+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Pediatric pulmonary hypertension and pulmonary arterial hypertension secondary to congenital heart diseases



Pediatric pulmonary hypertension and pulmonary arterial hypertension secondary to congenital heart diseases



Anadolu Kardiyoloji Dergisi 10(Suppl. 1): 50-56



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.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 054906070

Download citation: RISBibTeXText

PMID: 20819767

DOI: 10.5152/akd.2010.119


Related references

Pulmonary arterial capacitance in children with idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease: relation to pulmonary vascular resistance, exercise capacity, and survival. American Heart Journal 162(3): 562-568, 2011

Continuous intravenous prostacyclin treatment for primary pulmonary hypertension and pulmonary arterial hypertension associated with either connective tissue diseases or congenital heart disease. Circulation 104(17 Suppl.): II 250, October 23, 2001

Pulmonary hypertension/pulmonary arterial hypertension in congenital heart disease and therapy of pulmonary arterial hypertension in children. Deutsche Medizinische Wochenschrift 139(Suppl. 4): S166-S170, 2015

Management of pulmonary arterial hypertension associated with congenital heart disease with specific pulmonary arterial hypertension vasoactive drugs combined with transcatheter closure strategy:a case report. Zhonghua Xin Xue Guan Bing Za Zhi 42(11): 963-964, 2015

Lung neovascularity in pulmonary arterial hypertension associated with congenital heart defects and idiopathic pulmonary arterial hypertension: study of 198 patients. European Radiology 22(5): 1059-1066, 2012

Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications. Current Opinion in Cardiology 23(6): 545-554, 2008

Comparison of 18F-FDG uptake by right ventricular myocardium in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease. Pulmonary Circulation 2(3): 365-372, 2012

Standardized fluid-challenge testing to distinguish Pulmonary Arterial Hypertension (PAH) from pulmonary hypertension secondary to heart failure. European Heart Journal 34(Suppl 1): P233-P233, 2013

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with congenital heart disease. Annals of Thoracic Medicine 9(Suppl 1): S21-S25, 2014

Relaxin levels in pulmonary hypertension: a comparison between pulmonary arterial hypertension and diastolic heart failure-induced pulmonary hypertension. Journal of Heart and Lung Transplantation 32(3): 371-374, 2013

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy. Cardiovascular Diagnosis and Therapy 8(6): 716-724, 2019

Non-congenital heart disease associated pediatric pulmonary arterial hypertension. Progress in Pediatric Cardiology 27(1-2): 13-23, 2009

Pulmonary arterial hypertension in congenital heart diseases. Seminars in Respiratory and Critical Care Medicine 30(4): 421-428, 2009

Pulmonary Arterial Reactivity in Heart Failure with Preserved Ejection Fraction Induced Pulmonary Hypertension Similar to Pulmonary Arterial Hypertension. Journal of Cardiac Failure 19(8): S3-S4, 2013

Sildenafil for pulmonary hypertension secondary to congenital heart diseases. Indian Heart Journal 59(4): 342-345, 2009