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Pathology International 2015; 65: 619–621

doi:10.1111/pin.12313

Lettter to the Editor Cardiac sarcoidosis predominantly involved in right ventricle: An autopsy case To the Editor: Sarcoidosis is a systemic granulomatous disease of unknown etiology, and noncaseating granulomas are the pathological hallmark of this condition. Lung and lymph node are often involved by epithelioid granuloma with multinucleated giant cells. Although the overall fatal ratio of sarcoidosis is 1% to 5%, prognosis of sarcoid patients depends on the cardiac manifestation of this disease.1 In Japan, cardiac involvement is more common in older women and responsible for as many as 58% to 85% of deaths from sarcoidosis.2 Conduction abnormality are common in cardiac sarcoidosis (CS), ranging in prevalence from 12% to 62% in the study series. Ventricular tachycardia are usually caused by direct granulomatous involvement of the myocardium and the prevalence ranges from 2% to 42%. Congestive heart failure is known to be a complication of CS of about 20%. Sudden death, ranging in prevalence from 12% to 65% of CS patients, due to either ventricular arrhythmia or complete heart block, is the most feared manifestation of CS. On the one hand, there are patients whose clinical manifestation is isolated to the heart.3 The diagnosis of ‘isolated’ CS is difficult to make due to the restricted clinical manifestations in the heart. The ventricular septum and left ventricular free wall are frequently affected, and predominant involvement of sarcoidosis occurring in the right ventricle (RV) is rare.4 The present case manifested RV dominant biventricular heart failure and an autopsy revealed sarcoid scarring, mainly in the RV of heart. A 65-year-old man was admitted to the emergency room in our hospital because of loss of consciousness. His medical records revealed history of VDD pacemaker implantation due to complete atrioventricular block in another institute 8 years before. Previously, he was admitted to our hospital three times because of RV-dominant biventricular heart failure. During his second admission to our hospital 17 months pre-

viously, two pieces of endomyocardial biopsy specimens were obtained from the RV and one biopsy specimen demonstrated a non-necrotizing, tight, naked epithelioid granuloma with giant cell infiltration (Fig. 1a). Although hypertrophic myocardium with interstitial fibrosis was apparent, scar tissue was not identified in these biopsy tissues. High-power view of the granuloma showed an asteroid body in the multinucleated giant cell (Fig. 1b). Despite extensive clinical examination of whole body, no clinical evidence of sarcoidosis in the other organs was revealed. He was diagnosed as ‘isolated’ CS. The angiotensin converting enzyme (ACE) level was not analyzed, because he had been prescribed ACE inhibitor for his clinical manifestation of heart failure before the diagnosis of sarcoidosis. He did not undergo the cardiac magnetic resonance imaging because of the pacemaker implantation. He was re-hospitalized due to congestive heart failure 3 months before. The maximum value of brain natriuretic peptide (BNP) was 754 pg/mL at the time of his admission. He was prescribed predonisone 30 mg per day in addition to the diuretics and catecholamine. The symptoms of heart failure improved and he was discharged after 46 days of treatment. The dose of predonisone was tapered to 20 mg per day as an outpatient. The BNP value was shown to be between 350 and 500 pg/mL during the follow up period. Electrocardiogram demonstrated a pacemaker rhythm with sporadic premature ventricular contraction originating from the RV. No clinical symptoms, such as palpitation, chest discomfort or syncope were recorded. He was discovered to have fallen down the stairs of his apartment with loss of consciousness and was conveyed to our hospital. He regained consciousness after a passer-by called to him. Electrocardiogram showed RV paced rhythm. Examination of brain computed tomography revealed no evidence of intracranial bleeding. Complete blood count identified anemia (haemoglobin, 8.3 g/dL) and low blood pressure was sustained. Unfortunately, he died on the day of his last admission in spite of complete and adequate resuscitation efforts. An autopsy revealed liver contusion with massive

Figure 1 Photomicrograph of endomyocardial biopsy specimen 17 months before death (hematoxylin-eosin). (a) Endomyocardial biopsy of right ventricle illustrated epithelioid granuloma with multinucleated giant cell infiltration (scale bar, 200 μm). (b) High-power view of giant cell demonstrated presence of asteroid body (arrow; scale bar, 20 μm). © 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd

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Lettter to the Editor

Figure 2 Macroscopic and microscopic appearance of cardiac sarcoidosis predominantly involved in right ventricle. (a) Gross pathology of heart revealed scar tissue mainly involved in the right ventricle. (b) Enlarged right ventricle with mural thrombi (arrow) was identified. (c) Most of the myocardium was replaced with fibrous tissue (RV indicates right ventricle). Organized thrombi with fibrin adhered to the right ventricular wall (arrows, Th indicates thrombus). (c. Masson’s trichrome, scale bar, 1 mm).

