Anomalous hepatic drainage and platypnea-orthodeoxia in cyanotic children

Cardiol Young. 2023 Dec;33(12):2685-2689. doi: 10.1017/S1047951123003712. Epub 2023 Oct 25.

Abstract

The aetiology of cyanosis could be unclear in children, even for specialised paediatricians. Two cases were reported: first, a 6-year-old child with features of left isomerism and Fallot was fortuitously diagnosed with anomalous hepatic venous drainage before complete repair. Second, a newborn with an antenatal diagnosis of ductus venosus agenesis had an isolated intermittent right-to-left atrial shunt when upright, with favourable outcome, in contrast to the association with significant heart malformations including inferior caval vein interruption. Multimodality imaging and 3D printing helped to rule out extracardiac causes of persistent cyanosis and plan surgical repair.

Keywords: CHD; Paediatric; arantius; baffle; hepatic vein; saturation.

MeSH terms

  • Child
  • Cyanosis / diagnosis
  • Cyanosis / etiology
  • Dyspnea / complications
  • Female
  • Heterotaxy Syndrome* / complications
  • Humans
  • Infant, Newborn
  • Platypnea Orthodeoxia Syndrome
  • Pregnancy
  • Pulmonary Veins* / abnormalities
  • Vascular Malformations* / complications