Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Tetralogy of Fallot With Pulmonary Atresia Treatment & Management

  • Author: Michael D Pettersen, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Nov 22, 2015
 

Approach Considerations

Admit patients with tetralogy of Fallot with pulmonary atresia (TOF-PA) for testing and potential surgical intervention. Transfer to a tertiary care center is indicated for complete diagnostic evaluation and surgical intervention.

Newborn infants with cyanosis due to congenital heart disease almost always benefit from administration of prostaglandin E1 (PGE1) to maintain ductal patency while a definitive diagnosis is made. Once the diagnosis of TOF-PA is made, the need for a PGE1 infusion is dependent on whether a ductus arteriosus is, in fact, present. If the only sources of pulmonary blood flow are major aortopulmonary collateral arteries (MAPCAs), then a PGE1 infusion is not necessary.

Older infants with increased pulmonary blood flow may require treatment for heart failure.

All patients with TOF-PA who have undergone either palliative surgical intervention (shunt procedure) or complete repair (conduit placement) are required to take appropriate prophylactic antibiotics to avoid bacterial endocarditis.

Findings from a retrospective study of 12 patients with TOF-PA and MAPCAs suggest that use of pulmonary hypertension medications may provide symptomatic improvement and are well tolerated.[14] It remains unknown whether these medications confer any long-term survival benefit in those with complex congenital heart disease.[14]

Nutritional support

Infants who are born with multiple systemic-to-pulmonary collaterals and are in cardiac failure because of pulmonary overcirculation require caloric supplementation to establish a normal growth pattern. Caloric intake as high as 130-150 kcal/kg/d may be required to ensure adequate growth.

Children that undergo palliative procedures also require optimization of their caloric intake. Adequate nutritional supplementation in the form of total parental nutrition must also be ascertained in the perioperative period. These patients often have a prolonged postoperative recovery course.

Next

Surgical Intervention

Neonates with adequate-sized confluent pulmonary arteries may be amenable to primary definitive surgical repair. A palliative procedure with a systemic–to–pulmonary artery shunt may be performed to promote central pulmonary artery growth prior to complete repair at a later date. The ultimate surgical goals are: (1) to incorporate as many pulmonary artery segments as possible into a pulmonary artery confluence, (2) to place a conduit from the right ventricle to the pulmonary artery confluence, and (3) to close the ventricular septal defect (VSD). While the primary intervention in the majority of patients is surgical, selected cases may be amenable to transcatheter perforation of the right ventricular outflow tract followed by balloon dilation.[23]

Hypoplastic pulmonary arteries

When the pulmonary arteries are hypoplastic, nonconfluent, and supplied by aortopulmonary collaterals, a multistaged repair is often required.[24] Hypoplastic pulmonary arteries generally require palliative shunting to induce enlargement and growth of these vessels so they can be successfully incorporated into the complete repair. The shunts used may be a modified Blalock-Taussig or central shunt and may be unilateral or bilateral. Another important strategy to maximize the long-term outcome in this group of patients is the early unifocalization of as many of the aortopulmonary collaterals as possible into a central pulmonary artery confluence.[25, 26, 27, 28] This maximizes the recruitment of lung segments, increasing the cross-sectional area of the pulmonary vascular bed, and it may increase the likelihood of performing a definitive repair.

Complete repair following palliation

For complete repair to be performed in a child who has undergone palliation: (1) The central pulmonary arterial area must be greater than 50% of normal; (2) predominantly left-to-right shunting at the ventricular level (VSD) must be present; (3) the equivalent of an entire lung must be supplied by the central pulmonary artery confluence; and (4) stenotic lesions in the pulmonary artery outflow must be addressed.

