De Gasperis Cardio Center, Niguarda Hospital, Milano, Italy.
De Gasperis Cardio Center, Niguarda Hospital, Milano, Italy; Department of Health Sciences, University of Milano-Bicocca, Monza, Italy.
J Am Coll Cardiol. 2019 Jul 23;74(3):299-311. doi: 10.1016/j.jacc.2019.04.063.
Fulminant myocarditis (FM) is a form of acute myocarditis characterized by severe left ventricular systolic dysfunction requiring inotropes and/or mechanical circulatory support. A single-center study found that a patient with FM had better outcomes than those with acute nonfulminant myocarditis (NFM) presenting with left ventricular systolic dysfunction, but otherwise hemodynamically stable. This was recently challenged, so disagreement still exists.
This study sought to provide additional evidence on the outcome of FM and to ascertain whether patient stratification based on the main histologic subtypes can provide additional prognostic information.
A total of 220 patients (median age 42 years, 46.3% female) with histologically proven acute myocarditis (onset of symptoms <30 days) all presenting with left ventricular systolic dysfunction were included in a retrospective, international registry comprising 16 tertiary hospitals in the United States, Europe, and Japan. The main endpoint was the occurrence of cardiac death or heart transplantation within 60 days from admission and at long-term follow-up.
Patients with FM (n = 165) had significantly higher rates of cardiac death and heart transplantation compared with those with NFM (n = 55), both at 60 days (28.0% vs. 1.8%, p = 0.0001) and at 7-year follow-up (47.7% vs. 10.4%, p < 0.0001). Using Cox multivariate analysis, the histologic subtype emerged as a further variable affecting the outcome in FM patients, with giant cell myocarditis having a significantly worse prognosis compared with eosinophilic and lymphocytic myocarditis. In a subanalysis including only adults with lymphocytic myocarditis, the main endpoints occurred more frequently in FM compared with in NFM both at 60 days (19.5% vs. 0%, p = 0.005) and at 7-year follow up (41.4% vs. 3.1%, p = 0.0004).
This international registry confirms that patients with FM have higher rates of cardiac death and heart transplantation both in the short- and long-term compared with patients with NFM. Furthermore, we provide evidence that the histologic subtype of FM carries independent prognostic value, highlighting the need for timely endomyocardial biopsy in this condition.
暴发性心肌炎（FM）是一种急性心肌炎，其特征为严重的左心室收缩功能障碍，需要使用正性肌力药物和/或机械循环支持。一项单中心研究发现，与表现为左心室收缩功能障碍但血流动力学稳定的急性非暴发性心肌炎（NFM）患者相比，FM 患者的结局更好。然而，最近这一结论受到了挑战，因此仍然存在争议。
本研究旨在提供 FM 结局的更多证据，并确定基于主要组织学亚型的患者分层是否能提供额外的预后信息。
本回顾性国际注册研究纳入了 220 例经组织学证实的急性心肌炎（症状发作<30 天）患者，所有患者均表现为左心室收缩功能障碍，这些患者来自美国、欧洲和日本的 16 家三级医院。主要终点是入院后 60 天内和长期随访时发生心脏性死亡或心脏移植。
FM 组（n=165）的心脏性死亡和心脏移植发生率明显高于 NFM 组（n=55），两组患者在 60 天（28.0%比 1.8%，p=0.0001）和 7 年随访时（47.7%比 10.4%，p<0.0001）均如此。多变量 Cox 分析显示，组织学亚型是影响 FM 患者结局的另一个变量，巨细胞性心肌炎的预后明显差于嗜酸性粒细胞性和淋巴细胞性心肌炎。在仅纳入淋巴细胞性心肌炎成人患者的亚组分析中，FM 组的主要终点在 60 天（19.5%比 0%，p=0.005）和 7 年随访时（41.4%比 3.1%，p=0.0004）比 NFM 组更频繁发生。
这项国际注册研究证实，与 NFM 患者相比，FM 患者在短期和长期内心脏性死亡和心脏移植的发生率均更高。此外，本研究提供了证据表明，FM 的组织学亚型具有独立的预后价值，这凸显了在这种情况下及时进行心内膜心肌活检的必要性。
