Rapid Fire Abstracts
Greg Leonard, MD
Paediatric Resident
Evelina Children's Hospital, United Kingdom
Greg Leonard, MD
Paediatric Resident
Evelina Children's Hospital, United Kingdom
Tomas Woodgate, MD
Paediatric Cardiology Resident
Evelina Children's Hospital, United Kingdom
Wendy Norman, DCR(R), DRI
Senior Research MRI Radiographer, Early Life Imaging
Department of Early Life Imaging, Kings College London University, United Kingdom
John Simpson, MD
Professor of Paediatric and Fetal Cardiology
Evelina London Children’s Hospital, United Kingdom
Vita Zidere
Consultant Paediatric Cardiologist
Evelina London Children’s Hospital, United Kingdom
Owen Miller
Consultant in Paediatric and Fetal Cardiology
Evelina London Children’s Hospital, United Kingdom
Thomas Day, MD, PhD
Clinical Research Fellow
King's College London, United Kingdom
Alexia Egloff
Paediatric Radiologist
Guy's and St Thomas' NHS Foundation Trust, United Kingdom
Gema Priego, MD
Paediatric Radiologist
Evelina Children's Hospital, United Kingdom
Trisha Vigneswaran, MD
Consultant Paediatric Cardiologist
Evelina London Children’s Hospital, United Kingdom
Kuberan Pushparajah, MD, BMBS, BMedSc
Paediatric cardiology consultant
Evelina London Children’s Hospital/ King's College London, United Kingdom
David F. A Lloyd, MD, PhD
Consultant in Fetal Cardiology
King's College London, United Kingdom
Secondary pulmonary lymphangiectasia (PL) can develop in congenital heart disease (CHD) associated with pulmonary venous (PV) obstruction. Fetal MRI allows prenatal detection of PL, described a T2-hyperintense, heterogenous pattern on single-shot fast-spin echo sequences (“nutmeg lung”) often with pleural effusions. In hypoplastic left heart syndrome (HLHS) with restrictive/intact atrial septum, this appearance has been associated with high postnatal mortality, up to 100% in some series. However, some fetuses with a potential substrate for PL may show subtle lung abnormalities on fetal MRI, the implications of which are poorly understood. We describe a single centre experience of cases with a potential CHD substrate for PL undergoing fetal MRI.
Methods:
This retrospective study reviewed MRI data acquired between Jul 2019 and Dec 2022, including all cases of HLHS and/or total anomalous pulmonary venous drainage (TAPVD) referred for MRI following fetal echocardiography. MR images were reviewed independently by two paediatric radiologists, scoring 0 (no PL), 1 (some features of PL) or 2 (diagnostic for PL). Assessment was performed blinded, with any disagreements a) between both observers or b) between blinded and initial reports resolved by consensus review. Postnatal outcome data included: 1) need for immediate postnatal ventilation; 2) need for early neonatal procedure (< 24h); 3) survival to 28 days and 4) 1-year survival.
Results:
27 cases were identified (22 HLHS, 3 TAPVD, 2 mixed HLHS/APVD). Following consensus review, there were 2 cases with MRI diagnostic of PL (one TAPVD, one mixed HLHS/AVPD), 7 with features concerning for PL and 18 with no concerning features. 4/27 cases (3 HLHS, 1 mixed HLHS/APVD) were redirected to a non-surgical care pathway postnatally; there was no significant difference in the presence of any degree of PL in this group (2/4; 50%) compared to the active treatment group (7/23; 30%) (p=0.234). For the remaining cases, no HLHS fetuses (n=19) scored 2; however, the presence of some features of PL (score 1) was not a predictor for need for early ventilation or intervention, 28d survival or one year survival (Table 1). The presence of PL was also not predictive of outcome for TAPVD/mixed cases, however numbers were small. The one actively managed case with diagnostic features of PL (score 2) is alive at 1 year of age following early surgical repair of TAVPD.
Conclusion:
The relationship between obstructed PV drainage with severe PL and high early mortality is well described. However, more subtle lung changes of fetal MRI can be difficult to interpret. Whilst these changes may still reflect an underlying lymphatic abnormality where a substrate for PV obstruction exists, they were not universally associated with a higher rate of neonatal intervention, early or late mortality in our series. Future work, preferably multi-centre, combining fetal MRI findings with a more comprehensive set of MRI and fetal echo biomarkers may improve risk stratification in these groups.
