Association of asymmetric pulmonary artery growth following palliative surgery for hypoplastic left heart syndrome with ductal coarctation, neoaortic arch compression, and shunt-induced pulmonary artery stenosis

  • Howard S. Weber
    Dr. Weber’s address is: Department of Pediatrics (Cardiology), The Pennsylvania State University Children’s Hospital, Hershey, Pennsylvania 17033, USA.
    From the Departments of Department ofPediatrics (Cardiology), Hershey, Pennsylvania USA
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  • John L. Myers
    Department ofCardiothoracic Surgery, The Penn State University Children’s Hospital at the Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA
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      Survival after the Norwood stage I procedure for hypoplastic left heart syndrome has improved dramatically over the past decade, with many children proceeding to a successful Fontan-type operation. An important variable leading to “ideal” Fontan physiology is the presence of large unobstructed right and left pulmonary arteries that are preferably of equal size. In the case of hypoplastic left heart syndrome, the right and left branch pulmonary arteries are typically symmetric and of normal size immediately before the Norwood stage I procedure. However, palliative surgery for hypoplastic left heart syndrome requires placement of a systemic-to-pulmonary artery shunt, typically to the right pulmonary artery, which may result in stenosis and reconstruction of the neoaortic arch that lies superior to the left pulmonary artery. This in turn may lead to compression and ligation of the ductus arteriosus that can result in stenosis at the ductal insertion site. Therefore, these aspects of the Norwood stage I procedure may alter the symmetric growth pattern of the branch pulmonary arteries. This report retrospectively examines the incidence of and factors responsible for pulmonary artery growth abnormalities in a uniform cohort of patients with hypoplastic left heart syndrome who underwent operation at our institution.
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