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Last update 08 May 2025

Tricuspid Atresia

Last update 08 May 2025

Basic Info

Synonyms

Absent Right Atrioventricular Connection, Atresia, Tricuspid, Atresia, Tricuspid Valve

+ [30]

Introduction

Absence of the orifice between the RIGHT ATRIUM and RIGHT VENTRICLE, with the presence of an atrial defect through which all the systemic venous return reaches the left heart. As a result, there is left ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR) because the right ventricle is absent or not functional.

Drugs associated with Tricuspid Atresia

Bosentan

Target

ETA x ETB

Mechanism

ETA agonists [+1]

Active Org.

Janssen-Cilag International NV [+5]

Originator Org.

F. Hoffmann-La Roche Ltd.

Active Indication

Ulcer [+4]

Inactive Indication

Anemia, Sickle Cell [+19]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

First Approval Date20 Nov 2001

Clinical Trials associated with Tricuspid Atresia

ACTRN12622000050752

/ Not yet recruitingNot ApplicableIIT

The Effect of Exercise Prescription and Delivery in Congenital Heart Disease – The Congenital Heart Fitness Intervention Trial: CH-FIT

Start Date01 Mar 2022

Sponsor / Collaborator

The University of Sydney

NCT04467671

/ RecruitingPhase 2IIT

Prospective, Open-labeled, Single-arm Clinical Trial to Evaluate the Safety and Efficacy of the Second-generation Tissue Engineered Vascular Graft as Vascular Conduits for Extracardiac Total Cavopulmonary Connection.

A single arm clinical trial evaluating the safety and efficacy of the second generation TEVG as vascular conduits for extracardiac total cavopulmonary connection.

Start Date15 Jul 2020

Sponsor / Collaborator

Nationwide Children's Hospital

[+3]

DRKS00019889

/ RecruitingNot ApplicableIIT

Fetal cranial MRI und neurodevelopement of children with congenital heart disease - LEADER – Prenatal Imaging(LEADER = Long-term EArly DEvelopment Research)

Start Date13 Feb 2020

Sponsor / Collaborator-

100 Clinical Results associated with Tricuspid Atresia

100 Translational Medicine associated with Tricuspid Atresia

0 Patents (Medical) associated with Tricuspid Atresia

1,760

Literatures (Medical) associated with Tricuspid Atresia

01 Jun 2025·American Heart Journal

Transcatheter or surgical aortic valve replacement in patients with severe aortic stenosis aged 70 years or younger: A NOTION-2 substudy

Article

Author: Savontaus, Mikko ; Rück, Andreas ; Leon, Martin ; Christiansen, Evald H ; Thyregod, Hans Gustav Hørsted ; De Backer, Ole ; Prendergast, Bernard ; Laine, Mika ; Angerås, Oskar ; Niemela, Matti ; Gudmundsdóttir, Ingibjörg J ; Søndergaard, Lars ; Bleie, Öjvind ; Jørgensen, Troels Højsgaard

This NOTION-2 sub-study revealed distinct outcomes for transcatheter aortic valve replacement (TAVR) and surgical aortic valve replacement (SAVR) in low surgical risk patients aged ≤ 70 years with a tricuspid or bicuspid aortic valve stenosis (AS). One year after intervention, the risk of death, stroke or rehospitalization in patients with tricuspid AS was similar after TAVR when compared to SAVR (absolute risk difference: -2.0%; 95% confidence interval (CI): -11.8% to 7.7%) Conversely, in patients with bicuspid AS, TAVR was associated with a significantly higher risk of adverse outcomes (absolute risk difference: 13.8%; 95% CI: 1.2% to 26.3%). These analyses are exploratory, but highlight the importance of tailoring the intervention to the patient's clinical risk profile, life expectancy, native aortic valve morphology and the anticipated risks associated with TAVR or SAVR.

01 May 2025·The Journal of Thoracic and Cardiovascular Surgery

Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation

Article

Author: Niedermaier, Carolin ; Ono, Masamichi ; Borgmann, Kristina ; Grozdanov, Dimitrij ; Heinisch, Paul Philipp ; Schaeffer, Thibault ; Hager, Alfred ; Georgiev, Stanimir ; Piber, Nicole ; Hörer, Jürgen ; Palm, Jonas ; Ewert, Peter ; Osawa, Takuya

OBJECTIVEWe aimed to compare the pulmonary artery (PA) growth between infants with univentricular hearts who underwent a ductus stenting (DS) and those who received a systemic to pulmonary shunt (SPS) as an initial palliation.METHODSAll infants with ductal-dependent pulmonary blood flow who underwent initial palliation with either a DS or SPS between 2009 and 2022 in our institution were reviewed. PA development was compared between the groups using the PA index and the symmetry index.RESULTSA total of 130 patients were evaluated including 49 patients after DS and 81 after SPS. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19. At stage II palliation, PA index (P = .926), right PA index (P = .692), left PA index (P = .297), and the symmetry index (P = .650) were similar between the groups. At stage III Fontan completion, PA index (P = .115), right PA index (P = .868), and the symmetry index (P = .144) were similar between the groups. However, left PA index (60 vs 74 mm²/m²; P = .015) was lower, and the incidence of venovenous collaterals (24.2% vs 7.8%; P = .036) was higher in the DS group compared with the SPS group. Freedom from PA interventions between stage II and III palliation was lower in the DS group compared with the SPS group (P = .009).CONCLUSIONSIn infants with univentricular heart after DS, freedom from PA interventions after stage II was lower and the left PA index was smaller, compared with those after SPS. Long-term outcomes after the Fontan procedure should be addressed in patients after DS.

01 Mar 2025·World Journal for Pediatric and Congenital Heart Surgery

Management of Tricuspid Atresia With Normally Related Great Arteries and Left-Sided Obstruction

Article

Author: Alsoufi, Bahaaldin ; Karamlou, Tara ; Williams, William G. ; Bondarenko, Igor ; Palacio, Andres M. ; Welke, Karl F. ; Kirklin, James K. ; Jegatheeswaran, Anusha ; Argo, Madison B. ; Barron, David J. ; McCrindle, Brian W. ; Jacobs, Marshall L.

BackgroundTricuspid atresia (TA) is the second most common form of functionally univentricular heart. For patients with TA and normally related great arteries (Type I), left ventricular outflow tract obstruction (LVOTO) is rare.MethodsFrom the Congenital Heart Surgeons’ Society multi-institutional cohort of 445 patients with Type I TA enrolled from 1999 to 2024 from 42 sites, 14 infants (3%) had interventions for associated LVOTO, either at presentation or after their first TA-related intervention.ResultsOf seven infants initially undergoing Norwood/Damus-Kaye-Stansel (DKS), six survived to Stage II, of whom five survived with one developing pulmonary hypertension and four achieving Fontan. An additional seven infants who were first managed with pulmonary artery band placement subsequently had bidirectional superior cavopulmonary anastomosis (BCPA) and a DKS procedure; there were six survivors, all achieving Fontan. All ten survivors who underwent the Fontan procedure had normal left ventricular and mitral valve function at the latest follow-up. The overall Kaplan-Meier survival estimate at 20 years for these 14 patients was 79% (70% CI, 66%-88%), and the median follow-up was 8.3 years (0.24-21.5).ConclusionsWhile infants with TA and transposition of the great arteries are more likely to have LVOTO, this can also occur in the setting of normally related great arteries. Infants with Type I TA and LVOTO can be managed in the neonatal period with the Norwood procedure ensuring complete arch relief with acceptable outcomes. If LVOTO becomes evident after initial pulmonary artery band placement, a subsequent DKS procedure facilitates satisfactory success to Fontan.

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