Background: Late complications after Fontan procedure may be due to the absence of pump and pulsatile pulmonary blood flow in this type of palliation. Our aim was to quantify the degree of pulsation by echocardiographic method in patients with extracardiac total cavopulmonary connection (ECTCPC) in comparison with biventricular circulation and few cases of pulsatile Fontan.
Methods: In a case series study, pulsatility index (PI) derived by echocardiographic method were compared between 20 patients with ECTCPC, 6 patients with pulsatile Fontan and 18 normal individual aged 4 to 20 years old. All patients were in New York Heart Association class of I and there was no report of complication.
Results: In patients with ECTCPC pulmonary artery branches Doppler flow study showed lower peak and mean velocities compared to the pulsatile Fontan and normal groups. ECTCPC patients had PI of 0.59 ± 0.14 and 0.59 ± 0.09 for right and left pulmonary arteries (RPA and LPA) respectively. PI was higher in patients with preserved antegrade flow (RPA PI = 0.94 ± 0.26, LPA PI = 0.98 ± 0.27) and in normal individuals (RPA PI = 1.59 ± 0.12, LPA PI = 1.64 ± 0.17) for both branches (p = 0.000).
Conclusion: Using a Doppler derived index for pulsatility, patients with ECTCPC had the least pulsation. The pulmonary artery flow pattern in patients with preserved antegrade flow showed higher pulsatility indices in both branches. Normal individuals had the greatest pulsatility index.
Keywords: Cavopulmonary connection; Doppler flow velocity; Pulsatility index.