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

TY - JOUR

T1 - MRI shows limited mixing between systemic and pulmonary circulations in foetal transposition of the great arteries

T2 - a potential cause of in utero pulmonary vascular disease

AU - Porayette, Prashob

AU - van Amerom, Joshua

AU - Yoo, Shi-Joon

AU - Jaeggi, Edgar

AU - Macgowan, Christopher K

AU - Seed, Mike

PY - 2014

Y1 - 2014

N2 - 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.

AB - 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.

KW - received

KW - operation for transposition of

KW - arteries

KW - magnetic resonance imaging

KW - however

KW - circulation

KW - arteries are now excellent

KW - regional blood fl ow

KW - the great

KW - transposition of the great

KW - up to

KW - 14 october 2013

KW - 5 may 2014

KW - accepted

U2 - 10.1017/S1047951114000870

DO - 10.1017/S1047951114000870

M3 - Article

C2 - 24932863

SN - 1467-1107

SP - 1

EP - 8

JO - CARDIOLOGY IN THE YOUNG

JF - CARDIOLOGY IN THE YOUNG

ER -