eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Tetralogy of Fallot: Workup

Author: Shabir Bhimji, MD, PhD, Locum Cardiothoracic and Vascular Surgeon, Saudi Arabia and Middle East Hospitals
Coauthor(s): Mary C Mancini, MD, PhD, Director of Cardiothoracic Transplantation, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Contributor Information and Disclosures

Updated: May 1, 2008

Workup

Laboratory Studies

Hemoglobin and hematocrit values are usually elevated in proportion to the degree of cyanosis. The oxygen saturation in the systemic arterial blood typically varies from 65-70%. All patients with TOF who experience significant cyanosis have a tendency to bleed because of decreased clotting factors and low platelet count. The usual findings are diminished coagulation factors. The total fibrinogen levels are also diminished and are associated with prolonged prothrombin and coagulation times.

Imaging Studies

  • Radiography
    • Initially, chest radiographs may not reveal any abnormality; however, diminished vascularity in the lungs and diminished prominence of the pulmonary arteries gradually become apparent.
    • The hallmark of TOF is the classic boot-shaped heart (coeur en sabot).
  • Echocardiography
    • Today, ductus arteriosus, muscular VSD, or ASD are accurately diagnosed with color-flow Doppler echocardiography (ECG). The coronary anatomy can be revealed with some degree of accuracy, and valvar alterations can be detected with ease. In many institutions, echocardiography is the only diagnostic study used before surgery.
    • The use of ECG may be limited if multiple VSDs or coronary artery anomalies are present or if the distal pulmonary artery cannot be visualized adequately.
  • Magnetic resonance imaging
    • Magnetic resonance imaging (MRI) provides good delineation of the aorta, right ventricular outflow tract, VSDs, right ventricular hypertrophy, and the pulmonary artery and its branches.
    • MRI can be used to measure intracardiac pressures, gradients, and blood flows.
    • Drawbacks to MRI include the need for prolonged imaging times and the requirement for sedation in small children to prevent motion artifacts. Additionally, sick infants cannot be observed when enclosed in an MRI tunnel.

Other Tests

  • Electrocardiography
    • Right axis deviation with right ventricular enlargement may be seen.
    • If right ventricular hypertrophy is absent on ECG, the diagnosis of TOF should be suspected.

Diagnostic Procedures

  • Cardiac catheterization
    • Cardiac catheterization provides angiographic visualization of ventricular and pulmonary artery size. Catheterization also helps obtain pressure and oxygen saturation measurements in different chambers and identifies any possible shunts. In the presence of preexisting shunts, angiograms should be obtained before complete surgical repair.
    • Angiograms help identify coronary artery anomalies; however, catheterization is not mandatory in all patients. Cardiac catheterization is extremely useful if the anatomy cannot be completely defined by ECG, if disease in the pulmonary arteries is a concern, or if pulmonary vascular hypertension is possible.

More on Tetralogy of Fallot

Overview: Tetralogy of Fallot
Workup: Tetralogy of Fallot
Treatment: Tetralogy of Fallot
Follow-up: Tetralogy of Fallot
Multimedia: Tetralogy of Fallot
References
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References

  1. Aboulhosn J, Child JS. Management after childhood repair of tetralogy of fallot. Curr Treat Options Cardiovasc Med. Dec 2006;8(6):474-83. [Medline].

  2. Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Results of two-stage surgical treatment of tetralogy of Fallot. J Thorac Cardiovasc Surg. Jun 1980;79(6):876-83. [Medline].

  3. Boechat MI, Ratib O, Williams PL, Gomes AS, Child JS, Allada V. Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. Nov-Dec 2005;25(6):1535-46. [Medline].

  4. Borow KM, Green LH, Castaneda AR, et al. Left ventricular function after repair of tetralogy of fallot and its relationship to age at surgery. Circulation. Jun 1980;61(6):1150-8. [Medline].

  5. Devore GR, Polanko B. Tomographic ultrasound imaging of the fetal heart: a new technique for identifying normal and abnormal cardiac anatomy. J Ultrasound Med. Dec 2005;24(12):1685-96. [Medline].

