Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome

Michele D'Alto, Emanuele Romeo, Paola Argiento, Antonello D'Andrea, Berardo Sarubbi, Anna Correra, Giancarlo Scognamiglio, Silvia Papa, Eduardo Bossone, Raffaele Calabrò, Carmine D. Vizza, Maria G. Russo

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29 Citations (Scopus)

Abstract

Background: Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without Down's syndrome. Methods: WHO functional class, resting oxygen saturation, 6-minute walk test (6MWT) and hemodynamics were assessed at baseline and after 12 months of bosentan therapy in patients with CHD-related PAH with and without Down's syndrome. Results: Seventy-four consecutive patients were enrolled: 18 with and 56 without Down's syndrome. After 12 months of bosentan therapy, both with and without Down's syndrome patients showed an improvement in WHO functional class (Down: 2.5 ± 0.5 vs 2.9 ± 0.6, p = 0.005; controls: 2.5 ± 0.5 vs 2.9 ± 0.5, p = 0.000002), 6-minute walk distance (Down: 288 ± 71 vs 239 ± 74 m, p = 0.0007; controls: 389 ± 80 vs 343 ± 86 m, p = 0.00003), and hemodynamics (pulmonary flow, Down: 4.0 ± 1.6 vs 3.5 ± 1.4 l/m/m2, p = 0.006; controls: 3.5 ± 1.4 vs 2.8 ± 1.0 l/m/m2, p = 0.0005; pulmonary to systemic flow ratio, Down: 1.4 ± 0.7 vs 1.0 ± 0.4, p = 0.003; controls: 1.1 ± 0.7 vs 0.9 ± 0.3, p = 0.012; pulmonary vascular resistance index, Down: 15 ± 9 vs 20 ± 13 WU m2, p = 0.007; controls: 20 ± 10 vs 26 ± 15 WU m 2, p = 0.002). No differences in the efficacy of therapy were observed between the two groups. Conclusions: Bosentan was safe and well tolerated in adult patients with CHD-related PAH with and without Down's syndrome during 12 months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome.

Original languageEnglish
Pages (from-to)323-326
Number of pages4
JournalInternational Journal of Cardiology
Volume164
Issue number3
DOIs
Publication statusPublished - Apr 15 2013

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Down Syndrome
Pulmonary Hypertension
Heart Diseases
Hemodynamics
Lung
Therapeutics
Exercise Tolerance
Vascular Resistance
bosentan
Oxygen

Keywords

  • Congenital heart disease
  • Down's syndrome
  • Pulmonary arterial hypertension

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

D'Alto, M., Romeo, E., Argiento, P., D'Andrea, A., Sarubbi, B., Correra, A., ... Russo, M. G. (2013). Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome. International Journal of Cardiology, 164(3), 323-326. https://doi.org/10.1016/j.ijcard.2011.07.009

Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome. / D'Alto, Michele; Romeo, Emanuele; Argiento, Paola; D'Andrea, Antonello; Sarubbi, Berardo; Correra, Anna; Scognamiglio, Giancarlo; Papa, Silvia; Bossone, Eduardo; Calabrò, Raffaele; Vizza, Carmine D.; Russo, Maria G.

In: International Journal of Cardiology, Vol. 164, No. 3, 15.04.2013, p. 323-326.

