A 22-year-old woman (gravida 1, para 0) presented at the local hospital complaining of dyspnea. The patient described a cough, palpitation, and generalized myalgia. The patient had no prior medical history. She denied the use of tobacco products and said she consumed alcohol only occasionally. Her menstrual history revealed that she had experienced menarche at age 14, irregular cycles in the past, and occasional vaginal spotting in last two cycles. The chest radiographs revealed bilateral diffuse nodularity and a left pneumothorax. She was treated for pulmonary tuberculosis and pneumothorax with tuberculosis medications and the insertion of a chest tube; however, her symptoms worsened. A pulmonary thromboembolism was suspected, and chest enhanced computed tomography (CT) showed a filling defect in the left inferior pulmonary artery (Fig. 1A). Rivaroxaban was administered for the treatment of the pulmonary thromboembolism. Two weeks after starting treatment with rivaroxaban, the patient presented with aggravated dyspnea and massive hemoptysis and was urgently referred to the emergency department (ED).
Upon arrival at the ED, the patient was afebrile, with a heart rate of 138 beats per minute, blood pressure (BP) of 90/80 mm Hg, respiratory rate of 18 breaths per minute, and oxygen saturation of 100% in room air. She was alert and oriented. The chest examination for the breath sounds and auscultation revealed coarse crackles from the right lung and no sounds from the left.
The arterial blood gas levels under oxygen mask inhalation (5 L/min) were as follows: pH, 7.51; PaO2, 81 mm Hg; and PaCO2, 25 mm Hg. Her hemoglobin level was 13.1 g/dL, white blood cell count was 7,520/mL, and platelet count was 273,000/mL. The D-dimer was 2.72 mug/mL, and the troponin level was elevated slightly, at 0.01 ng/mL. Her chest radiograph revealed a normal cardiac field, diffuse nodularity in both lung fields, and a pneumothorax with chest tubing in the left lung zone. While repositioning the chest tube into the pneumothorax, she became mentally confused, and the laboratory results showed a partial pressure of oxygen (pO2) of 38 mm Hg. She was intubated, started on mechanical ventilation, and admitted to the intensive care unit for further evaluation.
On the second day after admission, her mean BP was decreased gradually. The patient remained hypotensive despite the intravenous fluid boluses that escalated the inotropic infusion with the maximum dose. VA ECMO was initiated to restore hemodynamic stability.
An echocardiogram revealed a severe left ventricular systolic dysfunction with an ejection fraction of 29% and a D-shaped left ventricle. Bronchoscopy revealed diffuse alveolar hemorrhage but no evidence of an endobronchial lesion. Therefore, the administration of rivaroxaban therapy was stopped. Pulmonary angiography was performed and showed a large filling defect in the left pulmonary artery (Fig. 1B). The patient underwent a pulmonary embolectomy in the presence of ECMO. At the site of the main pulmonary artery near its opening to the left pulmonary artery, a whitish tumor embolus was found in the left pulmonary artery. The tumor embolus was removed to the extent possible. Histologically, the pulmonary tumor embolism specimen revealed a malignancy consistent with choriocarcinoma (Fig. 2).
After a diagnosis of choriocarcinoma was made, the serum beta human chorionic gonadotropin (hCG) level was measured, and a gynecologic examination was performed. The serum beta hCG values were 260,000 mIU/mL. A metastatic workup revealed no lesions in the liver, spleen, kidney, pelvic cavity, and brain (International Federation of Gynecology and Obstetrics [FIGO] stage III, FIGO prognostic score 6). Although she was under an ECMO insertion state and her platelet level was below 75,000/muL, chemotherapy was started with the following conventional regimen after receiving fully informed consent: etoposide, 100 mg/m2 on days 1 and 2 ; methotrexate, 100 mg/m2 via intravenous bolus on day 1 and 200 mg/m2 via intravenous infusion over 12 hours on day 1; actinomycin D, 0.5 mg via intravenous bolus on day 1 and 2; cyclophosphamide, 600 mg via intravenous infusion on day 8; and vincristine, 1.0 mg/m2 on day 8 (EMA-CO). After the initial chemotherapy, her beta hCG level decreased to 2,296 mIU/mL, and the ECMO was removed as her heart function was restored. The second cycle of chemotherapy was postponed because of a poor general condition and grade 4 neutropenia. After a 2-month period of chemotherapy, the follow-up CT showed dissolved emboli in the pulmonary artery. The patient was discharged from hospital with a normal beta hCG level.
(A) A chest enhanced computed tomography, demonstrating a filling defect in the left inferior pulmonary artery.