Truncus Arteriosus Treatment & Management

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

Medical Care

  • Medical care before surgical repair depends on clinical presentation.
  • Most neonates with truncus arteriosus display some evidence of congestive heart failure; they are usually treated with digitalis and diuretic medicines.
  • Intravenous prostaglandin is often administered in patients with truncus arteriosus upon presentation because the differential diagnosis includes numerous anomalies with duct-dependent systemic or pulmonary blood flow. However, it is beneficial only in patients with associated interruption of the aortic arch or aortic coarctation.
  • Other preoperative medications are not generally indicated, although specific circumstances may dictate afterload reducing agents, inotropic medications, or antiarrhythmics.
  • Full-term and premature newborns with truncus arteriosus may be at increased risk for necrotizing enterocolitis, either preoperatively or postoperatively, and appropriate evaluation should be undertaken in any newborn exhibiting signs or symptoms of necrotizing enterocolitis.
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Surgical Care

  • Indications
    • Truncus arteriosus invariably requires operative repair.[8]
    • Symptoms typically develop in the early neonatal period, indicating that complete repair is required at this point.
  • Techniques
    • Surgical management of truncus arteriosus has undergone significant evolution over the past 30 years. Complete repair was first performed in 1967, but until neonatal and early infant repair became routine in the 1980s, palliative pulmonary artery banding was common, with complete repair performed at an older age. At most centers, primary complete repair in the neonate and young infant has become the accepted standard.
    • Currently, surgical management consists of complete primary repair, with closure of the ventricular septal defect, committing the common arterial trunk to the left ventricle, and reconstruction of the right ventricular outflow tract.
    • In patients with both branch pulmonary arteries arising from the common trunk, the standard method of right ventricular outflow tract reconstruction entails removing the central pulmonary arteries from the common trunk en bloc and placing a valved conduit from the right ventricle proximally to the central pulmonary arteries distally. Also, the most common type of conduit used is a cryopreserved valved aortic or pulmonary allograft. Before allograft conduits became widely available, other forms of pulmonary outflow reconstruction were used. These alternative forms remain in use in areas where the cost and availability of conduits prohibit routine use of modern techniques. Such alternatives include xenograft valves housed in synthetic tube grafts, direct anastomosis of the pulmonary arteries to the right ventriculotomy, or autologous flaps of pulmonary or aortic tissue augmented with synthetic patch material.
    • In patients with one pulmonary artery arising from the common trunk and one from the underside of the aortic arch, the pulmonary arteries are disconnected separately; they are then anastomosed together before being anastomosed to the conduit or are anastomosed to the conduit independently.
    • Coexisting anomalies are repaired as appropriate with cardiopulmonary bypass, cardioplegia, and sometimes deep hypothermic arrest, depending on anatomic features and the preference of the surgeon.
    • When the truncal valve is abnormal and repaired at the time of the initial truncus repair, outcomes appear to be significantly worse that when early truncal valve repair is not necessary.[9, 10]
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Consultations

  • Unless specific associated anomalies or problems are identified, consultations are not generally necessary.
  • Band 22q11 deletion is present in approximately one third of patients with truncus arteriosus, and consultation with a geneticist may be appropriate in some of these patients. Although patients with this chromosomal anomaly may have subtle associated abnormalities that are more likely to be identified if an experienced clinical geneticist is consulted, no evidence suggests that outcomes or management considerations differ in patients with or without chromosomal deletion.
  • When a chromosome 22q11 deletion is identified, comprehensive evaluation for associated conditions is indicated, including an otolaryngologist to evaluate for submucosal cleft palate, an immunologist to evaluate for immunodeficiency, and early intervention services for evaluation of learning and speech.
  • Consult a cardiologist before beginning, changing, or discontinuing cardiac medications in these patients.
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Diet

  • No special dietary considerations are indicated in patients with truncus arteriosus, other than a diet that might be dictated by associated conditions.
  • Resume enteral feeding once the patient is hemodynamically stable.
  • Resume oral feedings when the patient has been removed from mechanical ventilatory support and is adequately alert to take oral feedings safely. In patients with deletion in band 22q11, velopharyngeal insufficiency or cleft palate is frequently present, and oral feedings should be resumed with the aid of feeding specialists.
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Activity

  • Specific restrictions on activity are not indicated in patients with truncus arteriosus.
  • Patients with repaired truncus arteriosus and either residual defects or regurgitation of the right ventricle to pulmonary artery conduit may have limited exercise capacity best addressed on an individual basis.
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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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