title: Reduction in recalcitrant pulmonary hypertension after operation for atrial septal defect.
abstract: We present the case of a patient with atrial septal defect and severe pulmonary hypertension with pulmonary artery peak pressure greater than 110 mm Hg. Open lung biopsy was done prior to the corrective operation, and pathological findings in the small pulmonary arteries included "musculoelastosis" and complete occlusion of 70% of these small arteries and arterioles. The atrial septal defect was closed, and long-term oral prostacyclin therapy was initiated. Pulmonary artery peak pressure decreased to 65 mm Hg 2 years after the operation. This case demonstrates that in a patient with 70% complete occlusion of small pulmonary arteries and arterioles resulting from "musculoelastosis," not only is surgical intervention possible but also pulmonary artery pressure decreases in the long term after operation.
authors.affiliation: Department of Surgery II, Nippon Medical School, Tokyo, Japan. yamauchi_hitoshi/surg2@nms.ac.jp
authors.forename: H
authors.initials: H
authors.lastname: Yamauchi
authors.forename: S
authors.initials: S
authors.lastname: Yamaki
authors.forename: M
authors.initials: M
authors.lastname: Fujii
authors.forename: H
authors.initials: H
authors.lastname: Iwaki
authors.forename: S
authors.initials: S
authors.lastname: Tanaka
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001706:Biopsy; D001794:Blood Pressure; D004547:Elastic Tissue / Q000473:pathology; D011464:Epoprostenol / Q000627:therapeutic use; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D011651:Pulmonary Artery / Q000473:pathology
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol