Tetralogy of Fallot Workup

  • Author: Shabir Bhimji, MD, PhD; Chief Editor: Park W Willis IV, MD   more...
 
Updated: May 7, 2012
 

Hematologic Studies

Hemoglobin and hematocrit values are usually elevated in proportion to the degree of cyanosis. Prolonged cyanosis causes reactive polycythemia that increases the oxygen-carrying capacity. The oxygen saturation in systemic arterial blood typically varies from 65-70%. All patients with tetralogy of Fallot who experience significant cyanosis have a tendency to bleed because of decreased clotting factors and low platelet count. Hyperviscosity and coagulopathy often ensue and are particularly deleterious in patients with a right-to-left intracardiac shunt. The usual findings are diminished coagulation factors, and diminished total fibrinogen, which are associated with prolonged prothrombin and coagulation times.

Stroke and brain abscess are natural corollaries caused by paradoxical blood flow.

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ABG and Oximetry

Arterial blood gas (ABG) results show varying oxygen saturation, but pH and partial pressure of carbon dioxide (pCO2) are normal, unless the patient is in extremis, such as during a tet spell.

Oximetry is particularly useful in a dark-skinned patient or an anemic patient whose level of cyanosis is not apparent. Generally, cyanosis is not evident until 3-5 g/dL of reduced hemoglobin is present.

A decrease in systemic vascular resistance (SVR) during exercise, bathing, or fever potentiates a right-to-left shunt and precipitates hypoxemia.

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Radiologic Studies

Imaging studies used in the evaluation of tetralogy of Fallot (TOF) include echocardiography, chest radiographs, and magnetic resonance imaging (MRI).

Echocardiography

Ductus arteriosus, muscular ventricular septal defect (VSD), or atrial septal defect (ASD) is accurately diagnosed with color-flow Doppler echocardiography. 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.

Echocardiograms will usually reveal a large VSD with an overriding aorta and variable degrees of right ventricular (RV) outflow tract obstruction (RVOTO).

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 tetralogy of Fallot is the classic boot-shaped heart (coeur en sabot) (see the following image).

Uplifted apex and absence of pulmonary artery segmUplifted apex and absence of pulmonary artery segment typifies the "coeur en sabot" (ie, boot-shaped heart) of tetralogy of Fallot.

Magnetic resonance imaging

MRI provides good delineation of the aorta, RVOT, VSDs, RV hypertrophy, and the pulmonary artery and its branches.[9] MRI can also 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.

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Electrocardiography

The use of electrocardiography (ECG) may be limited if multiple ventricular septal defects (VSDs) or coronary artery anomalies are present or if the distal pulmonary artery cannot be visualized adequately.

Right axis deviation (+120° to +150°) with right ventricular (RV) enlargement may be seen.[10] Combined ventricular hypertrophy and right atrial hypertrophy may be present.

If RV hypertrophy is absent on ECG, the diagnosis of tetralogy of Fallot should be in doubt.

A typical preoperative ECG is shown below.

Typical preoperative electrocardiogram (ECG) for tTypical preoperative electrocardiogram (ECG) for tetralogy of Fallot.

Partial or complete right bundle branch block may be present; this is especially true of patients after surgical repair (see the following image).

Typical findings on postoperative electrocardiograTypical findings on postoperative electrocardiogram (ECG) for tetralogy of Fallot.
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Cardiac Catheterization and Angiography

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.

Cardiac catheterization findings include the following:

  • Assessment of the pulmonary annulus size and pulmonary arteries
  • Assessment of the severity of right ventricular (RV) outflow tract obstruction (RVOTO)
  • Location of the position and size of the ventricular septal defect (VSD)
  • Eliminating/ruling out possible coronary artery anomalies

Automatic internal cardiac defibrillator (AICD) placement is recommended in patients with sustained ventricular tachycardia and those resuscitated after a sudden death event.[11, 12]

Angiograms help identify coronary artery anomalies (see the image below); however, catheterization is not mandatory in all patients. Cardiac catheterization is extremely useful if the anatomy cannot be completely defined by echocardiography, if disease in the pulmonary arteries is a concern, or if pulmonary vascular hypertension is possible.

This angiogram shows a catheter in the right ventrThis angiogram shows a catheter in the right ventricle—severe infundibular stenosis.
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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)

Gary Setnik, MD  Chair, Department of Emergency Medicine, Mount Auburn Hospital; Assistant Professor, Division of Emergency Medicine, Harvard Medical School

Gary Setnik, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: SironaHealth Salary Management position; South Middlesex EMS Consortium Salary Management position; ProceduresConsult.com Royalty Other

Mary C Mancini, MD, PhD  Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in 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.

Theodore J Gaeta, DO, MPH, FACEP  Clinical Associate Professor, Department of Emergency Medicine, Weill Cornell Medical College; Vice Chairman and Program Director of Emergency Medicine Residency Program, Department of Emergency Medicine, New York Methodist Hospital; Academic Chair, Adjunct Professor, Department of Emergency Medicine, St George's University School of Medicine

Theodore J Gaeta, DO, MPH, FACEP is a member of the following medical societies: Alliance for Clinical Education, American College of Emergency Physicians, Clerkship Directors in Emergency Medicine, Council of Emergency Medicine Residency Directors, New York Academy of Medicine, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

David A Donson, MD  Assistant Medical Director, Department of Emergency Medicine, Maimonides Medical Center

David A Donson, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH  Professor of Medicine, Director of CME Programs, Team Leader, Root Cause Analysis, Tulane University Heart and Vascular Institute; Director of In-Patient Cardiology, Tulane Service, University Hospital; Visiting Physician, Medical Center of Louisiana at New Orleans; Faculty, Pennington Biomedical Research Institute, Louisiana State University; Professor, Tulane University School of Medicine

Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH is a member of the following medical societies: Alpha Omega Alpha, American Chemical Society, American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Society for Pharmacology and Experimental Therapeutics, American Society of Hypertension, American Thoracic Society, Heart Failure Society of America, Louisiana State Medical Society, National Lipid Association, and Southern Society for Clinical Investigation

Disclosure: Forest Labs Honoraria Speaking and teaching

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

Disclosure: Medscape 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; Medtronic Consulting fee Consulting; Novartis Consulting fee Consulting

David FM Brown, MD  Associate Professor, Division of Emergency Medicine, Harvard Medical School; Vice Chair, Department of Emergency Medicine, Massachusetts General Hospital

David FM Brown, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine

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.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Mark Spektor, DO, and Kurt Pflieger, MD, FAAP, to the development and writing of the source article.

References

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Anatomic findings in tetralogy of Fallot.
Typical preoperative electrocardiogram (ECG) for tetralogy of Fallot.
Typical findings on postoperative electrocardiogram (ECG) for tetralogy of Fallot.
Uplifted apex and absence of pulmonary artery segment typifies the "coeur en sabot" (ie, boot-shaped heart) of tetralogy of Fallot.
This angiogram shows a catheter in the right ventricle—severe infundibular stenosis.
This image shows completed blocking with a Taussig shunt
An opening in the right ventricle exposes ventricular septal defect
Interrupted pledgetted sutures are used to close a ventricular septal defect.
This image shows a closed ventricular septal defect and closure of right ventriculotomy with Gore-Tex.
Gore-Tex is used for complete closure of right ventriculotomy.
 
 
 
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