Toutai Chaimae, Kallel Oussama, Houssam Laachach, Ismaili Nabila, Elouafi Noha
Cardiology, Mohammed I University/Mohammed VI University Hospital/Epidemiological Laboratory of Clinical Research and Public Health, Oujda, MAR.
Cardiology, Mohammed I University/Mohammed VI University Hospital, Oujda, MAR.
Cureus. 2020 Dec 31;12(12):e12392. doi: 10.7759/cureus.12392.
Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure that occurs during the final month of pregnancy through about five months after delivery, without any other known cause, and it increases the risk of thromboembolic events by many folds. A 38-year-old female with a history of peripartum dilated cardiomyopathy was admitted to our hospital, one month after a cesarean section, for severe breathlessness. Examination revealed signs of global heart failure and right deep vein thrombosis. Pulmonary CT angiography revealed pulmonary embolism of the right pulmonary artery. The patient was treated by oral anticoagulation with acenocoumarol with all international normalized ratio (INR) values within the target range (2-3). One month later, she was admitted to the emergency department with acute dyspnea and superior vena cava syndrome. Thoracic CT angiogram showed bilateral pulmonary emboli associated with an extensive deep vein thrombosis of both internal jugular veins, sigmoid sinuses, subclavian veins, innominate venous trunks, and the origin of the superior vena cava without any lesion suspected of malignancy. The thrombophilia screen performed six weeks after the suspension of vitamin K antagonists (VKAs) revealed severe deficiencies of protein C and protein S. In this report, we present the first case of recurrence of fatal thromboembolic events under well-conducted oral anticoagulation in a patient with PPCM associated with severe protein C and protein S deficiencies.
围产期心肌病（PPCM）是一种罕见的心力衰竭病因，发生在妊娠最后一个月至产后约五个月期间，无任何其他已知病因，且会使血栓栓塞事件的风险增加数倍。一名有围产期扩张型心肌病病史的38岁女性，剖宫产术后一个月因严重呼吸困难入住我院。检查发现有全心衰和右下肢深静脉血栓形成的体征。肺部CT血管造影显示右肺动脉肺栓塞。患者接受口服醋硝香豆素抗凝治疗，所有国际标准化比值（INR）值均在目标范围内（2 - 3）。一个月后，她因急性呼吸困难和上腔静脉综合征入住急诊科。胸部CT血管造影显示双侧肺栓塞，伴有双侧颈内静脉、乙状窦、锁骨下静脉、无名静脉干及上腔静脉起始部广泛的深静脉血栓形成，未发现任何疑似恶性肿瘤的病变。停用维生素K拮抗剂（VKA）六周后进行的血栓形成倾向筛查显示蛋白C和蛋白S严重缺乏。在本报告中，我们呈现了首例在口服抗凝治疗良好的情况下，患有与严重蛋白C和蛋白S缺乏相关的PPCM患者发生致命血栓栓塞事件复发的病例。
