title: [Pediatric pulmonary hypertension and pulmonary arterial hypertension secondary to congenital heart diseases].
abstract: Causes of pulmonary arterial hypertension (PAH) are similar in adults and children. The main difference is that PAH secondary to congenital heart diseases, is the predominant cause in pediatric patients. Persistent pulmonary hypertension of the newborn shows completely different clinical course and pathophysiological mechanisms. It is usually seen in full term babies with a high morbidity and mortality rate. Improved prognosis has been reported with inhaled nitric oxide (NO) and extracorporeal membrane oxygenation therapy in babies hospitalized in well equipped and experienced newborn centers. Primary pulmonary hypertension and familial pulmonary hypertension are rare in pediatric age group because the diagnosis is initially made in adolescence. The incidence of PAH secondary to congenital heart disease is estimated as 1.6 - 12.5 case/million/year. Eisenmenger syndrome is diagnosed in 1% of patients with PAH. Patients with left to right shunts are the main group who develop pulmonary vascular disease if not treated in the early infancy. Some cyanotic congenital heart diseases are also the causes of PAH. The best treatment of patients at risk for the development of pulmonary vascular disease is prevention by early surgical elimination of defects or repairing the anatomy. Treatment options with vasodilating agents like NO, prostaglandin analogs, phosphodiesterase -5 inhibitors and endothelin receptor antagonists are used to improve survival and quality of life. Heart lung or bilateral lung transplantation is the only surgical option for many of these patients. Results of national and international registries will bring valuable epidemiological and prognostic perspectives to pediatric PAH.
authors.affiliation: Gazi Universitesi Tip Fakültesi, Pediatrik Kardiyoloji Anabilim Dali, Ankara, Türkiye. rana@gazi.edu.tr
authors.forename: Rana
authors.initials: R
authors.lastname: Olguntürk
mesh_terms: D006328:Cardiac Catheterization; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology* / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality / Q000517:prevention & control; D007223:Infant; D007231:Infant, Newborn; D016040:Lung Transplantation; D011652:Pulmonary Circulation; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D014665:Vasodilator Agents