Heart transplantation for end-stage heart failure due to cardiac sarcoidosis.
01 Jul 2013-Vol. 45, Iss: 6, pp 2384-2386
TL;DR: Patients with cardiac sarcoidosis undergoing heart transplantation have acceptable long-term outcomes without evidence of recurrence of sarcoideosis in the allograft when maintained on low-dose corticosteroids.
Abstract: Background Cardiac sarcoidosis with end-stage heart failure has a poor prognosis without transplantation The rates of sarcoid recurrence and rejection are not well established after heart transplantation Methods A total of 19 heart transplant recipients with sarcoid of the explanted heart were compared with a contemporaneous control group of 1,050 heart transplant recipients without cardiac sarcoidosis Assessed outcomes included 1st-year freedom from any treated rejection, 5-year actuarial survival, 5-year freedom from cardiac allograft vasculopathy (CAV), 5-year freedom from nonfatal major adverse cardiac events (NF-MACE), and recurrence of sarcoid in the allograft or other organs Patients with sarcoidosis were maintained on low-dose corticosteroids after transplantation Results There were no significant differences between the sarcoid and control groups in 1st-year freedom from any treated rejection (79% and 90%), 5-year posttransplantation survival (79% and 83%), 5-year freedom from CAV (68% and 78%), and 5-year freedom from NF-MACE (90% and 88%) Causes of death (n = 5) in the sarcoid group were coccidioidomycosis, pneumonia, rejection, hemorrhage, and CAV No patient had recurrence of sarcoidosis in the cardiac allograft Three of 19 patients (16%) experienced recurrence of extracardiac sarcoid, with no mortality Conclusions Patients with cardiac sarcoidosis undergoing heart transplantation have acceptable long-term outcomes without evidence of recurrence of sarcoidosis in the allograft when maintained on low-dose corticosteroids Progression of extracardiac sarcoid was uncommon, possibly related to immunosuppression In patients with cardiac sarcoidosis, heart transplantation is a viable treatment modality
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Osaka Medical College1, Nippon Medical School2, Tokyo Medical and Dental University3, Kitasato University4, Osaka University5, Kyushu University6, Saitama Prefecture7, Kagawa University8, Aichi Medical University9, Fujita Health University10, Hokkaido University11, Kyorin University12, Hiroshima University13, Nara Medical University14, Jichi Medical University15, Tokyo Medical University16
TL;DR: This English document is a digest version of JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis reported at the Japanese Circulation Society Joint Working Groups perfomed in 2016.
Abstract: J-STAGE Advance Publication released online October 9, 2019 Mailing address: Scientific Committee of the Japanese Circulation Society, 18F Imperial Hotel Tower, 1-1-1 Uchisaiwai-cho, Chiyoda-ku, Tokyo 100-0011, Japan. E-mail: meeting@j-circ.or.jp This English document is a digest version of JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis reported at the Japanese Circulation Society Joint Working Groups perfomed in 2016. (Website: http://www.j-circ.or.jp/guideline/pdf/ JCS2016_terasaki_d.pdf). Refer to Appendix 1 for the detailes of members. Joint Working Groups: The Japanese Circulation Society, Japanese College of Cardiology, The Japanese Heart Failure Society, Japan Society of Sarcoidosis and Other Granulomatous Disorders, The Japanese Society of Nuclear Cardiology, Japanese Heart Rhythm Society, The Research Group of Idiopathic Cardiomyopathy, Health, Labour and Welfare Sciences Research Grants, Research on Rare and Intractable Diseases. ISSN-1346-9843 All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: cj@j-circ.or.jp JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis ― Digest Version ―
185 citations
TL;DR: The prognosis of cardiac sarCOidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high, and corticosteroids remain the mainstay of treatment.
Abstract: Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter-defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy.
112 citations
TL;DR: The detection of infiltrative cardiomyopathies, particularly primary and secondary forms of iron overload, as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via disease-specific therapies.
44 citations
TL;DR: In the United States, patients with RCM, CHD, or prior heart transplantation had a higher risk for death while awaiting OHT than patients with DCM, ICM, HCM, or valvular heart disease.
