+ Site Statistics
References:
54,258,434
Abstracts:
29,560,870
PMIDs:
28,072,757
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications



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



To present the available data on pathophysiology, clinical presentation, prognosis, and especially management strategies for adult patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH). Particular emphasis is placed on differences between other types of PAH and CHD-related PAH, in which clinical presentation and management relate to a constellation of factors, both pulmonary and cardiac. Pulmonary vascular disease in adults with CHD and especially its extreme expression, the Eisenmenger syndrome, is a chronic disease with slow progression, leading to multiorgan failure and death, decades after its first clinical presentation. In the last few years, oral advanced therapies for PAH have emerged and are considered for mono or combination therapy for CHD, though the evidence is limited. Supportive care and prevention of complications seem to be at least as important in the overall care of these patients. Although new advanced therapies hold promise in PAH secondary to CHD, long-term data are clearly needed. Advanced therapies should be considered when other causes of functional limitation, such as iron deficiency, have been first addressed. Expertise in CHD as well as PAH is essential for providing adequate care for this patient group with a unique pathophysiology.

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

Accession: 055286819

Download citation: RISBibTeXText

PMID: 18830068

DOI: 10.1097/HCO.0b013e3283126954


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

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

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

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

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

Sex differences in the diagnosis, treatment, and outcome of patients with pulmonary arterial hypertension enrolled in the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Chest 141(2): 363-373, 2012

Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: a Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. Journal of Heart and Lung Transplantation 34(3): 356-361, 2015

Advanced therapies for the management of adults with pulmonary arterial hypertension due to congenital heart disease: a systematic review. Open Heart 5(1): E000744, 2018

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

Relationship between pulmonary arterial resistance and compliance among patients with pulmonary arterial hypertension and congenital heart disease. Journal of Thoracic and Cardiovascular Surgery 152(2): 507-513, 2018

Pulmonary arterial hypertension in adults with congenital heart disease. International Journal of Clinical Practice. Supplement 2010(165): 13-24, 2010

Pulmonary arterial hypertension in adults with congenital heart disease. Revista Espanola de Cardiologia 58(5): 465-469, 2005

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

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 Supplement): II 250, October 23, 2001

Other causes of pulmonary arterial hypertension: pulmonary veno-occlusive disease, pulmonary capillary hemangiomatosis, porto-pulmonary hypertension, HIV- associated pulmonary arterial hypertension. Anadolu Kardiyoloji Dergisi 10 Suppl 1: 63-68, 2010