Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Pulmonary Thromboendarterectomy Chronic Thromboembolic Pulmonary Hypertension Definition: mean pulmonary-artery pressure > 25mmHg persisting 6 months after pulmonary embolism Pathophysiology: Small-vessel arteriopathy with • Medial hypertrophy • Intimal proliferation • Microvascular thrombosis • Plexiform-lesion formation Clinical presentation • Exercise intolerance, dyspnea, fatigue • Decreasing right heart function (JVD, fixed splitting of S2, S3, tricuspid regurgitation) Diagnosis • TTE with Doppler 6 months after PE — sensitive but not specific • Chest CTA showing thromboembolic material, mosaic parenchymal pattern • Ventilation-perfusion lung scanning showing multiple bilateral defects • Confirm diagnosis with right heart catheterization and pulmonary angiography Pulmonary Thromboendarterectomy - Potentially curative procedure: • Median Sternotomy → Cardiopulmonary Bypass Started → Patient Cooling to 20 degrees C → Right Pulmonary Vessels Thrombi Removal → Left Pulmonary Vessels Thrombi Removal → Patient Warming (90-120m) → Removal of Cardiopulmonary Bypass Other Treatment Options Balloon Pulmonary-Artery Angioplasty • Used to reduce pulmonary-artery pressure in patients with CTEPH who have inoperable disease • Rarely performed Anticoagulation • Goal: prevent pulmonary-artery thrombosis and recurrent VTE • RCT data lacking for use in CTEPH Bosentan • Endothelin-receptor antagonist • BENEFiT Trial: significant improvements compared to placebo in hemodynamic variables, dyspnea, pro-BNP levels • Risk of hepatotoxicity Sildenafil - Phosphodiesterase inhibitor • Improvements in hemodynamic variables, WHO functional class Epoprostenol / Treprostinil - Prostacyclin analogues • Improvements in BNP, hemodynamic variables, WHO functional class • Promising inhaled and oral forms - Rebecca Steinberg @RebeccaSteinb17 #CTEPH #PHTN #Pulmonary #Hypertension #Thromboendarterectomy #diagnosis #management
