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.).