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Case report
Ebstein’s anomaly with ‘reversible’ functional pulmonary atresia
  1. João Rato1,
  2. Ana R Sousa1,
  3. Ana Teixeira1 and
  4. Rui Anjos2
  1. 1Pediatric Cardiology, Centro Hospitalar de Lisboa Ocidental EPE, Carnaxide, Lisboa, Portugal
  2. 2Pediatric Cardiology, Hospital Santa Cruz, Carnaxide, Portugal
  1. Correspondence to Dr João Rato; joaorato4{at}gmail.com

Abstract

We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein’s anomaly without anterograde flow from right ventricular to pulmonary atresia (PA)—functional PA with flow reversal in the ductus arteriosus. Prostaglandin E1 was started after birth. Chest X-ray showed severe cardiomegaly and echocardiogram confirmed Ebstein’s anomaly with a thickened non-opening pulmonary valve without anterograde flow but with mild regurgitation. Multidisciplinary team decision was to progressively reduce prostaglandins and have an expectant attitude. Peripheral oxygen saturation above 85% was maintained and serial echocardiograms documented progressive reduction of the ductus arteriosus and the opening of the pulmonic valve cusps, with the development of anterograde flow. The newborn was discharged at day 19 of life without the need for any intervention, and at last follow-up remains asymptomatic, with anterograde normal flow in the pulmonary valve.

  • congenital disorders
  • materno-fetal medicine
  • cardiovascular medicine
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Footnotes

  • Contributors JR authored the paper. ARS, AT and RA helped in its planning and carefully reviewed it.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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