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Truncus Arteriosus Follow-up

  • Author: Doff B McElhinney, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Jan 14, 2015
 

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.

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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.

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Transfer

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.

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Deterrence/Prevention

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.

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Patient Education

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.

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Prognosis

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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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, 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, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

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

Additional Contributors

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, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

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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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