There is no pumping subpulmonary ventricle in patients with univentricular hemodynamics after Fontan type physiological correction of complex congenital heart defects (CHD). Hence the blood &#64258; ow to the lungs is passive and depends on central venous pressure (CVP) and pulmonary vascular resistance (PVR) to provide adequate &#64257; lling of the single ventricle. The increase in PVR leads to a reduction in ventricular &#64257; lling and cardiac output, resulting in failure of the Fontan circulation (the so-called failing Fontan). Purpose. Invasive assessment of the hemodynamic effect of sildena&#64257; l treatment in children with single ventricle Fontan circulation. Material and Methods. Twenty-six children (12 girls, 14 boys) with completed stages of Fontan type surgery in whom a selective pulmonary vasodilator (sildena&#64257; l) has been administered during the follow-up have been investigated. An open-label, non-randomized, prospective invasive study of the hemodynamic effect of sildena&#64257; l treatment in patients with completed Fontan surgery stages was performed. Results. A signi&#64257; cant decrease in the cavopulmonary pressure (from 16.58 &plusmn; 1.88 mm Hg to 13.80 &plusmn; 2.20 mm Hg; p < 0.001) and the pulmonary vascular resistance (from 2.02 &plusmn; 0.72 WU to 1.42 &plusmn; 0.41 WU; p = 0.001), increase of the pulmonary/systemic blood &#64258; ow ratio (from 0.71 &plusmn; 0.21 to 0.83 &plusmn; 0.18; p < 0.05), as well as increase in the systemic oxygen saturation (from 85.65 &plusmn; 7.48% to 90.72 &plusmn; 4.53%; p = 0.005) were found. No signi&#64257; cant difference in hemodynamic parameters related to the ventricular morphology and the type of previous palliation was found. The only exception was the pulmonary blood &#64258; ow after sildena&#64257; l treatment,&nbsp;&nbsp;which was signi&#64257; cantly higher in children with &#1072; previous systemic to pulmonary shunt compared to children with &#1072; previous banding (p < 0.05). Conclusion. Our study showed signi&#64257; cant bene&#64257; cial changes in the main hemodynamic parameters after sildena&#64257; l treatment. The pharmacological modulation of the pulmonary vascular status is an important component of the treatment of patients with Fontan circulation.
Read full abstract