Tricuspid Atresia Workup

  • Author: Mary C Mancini, MD, PhD; Chief Editor: Richard A Lange, MD   more...
 
Updated: May 26, 2011
 

Laboratory Studies

  • CBC count: Because of cyanosis in this population, polycythemia may be present.
  • Prothrombin time and activated partial thromboplastin time: Findings from these studies may be abnormal secondary to the polycythemia.
Next

Imaging Studies

  • Chest radiography
    • Cardiomegaly is usually present, with a prominent right heart border that reflects enlargement of the right atrium.
    • In 80% of patients, pulmonary vascular markings are diminished because of diminished pulmonary blood flow. Pulmonary vascular markings may be increased when pulmonary flow is not obstructed.
    • A right aortic arch may be observed in 3-8% of cases.Tricuspid atresia. Frontal chest radiograph in a cTricuspid atresia. Frontal chest radiograph in a child with tricuspid atresia and a nonrestrictive ventricular septal defect. There is pulmonary plethora. Note the prominent right atrium. Tricuspid atresia. Frontal chest radiograph in a cTricuspid atresia. Frontal chest radiograph in a child with tricuspid atresia and a nonrestrictive ventricular septal defect, mild pulmonary plethora and, atypically, a right aortic arch (arrow). Note enlarged right atrium and the typical rounded configuration of the left cardiac apex. In the absence of the right ventricle, the left ventricle becomes hypertrophied and dilated, causing the development of a more rounded cardiac apex. Tricuspid atresia. Frontal chest radiograph in an Tricuspid atresia. Frontal chest radiograph in an adult with untreated tricuspid atresia. Increased pulmonary blood flow through a nonrestrictive ventricular septal defect has been tolerated for years but has led to the development of pulmonary hypertension, as shown by the large proximal pulmonary arteries (arrows) and pruned distal pulmonary arteries. The development of pulmonary hypertension prevents conventional surgical treatment.
  • Echocardiography
    • The diagnosis of tricuspid atresia can be reliably established with this noninvasive method.
    • The basic anatomy, size of the atrial septal defect, relationship of the great vessels, degree of pulmonary blood flow, ventricular function, and valvular function can be easily ascertained using a combination of M-mode, 2-dimensional, and color-flow echocardiography.[3]
Previous
Next

Other Tests

  • Electrocardiography
    • Sinus rhythm is generally present, with tall P waves indicative of atrial enlargement.
    • First-degree atrioventricular block may be observed.
    • Because of the origin of the left bundle branch from a common bundle, the frontal plane QRS axis may be leftward or superior.
Previous
Next

Procedures

  • Cardiac catheterization
    • In infants, the primary use of cardiac catheterization is to determine the source and reliability of pulmonary blood flow and, in particular, to assess the status of the patent ductus arteriosus. If a restrictive atrial septal defect is present, then balloon septostomy can be performed in this setting.
    • In the older population, arteriography is used to define details important in surgical management such as number and relationship of vena cavae, size of the pulmonary arteries, pulmonary artery resistance, mitral valve competency, and definition of prior operative procedures.
Previous
Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

Mary C Mancini, MD, PhD  Professor and Chief, Cardiothoracic Surgery, Department of Surgery, Louisiana State University Health Sciences Center-Shreveport

Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: eMedicine Salary Employment

Ronald J Oudiz, MD, FACP, FACC, FCCP  Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center

Ronald J Oudiz, MD, FACP, FACC, FCCP is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, and American Thoracic Society

Disclosure: Actelion Grant/research funds Clinical Trials + honoraria; Encysive Grant/research funds Clinical Trials + honoraria; Gilead Grant/research funds Clinical Trials + honoraria; Pfizer Grant/research funds Clinical Trials + honoraria; United Therapeutics Grant/research funds Clinical Trials + honoraria; Lilly Grant/research funds Clinical Trials + honoraria; LungRx Clinical Trials + honoraria; Bayer Grant/research funds Consulting

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD  Professor and Executive Vice Chairman, Department of Medicine, Director, Office of Educational Programs, University of Texas Health Science Center at San Antonio

Richard A Lange, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, and Association of Subspecialty Professors

Disclosure: Nothing to disclose.

