patients). Four of 12 patients died during follow-up: three patients died within 15 days after transplantation, one patient died suddenly at home after 27 months. The outcome of three remaining patients is unknown. Published evidence on the feasibility of heart transplantation in SSc patients remains limited. Our monocentric experience in three patients expands the limited published data indicating that cardiac transplantation is feasible and can be considered in selected patients. Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript. Disclosure statement: The authors have declared no conflicts of interest. References 1 Bennasar G , Carlevaris L , Secco A , Romanini F , Mamani M. Cardiac transplant in young female patient diagnosed with diffuse systemic sclerosis . Reumatol Clin 2016 ; 12 : 285 – 7 . Google Scholar CrossRef Search ADS PubMed 2 Quartier P , Bonnet D , Fournet JC et al. Severe cardiac involvement in children with systemic sclerosis and myositis . J Rheumatol 2002 ; 29 : 1767 – 73 . Google Scholar PubMed 3 Martens E , Lange P , Pohl T et al. Heart transplantation in a 36-year-old experiencing terminal heart failure caused by systemic sclerosis . Transplantation 2012 ; 94 : 13 – 5 . Google Scholar CrossRef Search ADS 4 Johnson JN , Kvistad BS , Reed AM et al. Cardiac transplantation in a pediatric patient with systemic sclerosis . Congenit Heart Dis 2013 ; 8 : 1 – 4 . Google Scholar CrossRef Search ADS PubMed 5 Yabuno J , Patel J , Kittleson M et al. Patients with auto-immune disease: not a contraindication for heart transplantation . Circulation 2015 ; 132 : 13688 . 6 Epailly E , Ikic A , Chatelus E et al. Heart transplantation in 8 patients with systemic sclerosis: results of a French national registry . Transpl Int 2015 ; 28 : 267 . 7 Goena VC , Quintas OL , Lluis SI et al. Heart involvement in systemic sclerosis: recognizing etiology and therapeutic options. A case report . Eur Heart J Acute Cardiovasc Care 2015 ; 4 : 235 . 8 Siegert E , Riemekasten G. Are we too lenient with immunosuppression in severe cases of systemic sclerosis . Rheumatology (Oxford) 2016 ; 55 : 1914 – 6 . Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

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RheumatologyOxford University Press

Published: Mar 7, 2018

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