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

Prashob Porayette, Joshua F P van Amerom, Shi-Joon Yoo, Edgar Jaeggi, Christopher K Macgowan, Mike Seed, Prashob Porayette, Joshua F P van Amerom, Shi-Joon Yoo, Edgar Jaeggi, Christopher K Macgowan, Mike Seed

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.

Figures

Figure 1
Figure 1
Flows in foetuses with transposition versus normal hearts. Normal foetal flows (black bars) are compared with transposition of the great arteries (TGA) (coloured symbols) in units of (a) ml/minute/kg, and (b) as percentage of combined ventricular output (CVO) (# denotes calculated flows). Significance level is indicated (*p<0.05, **p<0.01, ***p<0.001). AAo=ascending aorta; APC=aortopulmonary collateral; DA=arterial duct; DAo=descending aorta; FO=oval foramen; MPA=main pulmonary artery; PBF=pulmonary blood flow; SVC=superior caval vein; UV=umbilical vein.
Figure 2
Figure 2
Potential mechanism for development of foetal pulmonary vascular disease in transposition of the great arteries. Initial pulmonary vasodilation because of streaming of oxygen from the umbilical vein (UV) to the pulmonary arteries results in oval foramen (FO) restriction and arterial ductal (DA) constriction, producing “isolation” and hypoxia of the pulmonary circulation, which in turn drives increased aorto-pulmonary collateral (APC) flow. AAo=ascending aorta; DAo=descending aorta; GI=gastrointestinal; IVC=inferior caval vein; LA=left atrium; LHV=left hepatic vein; LPV=left portal vein; LV=left ventricle; MPA=main pulmonary artery; PBF=pulmonary blood flow; RA=right atrium; RHV=right hepatic vein; RPV=right portal vein; RV=right ventricle; SVC=superior caval vein; UA=umbilical arteries.

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Source: PubMed

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