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The Two Extremes of Cardiac Sarcoidosis and the Effect of Prednisone Therapy Danielle Armstrong, DOa, Gonzalo V. Gonzalez-Stawinski, MDb, Jong Mi Ko, BAc, Shelley A. Hall, MDd, and William C. Roberts, MDa,c,d,* Described herein are clinical and morphologic findings in 2 patients who underwent heart transplantation because of severe heart failure resulting from cardiac sarcoidosis. Although the explanted hearts in each patient had characteristic gross changes of cardiac sarcoidosis, one patient who had been treated with prednisone, had no residual sarcoid granulomas in the myocardium, whereas the other patient, in whom diagnosis was not made until heart transplantation, had innumerable sarcoid granulomas in her heart. This report suggests that prednisone can eliminate sarcoid granulomas in the heart but that their replacement is by dense fibrous tissue, something also likely the result of the granulomas themselves, creating a situation where the treated (prednisone) and the non-treated sarcoid heart may appear similar by gross examination. Ó 2015 Elsevier Inc. All rights reserved. (Am J Cardiol 2015;115:150e153) Although it is a systemic disease, sarcoidosis uncommonly affects the heart and when it does the non-cardiac organs are usually minimally affected and they function normally.1e6 On occasion, cardiac sarcoidosis may lead to such severe heart failure that heart transplantation (HT) is the only reasonable therapy.3e5 In such situations, diagnosis of cardiac sarcoidosis is usually not made until HT, the usual pre-HT diagnosis being “non-ischemic cardiomyopathy”.5 Recently, 2 patients underwent HT at Baylor University Medical Center (BUMC) in the same month and examination of the explanted heart in one disclosed innumerable myocardial sarcoid granulomas (not diagnosed clinically) and, in the other patient, despite some similar gross findings in the heart, no myocardial granulomas were seen (diagnosed clinically by an earlier biopsy). This report describes findings in these 2 patients to point out the huge spectrum of myocardial changes in patients with cardiac sarcoidosis, and the potential effect of long term prednisone therapy in this condition. Patients Studied Pertinent findings in each of the 2 patients are detailed in Table 1. Both patients were in their 50s; both were white; both had severe (4þ/4þ) heart failure; both had evidence of cardiac dysfunction for approximately 4 years; both had periodic runs of non-sustained ventricular tachycardia; both had some type of heart block (complete in case #1, and right bundle branch block in case #2); neither had dysfunction of a non-cardiac body organ, and neither patient had narrowing a b d Departments of Pathology, Cardiothoracic Surgery, and Internal Medicine (Division of Cardiology), Baylor University Medical Center, Dallas, Texas and cBaylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas. Manuscript received September 2, 2014; revised manuscript received and accepted October 5, 2014. Support for this investigation was provided by the Baylor Health Care System Foundation. See page 153 for disclosure information. *Corresponding author: Tel: 214-820-7911; fax: 214-820-7533. E-mail address: [email protected] (W.C. Roberts). 0002-9149/14/$ - see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjcard.2014.10.003 of the epicardial coronary arteries. Diagnosis of sarcoidosis before HT was made in patient #1 by biopsy of a mediastinal lymph node 21 months before HT although 2 years earlier a cardiac biopsy had shown “granulomas”. Patient #1, who had no granulomas in his explanted heart, was placed on prednisone after the granulomas were seen in the lymph node and he received this drug for 21 months, at very high doses for 10 months before dose-tapering Table 1 Clinical and morphologic findings in the 2 patients Variable Gender Age (years) At heart transplantation Onset of symptoms Electrophysiology Heart block Ventricular tachycardia Pacemaker Intracardiac defibrillator Systemic hypertension Body mass index (Kg/m2) Hemodynamic data Lowest left ventricular EF (%) Cardiac index (L/min/m2) Pressures (mm Hg) Blood pressure, indirect (s/d) Right atrial mean Right ventricle (s/d) Pulmonary artery (s/d) Pulmonary artery wedge mean Prednisone therapy (months) Heart weight (g) Cardiac granuloma (0-4þ) Left ventricular cavity (cm) Case #1 #2 Man Woman 53 49 57 53 Complete þ þ þ þ 31.8 RBBB þ 0 þ 0 29.9 20 2.01 15 1.36 100/85 6 26/10 26/14 11 þ (21) 535 0 7.5 85/40 2 48/6 44/11 14 0 380 4þ 5.5 EF ¼ ejection fraction; RBBB ¼ right bundle branch block; s/d ¼ peak systole/end diastole. www.ajconline.org Case Report/Cardiac Sarcoidosis With and Without Granulomas 151 Figure 1. Heart in patient #1. (A) View of the base of the heart showing marked dilatation of both right and left ventricular cavities and focal scars in the left and right ventricular free walls and ventricular septum. (B) Transverse cuts of the cardiac ventricles caudal to the view shown in (A). (C) Close up view of the wall of right ventricle showing extensive scarring and marked thinning of the wall (simulating arrhythmogenic right ventricular dysplasia/cardiomyopathy). (D) View of the ventricular septum showing the scarring primarily on the right side of the septum. Because the biotome to biopsy the heart extracts tissue from the right side of the ventricular septum that probably was the reason the biopsy was positive in this patient early in his course. 