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Prenatal diagnosis of pulmonary atresia: impact on clinical presentation and early outcome
  1. Aphrodite Tzifa1,
  2. Claire Barker2,
  3. Shane M Tibby2,
  4. John M Simpson1
  1. 1Department of Congenital Heart Disease, Guy’s and St Thomas’ Hospital, London, UK
  2. 2Department of Paediatric Intensive Care, Guy’s and St Thomas’ Hospital, London, UK
  1. Correspondence to:
    Dr John Simpson
    Department of Congenital Heart Disease, Evelina Children’s Hospital, St Thomas’ Hospital, Lambeth Palace Road, SE1 7EH, London, UK; john.simpson{at}


Aim: The impact of prenatal diagnosis on morbidity and mortality for certain types of congenital heart disease (obstructive left heart lesions and transposition of the great arteries) is well established. No data are available for lesions with duct dependent pulmonary flow. We aimed to assess the impact of prenatal diagnosis of pulmonary atresia on clinical presentation and neonatal outcome.

Method: Fifty-eight newborns with pulmonary atresia presenting to our centre were identified retrospectively between 1997 and 2004 (prenatal diagnosis n = 37, postnatal n = 21). Anatomical sub-types included intact ventricular septum (PAIVS, n = 33) and ventricular septal defect (PAVSD, n = 25); those with more complex anatomy were excluded.

Results: After adjusting for anatomical sub-type, postnatally diagnosed infants were significantly more hypoxic at presentation (mean oxygen saturation 65% vs 84%). However, they presented early (median age 1 day) and prostaglandin E was initiated promptly (median 3 hours) with rapid improvement of oxygen saturations (interaction p<0.001). This resulted in no appreciable differences in terms of pH, base deficit, blood pressure or heart rate between the groups by the time of the first catheter/surgical intervention. Postnatal infants did not differ in terms of length of intensive care unit (p = 0.18) or hospital stay (p = 0.86), incidence of complications (p = 0.72), or mortality (p = 0.77). Multivariable analysis revealed a positive association between occurrence of complications and both degree of cyanosis at presentation (rather than postnatal diagnosis per se) and anatomy (PAIVS).

Conclusion: Postnatal diagnosis of pulmonary atresia is associated with greater cyanosis at presentation. However this does not translate into greater neonatal morbidity or mortality provided that early recognition and prompt initiation of prostaglandin E therapy occur.

  • ANCOVA, analysis of covariance
  • HLHS, hypoplastic left heart syndrome
  • ICU, intensive care unit
  • PAIVS, pulmonary atresia with an intact ventricular septum
  • PAVSD, pulmonary atresia with ventricular septal defect
  • TGA, transposition of the great arteries

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  • Published Online First 13 July 2006

  • Competing interests: None.

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