title: A blue baby grows up.
abstract: This report describes the history of a 41-year-old female patient born with atresia of the right atrioventricular valve, hypoplasia of the right ventricle, an atrial septal defect, and malposed great arteries. The patient underwent surgical pulmonary artery banding in infancy but did not undergo corrective surgery due to the development of pulmonary arterial hypertension. This report touches on several important clinical issues that may arise when caring for the adult with complex palliated cyanotic congenital heart disease, including the development and treatment of pulmonary arterial hypertension.
authors.affiliation: Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, Calif 90095-1679, USA. jaboulhosn@mednet.ucla.edu
authors.forename: Jamil A
authors.initials: JA
authors.lastname: Aboulhosn
authors.forename: John S
authors.initials: JS
authors.lastname: Child
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D000328:Adult; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001145:Arrhythmias, Cardiac / Q000209:etiology / Q000628:therapy; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures; D003490:Cyanosis / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery; D018450:Disease Progression; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D007231:Infant, Newborn; D019028:Magnetic Resonance Imaging, Cine; D010166:Palliative Care; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D000889:Anti-Arrhythmia Agents; D000959:Antihypertensive Agents