Hostname: page-component-cb9f654ff-hqlzj Total loading time: 0 Render date: 2025-09-01T15:50:56.405Z Has data issue: false hasContentIssue false
  • English
  • Français

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle

Part of: Functionally Univentricular Heart Surgery

Published online by Cambridge University Press:  26 November 2019

Cheul Lee Open the ORCID record for Cheul Lee [Opens in a new window] ,
Kyung Min Kim ,
Jae Young Lee  and
Jihong Yoon
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
Kyung Min Kim
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
Jihong Yoon
Affiliation:
Department of Pediatrics, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
*
Author for correspondence: C. Lee, MD, Department of Thoracic and Cardiovascular Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 06591, Republic of Korea. Tel: +82 2 2258 6131; Fax: +82 2 594 8644; E-mail: tscheul@hanmail.net
Get access Rights & Permissions [Opens in a new window]

Abstract

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Keywords

Tricuspid atresiaabsent pulmonary valveintact ventricular septumbidirectional cavopulmonary anastomosis

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 30 , Issue 1 , January 2020 , pp. 126 - 128
Copyright
© Cambridge University Press 2019

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

References

Marin-Garcia, J, Roca, J, Blieden, LC, Lucas, RV, Edwards, JE.Congenital absence of the pulmonary valve associated with tricuspid atresia and intact ventricular septum. Chest 1973; 64: 658–661.CrossRefGoogle ScholarPubMed
Mori, K, Ando, M, Satomi, G, Nakazawa, M, Momma, K, Takao, A.Imperforate tricuspid valve with dysplasia of the right ventricular myocardium, pulmonary valve, and coronary artery: a clinicopathological study of nine cases. Pediatr Cardiol 1992; 13: 24–29.Google ScholarPubMed
Litovsky, S, Choy, M, Park, J, et al. Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature. Pediatr Dev Pathol 2000; 3: 353–366.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
Lopes, KR, Mennes, F, Delazoide, AL, Iserin, F, Azancot, A.Prenatal diagnosis of absent pulmonary valve with membranous tricuspid atresia and intact ventricular septum: report of one case and review of the literature. Prenat Diagn 2007; 27: 973–975.CrossRefGoogle ScholarPubMed
Lato, K, Gembruch, U, Geipel, A, et al. Tricuspid atresia with absent pulmonary valve and intact ventricular septum: intrauterine course and outcome of an unusual congenital heart defect. Ultrasound Obstet Gynecol 2010; 35: 243–245.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
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

Lee et al. supplementary material

Lee et al. supplementary material 1

Download Lee et al. supplementary material(Video)
Video 2.6 MB

Lee et al. supplementary material

Lee et al. supplementary material 2

Download Lee et al. supplementary material(Video)
Video 2.4 MB
Supplementary material: File

Lee et al. supplementary material

Lee et al. supplementary material 3

Download Lee et al. supplementary material(File)
File 14.2 KB