Fetal magnetic resonance imaging diagnosis of pulmonary lymphangiectasia in hypoplastic left heart syndrome: Association with fetal echocardiography and postnatal outcome

TL;DR

This study explores how fetal MRI can detect lung changes in fetuses with heart defects and how these findings relate to heart function and outcomes after birth.

Contribution

The study introduces a new approach to evaluating fetal MRI and echocardiography in hypoplastic left heart syndrome.

Findings

30% of fetuses showed suspicious or diagnostic features of pulmonary lymphangiectasia on MRI.

Pulmonary venous obstruction on echocardiography was significantly associated with MRI findings of PL.

Neither PL nor PVO predicted the need for early postnatal intervention or survival.

Abstract

Secondary 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.

A 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.

Of 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.

Fetuses 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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