TITLE

Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease

AUTHOR(S)
Engelfriet, Peter M.; Duffels, Marielle G. J.; Möller, Thomas; Boersma, Eric; Tijssen, Jan G. P.; Thaulow, Erik; Gatzoulis, Michael A.; Mulder, Barbara J. M.
PUB. DATE
June 2007
SOURCE
Heart;Jun2007, Vol. 93 Issue 6, p682
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Aim: To investigate the role of pulmonary arterial hypertension (PAH) in adult patients born with a cardiac septal defect, by assessing its prevalence and its relation with patient characteristics and outcome. Methods and results: From the database of the Euro Heart Survey on adult congenital heart disease (a retrospective cohort study with a 5-year follow-up), the relevant data on all 1877 patients with an atrial septal defect (ASD), a ventricular septal defect (VSD), or a cyanotic defect were analysed. Most patients (83%) attended a specialised centre. There were 896 patients with an ASD (377 dosed, 504 open without and 15 with Eisenmenger's syndrome), 710 with a VSD (275, 352 and 83, respectively), 1 33 with Eisenmenger's syndrome owing to another defect and 138 remaining patients with cyanosis. PAH was present in 531 (28%) patients, or in 34% of patients with an open ASD and 28% of patients with an open VSD, and 12% and 1 3% of patients with a closed defect, respectively. Mortality was highest in patients with Eisenmenger's syndrome (20.6%). In case of an open defect, PAH entailed an eightfold increased probability of functional limitations (New York Heart Association class >1), with a further sixfold increase when Eisenmenger's syndrome was present. Also, in patients with persisting PAH despite defect closure, functional limitations were more common. In patients with ASD, the prevalence of right ventricular dysfunction increased with systolic pulmonary artery pressure (OR = 1 .073 per mm Hg; p<0.001). Major bleeding events were more prevalent in patients with cyanosis with than without Eisenmenger's syndrome (17% vs 3%; p<0.001). Conclusion: In this selected population of adults with congenital heart disease, PAH was common and predisposed to more symptoms and further clinical deterioration, even among patients with previous defect closure and patients who had not developed Eisenmenger's physiology.
ACCESSION #
25512969

 

Related Articles

  • Bioabsorbable Atrial Septal Occluder for Percutaneous Closure of Atrial Septal Defect in Children. Baspinar, Osman; Kervancioglu, Mehmet; Kilinc, Metin; Irdem, Ahmet // Texas Heart Institute Journal;2012, Vol. 39 Issue 2, p184 

    The BioSTAR bioabsorbable septal repair implant is a new transcatheter secundum atrial septal defect occlusion device that is absorbed and replaced by healthy native tissue. This retrospective analysis was designed to determine the most significant factors for its successful use in children....

  • Pulmonary arterial hypertension in congenital cardiac disease -- the need for refinement of the Evian-Venice classification. van Albada, Mirjam E.; Berger, Rolf M. F. // Cardiology in the Young;Feb2008, Vol. 18 Issue 1, p10 

    Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial...

  • Ortner Syndrome in Infants. ZAKI, SYED AHMED; ASIF, SHUJAATH; SHANBAG, PREETI // Indian Pediatrics;Apr2010, Vol. 47 Issue 4, p351 

    Ortner syndrome or cardiovocal syndrome refers to hoarseness of voice due to recurrent laryngeal nerve paralysis secondary to cardiovascular disease. We present three cases of Ortner syndrome in infants with congenital heart disease. All the three cases had moderate to severe pulmonary...

  • Classification of Pulmonary Hypertension. Souza, Rogerio; Simonneau, Gerald // Advances in Pulmonary Hypertension;2014, Vol. 13 Issue 1, p17 

    Classification of pulmonary hypertension groups patients with similar pathological findings, hemodynamic profiles, and management strategies. Minor modifications have been made to the current classification system, particularly within Group 1 pulmonary arterial hypertension. This article...

  • Correlation between congenital heart disease complicated with pulmonary artery hypertension and circulating endothelial cells as well as endothelin-1. Xiaofei Li; Jun Qiu; Min Pan; Dongdong Zheng; Yamin Su; Meifang Wei; Xiangqing Kong; Wei Sun; Jiahua Zhu // International Journal of Clinical & Experimental Medicine;2015, Vol. 8 Issue 9, p10743 

    Objective: To investigate changes in the level of circulating endothelial cells (CECs) and endothelin-1 (ET-1) in peripheral venous blood of the patients with congenital heart disease (CHD) complicated with pulmonary artery hypertension (PAH), and research on their effects in the onset and...

  • Anaesthetic Management of Scimitar Syndrome: A Case Report. Singh, Ajmer; Sharma, Neeraj // Indian Anaesthetists' Forum;2010, Special section p1 

    Scimitar syndrome, a rare congenital cardiopulmonary anomaly, consists of partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia and anomalous systemic arterial supply to the right lung. A 5 month old child with scimitar syndrome and...

  • Anatomy of Congenital Heart Disease Lesions Associated With Pulmonary Arterial Hypertension. Kiefer, Todd L.; Bashore, Thomas // Advances in Pulmonary Hypertension;Winter2013, Vol. 11 Issue 4, p166 

    Adult congenital heart disease represents a growing population of patients. Many patients survive to adulthood and lead functional, productive lives. In fact, there are more adults living with congenital heart disease than pediatric patients. Many adult patients will have had prior surgical...

  • Advanced pulmonary vascular disease: the Eisenmenger syndrome. Kumar, R. Krishna; Sandoval, Julio // Cardiology in the Young;May2009, Vol. 19 Issue S1, p39 

    The article offers information concerning Eisenmenger syndrome, an advanced pulmonary vascular disease. It is viewed as a multisystem disease affecting multiple organ systems as a result of chronic hypoxia and heart failure. Common symptoms of such syndrome include dyspnea, chest pain, cyanosis...

  • Endothelin Receptor Antagonists are an Effective Long Term Treatment Option in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease With or Without Trisomy 21 Kermeen, F.D.; Franks, C.; O’Brien, K.; Seale, H.; Hall, K.; McNeil, K.; Radford, D. // Heart, Lung & Circulation;Oct2010, Vol. 19 Issue 10, p595 

    Introduction: Traditionally, treatment options for patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) are limited. Bosentan has been shown to improve pulmonary haemodynamics and exercise tolerance short term but long term clinical studies are...

Share

Read the Article

Courtesy of THE LIBRARY OF VIRGINIA

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics