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.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to American Journal of CardiologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
Mietus-Snyder M, Lang P, Mayer JE, Jones RA, Castanda AR, Lock JE. Childhood systemic-pulmonary shunts. Subsequent suitability for Fontan operation (abstr). Circulation 1987;76(suppl III):III–39
- Pulmonary artery configuration after palliative operations for hypoplastic left heart syndrome.J Thorac Cardiovasc Surg. 1989; 97: 878-885
- Hypoplastic left heart syndrome.J Am Coll Cardiol. 1991; 17: 1143-1149
- Transcatheter reconstruction using intravascular stents of acquired long-segment pulmonary artery discontinuity after the hemi-Fontan procedure associated with either congenital mitral valve stenosis or atresia and hypoplastic left ventricle.Am J Cardiol. 2002; 89: 1225-1229
Article info
Publication history
Accepted:
February 27,
2003
Received in revised form:
February 27,
2003
Received:
December 27,
2002
Identification
Copyright
© 2003 Excerpta Medica Inc. Published by Elsevier Inc. All rights reserved.
