LATE FENESTRATION CLOSURE IN THE HYPOPLASTIC LEFT-HEART SYNDROME - COMPARISON OF HEMODYNAMIC-CHANGES

Background and Objectives Although survival of patients with the hypop lastic left heart syndrome treated by staged surgical palliation has i mproved, hemodynamic data after fenestrated Fontan operation and after fenestration closure have not been reported in this patient populatio n. We sought to describe the hemodynamic status of these patients at c ardiac catheterization performed For the purpose of fenestration closu re and to compare these data with data from contemporary patients with other forms of univentricular heart. Methods and Results Hemodynamic responses to fenestration closure during cardiac catheterization were reviewed in 40 consecutive patients, including 20 with the hypoplastic left heart syndrome and 20 with other forms of univentricular heart d efects. Hemodynamics before fenestration closure (arterial saturation and pressure, Fontan baffle saturation and pressure, pulmonary capilla ry wedge pressure, systemic arteriovenous oxygen content difference, a nd right-to-left shunt fraction) were nearly identical between the two groups. Significant (p < 0.05) changes after Fenestration closure inc luded increases in arterial saturation (9%), mean arterial pressure (3 mm Hg), and baffle pressure (1 mm Hg) and arteriovenous oxygen conten t difference (18 ml/L), with near elimination of right-to-left shuntin g. Cardiac output decreased by 21% and systemic oxygen transport by 13 %, with no differences between the two patient groups. Mean baffle pre ssures were <17 mm Hg in 32 patients (80%). Conclusions Hemodynamics a fter fenestrated Fontan operation and responses to fenestration closur e in patients with the hypoplastic left heart syndrome were remarkably similar to that in patients with other univentricular heart defects.