title: Pulmonary arterial hypertension associated with congenital heart disease.
abstract: Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.
authors.affiliation: Dept of Cardiology, Monaldi Hospital, Second University of Naples, Naples, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.forename: Vaikom S
authors.initials: VS
authors.lastname: Mahadevan
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D002986:Clinical Trials as Topic; D015897:Comorbidity; D004314:Down Syndrome / Q000150:complications; D004541:Eisenmenger Complex / Q000150:complications* / Q000503:physiopathology; D004730:Endothelium, Vascular / Q000503:physiopathology; D011464:Epoprostenol / Q000627:therapeutic use; D015438:Health Behavior; D006330:Heart Defects, Congenital / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D016027:Heart Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*; D016040:Lung Transplantation; D010102:Oxygen Inhalation Therapy; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D017410:Practice Guidelines as Topic; D011652:Pulmonary Circulation / Q000502:physiology; D011687:Purines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D013927:Thrombosis / Q000209:etiology / Q000517:prevention & control
chemical_list: D000959:Antihypertensive Agents; D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D000077300:Bosentan