Advanced Heart Disease and Heart Transplant Programs, Cedars-Sinai Smidt Heart Institute, Los Angeles, California.
Advanced Heart Disease and Heart Transplant Programs, Cedars-Sinai Smidt Heart Institute, Los Angeles, California.
Transplant Proc. 2021 Jan-Feb;53(1):348-352. doi: 10.1016/j.transproceed.2020.10.047. Epub 2020 Dec 29.
Giant cell myocarditis (GCM) has a poor prognosis without heart transplant, but post-transplant survival is unknown.
To describe the post-transplant survival of patients with GCM at a large transplant center.
Seven patients underwent heart transplant for histologically confirmed GCM of the explanted heart. The median age was 59 years, and 43% (3 of 7) were female. All patients had cardiogenic shock, multiorgan failure, elevated troponin, and recurrent ventricular tachycardia, and some required mechanical circulatory support. All patients received rabbit antithymocyte globulin (rATG) in the perioperative period at a dose of 1.5 mg/kg daily for 1 to 5 days and 4 received intravenous immunoglobulin 1 g/kg daily for 2 days after rATG. All patients had early initiation of tacrolimus by first to third postoperative day depending on renal function, early mycophenolate, and high dose steroid. All were maintained using tacrolimus, mycophenolate, and prednisone.
One patient had asymptomatic recurrence of GCM at 3 months, managed by up-titration of tacrolimus, and had asymptomatic 2R cellular rejection at 4 months, managed with steroid bolus. No patient had high-grade rejection. One patient died at 267 days, possibly of GCM. Six of 7 (86%) remain alive at a median of 842 days (2.3 years) post transplant.
Patients with GCM have excellent post-transplant survival with use of rATG and triple drug immunosuppressive therapy; however, some patients remain at risk for GCM recurrence after transplant, which may respond to augmented immunosuppression.
巨细胞心肌炎(GCM)在没有心脏移植的情况下预后不良,但移植后的生存率尚不清楚。
描述大型移植中心接受 GCM 患者的移植后生存情况。
7 名患者因组织学证实的供体心脏 GCM 而行心脏移植。中位年龄为 59 岁,43%(7 例中有 3 例)为女性。所有患者均存在心源性休克、多器官衰竭、肌钙蛋白升高和复发性室性心动过速,部分患者需要机械循环支持。所有患者在围手术期接受兔抗胸腺细胞球蛋白(rATG)治疗,剂量为 1.5mg/kg,每日 1 至 5 天,并在 rATG 后接受静脉注射免疫球蛋白 1g/kg,每日 2 天。所有患者均根据肾功能在术后第 1 至第 3 天早期开始使用他克莫司、早期使用霉酚酸酯和大剂量类固醇。所有患者均使用他克莫司、霉酚酸酯和泼尼松维持治疗。
1 例患者在术后 3 个月时无症状复发 GCM,通过增加他克莫司剂量进行治疗,在术后 4 个月时无症状出现 2R 细胞排斥反应,采用类固醇冲击治疗。无一例患者发生高级别排斥反应。1 例患者在术后 267 天死亡,可能死于 GCM。7 例患者中有 6 例(86%)在移植后中位时间 842 天(2.3 年)时仍存活。
使用 rATG 和三联药物免疫抑制治疗,GCM 患者移植后具有优异的生存结果;然而,一些患者在移植后仍存在 GCM 复发的风险,可能需要增强免疫抑制治疗。