van der Ree Martijn H, Herrera Siklody Claudia, Le Bloa Mathieu, Pascale Patrizio, Porretta Alessandra P, Teres Cheryl C, Solana Munoz Jorge, Luca Adrian, Domenichini Giulia, Ozasahin Mahmut, Jumeau Raphael, Postema Pieter G, Ribi Camillo, Bourhis Jean, Schiappacasse Luis, Pruvot Etienne
Department of Cardiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland.
Department of Cardiology, Amsterdam UMC Location University of Amsterdam, Amsterdam, Netherlands.
Front Cardiovasc Med. 2023 Jul 21;10:1213165. doi: 10.3389/fcvm.2023.1213165. eCollection 2023.
Cardiac sarcoidosis is associated with heart failure, conduction abnormalities, and life-threatening arrhythmias including ventricular tachycardia (VT). Radiotherapy has been suggested as a treatment for extra-cardiac sarcoidosis in patients refractory to immunomodulatory treatment.
The effectiveness and safety of low-dose whole-heart radiotherapy for therapy refractory cardiac sarcoidosis were evaluated in a pre- and post-intervention case report comparing the 54 months before and after treatment. Immunomodulatory low-dose whole-heart irradiation as sarcoidosis treatment consisted of a 2 × 2 Gy scheme. Additionally, high-dose single-fraction stereotactic arrhythmia radioablation of 1 × 20 Gy was applied to the pro-arrhythmic region to manage the ventricular tachycardia episodes. Cardiac sarcoidosis disease activity was measured by hypermetabolic areas on repeated fluorodeoxyglucose ([F]FDG)-PET/computed tomography (CT) scans and by evaluating changes in ventricular tachycardia episodes before and after treatment.
One patient with therapy refractory progressive cardiac sarcoidosis and recurrent ventricular tachycardia was treated. The cardiac sarcoidosis disease activity showed a durable regression of inflammatory disease activity from 3 months onwards. The [F]FDG-PET/CT scan at 54 months did not show any signs of active cardiac sarcoidosis, and a state of remission was achieved. The number of sustained VT episodes was reduced by 95%. We observed that the development of moderate aortic valve regurgitation was likely irradiation-related. No other irradiation-related adverse events occurred, and the left ventricular ejection fraction remained stable.
We report here for the first time on the beneficial and lasting effects of combined immunomodulatory low-dose whole-heart radiotherapy and high-dose stereotactic arrhythmia radioablation in a patient with therapy refractory cardiac sarcoidosis and recurrent VT.
心脏结节病与心力衰竭、传导异常以及包括室性心动过速（VT）在内的危及生命的心律失常有关。对于免疫调节治疗无效的患者，放射治疗已被建议用于治疗心脏外结节病。
在一项干预前后的病例报告中，通过比较治疗前后54个月的情况，评估低剂量全心脏放疗对治疗难治性心脏结节病的有效性和安全性。作为结节病治疗的免疫调节低剂量全心脏照射采用2×2 Gy方案。此外，对致心律失常区域进行1×20 Gy的高剂量单次立体定向心律失常射频消融，以控制室性心动过速发作。通过重复的氟脱氧葡萄糖（[F]FDG）-正电子发射断层扫描/计算机断层扫描（CT）扫描上的高代谢区域以及评估治疗前后室性心动过速发作的变化来测量心脏结节病的疾病活动。
治疗了一名患有治疗难治性进行性心脏结节病和复发性室性心动过速的患者。心脏结节病的疾病活动从3个月起显示出炎症性疾病活动的持续消退。54个月时的[F]FDG-PET/CT扫描未显示任何活动性心脏结节病的迹象，实现了缓解状态。持续性VT发作的次数减少了95%。我们观察到中度主动脉瓣反流的发生可能与放疗有关。未发生其他与放疗相关的不良事件，左心室射血分数保持稳定。
我们首次报告了联合免疫调节低剂量全心脏放疗和高剂量立体定向心律失常射频消融对一名患有治疗难治性心脏结节病和复发性VT的患者具有有益且持久的效果。
