SummaryWe describe a case of a 41-year-old woman with acute exacerbation of chronic thromboembolic pulmonary hypertension (CTEPH) complicated by rapidly progressive respiratory failure and right heart failure with cardiogenic shock. A computed tomography (CT) showed thrombi in the right main pulmonary artery and bilateral peripheral pulmonary arteries, and echocardiography showed right ventricular dilatation and tricuspid regurgitation, with an estimated pressure gradient of 80 mmHg. The patient was initially diagnosed with acute pulmonary thromboembolism, and thrombolytic therapy was administered. Her condition subsequently deteriorated, however, necessitating mechanical ventilation and veno-arterial extracorporeal membrane oxygenation (VA-ECMO). We performed emergency catheter-directed thrombectomy and thrombus aspiration. Pulmonary hypertension (PH) temporarily improved, but subsequently worsened, and the patient was diagnosed with CTEPH. Pulmonary endarterectomy (PEA) was performed. After PEA, we were unable to wean the patient off VA-ECMO, and rescue balloon pulmonary angioplasty (BPA) to the middle and inferior lobe branches of the right lung was performed. Five days after BPA, the patient was removed from VA-ECMO and on the 57th day of hospitalization, she was weaned off the ventilator. The patient was discharged after 139 days of hospitalization. Rescue BPA represents a useful intervention for improving PH and weaning off VA-ECMO in a patient with acute exacerbation of CTEPH. (Int Heart J 2015; 56: 116-120) Key words: Right heart failure, Pulmonary endarterectomy, BPA, ECMO, CTEPH C hronic thromboembolic pulmonary hypertension (CTEPH) is caused by organized pulmonary thrombosis, and is considered to progress, at least in part, from acute pulmonary thromboembolism, though the exact etiology remains unknown. Five-year survival rates in patients with a mean pulmonary artery pressure (PAP) less than 30 mmHg is approximately 90%; a mean PAP over 50 mmHg confers a survival rate of approximately 10%, and very poor prognosis.1) Pulmonary endarterectomy (PEA) in patients with CTEPH can markedly decrease PAP and improve prognosis.
2)In peripheral types of CTEPH, however, PEA is ineffective and associated mortality rates are high. Recently, the efficacy of balloon pulmonary angioplasty (BPA) in inoperable CTEPH patients was reported.3) We successfully employed BPA under veno-arterial extracorporeal membrane oxygenation (VA-EC-MO) in a patient with acute exacerbation of CTEPH that did not improve with PEA.
Case ReportA 41-year-old woman presented at our hospital with dyspnea, edema, and weight gain. She had suffered from epilepsy since the age of 16, and at 36 had exhibited multiple lacunar infarctions on magnetic resonance imaging (MRI). She was a heavy smoker (40 cigarettes per day for 20 years), but had no family history of thromboembolic disease or blood coagulation disorders. She was taking phenytoin 100 mg tid, valproic acid 400 mg qid, and aspirin 100 mg sid.On admission, her blood pressure was 102/58 mmHg...