Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease
Peter M Engelfriet, Marielle G J Duffels, Thomas Möller, Eric Boersma, Jan G P Tijssen, Erik Thaulow, Michael A Gatzoulis, Barbara J M Mulder, Peter M Engelfriet, Marielle G J Duffels, Thomas Möller, Eric Boersma, Jan G P Tijssen, Erik Thaulow, Michael A Gatzoulis, Barbara J M Mulder
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 closed, 504 open without and 15 with Eisenmenger's syndrome), 710 with a VSD (275, 352 and 83, respectively), 133 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 13% 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.
Conflict of interest statement
Competing interests: None declared.
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References
- Engelfriet P, Boersma E, Oechslin al The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow‐up period: the Euro Heart Survey on adult congenital heart disease. Eur Heart J 2005262325–2333.
- Engelfriet P, Tijssen J, Kaemmerer al Adherence to guidelines in the clinical care for adults with congenital heart disease: the Euro Heart Survey on adult congenital heart disease. Eur Heart J 200627737–745.
- McQuillan B M, Picard M H, Leavitt al Clinical correlates and reference intervals for pulmonary artery systolic pressure among echocardiographically normal subjects. Circulation 20011042797–2802.
- Barst R J, McGoon M, Torbicki al Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 20044340S–47S.
- Duffels M G J, Engelfriet P M, Berger R M al Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardio December 18 2006 [Epub ahead print]
- Hoffman J I E, Rudolph A M. The natural history of ventricular septal defects in infancy. Am J Cardiol 196516634–653.
- Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. BMJ 19582701–9, 75562.
- Campbell M. Natural history of atrial septal defect. Br Heart J 197032820–826.
- Stein P D, Sabbah H N, Anbe D T, Marzilli M. Performance of the failing and nonfailing right ventricle of patients with pulmonary hypertension. Am J Cardiol 1979441050–1055.
- Daliento L, Somerville J, Presbitero al Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998191845–1855.
- Niwa K, Perloff J K, Kaplan al Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol 199934223–232.
- Cantor W J, Harrison D A, Moussadji J al Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol 199984677–681.
- Saha A, Balakrishnan K G, Jaiswal P al Prognosis for patients with Eisenmenger syndrome of various aetiology. Int J Cardiol 199445199–207.
- Edwards J E. Functional pathology of the pulmonary vascular tree in congenital cardiac disease (the Lewis A. Conner memorial lecture). Circulation 195715164–196.
- Wagenvoort C A, Nauta J, van der Schaar P al Effect of flow and pressure on pulmonary vessels: a semiquantitative study base on lung biopsies. Circulation 1967351028–1037.
Source: PubMed