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Tricuspid atresia with absent pulmonary valve and intact ventricular septum: Fontan operation after complete exclusion of the right ventricle

Published online by Cambridge University Press:  07 May 2025

Cheul Lee Open the ORCID record for Cheul Lee [Opens in a new window] ,
Won Young Lee ,
Ju Ae Shin  and
Jae Young Lee
Cheul Lee*
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Won Young Lee
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Ju Ae Shin
Affiliation:
Department of Pediatrics, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Jae Young Lee
Affiliation:
Department of Pediatrics, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
*
Corresponding author: Cheul Lee; Email: [email protected]
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Abstract

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an exceedingly rare CHD. Only few cases with completed single ventricle palliation have been reported in the literature. We present the follow-up case of a 4-year-old child with this cardiac anomaly who underwent Fontan operation after complete exclusion of the right ventricle.

Keywords

Tricuspid atresiaabsent pulmonary valveintact ventricular septumFontan operation
Type
Brief Report
Information
Cardiology in the Young , First View , pp. 1 - 3
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

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References

Lee, C, Kim, KM, Lee, JY, Yoon, J. Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle. Cardiol Young 2020; 30: 126–128.CrossRefGoogle ScholarPubMed
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Kasahara, H, Aeba, R, Yozu, R. Fenestrated exclusion of the right ventricle for tricuspid atresia and absent pulmonary valve. Ann Thorac Surg 2010; 90: 647–649.CrossRefGoogle ScholarPubMed
Hasegawa, M, Iwai, S, Kugo, Y. Fontan operation for tricuspid atresia with absent pulmonary valve: a case series. Cardiol Young 2022; 32: 813–814.CrossRefGoogle ScholarPubMed
Forrest, P, Bini, RM, Wilkinson, JL, et al. Congenital absence of the pulmonic valve and tricuspid valve atresia with intact ventricular septum. Am J Cardiol 1987; 59: 482–484.CrossRefGoogle ScholarPubMed
Best, TH, Vyas, HV, Jaquiss, RD, Sachdeva, R. Tricuspid atresia with absent pulmonary valve and intact ventricular septum: a rare association. Congenit Heart Dis 2011; 6: 393–396.CrossRefGoogle ScholarPubMed
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