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Fetal echocardiographic features associated with bicuspid aortic valve

Published online by Cambridge University Press:  12 May 2023

Joseph Burns Open the ORCID record for Joseph Burns [Opens in a new window] ,
Madhusudan Ganigara ,
Jane E. Cerise  and
Denise A. Hayes Open the ORCID record for Denise A. Hayes [Opens in a new window]
Joseph Burns
Affiliation:
Division of Pediatric Cardiology, Texas Children’s Hospital/Baylor College of Medicine, Houston, TX, USA
Madhusudan Ganigara
Affiliation:
Division of Pediatric Cardiology, University of Chicago, Chicago, IL, USA
Jane E. Cerise
Affiliation:
Feinstein Institute for Medical Research, Northwell Health, Manhasset, NY, USA
Denise A. Hayes*
Affiliation:
Division of Pediatric Cardiology, Cohen Children’s Medical Center Northwell Health, New Hyde Park, NY, USA
*
Corresponding author: Dr D. A. Hayes, MD, 1111 Marcus Ave, Division of Pediatric Cardiology, Cohen Children’s Medical Center Northwell Health, Suite M15, New Hyde Park, NY 11042, USA. Phone: 516-601-7200. E-mail: dhayes2@northwell.edu
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Abstract

Background:

Prenatal diagnosis of bicuspid aortic valve is challenging. Bicuspid aortic valve is often associated with aortic dilation.

Methods:

Fetuses with postnatally confirmed bicuspid aortic valve were gestational age-matched with normal controls. Complex lesions were excluded. Aortic valve and arch measurements by two blinded investigators were compared.

Results:

We identified 27 cases and 27 controls. Estimated fetal weight percentile was lower in cases than controls. Seven cases had one or more significant lesions including perimembranous ventricular septal defects (n = 2), isolated annular hypoplasia (n = 2), and/or arch hypoplasia/coarctation (n = 4). Fetuses with bicuspid aortic valves had significantly smaller median z-scores of the aortic annulus (–1.60 versus –0.53, p < 0.001) and root (–1.10 versus –0.53, p = 0.040), and larger ratios of root to annulus (1.32 versus 1.21, p < 0.001), sinotubular junction to annulus (1.07 versus 0.99, p < 0.001), ascending aorta to annulus (1.29 versus 1.18, p < 0.001), and transverse aorta to annulus (1.04 versus 0.96, p = 0.023). Leaflets were “doming” in 11 cases (41%) and 0 controls (p = 0.010), “thickened” in 10 cases (37%) and 0 controls (p = 0.002). We noted similar findings in the subgroup without significant additional cardiac defects.

Conclusions:

The appearance of doming or thickened aortic valve leaflets on fetal echocardiogram is associated with bicuspid aortic valve. Compared to controls, fetuses with bicuspid aortic valve had smaller aortic annulus sizes (possibly related to smaller fetal size) without proportionally smaller aortic measurements, resulting in larger aortic dimension to annulus ratios. Despite inherent challenges of diagnosing bicuspid aortic valve prenatally, these findings may increase suspicion and prompt appropriate postnatal follow-up.

Keywords

Pediatric cardiologyfetal echocardiographbicuspid aortic valve

Information

Type
Original Article
Information
Cardiology in the Young , Volume 34 , Issue 1 , January 2024 , pp. 50 - 55
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Copyright
© The Author(s), 2023. Published by Cambridge University Press

