Truncus Arteriosus Workup

  • Author: Doff B McElhinney, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: May 18, 2012
 

Laboratory Studies

  • Routine laboratory studies in the neonate with truncus arteriosus generally aid in determining therapeutic strategy rather than diagnosis.
    • An important exception in some cases is an arterial blood gas measurement, which helps to evaluate the degree of acidosis on presentation and may aid in differentiating cardiac disease from primary pulmonary pathology when performed before and after administration of 100% inspired oxygen (hyperoxia test).
    • Note that a hyperoxia test may be misleading in patients with truncus arteriosus and torrential pulmonary blood flow, both because of severe mismatch between pulmonary and systemic blood flow and, in some cases, because of streaming of left and right ventricular outflow into the systemic and pulmonary arterial systems, respectively.
  • Measurement of serum electrolytes, including total and ionized calcium, is important in patients with truncus arteriosus, given the common association with DiGeorge syndrome, which frequently may include hypoparathyroidism and hypocalcemia.
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Imaging Studies

  • Chest radiography
    • Upon presentation, obtain a chest radiograph in most patients with truncus arteriosus.
    • Cardiomegaly and increased pulmonary vascular markings are typically present, and fullness in the region of the truncal root may possibly be discerned. In patients with a right aortic arch, this radiographic finding in association with increased pulmonary vascular markings suggests truncus arteriosus.
  • Echocardiography
    • Echocardiography with cross-sectional and Doppler flow analysis is sufficient to confirm diagnosis of truncus arteriosus and fully characterize the various anatomic features in most patients. A full complement of echocardiographic views is necessary to ensure complete and accurate definition of the anatomy and potential associated anomalies.
    • Using subcostal coronal and parasternal long-axis images is the best way to demonstrate the single arterial trunk arising from the ventricles, with variable override of the ventricular septum. These views also demonstrate the thickness and mobility of the truncal valve leaflets. In general, the subcostal coronal view allows delineation of the pulmonary arterial origin(s) from the common trunk, although additional views are helpful to more completely characterize the pulmonary arterial anatomy. Morphology of the truncal valve and origins and course of the proximal coronary arteries are best observed from the parasternal short-axis window.
    • Doppler color imaging from these windows is critical to evaluate pulmonary arterial flow and regurgitation or stenosis of the truncal valve. Imaging from the high parasternal and suprasternal notch views is necessary to define the aortic arch and provide additional perspective on the anatomy of the central pulmonary arteries. Standard approaches to imaging of the ventricular masses, atrioventricular valves, and atriums are important for full characterization of their structure and function.
    • Increasingly, diagnosis of truncus arteriosus and other complex heart defects is made by prenatal echocardiography. Prenatal echocardiography is generally accurate and sensitive for the diagnosis of truncus arteriosus, although the precise diagnosis may be difficult to distinguish from similar anomalies, such as tetralogy of Fallot or double-outlet right ventricle with pulmonary atresia, or aortic atresia with a ventricular septal defect.[6]
    • An echocardiographic image of truncus arteriosus is shown in the image below.Echocardiographic images of truncus arteriosus (TAEchocardiographic images of truncus arteriosus (TA). The top image is from the subcostal coronal window (SC COR) and shows the common trunk (TR) arising from the left ventricle (LV), overriding the interventricular septum. The common trunk branches into the pulmonary trunk and the ascending aorta (AO). The left pulmonary artery (LPA) may be seen branching from the pulmonary trunk. RA=right atrium; RPA=right pulmonary artery. In the bottom image, which is from the suprasternal notch sagittal window, the truncal origin and course of the pulmonary trunk and left pulmonary artery can be appreciated. DAO=descending aorta; IV=innominate vein; LA=left atrium.
  • MRI
    • MRI is rarely necessary in patients with truncus arteriosus.
    • MRI modality provides excellent imaging for characterizing anatomy, and may be especially useful in reconstructing complex pulmonary arterial anatomy in older patients with truncus arteriosus.
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Other Tests

