Abstract Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but potentially curable form of pulmonary hypertension caused by incomplete resolution and fibrotic organization of pulmonary emboli, resulting in fixed vascular obstruction and remodeling. Classified as Group 4 pulmonary hypertension, it develops in about 3-4% of patients after acute pulmonary embolism, though true incidence is likely underrecognized. Early diagnosis is essential because pulmonary endarterectomy (PEA) offers a curative option and significantly improves survival. Case Description A 56-year-old woman with prior deep-vein thrombosis, hyperlipidemia, and ovarian cancer in remission presented with massive saddle pulmonary embolism and hemodynamic collapse. She underwent emergent thrombectomy and was maintained on lifelong warfarin (Goal INR 2-3). Despite compliance with therapeutic anticoagulation, she developed progressive exertional dyspnea and requiring nocturnal oxygen (2 L/min). Echocardiography showed right-ventricular dilation, septal flattening, and an estimated pulmonary-artery systolic pressure of about 70 mm Hg. Pulmonary-function testing revealed normal spirometry with moderately reduced diffusion capacity. A ventilation-perfusion (V/Q) scan demonstrated multiple bilateral mismatched perfusion defects consistent with chronic thromboembolic pulmonary hypertension (CTEPH); myocardial perfusion imaging was normal, excluding ischemic cardiomyopathy. Right-heart catheterization confirmed pre-capillary pulmonary hypertension (mean PAP 32 mm Hg, PCWP 8 mm Hg, PVR 4 Wood units). Extensive evaluation for alternative causes was unremarkable. The patient remained stable on warfarin and improved after starting sildenafil, which reduced pulmonary pressures on follow-up echocardiography. Due to residual functional limitation and persistent pulmonary hypertension, she was referred to a tertiary center for assessment of operability and possible PEA. Discussion Chronic thromboembolic pulmonary hypertension (CTEPH) results from incomplete resolution of pulmonary emboli, with organized thrombotic material becoming incorporated into the pulmonary arterial wall and causing progressive vascular remodeling and increased pulmonary vascular resistance. Persistent inflammation, abnormal fibrinolysis, and endothelial dysfunction contribute to disease progression. Although rare, CTEPH develops in approximately 3-4 % of patients after acute pulmonary embolism and should be suspected when dyspnea persists despite adequate anticoagulation. The ventilation-perfusion (V/Q) scan remains the most sensitive screening test; a normal scan essentially excludes CTEPH. Confirmation requires right-heart catheterization with evidence of pre-capillary pulmonary hypertension and imaging—CT pulmonary angiography or pulmonary angiography—demonstrating mismatched segmental defects. Pulmonary endarterectomy (PEA) is the treatment of choice for operable disease and can normalize pulmonary hemodynamics. For inoperable or residual cases, balloon pulmonary angioplasty and riociguat improve symptoms and exercise capacity. This case highlights the importance of recognizing CTEPH as a potentially curable cause of post-embolic dyspnea. This abstract is funded by: None
Asif et al. (Fri,) studied this question.
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