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References

Roberts, WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 1970; 26: 72–83.CrossRefGoogle ScholarPubMed
Nistri, S, Basso, C, Marzari, C, Mormino, P, Thiene, G. Frequency of bicuspid aortic valve in young male conscripts by echocardiogram. Am J Cardiol 2005; 96: 718–721.CrossRefGoogle Scholar
Basso, C, Boschello, M, Perrone, C, et al. An echocardiographic survey of primary school children for bicuspid aortic valve. Am J Cardiol 2004; 93: 661–663.CrossRefGoogle ScholarPubMed
Sillesen, AS, Vøgg, O, Pihl, C, etal, Bundgaard, H. Prevalence of bicuspid aortic valve and associated aortopathy in newborns in Copenhagen. Denmark JAMA 2021; 325: 561–567.CrossRefGoogle ScholarPubMed
Siu, SC, Silversides, CK. Bicuspid aortic valve disease. J Am Coll Cardiol 2010; 55: 2789–2800.CrossRefGoogle ScholarPubMed
Vedel, C, Rode, L, Bundgaard, H, et al. Prenatal cardiac biometries and flow assessments in fetuses at 20 weeks with a bicuspid aortic valve compared to healthy controls: a multicenter, cohort study. Ultrasound Obstet Gynecol 2021; 58: 846–852. DOI 10.1002/uog.23670.CrossRefGoogle Scholar
Mordi, I, Tzemos, N. Bicuspid aortic valve disease: a comprehensive review. Cardiol Res Pract 2012; 2012: 196037–7. DOI 10.1155/2012/196037.CrossRefGoogle ScholarPubMed
Verma, S, Siu, SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med 2014; 370: 1920–1929.CrossRefGoogle ScholarPubMed
Kiyota, Y, Della Corte, A, Montiero Vieira, V, et al. Risk and outcomes of aortic valve endocarditis among patients with bicuspid and tricuspid aortic valves. Open Heart 2017; 4: e000545. DOI 10.1136/openhrt-2016-000545.CrossRefGoogle ScholarPubMed
Grattan M, Prince A, Rumman, RK, etal. Predictors of bicuspid aortic valve-associated aortopathy in childhood: a report from the MIBAVA Consortium. Circ Cardiovasc Imaging 2020; 13: e009717. DOI 10.1161/CIRCIMAGING.119.009717.CrossRefGoogle Scholar
Kerstjens-Frederikse, WS, Du Marchie Sarvaas, GJ, Ruiter, JS, et al. Left ventricular outflow tract obstruction: should cardiac screening be offered to first-degree relatives? Heart 2011; 97: 1a228–32.CrossRefGoogle ScholarPubMed
Paladini, D, Russo, MG, Vassallo, M, et al. Ultrasound evaluation of aortic valve anatomy in the fetus. Ultrasound Obstet Gynecol 2002; 20: 30–34.CrossRefGoogle ScholarPubMed
Dumitrascu-Biris, I, Zidere, V, Vigneswaran, T, et al. Dilated ascending aorta in the fetus. Prenat Diagn 2021; 41: 1127–1133.CrossRefGoogle ScholarPubMed
Fowles, RE, Martin, RP, Abrams, JM, Schapira, JN, French, JW, Popp, RL. Two-dimensional echocardiographic features of bicuspid aortic valve. Chest 1979; 75: 434–440.CrossRefGoogle ScholarPubMed
BCH Z-Score Calculator: Boston Children’s Hospital Heart Center., https://zscore.chboston.org [2015].Google Scholar
Firpo, C, Hoffman, JI, Silverman, NH. Evaluation of fetal heart dimensions from 12 weeks to term. Am J Cardiol 2001 Mar 1; 87: 594–600.CrossRefGoogle Scholar
Michelena, HI, Desjardins, VA, Avierinos, JF, et al. Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation 2008; 117: 2776–2784.CrossRefGoogle ScholarPubMed
Niaz, T, Fernandes, SM, Sanders, SP, Michelena, H, Hagler, DJ. Clinical history and management of bicuspid aortic valve in children and adolescents. Prog Cardiovasc Dis 2020; 63: 425–433.CrossRefGoogle ScholarPubMed
Contro, E, Cattani, L, Balducci, A, et al. Prediction of neonatal coarctation of the aorta at fetal echocardiography: a scoring system. J Matern Fetal Neonatal Med 2020; 35: 4299–4305. DOI 10.1080/14767058.2020.1849109.CrossRefGoogle ScholarPubMed
Garne, E, Stoll, C, Clementi, M, Euroscan Group. Evaluation of prenatal diagnosis of congenital heart diseases by ultrasound: experience from 20 European registries. Ultrasound Obstet Gynecol 2001; 17: 386–391.CrossRefGoogle ScholarPubMed
Niaz, T, Poterucha, JT, Johnson, JN, et al. Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects. Congenit Heart Dis 2017; 12: 261–269.CrossRefGoogle Scholar
Familiari, A, Morlando, M, Khalil, A, et al. Risk factors for coarctation of the aorta on prenatal ultrasound: a systematic review and meta-analysis. Circulation 2017; 135: 772–785.CrossRefGoogle ScholarPubMed
Anuwutnavin, S, Satou, G, Chang, RK, DeVore, GR, Abuel, A, Sklansky, M. Prenatal sonographic predictors of neonatal coarctation of the aorta. J Ultrasound Med 2016; 35: 2353–2364.CrossRefGoogle ScholarPubMed
Warren, AE, Boyd, ML, OʼConnell, C, Dodds, L. Dilatation of the ascending aorta in paediatric patients with bicuspid aortic valve: frequency, rate of progression and risk factors. Heart 2006; 92: 1496–1500.CrossRefGoogle ScholarPubMed