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Tetralogy of Fallot With Pulmonary Stenosis Workup

  • Author: Michael D Pettersen, MD; Chief Editor: John Kupferschmid, MD  more...
 
Updated: Jan 08, 2014
 

Approach Considerations

The diagnosis of tetralogy of Fallot (TOF) with pulmonary stenosis may be established with fetal ultrasonography. Until the early to mid 1990s, the overwhelming majority of patients with tetralogy of Fallot underwent diagnostic cardiac catheterization before 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.

Routine blood studies indicated in patients with tetralogy of Fallot with pulmonary stenosis include a complete blood cell (CBC) count, chemistry panel, and coagulation studies, such as prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet count.

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Radiography

The radiographic appearance of tetralogy of Fallot (TOF) varies with whether the condition is cyanotic or acyanotic.

Cyanotic tetralogy of Fallot

The cardiac size appears normal or smaller than normal, and pulmonary vascular markings are decreased.

In tetralogy of Fallot with pulmonary atresia, black lung fields are seen.

A concave main pulmonary artery segment with an upturned cardiac apex (ie, coeur en sabot [boot-shaped heart]) is characteristic.

Right atrial enlargement (25%) and a 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 hypertrophy (RVH) rather than left ventricular hypertrophy (LVH) on the electrocardiogram (ECG).

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Electrocardiography

On electrocardiogram (ECG), right axis deviation +120 to ±150° is present in cyanotic tetralogy of Fallot (TOF). In the acyanotic form, the QRS axis is normal.

On ECG, right ventricular hypertrophy (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.

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Echocardiography

Two-dimensional (2-D) echocardiography and Doppler ultrasonographic studies are the diagnostic modalities of choice for Tetralogy of Fallot (TOF). Echocardiographic results confirm the diagnosis and help in quantitating the severity of tetralogy of Fallot.

A large, perimembranous, infundibular ventricular septal defect (VSD) and overriding of the aorta are depicted in the parasternal long-axis view.

The anatomy of the right ventricular (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, and anomalous coronary artery distribution can be accurately assessed with echocardiographic studies.

Perioperative echocardiographic studies

Echocardiography is the diagnostic modality of choice for the preoperative evaluation of patients with tetralogy of Fallot. This technique is also the diagnostic modality of choice for the postoperative follow-up evaluation of patients with both palliated and repaired tetralogy of Fallot.

Transesophageal echocardiography (TEE) is used in the operating room to plan the repair and to assess the success of the repair.

Preoperative versus postoperative findings

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. 2-D echocardiography and Doppler ultrasonographic 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.

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Cardiac Catheterization

In the modern era, preoperative cardiac catheterization is reserved for certain high-risk patients with tetralogy of Fallot (TOF).

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 these data can typically be obtained with echocardiography.

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Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

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, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Acknowledgements

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.

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.

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.

Vibhuti N Singh, MD, MPH, FACC, FSCAI Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine; Director, Cardiology Division and Cardiac Catheterization Lab, Chair, Department of Medicine, Bayfront Medical Center, Bayfront Cardiovascular Associates; President, Suncoast Cardiovascular Research

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.

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

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