References

  1. Tandon R, Edwards JE. Tricuspid atresia. A re-evaluation and classification. J Thorac Cardiovasc Surg. Apr 1974;67(4):530-42. [Medline].

  2. Weinberg PM. Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy. Ann Thorac Surg. Apr 1980;29(4):306-11. [Medline].

  3. Karamlou T, Ashburn DA, Caldarone CA, et al. Matching procedure to morphology improves outcomes in neonates with tricuspid atresia. J Thorac Cardiovasc Surg. Dec 2005;130(6):1503-10. [Medline].

  4. Airan B, Sharma R, Choudhary SK, et al. Univentricular repair: is routine fenestration justified?. Ann Thorac Surg. Jun 2000;69(6):1900-6. [Medline].

  5. Wong ML, Sim EK, Goh JJ, et al. Bidirectional cavopulmonary anastomosis. Ann Acad Med Singapore. Mar 1999;28(2):237-40. [Medline].

  6. Alexiou C, Delany DJ, Keeton BR, Monro JL. Double-barreled conduit for right atrioventricular connection in tricuspid atresia: a new technique. J Thorac Cardiovasc Surg. Oct 2000;120(4):820-2. [Medline].

  7. Annecchino FP, Fontan F, Chauve A, Quaegebeur J. Palliative reconstruction of the right ventricular outflow tract in tricuspid atresia: a report of 5 patients. Ann Thorac Surg. Apr 1980;29(4):317-21. [Medline].

  8. Behrendt DM, Rosenthal A. Cardiovascular status after repair by Fontan procedure. Ann Thorac Surg. Apr 1980;29(4):322-30. [Medline].

  9. Chopra PS, Rao PS. Corrective surgery for tricuspid atresia: which modification of Fontan- Kreutzer procedure should be used? A review. Am Heart J. Mar 1992;123(3):758-67. [Medline].

  10. Dore A, Somerville J. Right atrioventricular extracardiac conduit as a fontan modification: late results. Ann Thorac Surg. Jan 2000;69(1):181-5. [Medline].

  11. Freedom RM, Hamilton R, Yoo SJ, et al. The Fontan procedure: analysis of cohorts and late complications. Cardiol Young. Oct 2000;10(4):307-31. [Medline].

  12. Gale AW, Danielson GK, McGoon DC, et al. Fontan procedure for tricuspid atresia. Circulation. Jul 1980;62(1):91-6. [Medline].

  13. Haas GS, Hess H, Black M, et al. Extracardiac conduit fontan procedure: early and intermediate results. Eur J Cardiothorac Surg. Jun 2000;17(6):648-54. [Medline].

  14. Amanullah MM, Hasan A, Kirk R. Conduit from hypoplastic right ventricle to pulmonary artery in tricuspid atresia. Asian Cardiovasc Thorac Ann. Jan 2008;16(1):78-80. [Medline].

  15. Anderson RH, Wilkinson JL, Gerlis LM, et al. Atresia of the right atrioventricular orifice. Br Heart J. Apr 1977;39(4):414-28. [Medline].

  16. Durongpisitkul K, Porter CJ, Cetta F et al. Predictors of early- and late-onset supraventricular tachyarrhythmias after Fontan operation. Circulation. Sep 15 1998;98(11):1099-107. [Medline].

  17. Ensley AE, Lynch P, Chatzimavroudis GP, et al. Toward designing the optimal total cavopulmonary connection: an in vitro study. Ann Thorac Surg. Oct 1999;68(4):1384-90. [Medline].

  18. Facchini M, Guldenschuh I, Turina J, et al. Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia. J Cardiovasc Surg (Torino). Aug 2000;41(4):567-70. [Medline].

  19. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. May 1971;26(3):240-8. [Medline].

  20. Franklin RC, Spiegelhalter DJ, Sullivan ID, et al. Tricuspid atresia presenting in infancy. Survival and suitability for the Fontan operation. Circulation. Feb 1993;87(2):427-39. [Medline].

  21. Hager A, Fratz S, Schwaiger M, Lange R, Hess J, Stern H. Pulmonary blood flow patterns in patients with Fontan circulation. Ann Thorac Surg. Jan 2008;85(1):186-91. [Medline].