  6. Gustafson RA, Murray GF, Warden HE, et al. Early primary repair of tetralogy of Fallot. Ann Thorac Surg. Mar 1988;45(3):235-41. [Medline].

  7. Horer J, Friebe J, Schreiber C. Correction of tetralogy of Fallot and of pulmonary atresia with ventricular septal defect in adults. Ann Thorac Surg. 2005;80:2285-2291. [Medline].

  8. Kirklin JW, Blackstone EH, Kirklin JK, Pacifico AD, Aramendi J, Bargeron LM Jr. Surgical results and protocols in the spectrum of tetralogy of Fallot. Ann Surg. Sep 1983;198(3):251-65. [Medline].

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  11. Pacifico AD, Kirklin JK, Colvin EV, et al. Transatrial-transpulmonary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg. Jan 1990;2(1):76-82. [Medline].

  12. Pacifico AD, Ricchi A, Bargeron LM Jr, et al. Corrective repair of complete atrioventricular canal defects and major associated cardiac anomalies. Ann Thorac Surg. Dec 1988;46(6):645-51. [Medline].

  13. Patel CR, Agamanolis DP, Stewart JW. Prenatal diagnosis of tetralogy of Fallot with obstructed supracardiac totally anomalous pulmonary venous connection. Cardiol Young. Dec 2005;15(6):656-9. [Medline].

  14. Sakamoto T, Nagase Y, Hasegawa H, Shin'oka T, Tomimatsu H, Kurosawa H. One-stage intracardiac repair in combination with external stenting of the trachea and right bronchus for tetralogy of Fallot with an absent pulmonary valve and tracheobronchomalacia. J Thorac Cardiovasc Surg. Dec 2005;130(6):1717-8. [Medline].

  15. Sousa Uva M, Lacour-Gayet F, Komiya T, et al. Surgery for tetralogy of Fallot at less than six months of age. J Thorac Cardiovasc Surg. May 1994;107(5):1291-300. [Medline].

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Further Reading

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Keywords

tetralogy of Fallot, TOF, Fallot tetrad, pink tetralogy, wooden-shoe heart, boot-shaped heart, coeur en sabot, sabot heart, congenital heart disorders, CHD, congestive heart failure, CHF, cyanosis, cyanotic heart disorder, congenital cardiac defects, ventricular septal defect, VSD, atrial septal defect, ASD, pulmonic valve atresia, pulmonic valve stenosis, infundibular stenosis, dextroposition of the aorta, right ventricular hypertrophy, pulmonary atresia, paradoxical emboli, stroke, pulmonary embolus, subacute bacterial endocarditis, right ventricle outflow tract obstruction, RVOTO, cardiopulmonary bypass, CPB, Blalock-Taussig shunt, pentad of Fallot, cleft lip, cleft palate, hypospadias, pentalogy of Fallot

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Contributor Information and Disclosures

Author

Shabir Bhimji, MD, PhD, Locum Cardiothoracic and Vascular Surgeon, Saudi Arabia and Middle East Hospitals
Shabir Bhimji, MD, PhD is a member of the following medical societies: American Cancer Society, American College of Chest Physicians, American Lung Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Mary C Mancini, MD, PhD, Director of Cardiothoracic Transplantation, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Heart Association, American Medical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, International College of Surgeons, International Society for Heart and Lung Transplantation, New York Academy of Sciences, Phi Beta Kappa, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

Gary E Sander, MD, PhD, Professor, Department of Internal Medicine, Division of Cardiology, Tulane University Health Sciences Center
Gary E Sander, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Heart Association, American Society of Hypertension, Heart Failure Society of America, Louisiana State Medical Society, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ronald J Oudiz, MD, Director of Pulmonary Hypertension, Associate Professor, Department of Medicine, Division of Cardiology, Harbor-UCLA Medical Center, David Geffen School of Medicine at UCLA
Ronald J Oudiz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, and American Heart Association
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

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
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

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.

 
 
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