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Three-dimensional echocardiographic evaluation of Ebstein’s anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome

Part of: Echocardiography Hypoplastic Left Heart Syndrome (HLHS)

Published online by Cambridge University Press:  22 April 2018

Courtney Cassidy Open the ORCID record for Courtney Cassidy [Opens in a new window] ,
Max B. Mitchell  and
Pei-Ni Jone
Courtney Cassidy*
Affiliation:
Children’s Hospital Colorado, Aurora, CO, USA
Max B. Mitchell
Affiliation:
Cardiovascular Surgery, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA
Pei-Ni Jone
Affiliation:
Pediatric Cardiology, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA
*
Author for correspondence: C. Cassidy, RDCS, Children’s Hospital Colorado, 13123 East 16th Avenue, Aurora, CO 80045, USA. Tel: 720 777 2949; Fax: 1 720 777 7174; E-mail: courtney.cassidy@childrenscolorado.org
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Abstract

We report three-dimensional imaging of a rare finding of Ebstein’s anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome, which has been previously reported only by two-dimensional echocardiography. A fetal echocardiogram was performed at 19 weeks that showed a moderately hypoplastic left ventricle, severely hypoplastic mitral valve, a severely hypoplastic aortic valve, and a dysplastic tricuspid valve. Post Caesarean delivery at 40 weeks of gestation, a transthoracic echocardiogram confirmed the findings seen on the fetal echocardiogram. A three-dimensional echocardiogram was then performed, which demonstrated an Ebstein tricuspid valve with apical displacement of the septal leaflet, chordal attachments of the valve to the right ventricular outflow tract, and moderate tricuspid regurgitation. The patient underwent a successful heart transplantation.

Keywords

Ebstein’s anomalyhypoplastic left heart syndrome3D echocardiographytricuspid valve regurgitationechocardiography and scans

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 28 , Issue 6 , June 2018 , pp. 885 - 887
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Copyright
© Cambridge University Press 2018 

Hypoplastic left heart syndrome represents 2–3% of CHD cases.Reference Gobergs, Salputra and Lubaua 1 Ebstein’s anomaly represents <1% of CHD.Reference Luxford, Arora and Ayer 2 The combination of hypoplastic left heart syndrome with Ebstein’s anomaly is very rare in CHD, and the management of this type of anomaly is difficult as the prognosis is poor.Reference Stamm, Anderson and Ho 3 – Reference Castaneda-Zuniga, Nath, Moller and Edwards 6 We reported a case of hypoplastic left heart syndrome with Ebstein tricuspid valve using prenatal fetal imaging, and post-natal two-dimensional and three-dimensional echocardiographic imaging of this lesion.

Case presentation

A 37-year-old patient presented at 19 weeks and 3 days of gestation for a fetal echocardiogram as a referral from an outside institution for suspected fetal cardiac anomalies. A fetal echocardiogram was performed and it demonstrated a moderately hypoplastic left ventricle, severely hypoplastic mitral valve, a severely hypoplastic aortic valve, a dysplastic tricuspid valve, and significant left ventricular endocardial fibrosis.

The infant was born by Caesarean delivery at 40 weeks of gestation. A complete transthoracic echocardiogram confirmed a moderately hypoplastic left ventricle, severely hypoplastic mitral valve with minimal antegrade mitral valve flow, an atretic aortic valve, and a dysplastic tricuspid valve with moderate tricuspid valve insufficiency with concern for Ebstein’s anomaly (Supplementary Fig 1). A three-dimensional echocardiogram was then performed using the X7 transthoracic transducer with full-volume four-beat acquisition. After cropping, we demonstrated the Ebstein tricuspid valve from above (Supplementary video 1). The three-dimensional imaging was an additive component to the diagnosis of the tricuspid valve morphology in this complex patient. Using multi-plane reconstruction, we could demonstrate the chordal attachment of the anterior leaflet of the tricuspid valve to the right ventricular outflow tract (Fig 1). This patient did not qualify for a staged surgical palliation as the single-ventricle palliation with the addition of tricuspid valve repair would become very high risk. The patient was listed and received a heart transplantation at 55 days old. The explanted heart demonstrated a displaced tricuspid valve septal leaflet and anteriorly displaced anterior tricuspid valve leaflet (Fig 2).

Figure 1

( a ) The four-chamber view demonstrating the large section of atrialised right ventricle. ( b ) Right ventricular inflow/outflow view demonstrating the chordal attachments of the Ebstein valve to the right ventricular outflow tract. LA=left atrium; PA=pulmonary artery; RA=right atrium; RV=right ventricle, RVOT=right ventricular outflow tract.

Figure 2

( a ) Hypoplastic left heart syndrome (HLHS) with Ebstein’s anomaly: black arrow demonstrates transected hypoplastic ascending aorta, and white arrow demonstrates sail-like anterior leaflet with severely dysplastic leading edge. ( b ) HLHS with Ebstein’s anomaly: black arrow demonstrates the transected hypoplastic ascending aorta, white arrows demonstrate dysplastic septal leaflet with apical displacement, and white triangle is the atrialised portion of right ventricle.