intraperitoneal hemorrhage. We diagnosed the cause of death as hemorrhagic shock due to liver contusion. The heart weighed 470 g and gross pathology showed that the majority of the RV myocardium was replaced with scar tissue (Fig. 2a). Mural thrombus was found adhered to the RV despite anticoagulant therapy by warfarin, and subepicardial portions of the anterior and lateral wall in the left ventricle with the papillary muscle of the mitral valve were partially involved (Fig. 2b). Pulmonary thromboembolism was not identified by either macroscopic or microscopic examination. Histopathology revealed that most of the RV wall demonstrated matured fibrosis without any granulomatous inflammation. Organized thrombi adhered to the RV wall (Fig. 2c). The basal interventricular septum became thin and fibrous scar tissue was observed at the atrioventricular node. Although bilateral hilar lymphadenopathy was not apparent by gross pathology, histology identified hyalinization of the lymph nodes, which is consistent with burned-out granuloma. There was no histological evidence of sarcoidosis including scar stage of the disease in the other organs, namely lung, liver and spleen. We speculate that the lethal arrhythmia due to massive involvement of scar sarcoidosis in the RV might have induced the loss of consciousness. We herein described a case of CS mainly involved in the RV of heart. Endomyocardial biopsy of the RV 17 months before death demonstrated granuloma with giant cell infiltration. An autopsy revealed that gross scars were predominantly distributed within the RV. Although clinical manifestation was restricted to the heart, hyalinization of the bilateral hilar lymph nodes was identified, which is consistent with burned-out granuloma due to sarcoidosis. The present case demonstrated circumferential involvement of the RV with near-total loss of myocardium. There was no histological evidence of active inflammation with granuloma in the heart by autopsy. We diagnosed CS mainly involved in the RV. Several cases of predominantly RV involvement in CS, sometimes mimicking arrhythmogenic right ventricular cardiomyopathy (ARVC), have been reported previously.5–8 A

prospective study of 20 patients with suspected ARVC indicated that 3 (15%) patients were diagnosed with CS.9 Although this clinical evidence suggests the existence of CS cases showing predominant RV involvement, there are few reports clearly demonstrating the macroscopic appearance of such a heart. As far as we searched the previous literature, this is the first case report presenting the gross morphology of CS predominantly involved in RV. Although the clinical manifestation of sarcoidosis was restricted to the heart and ‘isolated’ CS was diagnosed, an autopsy revealed the hyalinization of hilar lymph nodes, which is consistent with burned-out granuloma.

DISCLOSURE STATEMENT None declared. Noriko Kajimoto,1 Hiroyuki Hao,1 Rika Kawakami,1 Yaemi Takagi,1 Akiko Fujino,1 Masataka Sugahara,2 Tohru Masuyama2 and Seiichi Hirota1 1 Department of Surgical Pathology and 2Division of Cardiology, Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Japan

REFERENCES 1 Lagana SM, Parwani AV, Nichols LC. Cardiac sarcoidosis: A pathology-focused review. Arch Pathol Lab Med 2010; 134: 1039–46. 2 Iwai K, Tachibana T, Takemura T, Matsui Y, Kitaichi M, Kawabata Y. Pathological studies on sarcoidosis autopsy. I. Epidemiological features of 320 cases in Japan. Acta Pathol Jpn 1993; 43: 372–6. 3 Isobe M, Tezuka D. Isolated cardiac sarcoidosis: Clinical characteristics, diagnosis and treatment. Int J Cardiol 2014; 182C: 132– 40. 4 Tavora F, Cresswell N, Li L, Ripple M, Solomon C, Burke A. Comparison of necropsy findings in patients with sarcoidosis dying suddenly from cardiac sarcoidosis versus dying suddenly from other causes. Am J Cardiol 2009; 104: 571–7.

© 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd

Lettter to the Editor

5 Noda T, Suyama K, Shimizu W et al. Ventricular tachycardia with figure eight pattern originating from the right ventricle in a patient with cardiac sarcoidosis. Pacing Clin Electrophysiol 2004; 27: 561–2. 6 Shiraishi J, Tatsumi T, Shimoo K et al. Cardiac sarcoidosis mimicking right ventricular dysplasia. Circ J 2003; 67: 169– 71. 7 Ott P, Marcus FI, Sobonya RE, Morady F, Knight BP, Fuenzalida CE. Cardiac sarcoidosis masquerading as right

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ventricular dysplasia. Pacing Clin Electrophysiol 2003; 26: 1498– 503. 8 Philips B, Madhavan S, James CA et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: Distinguishing features when the diagnosis is unclear. Circ Arrhythm Electrophysiol 2014; 7: 230–36. 9 Vasaiwala SC, Finn C, Delpriore J et al. Prospective study of cardiac sarcoid mimicking arrhythmogenic right ventricular dysplasia. J Cardiovasc Electrophysiol 2009; 20: 473–6.

© 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd

Cardiac sarcoidosis predominantly involved in right ventricle: An autopsy case.

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