Publication: Chronic Thromboembolic Pulmonary Hypertension Secondary to Thrombophilia and Incidentally Diagnosed Atrial Septal Defect
Abstract
A 46-year-old man developed chronic thromboembolic pulmonary hypertension and atrial fibrillation after acute pulmonary embolism. He was found incidentally to have an isolated secundum atrial septal defect, as well as a homozygous mutation for the plasminogen activator inhibitor-1 gene. He was successfully treated with pulmonary endarterectomy and atrial septal defect repair. He has continued to do well on a regimen of dabigatran. (Level of Difficulty: Beginner.).
Description
Keywords
AF, atrial fibrillation, ASD, atrial septal defect, CTEPH, chronic thromboembolic pulmonary hypertension, dabigatran, DOAC, direct oral anticoagulant agent, HF, heart failure, PAI, plasminogen activator inhibitor, PAP, pulmonary arterial pressure, PE, pulmonary embolism, PEA, pulmonary endarterectomy, PH, pulmonary hypertension, plasminogen activator inhibitor-1 (PAI-1) mutation, pulmonary endarterectomy, PVR, pulmonary vascular resistance, RA, right atrial, RHC, right-sided heart catheterization, RV, right ventricular, t-PA, tissue-type plasminogen activator, TAPSE, tricuspid annular excursion, u-PA, urokinase-type plasminogen activator, VTE, venous thromboembolism