Research output: Contribution to journalArticle

D'Alto, M, Romeo, E, Argiento, P, D'Andrea, A, Sarubbi, B, Correra, A, Scognamiglio, G, Papa, S, Bossone, E, Calabrò, R, Vizza, CD & Russo, MG 2013, 'Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome', International Journal of Cardiology, vol. 164, no. 3, pp. 323-326. https://doi.org/10.1016/j.ijcard.2011.07.009
D'Alto, Michele ; Romeo, Emanuele ; Argiento, Paola ; D'Andrea, Antonello ; Sarubbi, Berardo ; Correra, Anna ; Scognamiglio, Giancarlo ; Papa, Silvia ; Bossone, Eduardo ; Calabrò, Raffaele ; Vizza, Carmine D. ; Russo, Maria G. / Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome. In: International Journal of Cardiology. 2013 ; Vol. 164, No. 3. pp. 323-326.
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abstract = "Background: Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without Down's syndrome. Methods: WHO functional class, resting oxygen saturation, 6-minute walk test (6MWT) and hemodynamics were assessed at baseline and after 12 months of bosentan therapy in patients with CHD-related PAH with and without Down's syndrome. Results: Seventy-four consecutive patients were enrolled: 18 with and 56 without Down's syndrome. After 12 months of bosentan therapy, both with and without Down's syndrome patients showed an improvement in WHO functional class (Down: 2.5 ± 0.5 vs 2.9 ± 0.6, p = 0.005; controls: 2.5 ± 0.5 vs 2.9 ± 0.5, p = 0.000002), 6-minute walk distance (Down: 288 ± 71 vs 239 ± 74 m, p = 0.0007; controls: 389 ± 80 vs 343 ± 86 m, p = 0.00003), and hemodynamics (pulmonary flow, Down: 4.0 ± 1.6 vs 3.5 ± 1.4 l/m/m2, p = 0.006; controls: 3.5 ± 1.4 vs 2.8 ± 1.0 l/m/m2, p = 0.0005; pulmonary to systemic flow ratio, Down: 1.4 ± 0.7 vs 1.0 ± 0.4, p = 0.003; controls: 1.1 ± 0.7 vs 0.9 ± 0.3, p = 0.012; pulmonary vascular resistance index, Down: 15 ± 9 vs 20 ± 13 WU m2, p = 0.007; controls: 20 ± 10 vs 26 ± 15 WU m 2, p = 0.002). No differences in the efficacy of therapy were observed between the two groups. Conclusions: Bosentan was safe and well tolerated in adult patients with CHD-related PAH with and without Down's syndrome during 12 months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome.",
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AU - D'Alto, Michele

AU - Romeo, Emanuele

AU - Argiento, Paola

AU - D'Andrea, Antonello

AU - Sarubbi, Berardo

AU - Correra, Anna

AU - Scognamiglio, Giancarlo

AU - Papa, Silvia

AU - Bossone, Eduardo

AU - Calabrò, Raffaele

AU - Vizza, Carmine D.

AU - Russo, Maria G.

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N2 - Background: Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without Down's syndrome. Methods: WHO functional class, resting oxygen saturation, 6-minute walk test (6MWT) and hemodynamics were assessed at baseline and after 12 months of bosentan therapy in patients with CHD-related PAH with and without Down's syndrome. Results: Seventy-four consecutive patients were enrolled: 18 with and 56 without Down's syndrome. After 12 months of bosentan therapy, both with and without Down's syndrome patients showed an improvement in WHO functional class (Down: 2.5 ± 0.5 vs 2.9 ± 0.6, p = 0.005; controls: 2.5 ± 0.5 vs 2.9 ± 0.5, p = 0.000002), 6-minute walk distance (Down: 288 ± 71 vs 239 ± 74 m, p = 0.0007; controls: 389 ± 80 vs 343 ± 86 m, p = 0.00003), and hemodynamics (pulmonary flow, Down: 4.0 ± 1.6 vs 3.5 ± 1.4 l/m/m2, p = 0.006; controls: 3.5 ± 1.4 vs 2.8 ± 1.0 l/m/m2, p = 0.0005; pulmonary to systemic flow ratio, Down: 1.4 ± 0.7 vs 1.0 ± 0.4, p = 0.003; controls: 1.1 ± 0.7 vs 0.9 ± 0.3, p = 0.012; pulmonary vascular resistance index, Down: 15 ± 9 vs 20 ± 13 WU m2, p = 0.007; controls: 20 ± 10 vs 26 ± 15 WU m 2, p = 0.002). No differences in the efficacy of therapy were observed between the two groups. Conclusions: Bosentan was safe and well tolerated in adult patients with CHD-related PAH with and without Down's syndrome during 12 months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome.

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