Tricuspid valve (TV) atresia
- Sowmya Balasubramanian, MD, MSc
Sowmya Balasubramanian, MD, MSc
- Clinical Assistant Professor
- Lucile Packard Children’s Hospital and Stanford Medical School
- Theresa Tacy, MD
Theresa Tacy, MD
- Associate Professor of Pediatrics
- Stanford University Medical Center
Tricuspid valve (TV) atresia is a cyanotic congenital heart lesion that is characterized by congenital agenesis or absence of the tricuspid valve, resulting in no direct communication between the right atrium and ventricle . If untreated, TV atresia has a high mortality rate with a survival rate as low as 10 percent at one year of age, depending on the type of TV atresia and the presence of other cardiac lesions [2,3].
The constellation of anatomic variants, physiology, clinical presentation, diagnosis, and management of TV atresia will be reviewed here.
TV atresia is the third most common cyanotic heart lesion with an estimated prevalence of 0.5 to 1.2 per 10,000 live births [4-7]. There is no difference in the incidence based on gender.
Although TV atresia is characterized by the absence of the TV, there is a spectrum of anatomic variants based on the morphology of the atresia and the presence of other cardiac structural lesions.
Morphology of TV atresia — Morphologic variation of TV atresia includes the following [8,9]:To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Rao PS. Diagnosis and management of cyanotic congenital heart disease: part I. Indian J Pediatr 2009; 76:57.
- Dick M, Fyler DC, Nadas AS. Tricuspid atresia: clinical course in 101 patients. Am J Cardiol 1975; 36:327.
- Keating P, Van der Merwe, Shipton . Tricuspid atresia--profile and outcome. Cardiovasc J S Afr 2001; 12:202.
- Rao PS. Tricuspid Atresia. Curr Treat Options Cardiovasc Med 2000; 2:507.
- Report of the New England Regional Infant Cardiac Program. Pediatrics 1980; 65:375.
- Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39:1890.
- Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 2008; 153:807.
- Rao PS. A unified classification for tricuspid atresia. Am Heart J 1980; 99:799.
- Weinberg PM. Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy. Ann Thorac Surg 1980; 29:306.
- Rao PS, Jue KL, Isabel-Jones J, Ruttenberg HD. Ebstein's malformation of the tricuspid valve with atresia. Differentiation from isolated tricuspid atresia. Am J Cardiol 1973; 32:1004.
- Sittiwangkul R, Azakie A, Van Arsdell GS, et al. Outcomes of tricuspid atresia in the Fontan era. Ann Thorac Surg 2004; 77:889.
- Rao PS. Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications. Br Heart J 1977; 39:276.
- Tandon R, Edwards JE. Tricuspid atresia. A re-evaluation and classification. J Thorac Cardiovasc Surg 1974; 67:530.
- Svensson EC, Huggins GS, Lin H, et al. A syndrome of tricuspid atresia in mice with a targeted mutation of the gene encoding Fog-2. Nat Genet 2000; 25:353.
- Kumar A, Victorica BE, Gessner IH, Alexander JA. Tricuspid atresia and annular hypoplasia: report of a familial occurrence. Pediatr Cardiol 1994; 15:201.
- Wald RM, Tham EB, McCrindle BW, et al. Outcome after prenatal diagnosis of tricuspid atresia: a multicenter experience. Am Heart J 2007; 153:772.
- Tongsong T, Sittiwangkul R, Wanapirak C, Chanprapaph P. Prenatal diagnosis of isolated tricuspid valve atresia: report of 4 cases and review of the literature. J Ultrasound Med 2004; 23:945.
- Berg C, Lachmann R, Kaiser C, et al. Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome. Ultrasound Obstet Gynecol 2010; 35:183.
- Donofrio MT, Bremer YA, Moskowitz WB. Diagnosis and management of restricted or closed foramen ovale in fetuses with congenital heart disease. Am J Cardiol 2004; 94:1348.
- Galindo A, Comas C, Martínez JM, et al. Cardiac defects in chromosomally normal fetuses with increased nuchal translucency at 10-14 weeks of gestation. J Matern Fetal Neonatal Med 2003; 13:163.
- CAMPBELL M. Tricuspid atresia and its prognosis with and without surgical treatment. Br Heart J 1961; 23:699.
- Elliott L, Vanmierop L, Gleason DC, Schiebler GL. The roentgenology of tricuspid atresia. Seminars in Roentgenology 1968; 3:399.
- Davachi F, Lucas RV Jr, Moller JH. The electrocardiogram and vectorcardiogram in tricuspid atresia. Correlation with pathologic anatomy. Am J Cardiol 1970; 25:18.
- Mair DD, Puga FJ, Danielson GK. The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. J Am Coll Cardiol 2001; 37:933.
- Lee TM, Aiyagari R, Hirsch JC, et al. Risk factor analysis for second-stage palliation of single ventricle anatomy. Ann Thorac Surg 2012; 93:614.
- Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26:240.
- Kreutzer G, Galíndez E, Bono H, et al. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 1973; 66:613.
- Kreutzer GO, Schlichter AJ, Kreutzer C. The Fontan/Kreutzer procedure at 40: an operation for the correction of tricuspid atresia. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2010; 13:84.
- Sridhar A, Giamberti A, Foresti S, et al. Fontan conversion with concomitant arrhythmia surgery for the failing atriopulmonary connections: mid-term results from a single centre. Cardiol Young 2011; 21:665.
- Mavroudis C, Backer CL, Deal BJ. Late reoperations for Fontan patients: state of the art invited review. Eur J Cardiothorac Surg 2008; 34:1034.
- Sheikh AM, Tang AT, Roman K, et al. The failing Fontan circulation: successful conversion of atriopulmonary connections. J Thorac Cardiovasc Surg 2004; 128:60.
- Committee on Infectious Diseases. From the American Academy of Pediatrics: Policy statements--Modified recommendations for use of palivizumab for prevention of respiratory syncytial virus infections. Pediatrics 2009; 124:1694.
- Morphology of TV atresia
- Associated cardiac lesions
- CLINICAL PRESENTATION
- Fetal presentation
- Postnatal presentation
- - Physical examination
- - Initial tests
- Pulse oximetry
- - Chest radiography
- - Electrocardiogram
- Natural course
- Fetal diagnosis
- Postnatal diagnosis
- - Echocardiography
- - Other imaging tests
- DIFFERENTIAL DIAGNOSIS
- Initial medical management
- Surgical management
- - First stage
- - Second stage
- - Interstage management
- - Third stage
- LONG-TERM MANAGEMENT
- SUMMARY AND RECOMMENDATIONS