When a Peripartum Cardiomyopathy Patient Hides Various and Serious Risk Factors for Recurrent and Fatal Thromboembolic Events Even Under Well-Conducted Oral Anticoagulation

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.