Tricuspid Atresia

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.
Also Known As:
Absent Right Atrioventricular Connection; Atresia, Tricuspid Valve; Atresias, Tricuspid; Atresias, Tricuspid Valve; Tricuspid Atresias; Tricuspid Valve Atresias; Valve Atresia, Tricuspid; Valve Atresias, Tricuspid; Atresia, Tricuspid; Tricuspid Valve Atresia
Networked: 305 relevant articles (4 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Pulmonary Valve Stenosis (Pulmonary Stenosis)
2. Pulmonary Atresia (Pulmonary Valve Atresia)
3. Double Outlet Right Ventricle
4. Aortic Valve Stenosis (Aortic Stenosis)
5. Ventricular Heart Septal Defects (Ventricular Septal Defect)


1. Kagisaki, Koji: 4 articles (02/2011 - 10/2003)
2. Yagihara, Toshikatsu: 4 articles (02/2011 - 10/2003)
3. Hess, John: 3 articles (07/2010 - 02/2004)
4. Uemura, Hideki: 3 articles (05/2007 - 10/2003)
5. Williams, William G: 2 articles (11/2015 - 03/2004)
6. McCrindle, Brian W: 2 articles (11/2015 - 03/2004)
7. Vogt, Manfred: 2 articles (08/2015 - 07/2010)
8. Schreiber, Christian: 2 articles (08/2015 - 02/2004)
9. Rychik, Jack: 2 articles (01/2015 - 12/2010)
10. Heinle, Jeffrey S: 2 articles (01/2013 - 01/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Tricuspid Atresia:
1. Twin twin transfusion syndromeIBA
2. Complete atrioventricular septal defectIBA
3. Coarctation of the aortaIBA
4. tranilast (N 5')IBA
12/01/1991 - "Atrioventricular connection was double-inlet (n = 76), common (n = 29), absent left atrioventricular connection (n = 14), and absent right atrioventricular connection (n = 5). "
04/01/1988 - "During a 10-year period, 62 patients underwent the following modifications of the Fontan operation for repair of tricuspid atresia: direct atriopulmonary connection (N = 15), atriopulmonary connection using a conduit (N = 5), direct atrioventricular (AV) connection (N = 22), and AV connections with a valved conduit (N = 20), including 2 with combined Fontan-arterial switch procedures. "
06/01/2009 - "Fourteen patients were candidates for terminal biventricular repair [atrioventricular septal defect (AVSD) (n=6), double outlet right ventricle (DORV) +ventricular septal defect (VSD) (n=4), Swiss cheese VSD (n=2), transposition of the great arteries (TGA) +Swiss cheese VSD (n=1), ASD+VSD+aortic coarctation (n=1)], and the remaining 14 were candidates for terminal univentricular repair [double inlet left ventricle (n=5), double inlet right ventricle (n=2), AVSD+left ventricular (LV) hypoplasia (n=2), DORV+LV hypoplasia (n=2); tricuspid atresia (n=1), left AV valve atresia (n=1), TGA+Swiss cheese VSD+LV hypoplasia (n=1). "
07/01/1996 - "1994 50 neonates underwent cardiac surgery using CPB (n = 23 transposition of the great arteries; n = 4 pulmonary atresia; n = 6 critical pulmonary stenosis; n = 5 hypoplastic left heart syndrome; n = 3 Ebstein's anomaly; n = 2 interrupted arch with hypoplastic left ventricle; n = 2 single ventricle; n = 1 each: double outlet right ventricle, tricuspid atresia, critical aortic stenosis, rhabdo-myosarkoma, corrected transposition of the great arteries.) Thirty-one patients entered the study. "
02/01/2000 - "Sixty three patients had other lesions: hypoplastic left ventricle with ventricular septal defect (n = 18), unbalanced complete atrioventricular canal (n = 9), complex double-outlet right ventricle (n = 14), double-inlet left ventricle (n = 11), tricuspid atresia with transposition of the great arteries (n = 6), and others (n = 5), including heterotaxia. "
5. CollagenIBA
6. Right ventricle hypoplasiaIBA
7. Pulmonary venous return anomalyIBA
8. Congenitally corrected transposition of the great arteriesIBA
05/01/2001 - "In the other 38 children the PA-VSD was considered "complex" (group II) owing to the presence of heterotaxia, tricuspid atresia, a double-inlet left ventricle, transposition of the great arteries and congenitally corrected transposition of the great arteries. "
11/15/2000 - "Mortality was highest in patients with congenitally corrected transposition of the great arteries (26%), tricuspid atresia (25%), and univentricular connection (23%). "
09/01/2012 - "Subgroups were studied as follows: (1) single ventricle with pulmonary stenosis (n = 18), (2) single ventricle with tricuspid atresia and Glenn/Fontan surgery (n = 10), (3) congenitally corrected transposition of the great vessels (TGV) or with switch atrial surgery (n = 10), and (4) CHD with right ventricle overload (n = 17). "
12/01/1998 - "Diagnoses were double outlet right ventricle (6), univentricular heart (6), tricuspid atresia (5), congenitally-corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis (3), right isomerism (2) and pulmonary atresia with atrioventricular canal (1). "
01/01/1993 - "The diagnoses included univentricular connections (8 patients), complex morphology (4 patients), congenitally corrected transposition of the great arteries (TGA) (3 patients), hypoplastic left heart syndrome (2 patients), Fallot's tetralogy, tricuspid atresia, TGA, pulmonary atresia with intact ventricular septum and atrial septal defect (ASD) and ventricular septal defect (VSD). "
9. Polytetrafluoroethylene (Teflon)FDA Link
10. OxygenIBA
07/15/1994 - "Oxygen uptake transient kinetics during constant-load exercise in children after operations of ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, or tricuspid valve atresia."
01/01/2011 - "The majority were single (73%), employed or full-time students (93%), with health insurance (94%), had a single left ventricle (78%), the diagnosis of tricuspid atresia or double inlet left ventricle (59%), lateral tunnel Fontan type (44%), history of arrhythmias (76%), left ventricle ejection fraction percentage >50 (66%), oxygen saturations >90% (70%), frequent headaches (50%), scoliosis (22%), varicose veins, ascites, and liver cirrhosis (46%), normal body mass index (59%), and New York Heart Association class I (48%) and II-III (52%). "
03/01/2015 - "We compared the oxygen saturation of cerebral blood flow between newborns with tricuspid atresia (TA; a disease showing univentricular circulation and hypoplasia of the right ventricle), those with transposition of the great arteries (TGA; a disease showing abnormal mixing of arterial and venous blood), and those with HLHS. "
08/01/1989 - "The cardiac defects that best responded to PGE1 were pulmonary atresia with or without ventricular septal defect, Ebstein's anomaly, tricuspid atresia, pulmonic stenosis, double outlet right ventricle, and those that the arterial oxygen saturation increase was less than 10 vol. "
05/01/1997 - "In 3 neonates suffering from cyanotic congenital heart disease (1 with tricuspid atresia and 2 with transposition of the great arteries) with arterial desaturation despite application of prostaglandins, balloon atrioseptostomy, and eventually inhaled nitric oxide during intermittent positive-pressure ventilation with an inspired oxygen fraction of 1, oxygenation could only be established by means of preoperative extracorporeal membrane oxygenation. "

Therapies and Procedures

1. Fontan Procedure (Fontan Operation)
2. Transplants (Transplant)
3. Catheters
4. Right Heart Bypass (Cavopulmonary Anastomosis)
5. Blalock-Taussig Procedure