152 The American Journal of Cardiology (www.ajconline.org) Figure 2. Photomicrographs of portions of the heart in patient #1. (A) View of the epicardium, subepicardium, and outer myocardial wall in the anterior portion of left ventricle. The left anterior descending coronary artery and also a coronary vein are shown. The deep blue color represents scar tissue which is located primarily in the subepicardial region rather than the sub-endocardial region. No granulomas were found in the dense fibrous tissue despite examining numerous sections of myocardium. The coronary vessels are surrounded by mainly adipose tissue. (B) View of the right ventricular free wall also showing extensive scarring again involving the subepicardial portion more than the subendocardial portion. Trichrome stains, each 20. began. Patient #2, whose sarcoidosis was not diagnosed until HT, never received corticosteroid therapy before HT and her explanted heart contained innumerable granulomas typical of sarcoidosis. Photographs of the heart in each patient are shown in Figures 1 to 4. Discussion The explanted hearts in each of the 2 hitherto described patients had gross features characteristic of cardiac sarcoidosis: involvement of the walls of both right and left ventricles and the ventricular septum; multiple lesions in each of the cardiac walls; a tendency for the gross lesions to involve the subepicardial half of the left ventricular free wall and the right ventricular half of the ventricular septum, dilation of both ventricular cavities, and absence of narrowing of the epicardial coronary arteries. Yet one patient, who previously by Figure 3. Views of the heart in case #2. (A) View of the opened basal portion of the heart showing considerable dilatation of both ventricular cavities and severe scarring of the ventricular septum, posterolateral left ventricular free wall, and posterior wall of right ventricle. Numerous sarcoid granulomas were present in the dense fibrous tissue. (B) One-cm thick slices of the ventricles caudal to the upper view. Much of the left ventricular free wall, ventricular septum and right ventricular free wall are replaced by sarcoid granulomas with their surrounding fibrous tissue. both cardiac and lymph node biopsies showed sarcoidosis, had no granulomas in his explanted heart; the other patient had innumerable sarcoid granulomas in her heart and diagnosis of sarcoidosis was not made until examination of the explanted heart. Patient #1 who had no granulomas in his explanted heart had received prednisone therapy for 21 months, high doses during 10 of those months. The quantity of scar tissue in both patients’ hearts suggests that the sarcoid granulomas and their adjacent lymphocytes are strong fibrous-tissue stimulants, and, moreover, that prednisone therapy is a strong eliminator of sarcoid granulomas but the residue of that healing is extensive replacement fibrosis. (Sarcoidosis in the lung is also a strong fibrosis stimulator.7) It seems reasonable to believe that the heart in patient #1 earlier had been loaded with sarcoid granulomas as found in patient #2 but that the prednisone treatment likely played a role in eliminating the sarcoid granuloma by replacing them with dense fibrous Case Report/Cardiac Sarcoidosis With and Without Granulomas 153 Figure 4. Photomicrographs in patient #2. (A) View of a portion of left ventricular free wall showing numerous granulomas surrounded by dense fibrous tissue. (B) Close-up view of a single sarcoid granuloma. (C) Another view of sarcoid granulomas showing numerous nuclei in each of the giant cells. (D) Infiltration between myocardial fibers of lymphocytes typical of sarcoid myocarditis. Trichrome stain, 40 (A); trichrome stain, 400 (B); hematoxylin/eosin stain, 400 (C), and hematoxylin/eosin stain, 400 (D). tissue. Such a demonstration to our knowledge has not been provided previous to this report. Earlier we described a patient with typical gross lesions of cardiac sarcoidosis but study of numerous sections of the heart histologically failed to reveal any granulomas.6 This earlier patient had never received corticosteroid therapy,6 but earlier histologic examination of a portion of the left ventricular free wall excised to insert a left ventricular assist device did disclose sarcoid granulomas. Disclosures The authors have no conflicts of interest to disclose. 1. Roberts WC, McAllister HA Jr, Ferrans VJ. Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (Group I) and review of 78 previously described necropsy patients (Group II). Am J Med 1977;63:86e108. 2. Virmani R, Bures JC, Roberts WC. Cardiac sarcoidosis: a major cause of sudden death in young individuals. Chest 1980;77:423e428. 3. Donsky AS, Escobar J, Capehart J, Roberts WC. Heart Transplantation for undiagnosed cardiac sarcoidosis. Am J Cardiol 2002;89:1447e1450. 4. Roberts WC, Vowels TJ, Ko JM, Capehard JE, Hall SA. Cardiac transplantation for cardiac sarcoidosis with initial diagnosis by examination of the left ventricular apical “core” excised for insertion of a left ventricular assist device for severe chronic heart failure. Am J Cardiol 2009;103:110e114. 5. Sharma PS, Lubahn JG, Donsky AS, Yoon AD, Carry MM, Grayburn PA, Wood PB, Ko JM, Burton EC, Roberts WC. Diagnosing cardiac sarcoidosis clinically without tissue confirmation. Proc (Bayl Univ Med Cent) 2009;22:236e238. 6. Roberts WC, Chung MS, Ko JM, Capehart JE, Hall SA. Morphologic features of cardiac sarcoidosis in native hearts of patients having cardiac transplantation. Am J Cardiol 2014;113:706e712. 7. Crystal RG, Roberts WC, Hunninghake GW, Gadek JE, Fulmer JD, Line BR. Pulmonary sarcoidosis: a disease characterized and perpetuated by activated lung T-lymphocytes. Ann Intern Med 1981;94: 73e94.