Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany.
Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands.
Eur J Heart Fail. 2019 Jul;21(7):827-843. doi: 10.1002/ejhf.1493. Epub 2019 Jun 27.
Peripartum cardiomyopathy (PPCM) is a potentially life-threatening condition typically presenting as heart failure with reduced ejection fraction (HFrEF) in the last month of pregnancy or in the months following delivery in women without another known cause of heart failure. This updated position statement summarizes the knowledge about pathophysiological mechanisms, risk factors, clinical presentation, diagnosis and management of PPCM. As shortness of breath, fatigue and leg oedema are common in the peripartum period, a high index of suspicion is required to not miss the diagnosis. Measurement of natriuretic peptides, electrocardiography and echocardiography are recommended to promptly diagnose or exclude heart failure/PPCM. Important differential diagnoses include pulmonary embolism, myocardial infarction, hypertensive heart disease during pregnancy, and pre-existing heart disease. A genetic contribution is present in up to 20% of PPCM, in particular titin truncating variant. PPCM is associated with high morbidity and mortality, but also with a high probability of partial and often full recovery. Use of guideline-directed pharmacological therapy for HFrEF is recommended in all patients respecting contraindications during pregnancy/lactation. The oxidative stress-mediated cleavage of the hormone prolactin into a cardiotoxic fragment has been identified as a driver of PPCM pathophysiology. Pharmacological blockade of prolactin release using bromocriptine as a disease-specific therapy in addition to standard therapy for heart failure treatment has shown promising results in two clinical trials. Thresholds for devices (implantable cardioverter-defibrillators, cardiac resynchronization therapy and implanted long-term ventricular assist devices) are higher in PPCM than in other conditions because of the high rate of recovery. The important role of education and counselling around contraception and future pregnancies is emphasised.
围生期心肌病（PPCM）是一种潜在危及生命的疾病，通常表现为妊娠最后 1 个月或分娩后数月内射血分数降低的心力衰竭（HFrEF），且无其他已知心力衰竭病因。本更新立场声明总结了 PPCM 的病理生理机制、危险因素、临床表现、诊断和管理方面的知识。由于呼吸困难、疲劳和腿部水肿在围产期很常见，因此需要高度怀疑以避免漏诊。建议测量利钠肽、心电图和超声心动图以快速诊断或排除心力衰竭/PPCM。重要的鉴别诊断包括肺栓塞、心肌梗死、妊娠期间高血压性心脏病和先前存在的心脏病。高达 20%的 PPCM 存在遗传因素，特别是肌联蛋白截断变异。PPCM 发病率和死亡率高，但也有部分和常完全恢复的高概率。建议所有患者在妊娠/哺乳期禁忌证的情况下，使用指南指导的心衰治疗药物。催乳素在氧化应激介导下被切割成具有心脏毒性的片段，这已被确定为 PPCM 病理生理学的驱动因素。使用溴隐亭抑制催乳素释放作为心力衰竭治疗的特异性治疗药物联合标准心衰治疗，在两项临床试验中显示出有前景的结果。由于恢复率高，PPCM 患者的设备（植入式心律转复除颤器、心脏再同步治疗和植入式长期心室辅助设备）阈值高于其他疾病。强调了围绕避孕和未来妊娠进行教育和咨询的重要作用。
