The choice of echocardiographic parameters for determining the hemodynamics phenotype in children after Fontan procedure

Aim. Evaluation of echocardiographic parameters to characterize the hemodynamics phenotype in children after Fontan procedure. Methods. 30 children aged 8.06 ± 4.3 years, body surface area 0.95 ± 0.33 m2, 12 boys (40%) with a functionally single ventricle after total cavopulmonary connection with extracardiac conduit fenestration. In addition to traditional echocardiographic parameters we assessed: systemic ventricular preload (stroke volume index, SVI), ventricular afterload (arterial elastance), arterial compliance, end–systolic ventricular elastance (VE), ventricular–arterial coupling (VAC), transpulmonary gradient (TPG). Results. In 33.3% (n = 10) children, the parameters of the single ventricle function were comparable to the normative values in biventricular hemodynamics. In 3 (10%) patients decreased values of the single ventricle systolic function were found (ejection fraction 40.0 ± 3.5%, fraction area changes 44.6 ± 3.5%, longitudinal strain −4.03 ± 0.87%), preload (SVI 13.30 ± 0.64 ml/m2), arterial compliance, (0.47 ± 0.17 ml/mm Hg), increased VAC (1.81 ± 0.10). In 36.6% of children (n = 11), a decrease in preload was found (SVI 27.4 ± 1.2 ml/m2), an increase in ventricular-arterial coupling (2.17 ± 0.16), and a decrease VE (1.98 ± 0.45 mm Hg/ml), an increase E/e´ (13.2 ± 1.7). In 20% of cases (n = 6) an increase in TPG was determined (11.6 ± 0.9 mm Hg). Conclusion. In children after Fontan procedure, 4 hemodynamic phenotypes were revealed in accordance with echocardiographic parameters: compensated, hypocontractile, with impaired diastolic properties of the systemic ventricle and with increased pulmonary vascular resistance. With a hypocontractile phenotype of hemodynamics, a significantly increased concentration of the pro-brain natriuretic peptide is determined. The phenotype of hemodynamics with increased pulmonary vascular resistance is more often associated with a higher functional class of heart failure.