Truncus Arteriosus Follow-up

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

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 remainder of 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.
Next

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.
Previous
Next

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.
Previous
Next

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.
Previous
Next

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.
Previous
Next

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 Center.
Previous
 
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.

References

  1. Collett RW, Edwards JE. Persistent truncus arteriosus: a classification according to anatomic types. Surg Clin North Am. 1949;29:1245-70.

  2. Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol. Sep 1965;16(3):406-25. [Medline].

  3. Kodo K, Nishizawa T, Furutani M, Arai S, Yamamura E, Joo K, et al. GATA6 mutations cause human cardiac outflow tract defects by disrupting semaphorin-plexin signaling. Proc Natl Acad Sci U S A. Aug 18 2009;106(33):13933-8. [Medline]. [Full Text].

  4. Lisowski LA, Verheijen PM, Copel JA, Kleinman CS, Wassink S, Visser GH, et al. Congenital heart disease in pregnancies complicated by maternal diabetes mellitus. An international clinical collaboration, literature review, and meta-analysis. Herz. Jan 2010;35(1):19-26. [Medline].

  5. Chaoui R, Bollmann R, Zienert A, et al. [Prenatal diagnosis of truncus arteriosus communis (type I) in diabetic pregnancy]. Zentralbl Gynakol. 1992;114(4):198-200. [Medline].

  6. Swanson TM, Selamet Tierney ES, Tworetzky W, Pigula F, McElhinney DB. Truncus arteriosus: diagnostic accuracy, outcomes, and impact of prenatal diagnosis. Pediatr Cardiol. Apr 2009;30(3):256-61. [Medline].

  7. Rao PS. Diagnosis and management of cyanotic congenital heart disease: part I. Indian J Pediatr. Jan 2009;76(1):57-70. [Medline].

  8. [Guideline] Saxena A. Consensus on timing of intervention for common congenital heart disease. Indian Pediatr. Feb 2008;45(2):117-26. [Medline].

  9. Russell HM, Pasquali SK, Jacobs JP, Jacobs ML, O'Brien SM, Mavroudis C, et al. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg. Jan 2012;93(1):164-9; discussion 169. [Medline]. [Full Text].

  10. Lund AM, Vogel M, Marshall AC, Emani SM, Pigula FA, Tworetzky W, et al. Early reintervention on the pulmonary arteries and right ventricular outflow tract after neonatal or early infant repair of truncus arteriosus using homograft conduits. Am J Cardiol. Jul 1 2011;108(1):106-13. [Medline].

  11. Alexiou C, Keeton BR, Salmon AP, Monro JL. Repair of truncus arteriosus in early infancy with antibiotic sterilized aortic homografts. Ann Thorac Surg. May 2001;71(5 Suppl):S371-4. [Medline].

  12. Anderson RH, Thiene G. Categorization and description of hearts with a common arterial trunk. Eur J Cardiothorac Surg. 1989;3(6):481-7. [Medline].

  13. Bamforth SD, Braganca J, Eloranta JJ, et al. Cardiac malformations, adrenal agenesis, neural crest defects and exencephaly in mice lacking Cited2, a new Tfap2 co-activator. Nat Genet. Dec 2001;29(4):469-74. [Medline].

  14. Barbero-Marcial M, Tanamati C. Alternative nonvalved techniques for repair of truncus arteriosus: Long-term results. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 1999;2():121-130. [Medline].

  15. Bartelings MM, Gittenberger-de Groot AC. Morphogenetic considerations on congenital malformations of the outflow tract. Part 1: Common arterial trunk and tetralogy of Fallot. Int J Cardiol. Aug 1991;32(2):213-30. [Medline].

  16. Becker AE, Becker MJ, Edwards JE. Pathology of the semilunar valve in persistent truncus arteriosus. J Thorac Cardiovasc Surg. Jul 1971;62(1):16-26. [Medline].

  17. Bove EL, Lupinetti FM, Pridjian AK, et al. Results of a policy of primary repair of truncus arteriosus in the neonate. J Thorac Cardiovasc Surg. Jun 1993;105(6):1057-65; discussion 1065-6. [Medline].

  18. Brizard CP, Cochrane A, Austin C, et al. Management strategy and long-term outcome for truncus arteriosus. Eur J Cardiothorac Surg. Apr 1997;11(4):687-95; discussion 695-6. [Medline].

  19. Brown JW, Ruzmetov M, Okada Y, et al. Truncus arteriosus repair: outcomes, risk factors, reoperation and management. Eur J Cardiothorac Surg. Aug 2001;20(2):221-7. [Medline].

