Background: Patients with repaired coarctation of the aorta retain abnormal elastic properties of the aorta. It is not known whether patients with hypoplastic left heart syndrome also manifest abnormal elastic properties after palliative surgery. The presence of such abnormalities may have important clinical implications as reduced aortic compliance might adversely impact single right ventricular function.
Methods: We prospectively evaluated the elastic properties of the aorta in a cohort of patients with hypoplastic left heart syndrome who had undergone the Norwood procedure with aortic arch reconstruction and subsequent bidirectional Glenn or Fontan procedure. The hypoplastic left heart syndrome patients (n = 20) were compared with single-ventricle patients (n = 18) without history of arch reconstruction and patients with double-ventricular lesions (n = 22). Aortic elastic function was quantified by distensibility index and stiffness index. M-mode measurements of the transverse aortic arch were obtained with transesophageal echocardiography under general anesthesia. Patients were evaluated at a median age of 22.2 months with no age difference between patient subgroups.
Results: Distensibility index was significantly less (p = 0.007) and stiffness index greater (p = 0.005) in the reconstructed arch of hypoplastic left heart syndrome patients compared with single-ventricle and double-ventricle patients.
Conclusions: Patients with hypoplastic left heart syndrome after Norwood palliation have increased aortic stiffness and decreased distensibility in the reconstructed transverse arch. As previous studies in adults have shown that decreased aortic compliance increases the energy cost of cardiac ejection, examination of modifications to the surgical technique that might improve elastic properties is warranted.