Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with venous thromboembolism (VTE); however, in half of the cases, no prior deep vein thrombosis is ever found. The use of combined oral contraceptives (COCs) is a known risk factor for VTE. We present the case of a young woman with CTEPH in whom timely diagnosis and intervention prevented irreversible pulmonary hypertension (PH). Case Presentation: A 27-year-old woman with 10-year use of COCs presented with progressive dyspnea for 3 weeks. The ECG showed right ventricular systolic overload and right atrial enlargement. Laboratory tests showed elevated pro-BNP and D-dimer. TTE showed right atrium and right ventricle (RV) enlargement and elevated right ventricular systolic pressure (RVSP) of 83 mmHg. The ventilation/perfusion scintigraphy showed mismatched segmental perfusion defects in both lung fields with greater involvement of the left lung, and oral anticoagulation was started. Lower extremity venous ultrasound and hypercoagulability studies were normal. CTPA revealed filling defects in the left main pulmonary artery and bilateral segmental branches. After 3 months of anticoagulation, she persisted in NYHA class III, and right heart catheterization showed mean PAP of 38 mmHg, PCWP of 8 mmHg, and PVR of 9 WU, confirming CTEPH. Due to unavailability in Peru, she was referred to a US center for pulmonary endarterectomy (PEA). After a successful intervention, she improved clinically with a reduction in RVSP to 34 mmHg. This case highlights the importance of rapid intervention in CTEPH even in situations where optimal treatment is not readily available. Conclusion: CTEPH may be associated with the prolonged use of COCs in young women. Timely intervention can reverse PH and prevent RV remodeling. Unfortunately, Peru does not have a PEA center and not every case will undergo treatment. We call for the training of local surgeons to ensure fair and equitable access for the population.
Read full abstract