Vanzetto Gérald, Martin Alix, Bouvaist Hélène, Marlière Stéphanie, Durand Michel, Chavanon Olivier
Centre hospitalier universitaire de Grenoble, cliniques universitaires de cardiologie, BP 217, 38043 Grenoble cedex 7, France.
Presse Med. 2012 Jun;41(6 Pt 1):613-20. doi: 10.1016/j.lpm.2012.03.014. Epub 2012 May 1.
Peripartum cardiomyopathy (PPCMP) is a dilated and hypokinetic cardiomyopathy occurring during pregnancy or after delivery, with an estimated incidence between 1/1000 and 1/4000 births. It has been defined as a new onset of heart failure in the month preceding or following delivery, without demonstrated aetiology nor previously known heart disease, and with echocardiographic evidences of left ventricular (LV) dysfunction (LV ejection fraction<0.45). It's a multifactorial disease, immunologic, hormonal, and possibly viral mechanisms playing a determinant pathophysiological role. The classical clinical presentation is a rapid and unexpected onset of heart failure in a previously healthy woman, echocardiography being the key examination for positive and differential diagnosis, prognostication, therapeutic decision-making, and follow-up. The potential severity of PPCMP, and its unpredictable evolution in the first days following diagnosis, require that patients be referred to a tertiary care centre with a high skill in intensive cardiology care. Therapeutic management of PPCMP does not offer any specificity when compared to other causes of acute or chronic heart failure (from diuretics to extracorporeal life support), except for ACE-inhibitors, that are contraindicated before delivery. The high incidence of thrombo-embolic complications observed in the disease requires however rapid and curative anticoagulation, and immuno-suppressive treatment has been proposed in fulminant and highly inflammatory presentation, but its efficacy remains controversial. Very recently, promising results have been reported with bromocriptin-a prolactin secretion inhibitor-for reducing 6-month morbidity and mortality, but these findings have to be confirmed in larger scale randomised trials. As for the long-term evolution, approximately half of the patients will heal, while half of the women will keep some degree of LV dysfunction, 25% of them developing moderate to severe chronic heart failure.
围产期心肌病（PPCMP）是一种在妊娠期间或分娩后发生的扩张型和运动功能减退型心肌病，估计发病率为每1000至4000例分娩中有1例。它被定义为在分娩前或分娩后一个月内新发生的心力衰竭，无明确病因且既往无已知心脏病史，并有左心室（LV）功能障碍的超声心动图证据（LV射血分数<0.45）。这是一种多因素疾病，免疫、激素以及可能的病毒机制在病理生理过程中起决定性作用。典型的临床表现是先前健康的女性突然快速发生心力衰竭，超声心动图是进行阳性和鉴别诊断、预后评估、治疗决策及随访的关键检查。PPCMP的潜在严重性及其在诊断后最初几天不可预测的病情发展，要求将患者转诊至具备高水平重症心脏病护理技能的三级医疗中心。与其他急性或慢性心力衰竭病因相比，PPCMP的治疗管理并无特殊之处（从利尿剂到体外生命支持），但分娩前禁用血管紧张素转换酶抑制剂（ACE抑制剂）。然而，该疾病中观察到的血栓栓塞并发症发生率很高，需要迅速进行有效抗凝治疗，对于暴发性和高度炎症性表现，有人提出免疫抑制治疗，但其疗效仍存在争议。最近，有报道称使用溴隐亭（一种催乳素分泌抑制剂）在降低6个月发病率和死亡率方面取得了有前景的结果，但这些发现必须在更大规模的随机试验中得到证实。至于长期发展，大约一半的患者会康复，而另一半女性会保留一定程度的LV功能障碍，其中25%会发展为中度至重度慢性心力衰竭。
