title: Impact of pulmonary vascular resistances in heart transplantation for congenital heart disease.
abstract: Congenital heart disease is one of the major diagnoses in pediatric heart transplantation recipients of all age groups. Assessment of pulmonary vascular resistance in these patients prior to transplantation is crucial to determine their candidacy, however, it is frequently inaccurate because of their abnormal anatomy and physiology. This problem places them at significant risk for pulmonary hypertension and right ventricular failure post transplantation. The pathophysiology of pulmonary vascular disease in children with congenital heart disease depends on their pulmonary blood flow patterns, systemic ventricle function, as well as semilunar valves and atrioventricular valves structure and function. In our review we analyze the pathophysiology of pulmonary vascular disease in children with congenital heart disease and end-stage heart failure, and outline the state of the art pre-transplantation medical and surgical management to achieve reverse remodeling of the pulmonary vasculature by using pulmonary vasodilators and mechanical circulatory support.
authors.affiliation: Divisions of Critical Care and Cardiology, Department of Pediatrics, Saint Louis Children's Hospital, Washington University in Saint Louis, Missouri, USA. gazit_a@kids.wustl.edu
authors.forename: Avihu Z
authors.initials: AZ
authors.lastname: Gazit
authors.forename: Charles E
authors.initials: CE
authors.lastname: Canter
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006330:Heart Defects, Congenital / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D006331:Heart Diseases / Q000151:congenital* / Q000601:surgery*; D006333:Heart Failure / Q000209:etiology; D016027:Heart Transplantation / Q000009:adverse effects / Q000379:methods; D006352:Heart Ventricles / Q000503:physiopathology; D018056:Hemorheology / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007223:Infant; D011652:Pulmonary Circulation / Q000502:physiology; D011660:Pulmonary Heart Disease / Q000503:physiopathology*; D014655:Vascular Resistance / Q000502:physiology*; D018497:Ventricular Dysfunction, Right / Q000209:etiology