Discussion

Hypoplastic left heart syndrome with Ebstein’s anomaly is a rare congenital heart lesion that has poor prognosis.Reference Hammel, Danford, Spicer and Kutty 5 Hammel et al reported a case with hypoplastic left heart syndrome with Ebstein’s anomaly with severe tricuspid regurgitation who did not survive. Tricuspid valve regurgitation is a poor prognostic factor that leads to poor outcomes in a single-stage palliation.Reference Kutty, Graney and Khoo 7 The Ebstein valve would not be suited to function as a systemic atrioventricular valve. Three-dimensional echocardiographic imaging complemented our two-dimensional imaging by providing better delineation of the tricuspid valve function with chordal attachment of the papillary muscle to the right ventricular outflow tract. Multi-plane reconstruction using the three-dimensional data set was helpful in delineating the anatomy well and in understanding the anatomy in different cut planes.Reference Bharucha, Anderson, Lim and Vettukattil 8 Multi-plane reconstruction allows for quick interrogation of the anatomy of the chordae tendinae and papillary muscles from one single three-dimensional data set, and provides cut planes that would not have been obtained from the traditional two-dimensional imaging. This allows for understanding the anatomy well in three dimensions and assists in communicating this anatomy to the surgeon.Reference Acar, Abadir and Roux 9 The explanted heart anatomy corresponded well to the three-dimensional images and multi-plane reconstruction.

Conclusion

We report a three-dimensional evaluation of a patient with hypoplastic left heart syndrome and Ebstein’s anomaly with significant tricuspid valve regurgitation. Owing to the severity of the tricuspid valve dysfunction, the patient was listed and received a heart transplantation.

Acknowledgements

None.

Financial Support

This research received no specific grant from any funding agency or from commercial, or not-for-profit sectors.

Conflicts of Interest

None.

Supplementary material

To view supplementary material for this article, please visit https://doi.org/10.1017/S1047951118000367

References

1. Gobergs, R, Salputra, E, Lubaua, I. Hypoplastic left heart syndrome: a review. Acta Med Litu 2016; 23: 86–98.Google ScholarPubMed
2. Luxford, JC, Arora, N, Ayer, JG, et al. Neonatal Ebstein anomaly: a 30-year institutional review. Semin Thorac Cardiovasc Surg 2017; 29: 206–212.Google Scholar
3. Stamm, C, Anderson, RH, Ho, SY. The morphologically tricuspid valve in hypoplastic left heart syndrome. Eur J Cardiothorac Surg 1997; 12: 587–592.Google Scholar
4. Werho, DK, Thorsson, T, Owens, ST, Fifer, C. Biventricular badness: rare images of Ebstein anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome. Pediatr Cardiol 2015; 36: 1310–1311.CrossRefGoogle Scholar
5. Hammel, JM, Danford, DA, Spicer, RL, Kutty, S. Fetal and neonatal imaging and strategy of primary neonatal heart transplantation in hypoplastic left heart with Ebstein’s anomaly. Echocardiography 2015; 32: 598–601.CrossRefGoogle ScholarPubMed
6. Castaneda-Zuniga, W, Nath, HP, Moller, JH, Edwards, JE. Left-sided anomalies in Ebstein’s malformation of the tricuspid valve. Pediatr Cardiol 1982; 3: 181–185.CrossRefGoogle ScholarPubMed
7. Kutty, S, Graney, BA, Khoo, NS, et al. Serial assessment of right ventricular volume and function in surgically palliated hypoplastic left heart syndrome using real-time transthoracic three-dimensional echocardiography. J Am Soc Echocardiogr 2012; 25: 682–689.CrossRefGoogle ScholarPubMed
8. Bharucha, T, Anderson, RH, Lim, ZS, Vettukattil, JJ. Multiplanar review of three-dimensional echocardiography gives new insights into the morphology of Ebstein’s malformation. Cardiol Young 2010; 20: 49–53.CrossRefGoogle ScholarPubMed
9. Acar, P, Abadir, S, Roux, D, et al. Ebstein’s anomaly assessed by real-time 3-D echocardiography. Ann Thorac Surg 2006; 82: 731–733.Google Scholar
Figure 0

Figure 1 (a) The four-chamber view demonstrating the large section of atrialised right ventricle. (b) Right ventricular inflow/outflow view demonstrating the chordal attachments of the Ebstein valve to the right ventricular outflow tract. LA=left atrium; PA=pulmonary artery; RA=right atrium; RV=right ventricle, RVOT=right ventricular outflow tract.

Figure 1

Figure 2 (a) Hypoplastic left heart syndrome (HLHS) with Ebstein’s anomaly: black arrow demonstrates transected hypoplastic ascending aorta, and white arrow demonstrates sail-like anterior leaflet with severely dysplastic leading edge. (b) HLHS with Ebstein’s anomaly: black arrow demonstrates the transected hypoplastic ascending aorta, white arrows demonstrate dysplastic septal leaflet with apical displacement, and white triangle is the atrialised portion of right ventricle.

Supplementary material: Image

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