The choice of the optimal type of conduit for a growing child remains controversial. Current options include cryopreserved aortic or pulmonary homografts, glutaraldehyde fixed bovine jugular vein grafts, and synthetic conduits, with variable intermediate-term results reported in the medical literature.[29, 30, 31] Patients with membranous pulmonary atresia may be amenable to repair using a pulmonary transannular patch. These patients have an improved freedom from reintervention compared with patients who receive right ventricle-to-pulmonary artery conduits.[32]

Significant pulmonic valve regurgitation often occurs regardless of the type of conduit placed between the right ventricle and the pulmonary arteries. Some patients develop substantial right ventricular dilation and right ventricular dysfunction. Surgical placement of a pulmonic valve may significantly benefit these patients. More recently, a transcatheter-placed pulmonary valve comprising a valved segment of bovine jugular vein sewn within a balloon-expandable stent has been made commercially available. This valve can be placed in patients with postoperative conduit dysfunction consisting of pulmonary regurgitation, obstruction, or both. However, the existing conduit has to be approximately 16 mm in diameter at the time of its original implantation. Early and midterm results with this valve suggest a high rate of procedural success and encouraging valve function.[33]

Single-stage repair

Some centers have shifted toward performing a single-stage repair, wherein all the multiple aortopulmonary collaterals (MAPCAs) are ligated at the aorta.[34, 35] These MAPCAs are then mobilized toward the posterior mediastinum to construct a pulmonary artery confluence, followed by insertion of a pulmonary allograft to establish continuity between these neopulmonary arteries and the right ventricle. The ventricular septal defect (VSD) is closed.

These centers have reported good results. Infants with postunifocalization pulmonary arteries that, combined, are only mildly hypoplastic (> 200 mm2/m2) have a lower mortality rate and acceptable right ventricular pressures. However, many patients require repeat catheterizations for balloon dilation or stent placements in stenotic pulmonary artery segments to alleviate elevated right ventricular pressures.[36]

Previous
Next

Consultations

The following consultations are advised:

  • Pediatric cardiology consultation
  • Geneticist consultation to evaluate the presence of syndromic associations and gene deletions, especially in the presence of associated anomalies or dysmorphic features
  • Cardiovascular surgical consultation, once the anatomy of a child with tetralogy of Fallot with pulmonary atresia (TOF-PA) is determined by echocardiography and angiography findings (see Workup);the caregivers need to be aware of the possibility of a multistage repair and repeated surgeries and catheterizations
  • Consultations and follow-up with the appropriate specialists for anomalies involving other systems
Previous
Next

Long-Term Monitoring

Infants with multiple aortopulmonary collaterals may require outpatient medical management of heart failure.

Residual right ventricular hypertension with right ventricular dysfunction from hypoplastic pulmonary arteries may be present.

After each stage of surgical reconstruction, echocardiographic evaluation of hemodynamic adequacy should be performed. After complete repair and during clinical follow-up, the patient needs to be evaluated for the development of right ventricle–to–pulmonary artery conduit stenosis as well as pulmonary valve regurgitation.

Some patients may never reach the stage of complete repair because of very hypoplastic and discontinuous pulmonary arteries. These patients are often hypoxemic and polycythemic, requiring oxygen supplementation. Patients who are chronically cyanotic should be carefully monitored for complications related to polycythemia and iron deficiency anemia.

Previous
 
 
Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

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

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

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Aparna Kulkarni, MBBS, MD, to the development and writing of the source article.

References
  1. Tchervenkov CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg. 2000 Apr. 69(4 Suppl):S97-105. [Medline].

  2. Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol. 1985 Jan. 121(1):31-6. [Medline].

  3. Garg P, Talwar S, Kothari SS, et al. Management of pulmonary arterial supply dependent on a coronary arterial fistula in a patient with tetralogy of fallot with pulmonary atresia. World J Pediatr Congenit Heart Surg. 2012 Oct 1. 3(4):499-503. [Medline].

  4. Van Praagh R, Van Praagh S, Nebesar RA, et al. Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and its sequelae. Am J Cardiol. 1970 Jul. 26(1):25-33. [Medline].

  5. Marino B, Digilio MC, Toscano A, et al. Anatomic patterns of conotruncal defects associated with deletion 22q11. Genet Med. 2001 Jan-Feb. 3(1):45-8. [Medline].

