MRI shows limited mixing between systemic and pulmonary circulations in foetal transposition of the great arteries: a potential cause of in utero pulmonary vascular disease

Cardiol Young. 2015 Apr;25(4):737-44. doi: 10.1017/S1047951114000870. Epub 2014 Jun 16.

Abstract

Objectives: To investigate the relationship between foetal haemodynamics and postnatal clinical presentation in patients with transposition of the great arteries using phase-contrast cardiovascular magnetic resonance.

Background: A severe and irreversible form of persistent pulmonary hypertension of the newborn occurs in up to 5% of patients with transposition and remains an important cause of morbidity and mortality in these infants. Restriction at the foramen ovale and ductus arteriosus has been identified as a risk factor for the development of pulmonary hypertension, and this can now be studied with magnetic resonance imaging using a new technique called metric optimised gating.

Methods: Blood flow was measured in the major vessels of four foetuses with transposition with intact ventricular septum (gestational age range: 35-38 weeks) and compared with values from 12 normal foetuses (median gestational age: 37 weeks; range: 34-40 weeks).

Results: We found significantly reduced flows in the ductus arteriosus (p<0.01) and foramen ovale (p=0.03) and increased combined ventricular output (p=0.01), ascending aortic (p=0.001), descending aortic (p=0.03), umbilical vein (p=0.03), and aorto-pulmonary collateral (p<0.001) flows in foetuses with transposition compared with normals. The foetus with the lowest foramen ovale shunt and highest aorto-pulmonary collateral flow developed fatal pulmonary vascular disease.

Conclusions: We found limited mixing between the systemic and pulmonary circulations in a small group of late-gestation foetuses with transposition. We propose that the resulting hypoxia of the pulmonary circulation could be the driver behind increased aorto-pulmonary collateral flow and contribute to the development of pulmonary vascular disease in some foetuses with transposition.

Keywords: circulation.

MeSH terms

  • Case-Control Studies
  • Female
  • Gestational Age
  • Hemodynamics
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / congenital
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Ontario
  • Pregnancy
  • Pulmonary Artery / abnormalities
  • Pulmonary Artery / physiopathology*
  • Pulmonary Circulation / physiology*
  • Pulmonary Veno-Occlusive Disease / complications
  • Pulmonary Veno-Occlusive Disease / congenital
  • Regional Blood Flow / physiology
  • Transposition of Great Vessels / complications*
  • Transposition of Great Vessels / physiopathology*
  • Transposition of Great Vessels / surgery
  • Treatment Outcome