Abstract

Introduction: We report the case of a man with chronic thromboembolic pulmonary hypertension (CTEPH) who developed mixed shock from a unique source and required veno-arterial extracorporeal membranous oxygenation (VA ECMO) for refractory cardiopulmonary failure. Description: A 19-year-old man with asthma and progressive dyspnea presented to an outside hospital after developing spontaneous pneumomediastinum. Computed tomography (CT) chest showed coarse calcifications in the right pulmonary artery (RPA) and lung nodules concerning for malignancy. Initial evaluation was consistent with CTEPH and antiphospholipid antibodies. PET scan and cardiac MRI confirmed thrombus. Endobronchial ultrasound (EBUS) and biopsy were then performed. The patient was transferred to our center for surgical pulmonary thromboendarterectomy (PTE). On hospital day (HD) 2, he acutely decompensated with tachycardia, hypoxemia, hypotension, and rigors. Broad-spectrum antibiotics, vasopressors, and non-invasive positive pressure ventilation (NIPPV) were started. He developed worsening hypoxemia and shock and was cannulated with VA ECMO, which rapidly improved his hemodynamics and oxygenation. He was quickly liberated from vasopressors and NIPPV. CT pulmonary angiography showed new foci of gas within the RPA clot, and blood cultures grew Streptococcus mitis and Granulicatella adiacens. Hypercoagulability evaluation revealed warm and cold agglutinins. After two sessions of plasmapheresis, he underwent PTE on HD 7. Purulence was noted around the RPA thrombus. Following successful PTE, he separated from cardiopulmonary bypass and ECMO. He liberated from vasopressors and inotropes and extubated on postoperative day (POD) 1. RPA fluid culture grew Actinomyces odontolyticus. He was discharged on POD 14. At 3-month follow-up, he had less dyspnea and no serious complications. Discussion: Patients with CTEPH have a high risk of acute right ventricular (RV) failure from worsening pulmonary hypertension or RV ischemia from systemic hypotension (e.g., sepsis). In this case, it is likely that biopsy of the RPA thrombus introduced bacteria, leading to bacteremia, septic shock and rapid hemodynamic collapse. VA ECMO was a successful rescue therapy, minimizing the need for escalating vasopressors and PPV, which may worsen RV dysfunction.

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