The efficiency of a specific treatment in a child with pulmonary arterial hypertension

Abstract

Introduction- Pulmonaryarterial hypertension (PAH) is a severe disease that requests modern methods of diagnosis as cardiac catheterization and a timely initiated specific treatment with Bosentan and Sildenafil contributes to a significant improvement of the health condition. Purpose- To present a case report of a child with PAH treated with Bosentan and Sildenafil with dynamic evaluation of the efficiency criteria. Material and methods- A 5 years old child was diagnosed by ECHO and CT with CHD, PAD (7 mm length and 9*7 mm in diameter) and severe PAH (mPAP- 70 mm/Hg). According to cardiac catheterization data PVR/SVRratio - 1.03, the surgery is questionable. Initiated Sildenafil 10 mg 3 times/day and Bosentan 16 mg 2 times/day gives us better results at 6 and 12 months for following criteria: FC WHO/NYHA, Oxygen saturation 6MWT (6-min walk test) mPAP (mean pulmonary artery pressure) PVR (pulmonary vascular resistance) Conclusions- The combined and specific treatment with Bosentan and Sildenafil initiated in children with PAH reveals a significant positive dynamic of the efficiency criteria.