Internal Medicine Resident Columbus, Florida, United States
Disclosure(s):
Kenzo Ramos, MD: No financial relationships to disclose
Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare yet curable cause of pulmonary hypertension from unresolved pulmonary emboli. This case highlights an atypical presentation evolving from subacute dyspnea to massive pulmonary embolism with cardiac arrest, necessitating extracorporeal cardiopulmonary resuscitation and definitive surgical intervention.
Methods: This is a single-patient case report describing the clinical presentation, diagnostic evaluation, and multidisciplinary management of massive pulmonary embolism complicated by cardiac arrest. Data were obtained from a review of the electronic medical record, including laboratory studies, imaging, invasive hemodynamics, echocardiography, procedural reports, and operative findings across one tertiary care and one quaternary center.
Outcome: A 48-year-old man with a history of testicular cancer, alcohol use disorder, and tobacco use presented with several weeks of progressive dyspnea and right lower extremity swelling. Evaluation revealed tachycardia, tachypnea, elevated transaminases, lactic acidosis, and a markedly elevated NT-proBNP of 20,000 pg/mL. Computed tomography pulmonary angiography demonstrated bilateral pulmonary emboli with significant clot burden and right heart strain. Lower extremity Doppler ultrasound revealed bilateral deep venous thromboses. The patient was started on systemic anticoagulation and taken for planned mechanical thrombectomy. During the procedure, he suffered cardiac arrest and was emergently placed on veno-arterial extracorporeal membrane oxygenation for extracorporeal cardiopulmonary resuscitation. Transthoracic echocardiography demonstrated severe right ventricular enlargement with interventricular septal flattening, consistent with marked right-sided pressure and volume overload. Thrombectomy was aborted due to hemodynamic instability and concern for chronic thromboembolic disease. He was initiated on inhaled pulmonary vasodilators and transferred to a quaternary center. Pulmonary angiography demonstrated acute emboli superimposed on extensive chronic thromboembolic disease with severe pulmonary hypertension. Aspiration thrombectomy retrieved significant clot burden but revealed persistent pulmonary hypertension. The patient subsequently underwent successful pulmonary endarterectomy with ECMO decannulation. Follow-up echocardiography demonstrated normalization of right ventricular size, function, and systolic pressure. He was discharged in stable condition.
Conclusion: Previously unrecognized CTEPH may present as catastrophic massive pulmonary embolism with cardiac arrest. Early extracorporeal cardiopulmonary resuscitation enabled survival, definitive diagnosis, and curative pulmonary endarterectomy, highlighting the importance of early mechanical support and suspicion for chronic disease in refractory right ventricular failure.