  22. Hess J. Long-term problems after cavopulmonary anastomosis: diagnosis and management. Thorac Cardiovasc Surg. Apr 2001;49(2):98-100. [Medline].

  23. Humes RA, Porter CJ, Mair DD, et al. Intermediate follow-up and predicted survival after the modified Fontan procedure for tricuspid atresia and double-inlet ventricle. Circulation. Sep 1987;76(3 Pt 2):III67-71. [Medline].

  24. Kirklin JK, Blackstone EH, Kirklin JW, et al. The Fontan operation. Ventricular hypertrophy, age, and date of operation as risk factors. J Thorac Cardiovasc Surg. Dec 1986;92(6):1049-64. [Medline].

  25. Lee CN, Schaff HV, Danielson GK, et al. Comparison of atriopulmonary versus atrioventricular connections for modified Fontan/Kreutzer repair of tricuspid valve atresia. J Thorac Cardiovasc Surg. Dec 1986;92(6):1038-43. [Medline].

  26. Ovroutski S, Ewert P, Alexi-Meskishvili V, et al. Comparison of somatic development and status of conduit after extracardiac Fontan operation in young and older children. Eur J Cardiothorac Surg. Dec 2004;26(6):1073-9. [Medline].

  27. Park SC, Neches WH, Mullins CE, et al. Blade atrial septostomy: collaborative study. Circulation. Aug 1982;66(2):258-66. [Medline].

  28. Rao PS. Tricuspid atresia. Mount Kisco, NY: Futura Publishing Co; 1982:13-24.

  29. Sanders SP, Wright GB, Keane JF, et al. Clinical and hemodynamic results of the Fontan operation for tricuspid atresia. Am J Cardiol. May 1982;49(7):1733-40. [Medline].

  30. Sano S, Ishino K, Kawada M, et al. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg. Nov 2000;70(5):1501-6. [Medline].

  31. Sarkozy A, Conti E, D'Agostino R, et al. ZFPM2/FOG2 and HEY2 genes analysis in nonsyndromic tricuspid atresia. Am J Med Genet A. Feb 15 2005;133(1):68-70. [Medline].

  32. Stefanelli G, Kirklin JW, Naftel DC, et al. Early and intermediate-term (10-year) results of surgery for univentricular atrioventricular connection ("single ventricle"). Am J Cardiol. Oct 1 1984;54(7):811-21. [Medline].

  33. Takeda M, Shimada M, Sekiguchi A, Ishizawa A. Long-term results of the fenestrated Fontan operation. Progress of patients with patent fenestrations. Jpn J Thorac Cardiovasc Surg. Sep 1999;47(9):432-9. [Medline].

  34. 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. Jul 2004;23(7):945-50. [Medline].

  35. Tzifa A, Gauvreau K, Geggel RL. Factors associated with development of atrial septal restriction in patients with tricuspid atresia involving the right-sided atrioventricular valve. Am Heart J. Dec 2007;154(6):1235-41. [Medline].

  36. van Doorn CA, de Leval MR. The Fontan operation in clinical practice: indications and controversies. Nat Clin Pract Cardiovasc Med. Mar 2005;2(3):116-7. [Medline].

  37. van Son JA, Mohr FW, Hambsch J, et al. Conversion of atriopulmonary or lateral atrial tunnel cavopulmonary anastomosis to extracardiac conduit Fontan modification. Eur J Cardiothorac Surg. Feb 1999;15(2):150-7; discussion 157-8. [Medline].