  6. Carotti A, Digilio MC, Piacentini G, Saffirio C, Di Donato RM, Marino B. Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome. Dev Disabil Res Rev. 2008. 14(1):35-42. [Medline].

  7. Digilio MC, Marino B, Grazioli S, et al. Comparison of occurrence of genetic syndromes in ventricular septal defect with pulmonic stenosis (classic tetralogy of Fallot) versus ventricular septal defect with pulmonic atresia. Am J Cardiol. 1996 Jun 15. 77(15):1375-6. [Medline].

  8. Bertranou EG, Blackstone EH, Hazelrig JB, et al. Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol. 1978 Sep. 42(3):458-66. [Medline].

  9. Leonard H, Derrick G, O'Sullivan J, Wren C. Natural and unnatural history of pulmonary atresia. Heart. 2000 Nov. 84(5):499-503. [Medline].

  10. Fukui D, Kai H, Takeuchi T, et al. Longest survivor of pulmonary atresia with ventricular septal defect: well-developed major aortopulmonary collateral arteries demonstrated by multidetector computed tomography. Circulation. 2011 Nov 8. 124(19):2155-7. [Medline].

  11. Marrelli AJ, Perloff JK, Child JS, Laks H. Pulmonary atresia with ventricular septal defect in adults. Circulation. 1994. 89(1):243-51. [Medline].

  12. Dearani JA, Danielson GK, Puga FJ, et al. Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Ann Thorac Surg. 2003 Feb. 75(2):399-410; discussion 410-1. [Medline].

  13. 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. 2005 Aug. 28(2):217-22. [Medline].

  14. Grant EK, Berger JT. Use of pulmonary hypertension medications in patients with tetralogy of Fallot with pulmonary atresia and multiple aortopulmonary collaterals. Pediatr Cardiol. 2015 Oct 28. [Medline].

  15. Lewis M, Ginns J, Schulze C, et al. Outcomes of adult patients with congenital heart disease after heart transplantation: impact of disease type, previous thoracic surgeries, and bystander organ dysfunction. J Card Fail. 2015 Nov 11. [Medline].

  16. Geva T, Greil GF, Marshall AC, et al. Gadolinium-enhanced 3-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with x-ray angiography. Circulation. 2002 Jul 23. 106(4):473-8. [Medline]. [Full Text].

  17. Bernardes RJ, Marchiori E, Bernardes PM, Monzo Gonzaga MB, Simoes LC. A comparison of magnetic resonance angiography with conventional angiography in the diagnosis of tetralogy of Fallot. Cardiol Young. 2006 Jun. 16(3):281-8. [Medline].

  18. Rajeshkannan R, Moorthy S, Sreekumar KP, Ramachandran PV, Kumar RK, Remadevi KS. Role of 64-MDCT in evaluation of pulmonary atresia with ventricular septal defect. AJR Am J Roentgenol. 2010 Jan. 194(1):110-8. [Medline].

  19. Rajeshkannan R, Moorthy S, Sreekumar KP, Ramachandran PV, Kumar RK, Remadevi KS. Role of 64-MDCT in evaluation of pulmonary atresia with ventricular septal defect. AJR Am J Roentgenol. 2010 Jan. 194(1):110-8. [Medline].

  20. O'Meagher S, Seneviratne M, Skilton MR, et al. Right ventricular mass is associated with exercise capacity in adults with repaired tetralogy of Fallot. Pediatr Cardiol. 2015 Aug. 36 (6):1225-31. [Medline].

  21. Mackie AS, Gauvreau K, Perry SB, et al. Echocardiographic predictors of aortopulmonary collaterals in infants with tetralogy of fallot and pulmonary atresia. J Am Coll Cardiol. 2003 Mar 5. 41(5):852-7. [Medline].

  22. Mair DD, Julsrud PR. Diagnostic evaluation of pulmonary atresia and ventricular septal defect cardiac catheterization and angiography. Prog Pediatr Cardiol. 1992. 1(1):23-26.

