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摘要: 围产期心肌病(peripartum cardiomyopathy，PPCM)是在妊娠晚期和分娩后6个月内发生的心肌功能障碍。其发病早期不易诊断，受多种危险因素的影响，病理生理机制复杂，预后差异大，近年来逐渐引起关注。本文将详细综述PPCM在临床表现、诊断、治疗、预后、发病机制和研究现状等方面的进展。Abstract: Peripartum cardiomyopathy(PPCM) is a myocardial dysfunction that occurs in late pregnancy or within 6 months after delivery. The early diagnosis of PPCM is difficult, and the disease is influenced by multiple risk factors, with a complex pathophysiological mechanism and significant variability in prognosis. In recent years, it has gradually attracted attention. This article will comprehensively review the progress of PPCM in clinical manifestations, diagnosis, treatment, prognosis, pathogenesis, and research status.
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Key words:
- peripartum cardiomyopathy /
- heart failure /
- angiogenesis /
- oxidative stress
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图 5 心肌特异性敲除ZFP36L2的PPCM模型机制
Figure 5. Mechanism diagram of the PPCM model with myocardium-specific knockout of ZFP36L2
表 1 PPCM的鉴别诊断
Table 1. Differential diagnosis of PPCM
疾病 鉴别要点 发病时间 生物标志物 UCG特点 与PPCM的区别 PPCM 没有已知的心脏病，怀孕前没有心衰体征和(或)症状 妊娠末期和产后1~6个月内 利钠肽、BNP、pro-BNP 左心室收缩功能降低，LVEF < 45% 扩张型心肌病 心衰体征和(或)症状和(或)怀孕前已知的心脏病，劳力性呼吸困难、双下肢水肿、颈静脉怒张、心音增强 怀孕中期 利钠肽 收缩性左心室功能下降，可能出现右心室功能障碍、心室腔扩大、心室壁运动减弱 病史、UCG、心脏MRI 肥厚型心肌病 呼吸困难、胸痛、心室腔变小、室间隔增厚、蹲踞和服用β受体阻滞剂后有好转、青少年运动员和家族遗传史多见 怀孕中期 利钠肽 左心室肥厚，典型的心肌晚期增强模式，左心室流出道梗阻(肥厚型梗阻性心肌病) 病史、UCG、心脏MRI 高血压性心脏病/重度先兆子痫 有长期高血压病史或新发高血压、蛋白尿 怀孕中期 利钠肽 左心室肥厚、舒张功能障碍、一过性左心室功能障碍 病史、UCG 冠心病 劳力型心绞痛、胸痛呈放射性、发病时头晕黑矇，舌下含服硝酸甘油可以缓解、持续时间不超过15 min 怀孕中期 CM-MB、TnI 病史、ECG、冠状动脉造影、心脏MRI 心肌梗死 剧烈胸痛、持续时间大于15 min、舌下含服硝酸甘油不能缓解、呼吸困难、大汗淋漓、恶心呕吐 急性发作、怀孕期间或分娩后立即 CM-MB、TnI 局部室壁运动异常、缺血性心肌瘢痕 病史、ECG、冠状动脉造影、心脏MRI 先兆子痫 孕产妇有高血压、糖尿病、多胎妊娠、流产史 怀孕中期 收缩功能保留 高血压病史、蛋白尿、UCG Takotsubo综合征 胸痛、分娩压力很大或胎儿并发症导致的紧急情况 急性发作、分娩期间或分娩后立即 利钠肽 具有典型解剖模式的区域壁运动异常 病史、UCG 先天性心脏病 可能在妊娠期间首次通过UCG诊断，怀孕前心衰体征和(或)症状、已知心脏病、既往心脏手术 怀孕中期 利钠肽 心脏结构异常或心脏血液反流等 UCG、病史 瓣膜性心脏病 怀孕前的心衰体征和(或)症状，已知的心脏病。UCG检查结果先天性主动脉瓣狭窄、风湿性心脏病引起的二尖瓣狭窄。PPCM患者也可能有瓣膜疾病，即二尖瓣反流 怀孕中期 利钠肽 瓣膜狭窄或反流、人工心脏瓣膜关闭不全 UCG、病史 阿霉素诱导的心肌病 阿霉素药物治疗史、心衰的典型症状 怀孕中期 利钠肽 左心扩大、左心收缩功能降低 UCG、病史 病毒性心肌炎 感染病毒前驱症状、组织学诊断、暴发性表现 病毒感染后急性或亚急性发作 肌钙蛋白和CRP升高 左心室收缩功能正常或降低，典型的心肌晚期钆增强模式，心包积液 心脏MRI、心肌活检 心律失常 存在特定的潜在节律异常，窦性心动过速可能继发于妊娠期心衰 怀孕中期 ECG 注：MRI：磁共振成像；CM-MB：肌酸激酶同工酶；TnI：肌钙蛋白I；CRP：C反应蛋白。 
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