ArticleJournal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance2025
Fetal magnetic resonance imaging diagnosis of pulmonary lymphangiectasia in hypoplastic left heart syndrome: Association with fetal echocardiography and postnatal outcome.
Article in Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundSecondary pulmonary lymphangiectasia (PL) is a recognised complication of hypoplastic left heart syndrome (HLHS) with an intact or restrictive atrial septum, associated with poor postnatal outcomes. Fetal MRI has been increasingly used to assess pulmonary abnormalities in HLHS, but the prognostic significance of subtle PL-like changes remains unclear. In this study, we evaluate the relationship between fetal MRI lung findings, echocardiographic markers of pulmonary venous obstruction, and postnatal outcomes.
methodsA retrospective analysis of all fetuses with HLHS who underwent fetal MRI between July 2019 and December 2022 was performed. MRI images were reviewed for features of PL and categorised as "normal," "suspicious," or "diagnostic" of PL. Pulmonary venous Doppler velocity-time integral (VTI) ratios from the most recent fetal echocardiogram were then compared to MRI findings. Postnatal outcomes, including early ventilation, need for intervention, and survival at 28 days and 1 year, were assessed.
resultsOf 20 fetuses with HLHS who underwent MRI, 6/20 (30%) showed features suspicious or diagnostic of PL (5 "suspicious" and 1 "diagnostic"), and 6/20 (30%) showed some evidence of pulmonary venous obstruction (PVO) on echo. While echo markers of PVO were significantly associated with some degree of PL on MRI (p=0.006), neither PL nor PVO predicted the need for early support/intervention or survival in fetuses who underwent active postnatal management.
conclusionFetuses with HLHS may exhibit a spectrum of lung changes on fetal MRI related to pulmonary venous obstruction. Whilst technical factors may also play a role, a degree of caution is advisable when interpreting more subtle forms of PL in fetal life, particularly in the absence of echocardiographic markers of severe atrial restriction. Larger, multi-centre prospective studies are needed to refine diagnostic criteria for PL in HLHS and better understand its prognostic significance in terms of both early and long-term outcome.
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