eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Tetralogy of Fallot: Surgical Perspective: Workup

Author: Vibhuti N Singh, MD, MPH, FACC, FSCAI, Director, Suncoast Cardiovascular Center; Chair, Cardiology Division and Cath Labs, Department of Medicine, Bayfront Medical Center; Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine
Coauthor(s): Jeffrey P Jacobs, MD, FACS, FACC, FCCP, Clinical Associate Professor, Department of Surgery, University of South Florida College of Medicine; Medical Director, ECMO Program, Division of Thoracic and Cardiovascular Surgery, All Children's Hospital/Bayfront Medical Center
Contributor Information and Disclosures

Updated: Nov 13, 2008

Workup

Laboratory Studies

Routine blood studies are indicated in patients with tetralogy of Fallot (TOF).

  • CBC count
  • Chemistry panel
  • Coagulation parameters
    • Prothrombin time (PT)
    • Activated partial thromboplastin time (aPTT)
    • Platelet count

Imaging Studies

  • Radiography
    • Cyanotic tetralogy of Fallot 
      • Cardiac size is normal or smaller than normal, and pulmonary vascular markings are decreased.
      • Black lung fields are seen in tetralogy of Fallot with pulmonary atresia.
      • Concave main pulmonary artery segment with an upturned cardiac apex (ie, coeur en sabot [boot-shaped heart]) is characteristic.
      • Right atrial enlargement (25%) and right aortic arch (25%) may be present.
    • Acyanotic tetralogy of Fallot: Radiographic findings of acyanotic tetralogy of Fallot are indistinguishable from those in a small-to-moderate ventricular septal defect (VSD), but patients with tetralogy of Fallot have right ventricular (RV) hypertrophy (RVH) rather than left ventricular hypertrophy (LVH) on the ECG.
  • Echocardiography
    • Two-dimensional echocardiography and Doppler studies are the diagnostic modalities of choice. Echocardiographic results confirm the diagnosis and help in quantitating the severity of tetralogy of Fallot.
      • A large, perimembranous, infundibular VSD and overriding of the aorta are depicted in the parasternal long-axis view.
      • The anatomy of the RV outflow tract (RVOT), the pulmonary valve, the pulmonary annulus, and the main pulmonary artery and its branches is depicted in the parasternal short-axis view.
    • Doppler studies are helpful to estimate the pressure gradient across the obstruction in the RVOT.
    • Associated anomalies, such as atrial septal defect (ASD) and persistence of the left superior vena cava (SVC), can be imaged.
    • Anomalous coronary artery distribution can be accurately assessed by means of echocardiographic studies.
    • Until the early to mid 1990s, the overwhelming majority of patients with tetralogy of Fallot underwent diagnostic cardiac catheterization prior to surgical repair. Since the mid 1990s, most centers have surgically repaired the majority of patients with tetralogy of Fallot with preoperative echocardiography without preoperative cardiac catheterization.
    • Echocardiography is the diagnostic modality of choice for the preoperative evaluation of patients with tetralogy of Fallot.
    • Transesophageal echocardiography (TEE) is used in the operating theatre to plan the repair and to assess the success of the repair.
    • Echocardiography is the diagnostic modality of choice for the postoperative follow-up evaluation of patients with both palliated and repaired tetralogy of Fallot.
  • Preoperative versus postoperative findings may be summarized as follows: Before surgery, tetralogy of Fallot represents a broad spectrum of VSD sizes and RVOT obstructions (RVOTOs). After surgery, residual abnormalities range from a nearly normal-appearing heart to one with substantial RV dysfunction and residual RVOTO. Two-dimensional echocardiography and Doppler techniques can be definitive means for monitoring patients with respect to their recovery of RV function and complications, such as recurrent RVOTO and residual or recurrent VSD.

Other Tests

  • On ECG, right axis deviation +120 to ± 150° is present in cyanotic tetralogy of Fallot. In the acyanotic form, the QRS axis is normal.
  • On ECG, RVH is usually present, but the strain pattern is unusual. Combined ventricular hypertrophy (CVH) may be seen in the acyanotic form. Right atrial hypertrophy (RAH) is occasionally present.

Diagnostic Procedures

  • In the modern era, preoperative cardiac catheterization is reserved for certain high-risk patients with tetralogy of Fallot.
    • In patients with tetralogy of Fallot with pulmonary atresia, cardiac catheterization is used to assess the anatomy, size, and distribution of the peripheral pulmonary artery. The presence, origin, and insertion of major aortopulmonary collateral arteries (MAPCAs) should be documented.
    • In preoperative patients before complete repair status but after previous systemic-to-pulmonary artery shunting, cardiac catheterization allows visualization of the shunt and the pulmonary artery at the shunt insertion site.
    • Preoperative cardiac catheterization solely for the assessment of coronary artery anatomy is not necessary because this data can typically be obtained with echocardiography.
  • Preoperative diagnostic studies must provide the surgeon with the following data:
    • The number, size, and location of all VSDs
    • The severity and location of RVOTO
    • The size and distribution of the pulmonary artery
    • The origins and branching pattern of the coronary arteries
    • The origin and distribution of all sources of pulmonary blood flow, including MAPCAs

More on Tetralogy of Fallot: Surgical Perspective

Overview: Tetralogy of Fallot: Surgical Perspective
Workup: Tetralogy of Fallot: Surgical Perspective
Treatment: Tetralogy of Fallot: Surgical Perspective
Follow-up: Tetralogy of Fallot: Surgical Perspective
References
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References

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Further Reading

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Keywords

tetralogy of Fallot, TOF, Fallot's tetralogy, Fallot's tetrad, ventricular septal defect, VSD, right ventricular outflow tract obstruction, RVOTO, aorta overriding the ventricular septum, right ventricular hypertrophy, RVH, common atrioventricular canal, atrioventricular septal defect, AVSD, complete AVSD, cyanotic heart defect, absent pulmonary valve syndrome, pulmonary atresia, pulmonary stenosis, infundibular stenosis, polycythemia, growth retardation, brain abscess, stroke, aortic regurgitation, atrial septal defect, secundum atrial septal defect, patent foramen ovale, patent ductus arteriosus 

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Contributor Information and Disclosures

Author

Vibhuti N Singh, MD, MPH, FACC, FSCAI, Director, Suncoast Cardiovascular Center; Chair, Cardiology Division and Cath Labs, Department of Medicine, Bayfront Medical Center; Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine
Vibhuti N Singh, MD, MPH, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, and Florida Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Jacobs, MD, FACS, FACC, FCCP, Clinical Associate Professor, Department of Surgery, University of South Florida College of Medicine; Medical Director, ECMO Program, Division of Thoracic and Cardiovascular Surgery, All Children's Hospital/Bayfront Medical Center
Jeffrey P Jacobs, MD, FACS, FACC, FCCP is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Congenital Heart Surgeons Society, Society of Thoracic Surgeons, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

Jeff L Myers, MD, PhD, Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School
Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American Heart Association, and International Society for Heart and Lung Transplantation
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Robert DB Jaquiss, MD, Professor of Surgery, University of Arkansas for Medical Sciences; Chief, Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital and Chief, Cardiothoracic Surgery, University of Arkansas for Medical Sciences
Robert DB Jaquiss, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, Congenital Heart Surgeons Society, International Society for Heart and Lung Transplantation, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting; Pfizer Honoraria Consulting

Chief Editor

John Kupferschmid, MD, Director of Congenital Heart Surgery, Department of Surgery, Methodist Children's Hospital at San Antonio
John Kupferschmid, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, Society of Thoracic Surgeons, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

 
 
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