Prognosis and Outcomes of Clinically Diagnosed Cardiac Sarcoidosis Without Positive Endomyocardial Biopsy Findings

Kengo Kusano; Kohei Ishibashi; Takashi Noda; Kenzaburo Nakajima; Kosuke Nakasuka; Satoshi Terasaki; Yusuke Hattori; Tomomi Nagayama; Kazuki Mori; Yoichi Takaya; Koji Miyamoto; Satoshi Nagase; Takeshi Aiba; Satoshi Yasuda; Masafumi Kitakaze; Shiro Kamakura; Yoshikazu Yazaki; Shin-Ichiro Morimoto; Mitsuaki Isobe; Fumio Terasaki

doi:10.1016/j.jacasi.2021.09.005

Prognosis and Outcomes of Clinically Diagnosed Cardiac Sarcoidosis Without Positive Endomyocardial Biopsy Findings

JACC Asia. 2021 Dec 7;1(3):385-395. doi: 10.1016/j.jacasi.2021.09.005. eCollection 2021 Dec.

Authors

Kengo Kusano¹, Kohei Ishibashi¹, Takashi Noda¹, Kenzaburo Nakajima¹, Kosuke Nakasuka², Satoshi Terasaki¹, Yusuke Hattori³, Tomomi Nagayama⁴, Kazuki Mori⁵, Yoichi Takaya⁶, Koji Miyamoto¹, Satoshi Nagase¹, Takeshi Aiba¹, Satoshi Yasuda¹, Masafumi Kitakaze¹, Shiro Kamakura¹, Yoshikazu Yazaki⁷, Shin-Ichiro Morimoto⁸, Mitsuaki Isobe⁹, Fumio Terasaki¹⁰

Affiliations

¹ Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.
² Department of Cardio-Renal Medicine and Hypertension, Nagoya City University, Nagoya, Japan.
³ Department of Cardiovascular Medicine, Shizuoka City Shizuoka Hospital, Shizuoka, Japan.
⁴ Department of Cardiovascular Medicine, Fukuoka City Hospital, Fukuoka, Japan.
⁵ Department of Cardiovascular Medicine, Hyogo Prefectural Amagasaki General Medical Center, Hyogo, Japan.
⁶ Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Okayama, Japan.
⁷ Department of Cardiovascular Medicine, Saku Central Hospital Advanced Care Center, Nagano, Japan.
⁸ Department of Cardiovascular Medicine, Fujita Health University, Aichi, Japan.
⁹ Department of Cardiovascular Medicine, Sakakibara Heart Institute, Tokyo, Japan.
¹⁰ Medical Education Center/Department of Cardiology, Osaka Medical College, Osaka, Japan.

Abstract

Background: Diagnosis of cardiac sarcoidosis (CS) is sometimes difficult due to a low positive rate of epithelioid granulomas by endomyocardial biopsy (EMB). Accordingly, Japanese guidelines can allow the CS diagnosis using clinical data alone without EMB results (clinical CS) since 2006. However, little is known about prognosis and outcome of clinical CS.

Objectives: Purpose of this study was to analyze the prognosis, outcomes, and response to corticosteroid of clinical CS using large-scale cohort survey.

Methods: Overall, 422 CS patients (mean age 60 ± 13 years, 68% female, median follow-up period of 5 years), including 345 clinical CS and 77 EMB-positive patients, histologically diagnosed CS (histological CS) by Japanese guidelines, were enrolled and examined.

Results: Clinical profile (age, sex, initial cardiac arrhythmias, and abnormal uptake of gallium-67 scintigraphy or ¹⁸F-fluorodeoxyglucose positron emission tomography in heart) was similar in both groups. Although clinical CS had better prognosis (P = 0.018) and outcome (all-cause death, appropriate defibrillator therapy, and heart transplantation; P = 0.008), multivariate Cox hazard analysis revealed that left ventricular ejection fraction (LVEF) and sustained ventricular tachycardia history were independently associated with outcome (P < 0.001 and P = 0.002, respectively), but not with the diagnosed CS category. Moreover, similar LVEF recovery after corticosteroid was observed in both groups with low LVEF (≤35%) at the 1-year follow-up period (P < 0.001).

Conclusions: In clinical CS according to the Japanese guideline, prophylactic implantable-cardioverter-defibrillator and immunosuppressive therapy are important in patients with low LVEF or ventricular tachycardia history, similar to histological CS.

Keywords: 18F-FDG, 18F-fluorodeoxyglucose; 67Ga, gallium-67; AVB, atrioventricular block; BAL, bronchoalveolar lavage; CMR, cardiac magnetic resonance; CRT, cardiac resynchronization therapy; CS, cardiac sarcoidosis; EMB, endomyocardial biopsy; HRS, Heart Rhythm Society; ICD, implantable cardioverter-defibrillator; JCS, Japanese Circulation Society; JSSOG, Japanese Society of Sarcoidosis and Other Granulomatous diseases; LV, left ventricular; LVEF, left ventricular ejection fraction; PET, positron emission tomography; RFCA, radiofrequency catheter ablation; RV, right ventricle/ventricular; VT, ventricular tachycardia; cardiac sarcoidosis; clinical diagnosis; histological diagnosis; prognosis.