  20. Chen JM, Glickstein JS, Davies RR, et al. The effect of repair technique on postoperative right-sided obstruction in patients with truncus arteriosus. J Thorac Cardiovasc Surg. Mar 2005;129(3):559-68. [Medline].

  21. Crupi G, Macartney FJ, Anderson RH. Persistent truncus arteriosus. A study of 66 autopsy cases with special reference to definition and morphogenesis. Am J Cardiol. Oct 1977;40(4):569-78. [Medline].

  22. Danton MH, Barron DJ, Stumper O, et al. Repair of truncus arteriosus: a considered approach to right ventricular outflow tract reconstruction. Eur J Cardiothorac Surg. Jul 2001;20(1):95-103. discussion 103-4. [Medline].

  23. de la Cruz MV, Cayre R, Angelini P, et al. Coronary arteries in truncus arteriosus. Am J Cardiol. Dec 15 1990;66(20):1482-6. [Medline].

  24. Di Donato RM, Fyfe DA, Puga FJ, et al. Fifteen-year experience with surgical repair of truncus arteriosus. J Thorac Cardiovasc Surg. Mar 1985;89(3):414-22. [Medline].

  25. Digilio MC, Angioni A, Giannotti A, et al. Truncus arteriosus and duplication 8q. Am J Med Genet A. Aug 15 2003;121(1):79-81. [Medline].

  26. Ebert PA, Turley K, Stanger P, et al. Surgical treatment of truncus arteriosus in the first 6 months of life. Ann Surg. Oct 1984;200(4):451-6. [Medline].

  27. Elkins RC, Steinberg JB, Razook JD, et al. Correction of truncus arteriosus with truncal valvar stenosis or insufficiency using two homografts. Ann Thorac Surg. Nov 1990;50(5):728-33. [Medline].

  28. Feiner L, Webber AL, Brown CB, et al. Targeted disruption of semaphorin 3C leads to persistent truncus arteriosus and aortic arch interruption. Development. Aug 2001;128(16):3061-70. [Medline].

  29. Fyfe DA, Driscoll DJ, Di Donato RM, et al. Truncus arteriosus with single pulmonary artery: influence of pulmonary vascular obstructive disease on early and late operative results. J Am Coll Cardiol. May 1985;5(5):1168-72. [Medline].

  30. Gerlis LM, Ho SY, Smith A, et al. The site of origin of nonconfluent pulmonary arteries from a common arterial trunk or from the ascending aorta: its morphological significance. Am J Cardiovasc Pathol. 1990;3(2):115-20. [Medline].

  31. Hanley FL, Heinemann MK, Jonas RA, et al. Repair of truncus arteriosus in the neonate. J Thorac Cardiovasc Surg. Jun 1993;105(6):1047-56. [Medline].

  32. Heathcote K, Braybrook C, Abushaban L, et al. Common arterial trunk associated with a homeodomain mutation of NKX2.6. Hum Mol Genet. Mar 1 2005;14(5):585-93. [Medline].

  33. Heinemann MK, Hanley FL, Fenton KN, et al. Fate of small homograft conduits after early repair of truncus arteriosus. Ann Thorac Surg. Jun 1993;55(6):1409-11; discussion 1411-2. [Medline].

  34. Imamura M, Drummond-Webb JJ, Sarris GE, et al. Improving early and intermediate results of truncus arteriosus repair: a new technique of truncal valve repair. Ann Thorac Surg. Apr 1999;67(4):1142-6. [Medline].

  35. Jahangiri M, Zurakowski D, Mayer JE, et al. Repair of the truncal valve and associated interrupted arch in neonates withtruncus arteriosus. J Thorac Cardiovasc Surg. Mar 2000;119(3):508-14. [Medline].

  36. Kaartinen V, Dudas M, Nagy A, et al. Cardiac outflow tract defects in mice lacking ALK2 in neural crest cells. Development. Jul 2004;131(14):3481-90. [Medline].

  37. Kirklin JW, Barratt-Boyes BG. Truncus arteriosus. In: Cardiac Surgery. New York, NY: John Wiley & Sons; 1992:1131-51.

  38. Lacour-Gayet F, Serraf A, Komiya T, et al. Truncus arteriosus repair: influence of techniques of right ventricular outflow tract reconstruction. J Thorac Cardiovasc Surg. Apr 1996;111(4):849-56. [Medline].

  39. Litovsky SH, Ostfeld I, Bjornstad PG, et al. Truncus arteriosus with anomalous pulmonary venous connection. Am J Cardiol. Mar 1 1999;83(5):801-4, A10. [Medline].

  40. Mavroudis C, Backer CL. Surgical management of severe truncal insufficiency: experience with truncal valve remodeling techniques. Ann Thorac Surg. Aug 2001;72(2):396-400. [Medline].

