title: Management of a child with pulmonary arterial hypertension presenting with systemic hypertension.
abstract: We describe the course and management of a 12-year-old girl with severe pulmonary arterial hypertension who initially presented with severe systemic hypertension. Successful therapy included pulmonary vasodilators and an atrial septostomy, while ensuring adequate maintenance of her systemic vascular resistance to maintain cardiac output. Clear understanding of the physiology and judicious medical management in patients with severe pulmonary arterial hypertension using extreme compensatory mechanisms is vitally important.
authors.affiliation: 1Heart Institute,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America.
authors.forename: Saul
authors.initials: S
authors.lastname: Flores
authors.affiliation: 1Heart Institute,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America.
authors.forename: Joshua
authors.initials: J
authors.lastname: Daily
authors.affiliation: 1Heart Institute,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America.
authors.forename: Jayant Nick
authors.initials: JN
authors.lastname: Pratap
authors.affiliation: 1Heart Institute,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America.
authors.forename: Michelle C
authors.initials: MC
authors.lastname: Cash
authors.affiliation: 1Heart Institute,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America.
authors.forename: Russel
authors.initials: R
authors.lastname: Hirsch
mesh_terms: D001794:Blood Pressure / Q000187:drug effects*; D006328:Cardiac Catheterization; D002648:Child; D019468:Disease Management; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006973:Hypertension / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D014655:Vascular Resistance / Q000502:physiology*; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents
keywords: Pulmonary arterial hypertension; paediatrics; systemic hypertension