| Grant number: | 11/09341-0 |
| Support Opportunities: | Regular Research Grants |
| Start date: | October 01, 2011 |
| End date: | September 30, 2014 |
| Field of knowledge: | Health Sciences - Medicine - Medical Clinics |
| Principal Investigator: | Antonio Augusto Barbosa Lopes |
| Grantee: | Antonio Augusto Barbosa Lopes |
| Host Institution: | Instituto do Coração Professor Euryclides de Jesus Zerbini (INCOR). Hospital das Clínicas da Faculdade de Medicina da USP (HCFMUSP). Secretaria da Saúde (São Paulo - Estado). São Paulo , SP, Brazil |
| Associated researchers: | Ana Maria Thomaz ; Arlindo de Almeida Riso ; Filomena Regina Barbosa Gomes Galas ; Marcelo Biscegli Jatene ; Nair Yukie Maeda ; Rogério dos Anjos Miranda ; Rosangela de Paula Silva Soares ; Sergio Paulo Bydlowski ; Vera Demarchi Aiello |
Abstract
Pulmonary arterial hypertension (PAH) is a complicating factor in the management of congenital heart disease (CHD) with intracardiac or extracardiac shunts. In children with moderate to severe PAH, the risk of serious complications following the surgical repair of shunts (including right cardiac failure and death) is 15% to 20%, and the risk of late, postoperative residual PAH is up to 25%. We therefore intend to conduct a study aimed at: 1- reducing the risk of immediate postoperative complications to less than 10%; 2- reducing the likelihood of late postoperative (residual) PAH to less than 10% in children with moderate PAH; 3- promoting a significant reduction in pulmonary artery pressure and vascular resistance, at six month postoperatively, in children with moderate to severe PAH. We hypothesized that these goals could be achieved by treating patients preoperatively and for six months postoperatively with sildenafil, either singly or combined with bosentan. Both drugs have been approved for treatment of PAH on the basis of randomized clinical trials. Preoperative and postoperative (on treatment) hemodynamic evaluation will be based on noninvasive and invasive diagnostic procedures. As a second objective, we intend to analyse possible abnormalities in three genes that have been associated with PAH in CHD, and 36 inflammatory mediators. The idea is to investigate whether changes in these biomarkers correlate with the response to combined medical and surgical treatments. (AU)
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