Fetal case of rare association of hypoplastic left heart syndrome and absent atrial septum accompanied by mixed form of supracardiac total anomalous pulmonary venous connection

Echocardiography. 2022 Feb;39(2):375-380. doi: 10.1111/echo.15272. Epub 2022 Jan 6.

Abstract

Abnormal pulmonary venous flow patterns on fetal echocardiography and a nutmeg lung pattern on fetal magnetic resonance imaging are seen in patients with pulmonary venous stenosis. The association between these findings and the degree of pulmonary venous stenosis remains unknown. We report an extremely rare case of a fetus diagnosed with hypoplastic left heart syndrome complicated by an absent atrial septum and supracardiac total anomalous pulmonary venous connection with left pulmonary venous congestion. This case suggests that compared to non-pulsatile continuous pulmonary venous flow, the nutmeg lung pattern can only be observed with severe pulmonary congestion and advanced pulmonary lymphangiectasia.

Keywords: absent atrial septum; case report; hypoplastic left heart syndrome; supracardiac total anomalous pulmonary venous connection.

Publication types

  • Case Reports

MeSH terms

  • Atrial Septum*
  • Fetus
  • Humans
  • Hypoplastic Left Heart Syndrome* / complications
  • Hypoplastic Left Heart Syndrome* / diagnostic imaging
  • Pulmonary Veins* / abnormalities
  • Scimitar Syndrome* / complications
  • Scimitar Syndrome* / diagnostic imaging