Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy?　― Revisiting Histopathological Characteristics Using 282 Lung Biopsy Specimens

• Published in: Circulation Journal Vol. 82; no. 6; pp. 1682 - 1687
• Main Authors: Masaki, Naoki, Saiki, Yuriko, Endo, Masato, Maeda, Kay, Adachi, Osamu, Akiyama, Masatoshi, Kawamoto, Shunsuke, Saiki, Yoshikatsu
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Circulation Society 25.05.2018
• Subjects: Age Factors; Atrioventricular septal defect; Biopsy; Blood Pressure; Disease Progression; Down Syndrome - complications; Female; Humans; Hypertension; Infant; Male; Pulmonary arteriopathy; Pulmonary Artery - pathology; Pulmonary Artery - physiopathology; Retrospective Studies; Risk Factors; Trisomy 21, Ventricular septal defect; Vascular Calcification; Vascular Resistance; Trisomy 21, Ventricular septal defect; Pulmonary arteriopathy; Atrioventricular septal defect

Background:Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patien...