Abstract: Objectives The aim of this study was to identify differences in survival on the basis of type of heart disease while awaiting orthotopic heart transplantation (OHT) Background Patients with restrictive cardiomyopathy (RCM), congenital heart disease (CHD), or hypertrophic cardiomyopathy (HCM) may be at a disadvantage while awaiting OHT because they often are poor candidates for mechanical circulatory support and/or inotropes Methods The study included all adults in the Scientific Registry of Transplant Recipients database awaiting OHT from 2004 to 2014, and outcomes were evaluated on the basis of type of heart disease The primary endpoint was time to all-cause mortality, censored at last patient follow-up and time of transplantation Multivariate Cox proportional hazards modeling was performed to evaluate survival by type of cardiomyopathy Results There were 14,447 patients with DCM, 823 with RCM, 11,799 with ischemic cardiomyopathy (ICM), 602 with HCM, 964 with CHD, 584 with valvular disease, and 1,528 in the “other” category (including 1,216 for retransplantation) During median follow-up of 37 months, 4,943 patients died (1,253 women, 3,690 men) After adjusting for possible confounding variables including age, renal function, inotropes, mechanical ventilation, and mechanical circulatory support, the adjusted hazard ratios by diagnoses relative to DCM were 170 for RCM (95% confidence interval [CI]: 143 to 202), 110 for ICM (95% CI: 103 to 118), 123 for HCM (95% CI: 098 to 154), 130 for valvular disease (95% CI: 107 to 157), 137 for CHD (95% CI: 117 to 161), and 151 for “other” diagnoses (95% CI: 134 to 169) Sex was a significant modifier of mortality for ICM, RCM, and “other” diagnoses (p Conclusions In the United States, patients with RCM, CHD, or prior heart transplantation had a higher risk for death while awaiting OHT than patients with DCM, ICM, HCM, or valvular heart disease
43 citations
TL;DR: The clinical parameters and pathologic findings of cardiac sarcoidosis and the advanced imaging features and differential diagnostic challenges that must be considered for a successful diagnostic approach are described.
Abstract: Cardiac sarcoidosis is a rare but potentially fatal disorder with a nonspecific spectrum of clinical manifestations, including conduction disorders, congestive heart failure, ventricular arrhythmias, and sudden cardiac death. Although early treatment to improve morbidity and mortality is desirable, sensitive and accurate detection of cardiac sarcoidosis remains a challenge. Except for the histopathologic finding of noncaseating granulomas in an endomyocardial biopsy specimen, most diagnostic tests are limited and nonspecific at best. Therefore, the decision to initiate treatment is based largely on the patient's clinical symptoms and the course of the disease, rather than histologic confirmation. Successful recognition of cardiac sarcoidosis ultimately requires rigorous collaboration among a clinician, radiologist, and pathologist. Advanced imaging modalities, such as cardiac magnetic resonance imaging and positron emission tomography with fluorodeoxyglucose, have become increasingly useful in facilitating diagnosis and therapeutic monitoring, although limited prospective studies exist. This article describes the clinical parameters and pathologic findings of cardiac sarcoidosis and the advanced imaging features and differential diagnostic challenges that must be considered for a successful diagnostic approach. In addition, to improve the understanding of abnormalities detected with different imaging modalities, we suggest a unified terminology in describing radiologic findings related to cardiac sarcoidosis.
36 citations
References
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TL;DR: The severity of heart failure was one of the most significant independent predictors of mortality for cardiac sarcoidosis and starting corticosteroids before the occurrence of systolic dysfunction resulted in an excellent clinical outcome.
Abstract: Cardiac involvement is an important prognostic factor in sarcoidosis, but reliable indicators of mortality risk in cardiac sarcoidosis are unstudied in a large number of patients. To determine the significant predictors of mortality and to assess the efficacy of corticosteroids, we analyzed clinical findings, treatment, and prognosis in 95 Japanese patients with cardiac sarcoidosis. Twenty of these 95 patients had cardiac sarcoidosis proven by autopsy; none of these patients had received corticosteroids. We assessed 12 clinical variables as possible predictors of mortality by Cox proportional hazards model in 75 steroid-treated patients. During the mean follow-up of 68 months, 29 patients (73%) died of congestive heart failure and 11 (27%) experienced sudden death. Kaplan-Meier survival curves showed 5-year survival rates of 75% in the steroid-treated patients and of 89% in patients with a left ventricular ejection fraction > or = 50%, whereas there was only 10% 5-year survival rate in autopsy subjects. There was no significant difference in survival curves of patients treated with a high initial dose (> 30 mg) and a low initial dose (> or = 30 mg) of prednisone. Multivariate analysis identified New York Heart Association functional class (hazard ratio 7.72 per class I increase, p = 0.0008), left ventricular end-diastolic diameter (hazard ratio 2.60/10 mm increase, p = 0.02), and sustained ventricular tachycardia (hazard ratio 7.20, p = 0.03) as independent predictors of mortality. In conclusion, the severity of heart failure was one of the most significant independent predictors of mortality for cardiac sarcoidosis. Starting corticosteroids before the occurrence of systolic dysfunction resulted in an excellent clinical outcome. A high initial dose of prednisone may not be essential for treatment of cardiac sarcoidosis.
513 citations
TL;DR: The sarcoidosis patients had different clinical features, including female predominance, a high incidence of grave conduction disturbance and abnormal wall thickness, uneven wall motion abnormalities, and perfusion defects preferentially affecting the anteroseptal and apical regions, and poor prognosis compared with those with idiopathic dilated cardiomyopathy.
Abstract: In the present study, clinical findings of 15 patients with cardiac sarcoidosis presenting as dilated cardiomyopathy were compared with those of 30 consecutive patients with idiopathic dilated cardiomyopathy. The sarcoidosis patients had different clinical features, including female predominance, a high incidence of grave conduction disturbance and abnormal wall thickness, uneven wall motion abnormalities, and perfusion defects preferentially affecting the anteroseptal and apical regions, and poor prognosis compared with those with idiopathic dilated cardiomyopathy.
118 citations
TL;DR: Patients with sarcoidosis undergoing orthotopic heart transplant had better short- and intermediate-term survival than the majority of heart transplant recipients and should not disqualify potential transplant candidates.
Abstract: Background Cardiac transplantation has been utilized increasingly in patients with sarcoid cardiomyopathy. We sought to review outcomes in patients with sarcoidosis who had heart transplants and compared their outcomes with those of transplanted patients without sarcoidosis. Methods We retrospectively reviewed the United Network for Organ Sharing (UNOS) database to assess the survival of patients with sarcoidosis who were treated by heart transplantation. Results Over an 18-year period, 65 patients (40 men and 25 women) with sarcoidosis underwent orthotopic heart transplantation. There were 4 operative deaths and 12 late deaths at a mean follow-up of 40 months. One-year post-transplant survival was significantly better for sarcoid patients receiving orthotopic transplantation compared with contemporaneous patients receiving transplantation for all other diagnoses (87.7% vs 84.5%, p = 0.030). Conclusions Patients with sarcoidosis undergoing orthotopic heart transplant had better short- and intermediate-term survival than the majority of heart transplant recipients. The diagnosis of sarcoidosis should not disqualify potential transplant candidates.
90 citations
01 Jan 2005
TL;DR: The salient clinical features, diagnostic evaluation, treatment, and prognosis of cardiac sarcoidosis are discussed and a sensible management strategy is selected.
Abstract: Sarcoidosis is a systemic disease of unknown aetiology characterised by the formation of noncaseating epitheloid cell granulomas, which can occur in virtually any organ. Cardiac involvement is of critical importance, due to the poor prognosis if this organ manifestation is left undiagnosed and untreated. Early initiation of therapy seems to be associated with a good prognosis; therefore, the diagnosis of cardiac sarcoidosis should be made before irreversible damage has occurred. Nevertheless, the diagnosis of cardiac sarcoidosis still remains "an imperfect science, a hesitant art" [1].
66 citations
TL;DR: In this article, the salient clinical features, diagnostic evaluation, treatment, and prognosis of cardiac sarcoidosis are discussed, and a detailed review of the clinical features of myocardial sarcoideosis is presented.
Abstract: Cardiac sarcoidosis is a challenging diagnostic entity Manifestations range from an incidentally discovered, benign condition to fatal cardiomyopathy and arrhythmias In this review, we discuss the salient clinical features, diagnostic evaluation, treatment, and prognosis of cardiac sarcoidosis A definitive histologic diagnosis of myocardial sarcoidosis is difficult, and treatment may be necessary in some patients with suspected cardiac involvement even in the absence of histologic confirmation Optimal strategies to diagnose cardiac involvement have not been clarified Thallium (201) radionuclide scintigraphy has been most extensively studied; 2-D echocardiography is less sensitive, but may have a complementary role Additional radionuclide techniques (eg, gallium (67) or technetium (99) ) may have a role in complex cases Given the life-threatening nature of cardiac involvement, prompt and aggressive treatment with corticosteroids or immunosuppressive agents, or both, is warranted Long-term, possibly lifelong, therapy may be required Serious, recurrent tachyarrhythmias may require implantation of an automatic cardioverter-defibrillator Recognizing cardiac sarcoidosis and selecting a sensible management strategy remain the major obstacles facing clinicians
48 citations