  41. McElhinney DB, Driscoll DA, Emanuel BS, Goldmuntz E. Chromosome 22q11 deletion in patients with truncus arteriosus. Pediatr Cardiol. Nov-Dec 2003;24(6):569-73. [Medline].

  42. McElhinney DB, Hedrick HL, Bush DM, et al. Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes. Pediatrics. Nov 2000;106(5):1080-7. [Medline].

  43. McElhinney DB, Rajasinghe HA, Mora BN, et al. Reinterventions after repair of common arterial trunk in neonates and young infants. J Am Coll Cardiol. Apr 2000;35(5):1317-22. [Medline].

  44. McElhinney DB, Reddy VM, Rajasinghe HA, et al. Trends in the management of truncal valve insufficiency. Ann Thorac Surg. Feb 1998;65(2):517-24. [Medline].

  45. Mohammadi S, Belli E, Martinovic I, et al. Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation. Eur J Cardiothorac Surg. Aug 2005;28(2):217-22. [Medline].

  46. Momma K, Ando M, Matsuoka R. Truncus arteriosus communis associated with chromosome 22q11 deletion. J Am Coll Cardiol. Oct 1997;30(4):1067-71. [Medline].

  47. Niwa K, Perloff JK, Kaplan S, et al. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol. Jul 1999;34(1):223-32. [Medline].

  48. Rajasinghe HA, McElhinney DB, Reddy VM, et al. Long-term follow-up of truncus arteriosus repaired in infancy: a twenty- year experience. J Thorac Cardiovasc Surg. May 1997;113(5):869-78; discussion 878-9. [Medline].

  49. Reddy VM, Rajasinghe HA, McElhinney DB, et al. Performance of right ventricle to pulmonary artery conduits after repair of truncus arteriosus: a comparison of Dacron-housed porcine valves and cryopreserved allografts. Semin Thorac Cardiovasc Surg. Jul 1995;7(3):133-8. [Medline].

  50. Schreiber C, Eicken A, Balling G, et al. Single centre experience on primary correction of common arterial trunk: overall survival and freedom from reoperation after more than 15 years. Eur J Cardiothorac Surg. Jul 2000;18(1):68-73. [Medline].

  51. Silverman NH. Truncus arteriosus. In: Pediatric Echocardiography. Baltimore, Md: Williams & Wilkins; 1993:229-43.

  52. Stewart DE, Kirby ML, Sulik KK. Hemodynamic changes in chick embryos precede heart defects after cardiac neural crest ablation. Circ Res. Nov 1986;59(5):545-50. [Medline].

  53. Suzuki A, Ho SY, Anderson RH, et al. Coronary arterial and sinusal anatomy in hearts with a common arterial trunk. Ann Thorac Surg. Dec 1989;48(6):792-7. [Medline].

  54. Takeuchi JK, Mileikovskaia M, Koshiba-Takeuchi K, et al. Tbx20 dose-dependently regulates transcription factor networks required for mouse heart and motoneuron development. Development. May 2005;132(10):2463-74. [Medline].

  55. Thompson LD, McElhinney DB, Reddy M, et al. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg. Aug 2001;72(2):391-5. [Medline].

  56. Tlaskal T, Hucin B, Kostelka M, et al. Successful reoperation for severe left bronchus compression after repair of persistent truncus arteriosus with interrupted aortic arch. Eur J Cardiothorac Surg. Mar 1998;13(3):306-9. [Medline].

  57. Tometzki AJ, Suda K, Kohl T, et al. Accuracy of prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies. J Am Coll Cardiol. May 1999;33(6):1696-701. [Medline].

  58. Tworetzky W, McElhinney DB, Brook MM, et al. Echocardiographic diagnosis alone for the complete repair of major congenital heart defects. J Am Coll Cardiol. Jan 1999;33(1):228-33. [Medline].

  59. Van Mierop LHS, Patterson DF, Schnarr WR. Pathogenesis of persistent truncus arteriosus in light of observations made in a dog embryo with the anomaly. Am J Cardiol. 1978;41(4):755-62. [Medline].

  60. Van Praagh R. Truncus arteriosus: what is it really and how should it be classified?. Eur J Cardiothorac Surg. 1987;1(2):65-70. [Medline].

  61. Volpe P, Paladini D, Marasini M, et al. Common arterial trunk in the fetus: characteristics, associations, and outcome in a multicentre series of 23 cases. Heart. Dec 2003;89(12):1437-41. [Medline].

  62. Williams JM, de Leeuw M, Black MD, et al. Factors associated with outcomes of persistent truncus arteriosus. J Am Coll Cardiol. Aug 1999;34(2):545-53. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.