A case of maternal death diagnosed as idiopathic pulmonary arterial hypertension by pathological autopsy

Kurotaki Saki, Ito Asami, Maeda Juria, Yokoyama Minako, Matsukura Daisuke, Tanaka Kanji

doi:10.34456/jjspnm.57.2_399

<p> The patient was a 32-year-old woman with gravida 4 para 2 and had a medical history of childhood asthma. After spontaneous pregnancy, she was managed for pregnancy and underwent preventive cervical cerclage at 15 weeks of gestation at her prior hospital. She was aware of shortness of breath, respiratory distress, and edema, and she consulted her previous internal medicine facility at 19 weeks of gestation. The results of the examination showed right heart failure, pulmonary hypertension, and thrombocytopenia. Contrast-enhanced computed tomography showed no pulmonary thromboembolism, and the patient was transferred to our hospital for examination and treatment. The echocardiography showed marked right heart load, and her platelet number decreased to 29,000/μL, suggesting thrombotic microangiopathy. She was scheduled to be treated after a platelet transfusion on the next day, but her condition suddenly changed and she died. A pathological autopsy was performed, and confirmed a diagnosis of idiopathic pulmonary arterial hypertension（IPAH）. IPAH is more prevalent in young women, with a high mortality rate of 30-50% in cases of perinatal complications, and pregnancy is generally contraindicated. Secondary pulmonary hypertension was ruled out in this case because there was no history of collagen disease, cardiac disease, or pulmonary or liver disease. If a patient complains of shortness of breath or respiratory distress during pregnancy, we must keep IPAH in mind and provide prompt examination and treatment.</p>

A case of maternal death diagnosed as idiopathic pulmonary arterial hypertension by pathological autopsy

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