Chronic Thromboembolic Pulmonary Disease: Right Ventricular Function and Pulmonary Hemodynamics in a 4-Year Follow-Up

Chronic thromboembolic pulmonary disease (CTEPD) with or without pulmonary hypertension (PH) is characterized by persistent perfusion defects and progressive pulmonary vascular dysfunction after acute pulmonary embolism (PE), despite adequate anticoagulant therapy. We aimed at assessing clinical, hemodynamic, and functional evolution in patients screened for CTEPD with persistent lung perfusion scintigraphy (Q-scan) defects to identify non-invasive predictors of right ventricular (RV) impairment and development of exercise-induced pulmonary hypertension (ExPH). We analyzed 55 patients with a history of PE and no prior cardiopulmonary disease, stratified by perfusion (Q)-scan at 4 months into Q-scan-positive (n = 35) and Q-scan-negative (n = 20). At that time, all patients underwent echocardiography, cardiopulmonary exercise testing (CPET), and exercise stress echocardiography (ESE). Clinical evaluation and resting echocardiography were repeated at 24, 36, and 48 months. At baseline, Q-positive patients had higher NT-proBNP levels and greater PESI scores. At 4 months, they exhibited a higher prevalence of exercise-induced pulmonary hypertension (ExPH) on both CPET and ESE (p < 0.001). Both groups showed a partial recovery of echocardiographic parameters over time; however, Q-positive patients featured significantly higher systolic (s) pulmonary artery pressure (PAP) and mean PAP and a lower TAPSE/sPAP ratio (p < 0.001), increased eccentricity index, and shorter right ventricle (RV) outflow tract acceleration time at 48 months, suggestive of persistent RV-PA uncoupling and of a higher subclinical hemodynamic burden. Persistent Q-scan defects identify a post-PE population at risk for long-term RV dysfunction and ExPH, even in the absence of pulmonary hypertension at rest. CPET and ESE at 4 months provide useful prognostic information, supporting their integration into structured follow-up strategies to identify patients early on with evolving pulmonary vascular disease.