  • Electrocardiography
    • Electrocardiographic (ECG) findings in young infants with truncus arteriosus do not distinguish this lesion from others on the differential diagnosis.
    • A normal sinus rhythm, normal intervals, and either a normal QRS axis or minimal right-axis deviation are generally observed. Biventricular hypertrophy is a characteristic finding.
    • In patients with substantial pulmonary overcirculation, left ventricular forces are especially prominent with evidence of left atrial enlargement.
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Procedures

  • Cardiac catheterization
    • Standard angiographic images from the truncal root can aid in the assessment of coronary arterial anatomy, if echocardiography is inadequate, and in the assessment of regurgitation through the truncal valve.
    • Cardiac catheterization is generally not required prior to repair in neonates and young infants with truncus arteriosus.[7]
    • Catheterization is an important tool for evaluating some of the most common anatomic problems in patients with repaired truncus arteriosus, such as obstruction of the surgical reconstructed right ventricular outflow tract, branch pulmonary arterial stenosis, truncal valve regurgitation, and, in patients repaired later in life, pulmonary vascular obstructive disease.
  • Transcatheter balloon dilation: Treatment of pulmonary outflow tract or pulmonary arterial obstruction with transcatheter balloon dilation or stenting is an effective therapy for these problems in many patients.
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Histologic Findings

Histologic examination is not generally indicated in the evaluation of patients with truncus arteriosus. In the rare older patient with evidence of elevated pulmonary vascular resistance, pulmonary biopsy is occasionally performed as a means of assessing the extent of pulmonary vascular obstructive disease.

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Contributor Information and Disclosures
Author

Doff B McElhinney, MD  Assistant Professor of Pediatrics, Harvard Medical School; Associate in Cardiology, Department of Cardiology, Children's Hospital of Boston

Doff B McElhinney, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

Disclosure: Nothing to disclose.

Coauthor(s)

Gil Wernovsky, MD, FACC, FAAP  Professor, Department of Pediatrics, University of Pennsylvania, Children's Hospital of Philadelphia

Gil Wernovsky, MD, FACC, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Juan Carlos Alejos, MD  Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation

Disclosure: Actelion Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD  Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

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Anatomic subtypes of truncus arteriosus (TA), according to the classification systems of both Collett and Edwards (I, II, III) and the Van Praaghs (A1, A2, A3, A4).
Pathologic specimen with truncus arteriosus (TA), viewed through the opened right ventricle and truncal valve. The common trunk (CT) can be seen giving off the ascending aorta (AA) as well as the left (LPA) and right (RPA) pulmonary arteries. The truncal valve straddles the ventricular septal defect (VSD). The tricuspid valve (TV) also is labeled. Photograph courtesy of Robert H. Anderson, MD.
Pathologic specimen with truncus arteriosus (TA) and interruption of the aortic arch between the left (L) common carotid (CCA) and subclavian (SCA) arteries, viewed from the anterior aspect. The common trunk (CT) is seen arising from the ventricular mass, including the right ventricular (RV) infundibulum. Pulmonary arteries arise as a single trunk from the leftward aspect of the common trunk, which then divides into left and right branches (not shown) and the arterial duct (DA), which continues into the descending aorta, from which the left subclavian artery arises. The ascending aorta (AA), which supplies only the right (R) and left common carotid arteries (the right subclavian artery, which arises anomalously as the last brachiocephalic branch, is not shown), continues from the rightward aspect of the common trunk and is much smaller than in patients without an interrupted arch. RA=right atrial appendage. Photograph courtesy of Robert H. Anderson, MD.
Echocardiographic images of truncus arteriosus (TA). The top image is from the subcostal coronal window (SC COR) and shows the common trunk (TR) arising from the left ventricle (LV), overriding the interventricular septum. The common trunk branches into the pulmonary trunk and the ascending aorta (AO). The left pulmonary artery (LPA) may be seen branching from the pulmonary trunk. RA=right atrium; RPA=right pulmonary artery. In the bottom image, which is from the suprasternal notch sagittal window, the truncal origin and course of the pulmonary trunk and left pulmonary artery can be appreciated. DAO=descending aorta; IV=innominate vein; LA=left atrium.
 
 
 
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