  23. Hugues N, Abadir S, Dragulescu A, et al. Transcatheter perforation followed by pulmonary valvuloplasty in neonates with pulmonary atresia and ventricular septal defect. Arch Cardiovasc Dis. 2009 May. 102(5):427-32. [Medline].

  24. Duncan BW, Mee RB, Prieto LR, et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2003 Sep. 126(3):694-702. [Medline].

  25. Davies B, Mussa S, Davies P, et al. Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology. J Thorac Cardiovasc Surg. 2009 Dec. 138(6):1269-75.e1. [Medline].

  26. Malhotra SP, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009. 145-51. [Medline].

  27. Maskatia SA, Feinstein JA, Newman B, Hanley FL, Roth SJ. Pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2012 Jul. 144(1):184-9. [Medline].

  28. Fouilloux V, Bonello B, Kammache I, Fraisse A, Mace L, Kreitmann B. Management of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals: Focus on the strategy of rehabilitation of the native pulmonary arteries. Arch Cardiovasc Dis. 2012 Dec. 105(12):666-75. [Medline].

  29. Sierra J, Christenson JT, Lahlaidi NH, Beghetti M, Kalangos A. Right ventricular outflow tract reconstruction: what conduit to use? Homograft or Contegra?. Ann Thorac Surg. 2007 Aug. 84(2):606-10; discussion 610-1. [Medline].

  30. Niemantsverdriet MB, Ottenkamp J, Gauvreau K, Del Nido PJ, Hazenkamp MG, Jenkins KJ. Determinants of right ventricular outflow tract conduit longevity: a multinational analysis. Congenit Heart Dis. 2008 May. 3(3):176-84. [Medline].

  31. Belli E, Salihoglu E, Leobon B, et al. The performance of Hancock porcine-valved Dacron conduit for right ventricular outflow tract reconstruction. Ann Thorac Surg. 2010 Jan. 89(1):152-7; discussion 157-8. [Medline].

  32. Kaza AK, Lim HG, Dibardino DJ, et al. Long-term results of right ventricular outflow tract reconstruction in neonatal cardiac surgery: options and outcomes. J Thorac Cardiovasc Surg. 2009 Oct. 138(4):911-6. [Medline].

  33. Cheatham JP, Hellenbrand WE, Zahn EM, et al. Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial. Circulation. 2015 Jun 2. 131(22):1960-70. [Medline].

  34. Lofland GK. The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy. Eur J Cardiothorac Surg. 2000 Oct. 18(4):480-6. [Medline].

  35. Reddy VM, Petrossian E, McElhinney DB, et al. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed?. J Thorac Cardiovasc Surg. 1997 May. 113(5):858-66; discussion 866-8. [Medline].

  36. Learn C, Phillips A, Chisolm J, et al. Pulmonary atresia with ventricular septal defect and multifocal pulmonary blood supply: does an intensive interventional approach improve the outcome?. Congenit Heart Dis. 2012 Mar-Apr. 7(2):111-21. [Medline].

 
Previous
Next
 
Parasternal long axis two-dimensional echocardiographic image demonstrating a large malalignment ventricular septal defect with overriding of the aorta over the ventricular septum.
Subcostal sagittal plane two-dimensional echocardiographic image showing pulmonary valve atresia, with confluent and well-developed pulmonary artery branches.
Suprasternal long axis color flow echocardiographic image showing a large patent ductus arteriosus supply confluent pulmonary arteries.
Aortopulmonary view angiogram, with injection in the descending thoracic aorta demonstrating multiple aortopulmonary collaterals supplying pulmonary blood flow.
Parasternal long axis two-dimensional echocardiographic image in a patient status post complete repair of tetralogy of Fallot with pulmonary atresia. A patch is visualized closing the ventricular septal defect.
Parasternal long axis color compare echocardiographic image showing the pulmonary artery conduit arising from the right ventricle.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.