Truncus Arteriosus Treatment & Management
- Author: Doff B McElhinney, MD; Chief Editor: Howard S Weber, MD, FSCAI more...
Medical care before surgical repair depends on the neonate's 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.
Truncus arteriosus invariably requires operative repair. Symptoms typically develop in the early neonatal period, indicating that complete repair is required at this point.
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.[12, 13]
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.
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.
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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