Truncus Arteriosus Follow-up
- Author: Doff B McElhinney, MD; Chief Editor: Howard S Weber, MD, FSCAI more...
Further Outpatient Care
Maintain close follow-up care in young children after repair of truncus arteriosus. Young infants are often discharged on cardiac medications and may usually be weaned over the following months.
Frequently, a mild degree of regurgitation occurs through the right ventricle–to–pulmonary arterial conduit but, in most cases, does not pose a significant load on the heart.
In most patients, conduit regurgitation and obstruction becomes an important issue after early repair; however, reintervention usually is not required for a year or more.
Truncal valve regurgitation, which may progress even if it was not severe before repair, may become an important cause of persistent failure to thrive, and repair or replacement of the valve may be indicated.
In patients with associated interruption of the aortic arch, pay particular attention to potential recurrent arch obstruction and compression of the bronchi, both of which may manifest within weeks or months of the initial repair.
Routine clinical and echocardiographic follow-up care is sufficient to monitor most patients. Cardiac catheterization may be performed for the purpose of balloon dilation, stenting, or both of the pulmonary arteries or pulmonary outflow conduit, for evaluation of the pulmonary vascular bed in patients who are older and have evidence of pulmonary hypertension, or for other diagnostic indications according to the preference of the physicians.
Further Inpatient Care
Administer routine postoperative care, initially in the cardiac intensive care unit, following correction of truncus arteriosus. Support patients with mechanical ventilation, inotropic medications, and sedation as necessary. Restore fluid balance with diuretic therapy and continue tube thoracostomy until pleural and pericardial effusions have resolved.
Focus the remainder of the inpatient stay on providing sufficient enteral nutrition, parental education, and elucidation of the maintenance pharmacologic regimen (if any) that is adopted.
Postoperative care after repair of truncus arteriosus requires attention to issues that are common to patients with complex congenital heart disease (eg, support of cardiac output) and prevention or management of arrhythmias and end-organ dysfunction.
Management issues include maintenance of intravascular volume and ventricular filling, inotropic support, and acid-base and electrolyte homeostasis. In addition, potential issues that are of particular concern in patients with truncus arteriosus include pulmonary hypertensive crisis and volume overload in patients with persistent truncal valve regurgitation. Because of the lability of the pulmonary resistance vessels that may occur with and following elimination of elevated pulmonary blood flow at high pressures, pulmonary hypertensive crisis currently is less of an issue in early neonatal repair than it was with later repair. Nevertheless, patients may experience episodes of paroxysmal elevation of pulmonary vascular resistance.
Management with extended periods of anesthesia, including neuromuscular blockade and continuous fentanyl infusion, often is helpful. Ventilatory strategies aimed at minimizing pulmonary vascular resistance also may be effective. In refractory cases, inhaled nitric oxide or extracorporeal membrane oxygenation may be indicated.
After stabilization in the intensive care unit, removal from mechanical ventilatory and inotropic support, and discontinuation of intracardiac monitoring catheters, transfer the patient to the regular inpatient care area for advancement of feedings and additional postoperative care, depending on the experience and comfort level of the nursing staff on the ward.
No known methods to prevent the development of truncus arteriosus in the fetus are known. On screening obstetric ultrasonography findings, 4-chamber and great vessel views are sufficient to identify that cardiac anomalies are present. In such an event, the parents should be referred for fetal echocardiography, with which the anatomy of truncus arteriosus can be more fully defined. Diagnosis in utero allows for greater parental choice, and may facilitate planned delivery at a tertiary care center and immediate neonatal stabilization, thus preventing the potential hemodynamic sequelae that can result from the natural history of the lesion.
For the early posthospital period, educate parents about the signs and symptoms of congestive heart failure, proper administration and potential adverse effects of any maintenance medications, and management of the sternotomy incision.
For patient education resources, see the Heart Health Center.
Among patients surviving the early postoperative period, prognosis is generally very good. Few published long-term follow-up data are available on patients undergoing repair in the neonatal and early infant periods because this management strategy came into widespread application in the mid to late 1980s. Moreover, techniques of myocardial protection and perioperative management have changed dramatically even within this period; thus, existing data, limited as they may be, are still likely to underestimate outcome in contemporary patients.
Although late mortality among patients undergoing early repair is minimal, a substantial proportion of premature deaths among such patients are likely to be related to reinterventions. Because the right ventricular outflow tract is usually reconstructed with a nonviable conduit, which does not grow along with the patient, reinterventions for conduit replacement, revision, or dilation are essentially inevitable. In a series following infants younger than 4 months with surgically repaired truncus arteriosus, freedom from conduit-related reintervention was less than 50% at 5 years and less than 10% at 10 years.
Patients who have the conduit replaced earlier in life often require at least one subsequent intervention on the right ventricular outflow tract. Reintervention for truncal valve regurgitation (often within the first year after repair) or for branch pulmonary arterial stenosis is also required in a substantial number of patients.
At major centers in North America, survival to hospital discharge after complete repair of truncus arteriosus is approximately 90-95%. Prognosis appears somewhat less favorable for patients with complicating associated conditions, such as severe truncal valve regurgitation of interruption of the aortic arch. Significant perioperative morbidity is uncommon and includes issues common to many forms of complex congenital heart disease, such as transient arrhythmias, low cardiac output, and sequelae of cardiopulmonary bypass.
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