 
Previous
Next
 
Fontan procedure: Illustration of the atrial-to-pulmonary artery anastomosis.
Tricuspid atresia. Frontal chest radiograph in a child with tricuspid atresia and a nonrestrictive ventricular septal defect. There is pulmonary plethora. Note the prominent right atrium.
Tricuspid atresia. Frontal chest radiograph in a child with tricuspid atresia and a nonrestrictive ventricular septal defect, mild pulmonary plethora and, atypically, a right aortic arch (arrow). Note enlarged right atrium and the typical rounded configuration of the left cardiac apex. In the absence of the right ventricle, the left ventricle becomes hypertrophied and dilated, causing the development of a more rounded cardiac apex.
Tricuspid atresia. Frontal chest radiograph in an adult with untreated tricuspid atresia. Increased pulmonary blood flow through a nonrestrictive ventricular septal defect has been tolerated for years but has led to the development of pulmonary hypertension, as shown by the large proximal pulmonary arteries (arrows) and pruned distal pulmonary arteries. The development of pulmonary hypertension prevents conventional surgical treatment.
Tricuspid atresia. Axial ECG-gated spin-echo MRI in an adult patient with tricuspid atresia shows the high signal from atrioventricular sulcus tissue (black arrow), replacing the tricuspid valve, and an enlarged right atrium. Note how the mitral valve orientation (white arrows) is abnormal. The right ventricular outflow chamber (R) is anterior.
Tricuspid atresia. Axial ECG-gated spin-echo MRI (10 mm caudad to previous Image ) shows the high signal intensity from atrioventricular sulcus tissue and the restrictive ventricular septal defect (arrow) between the ventricle and the right ventricular outflow chamber. Note the dilated and rounded left ventricular cavity.
Tricuspid atresia. Axial ECG-gated spin-echo MRI in an adolescent patient with tricuspid atresia with modified Fontan repair. The Fontan conduit (white arrow) runs from the right atrium (A) around the front of the heart towards the pulmonary artery. Note that the front of the heart is identified by the anterior atrioventricular sulcus tissue containing the signal void of the right coronary artery (black arrow).
Tricuspid atresia. Axial ECG-gated spin-echo MRI in an adolescent patient with tricuspid atresia with modified Fontan repair (10 mm inferior to previous Image ). Thick atrioventricular sulcus tissue (arrow) is noted replacing the tricuspid valve. The ventricular septal defect has been repaired, and the ventricular septum is now intact.
Tricuspid atresia. Apical 4-chamber 2-dimensional echocardiogram shows atrioventricular sulcus tissue (solid arrow) replacing the tricuspid valve in a patient with tricuspid atresia. Note the enlarged right atrium posterior to the abnormal atrioventricular sulcus tissue. A moderate-sized ventricular septal defect (open arrow) is noted between the ventricle (V) and outflow chamber (C).
Tricuspid atresia. Fluoroscopic image shows a Park blade septostomy catheter with cutting blade extended in a patient with tricuspid atresia. The catheter has been passed through a restrictive atrial septal defect, which was resistant to balloon septostomy. The blade was used to make 2 cuts in the atrial septum, starting a tear, which then was completed using balloon septostomy.
Tricuspid atresia. Frontal ventriculogram in a patient with tricuspid atresia shows the pulmonary arteries arising from a small right ventricular type outflow chamber (arrow). A restrictive ventricular septal defect and a large globular ventricle (V) are noted.
Tricuspid atresia. Steep left anterior oblique ventriculogram in a patient with tricuspid atresia shows a restrictive ventricular septal defect (between arrows) and a typically large globular ventricle (V).
Tricuspid atresia. Steep left anterior oblique ventriculogram in a patient with tricuspid atresia shows a larger nonrestrictive ventricular septal defect (white arrow). A typically large globular ventricle (V) is seen, which is receiving inflow from a single atrioventricular valve (mitral valve, black arrows). Note how the aorta and pulmonary arteries are superimposed, making interpretation of their attachments difficult. Angiography must be performed in multiple projections to fully define complex relationships accurately.
Tricuspid atresia. Shallow right anterior oblique view from a ventriculogram in a patient with tricuspid atresia shows mitral regurgitation with contrast filling in both the left atrium (LA) and right atrium (RA), through the atrial septal defect. Contrast outlines the thick band of atrioventricular sulcus tissue (arrow), which is demonstrated well on cross-sectional imaging techniques.
Tricuspid atresia. Right anterior oblique ventriculogram in a patient with tricuspid atresia shows simultaneous filling of the aorta (Ao) and pulmonary arteries (PA). Nonrestrictive ventricular septal defect was present, which necessitated pulmonary artery banding (arrow) to reduce pulmonary blood flow and protect against development of pulmonary hypertension before proceeding to a Fontan procedure.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.