Paeds Vivas · fetal-neonatal-and-perinatal
Congenital heart disease presenting in the newborn — branching viva
Branching viva from the cyanotic-versus-duct-dependent bedside split, through the day-three collapsed neonate, the positive pulse-oximetry screen, and the prostaglandin-first resuscitation and its pitfalls.
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Target exams
RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
You are the paediatric registrar covering the postnatal ward and the neonatal unit. The midwife asks you to review three newborns: a cyanotic baby found on pulse-oximetry screening, a previously well baby who has collapsed on day three, and a baby who has just started prostaglandin E1 after a positive assessment. The examiner releases information in stages.
Station opening
Examiner: "Tell me the single bedside decision that frames every newborn with suspected congenital heart disease, and why it matters." [6]
Strong candidate (must-hit)
- The decision is whether the lesion is duct-dependent: whether systemic or pulmonary blood flow can only occur through an open ductus arteriosus. It matters because the ductus closes functionally over 24 to 48 hours, so a duct-dependent baby who looks well initially will collapse — with shock (left obstruction) or cyanosis (right obstruction) — as the duct closes. Making that call early and starting prostaglandin before the echo is the difference between a planned transfer and an arrest. [6]
Weak candidate
- "We look for a murmur and do an echocardiogram." [6]
References7ShowHide
- [1]de-Wahl Granelli A; Wennergren M; Sandberg K; et al Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: a Swedish prospective screening study in 39,821 newborns. BMJ, 2009.PMID 19131383
- [2]Thangaratinam S; Brown K; Zamora J; et al Pulse oximetry screening for critical congenital heart defects in asymptomatic newborn babies: a systematic review and meta-analysis. Lancet, 2012.PMID 22554860
- [4]Mahle WT; Martin GR; Beekman RH 3rd; et al Endorsement of Health and Human Services recommendation for pulse oximetry screening for critical congenital heart disease. Pediatrics, 2012.PMID 22201143
- [6]Wren C; Reinhardt Z; Khawaja K Twenty-year trends in diagnosis of life-threatening neonatal cardiovascular malformations. Arch Dis Child Fetal Neonatal Ed, 2008.PMID 17556383
- [7]Ailes EC; Gilboa SM; Honein MA; et al Estimated number of infants detected and missed by critical congenital heart defect screening. Pediatrics, 2015.PMID 25963011
- [9]Browning Carmo KA; Barr P; West M; et al Transporting newborn infants with suspected duct dependent congenital heart disease on low-dose prostaglandin E1 without routine mechanical ventilation. Arch Dis Child Fetal Neonatal Ed, 2007.PMID 16905574
- [10]Escobar-Diaz MC; Freud LR; Bueno A; et al Prenatal diagnosis of transposition of the great arteries over a 20-year period: improved but imperfect. Ultrasound Obstet Gynecol, 2015.PMID 25484180