Abstract

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. 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. 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). 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. 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.

Highlights

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

  • It has been suggested that the normal streaming of well-oxygenated blood from the umbilical vein across the oval foramen would result in increased pulmonary blood flow and oxygen mediated arterial ductal constriction in foetuses with transposition, because in transposition the left ventricle is connected to the pulmonary artery.[5]

  • The increased pulmonary venous return associated with the drop in pulmonary vascular resistance resulting from higher oxygen saturations in the pulmonary arteries might help to explain the reduced oval foramen shunting that we observed in transposition foetuses

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Summary

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. Restriction at the foramen ovale and ductus arteriosus has been identified as a risk factor for the development of pulmonary hypertension, and this can be studied with magnetic resonance imaging using a new technique called metric optimised gating. 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. 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

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