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Ductus-associated proximal pulmonary artery stenosis in patients with right heart obstruction

Anita J Moon-Grady1, David F Teitel, Frank L Hanley

  • 1Division of Pediatric Cardiology, University of California, San Francisco, 505 Parnassus Avenue Box 0214, San Francisco, CA 94143, United States.

International Journal of Cardiology
|April 29, 2006
PubMed

Insights

Proximal pulmonary artery stenosis often develops at the ductal insertion site in infants with congenital heart disease, increasing the risk of pulmonary artery hypoplasia. Early detection requires a high index of suspicion.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Vascular Biology

Background:

  • Proximal pulmonary artery stenosis is a frequent acquired condition in infants undergoing treatment for congenital heart disease.
  • Stenosis at the ductal insertion site is hypothesized to be common in patients with right ventricular outflow tract obstruction (RVOTO).

Purpose of the Study:

  • To investigate the incidence of ductal insertion site stenosis in infants with specific congenital heart defects.
  • To determine the association between RVOTO, ductal insertion site stenosis, and ipsilateral pulmonary artery hypoplasia.

Main Methods:

  • Retrospective review of surgical and cardiac catheterization databases (1988-2000).
  • Inclusion criteria: infants (<1 year) diagnosed with pulmonary atresia with intact ventricular septum (PA), tetralogy of Fallot (TOF), or pulmonary stenosis (PS).
  • Analysis of proximal pulmonary artery stenosis associated with the ductal insertion site and distal pulmonary arterial hypoplasia.

Main Results:

  • Proximal pulmonary artery stenosis at the ductal insertion site was identified in 33 infants.
  • This lesion was present in 29% of PA patients, 1% of TOF patients, and 2% of PS patients.
  • Fifty-nine percent of infants with RVOTO and ductal insertion site stenosis also had distal pulmonary arterial hypoplasia.

Conclusions:

  • A significant proportion of infants with PA and RVOTO develop proximal pulmonary artery stenosis at the ductal insertion site.
  • Associated distal pulmonary arterial hypoplasia is common in these patients.
  • Early detection of this lesion necessitates a high index of clinical suspicion as symptomatology is unreliable.

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