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The Belgian Eisenmenger syndrome registry: Implications for treatment strategies?

(2009) ACTA CARDIOLOGICA. 64(4). p.447-453
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Organization
Abstract
Objective - Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD), usually results from a systemic-to-pulmonary shunt. Eisenmenger syndrome (ES) is characterised by severe irreversible PAH and reversal of a previous systemic-to-pulmonary shunt.A national registry of ES patients was initiated to optimise patient care and to provide epidemiological information regarding PAH and CHD in Belgium. Methods - All ES patients, older than 18 years, were selected through the local databases of ten centres in Belgium. After written informed consent, demographic, clinical, biochemical, technical, and treatment data were entered into the web-based registry. Results - Ninety-one patients were included in the registry. Mean age was 36 +/- 11 years (range 18-59 years). Complete atrioventricular septal defect (N = 26, 28.6%), followed by ventricular septal defect (N = 25, 27.5%) were the commonest defects. Forty-five percent were patients with Down syndrome. Down patients were younger (32 +/- 9 versus 40 +/- 12 years; P = 0.039), had worse functional capacity (class II/III ratio: 15/16 versus 21/8; P = 0.035) and received significantly less specific PAH treatment (7% versus 38%; P = 0.002). Conclusion - Through the national Eisenmenger registry, 91 adult patients with ES were identified (estimated prevalence 11 per million inhabitants). Almost half of them were Down patients. Although having worse functional capacity, significantly less Down patients were receiving specific PAH treatment.
Keywords
GUIDELINES, ADULTS, CONGENITAL-HEART-DISEASE, PULMONARY ARTERIAL-HYPERTENSION, ATRIOVENTRICULAR SEPTAL-DEFECT, VASCULAR-DISEASE, DOWN-SYNDROME, DEATH, BOSENTAN THERAPY, NATIONAL REGISTRY

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MLA
Van de Bruaene, Alexander et al. “The Belgian Eisenmenger Syndrome Registry: Implications for Treatment Strategies?” ACTA CARDIOLOGICA 64.4 (2009): 447–453. Print.
APA
Van de Bruaene, A., Delcroix, M., Pasquet, A., De Backer, J., De Pauw, M., Naeije, R., Vachiery, jean-L., et al. (2009). The Belgian Eisenmenger syndrome registry: Implications for treatment strategies? ACTA CARDIOLOGICA, 64(4), 447–453.
Chicago author-date
Van de Bruaene, Alexander, Marion Delcroix, Agnes Pasquet, Julie De Backer, Michel De Pauw, Robert Naeije, jean-Luc Vachiery, Bernard Paelinck, Marielle Morissens, and Werner Budts. 2009. “The Belgian Eisenmenger Syndrome Registry: Implications for Treatment Strategies?” Acta Cardiologica 64 (4): 447–453.
Chicago author-date (all authors)
Van de Bruaene, Alexander, Marion Delcroix, Agnes Pasquet, Julie De Backer, Michel De Pauw, Robert Naeije, jean-Luc Vachiery, Bernard Paelinck, Marielle Morissens, and Werner Budts. 2009. “The Belgian Eisenmenger Syndrome Registry: Implications for Treatment Strategies?” Acta Cardiologica 64 (4): 447–453.
Vancouver
1.
Van de Bruaene A, Delcroix M, Pasquet A, De Backer J, De Pauw M, Naeije R, et al. The Belgian Eisenmenger syndrome registry: Implications for treatment strategies? ACTA CARDIOLOGICA. BRUSSELS: ACTA CARDIOLOGICA; 2009;64(4):447–53.
IEEE
[1]
A. Van de Bruaene et al., “The Belgian Eisenmenger syndrome registry: Implications for treatment strategies?,” ACTA CARDIOLOGICA, vol. 64, no. 4, pp. 447–453, 2009.
@article{889442,
  abstract     = {Objective - Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD), usually results from a systemic-to-pulmonary shunt. Eisenmenger syndrome (ES) is characterised by severe irreversible PAH and reversal of a previous systemic-to-pulmonary shunt.A national registry of ES patients was initiated to optimise patient care and to provide epidemiological information regarding PAH and CHD in Belgium.

Methods - All ES patients, older than 18 years, were selected through the local databases of ten centres in Belgium. After written informed consent, demographic, clinical, biochemical, technical, and treatment data were entered into the web-based registry.

Results - Ninety-one patients were included in the registry. Mean age was 36 +/- 11 years (range 18-59 years). Complete atrioventricular septal defect (N = 26, 28.6%), followed by ventricular septal defect (N = 25, 27.5%) were the commonest defects. Forty-five percent were patients with Down syndrome. Down patients were younger (32 +/- 9 versus 40 +/- 12 years; P = 0.039), had worse functional capacity (class II/III ratio: 15/16 versus 21/8; P = 0.035) and received significantly less specific PAH treatment (7% versus 38%; P = 0.002).

Conclusion - Through the national Eisenmenger registry, 91 adult patients with ES were identified (estimated prevalence 11 per million inhabitants). Almost half of them were Down patients. Although having worse functional capacity, significantly less Down patients were receiving specific PAH treatment.},
  author       = {Van de Bruaene, Alexander and Delcroix, Marion and Pasquet, Agnes and De Backer, Julie and De Pauw, Michel and Naeije, Robert and Vachiery, jean-Luc and Paelinck, Bernard and Morissens, Marielle and Budts, Werner},
  issn         = {0001-5385},
  journal      = {ACTA CARDIOLOGICA},
  keywords     = {GUIDELINES,ADULTS,CONGENITAL-HEART-DISEASE,PULMONARY ARTERIAL-HYPERTENSION,ATRIOVENTRICULAR SEPTAL-DEFECT,VASCULAR-DISEASE,DOWN-SYNDROME,DEATH,BOSENTAN THERAPY,NATIONAL REGISTRY},
  language     = {eng},
  number       = {4},
  pages        = {447--453},
  publisher    = {ACTA CARDIOLOGICA},
  title        = {The Belgian Eisenmenger syndrome registry: Implications for treatment strategies?},
  url          = {http://dx.doi.org/10.2143/AC.64.4.2041608},
  volume       = {64},
  year         = {2009},
}

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