Polyviou S, Charakida M, Miller OI, Rscn TW, Vigneswaran TV, Zidere V, Day TG, Lloyd DFA, Sharland GK, Simpson JM.
Am J Cardiol. 2022 Mar 1;166:114-121. doi: 10.1016/j.amjcard.2021.11.023. Epub 2021 Dec 21.
- There is an increased burden of associated left-sided cardiac lesions in fetuses with antenatal diagnosis of coarctation of the aorta, with the bicuspid aortic valve being the most common
- Fetal lie impeding short-axis imaging, maternal body habitus, and image resolution pose diagnostic challenges to identifying associated left heart lesions
- Comprehensive antenatal counseling should include the potential of postnatally diagnosing associated left heart lesions while discussing the prognosis of primary coarctation.
Commentary from Dr. Venu Amula (Salt Lake City, UT, USA), section editor of Pediatric & Fetal Cardiology Journal Watch
Polyviou et al. report a single-center retrospective cohort study aiming at the medium-term outcomes of fetuses with antenatally suspected and postnatally confirmed coarctation of the Aorta, emphasizing the requirement of intervention not only to CoA but also for associated left heart lesions. Although it is a common experience that left-sided obstructive lesions cluster, the data on the associated left heart lesions and the need for interventions are limited, and this paper intends to address this knowledge gap.
The study sample was derived by reviewing all infants with the repair of CoA during the period Jan 2010 to Dec 2019 on a background of antenatally suspected COA. The left-sided lesions defined were supramitral membrane, mitral parachute valve, mitral valve stenosis, subaortic stenosis, bicuspid aortic valve, and supravalvular aortic stenosis. The authors identified 119 patients, of which 89 met the inclusion criteria. 72% of patients had left-sided cardiac lesions, with the majority having bicuspid aortic valves. Eleven ( 12%) patients had surgical/transcatheter reintervention on the aortic arch after the initial repair. Twelve patients underwent 30 interventions for additional heart lesions, 6 for supramitral membrane, 10 for MV stenosis, 8 for subaortic membrane, one for Ross-Konno, 4 for aortic valve stenosis, and one for aortic valve stenosis, and one for supravalvar stenosis. There were four deaths in the follow-up period. Survival analysis with Kaplan Meier estimate showed 5-year survival free of intervention for additional left suited cardiac lesions or reintervention on the aortic arch of 71.6% ( 95% CI 60.4 to 82.8%)
The paper highlights the challenges of prenatal diagnosis of associated left heart lesions and how they could impact postnatal outcomes. Although the bicuspid aortic valve is the most common postnatal diagnosis, the other associated left heart lesions occur infrequently and cause high morbidity.
The study is from a single center and has inherent limitations. The diagnostic accuracy and challenges may not be uniform when applied to other centers. The outcomes are reflective of single-center management and may not be generalizable. However, it does bring to the forefront the challenges of accurate assessment of left heart structures and the need for caution in antenatal counseling.
Requirement of intervention for additional left-sided cardiac lesions. Thirty procedures were performed on individual lesions during 21 surgical o transcatheter interventions, some of which involved intervention on more than one lesion.
Kaplan-Meier curve for survival free of (surgical or transcatheter) intervention for additional left-sided cardiac lesions.