eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Tetralogy of Fallot With Pulmonary Atresia: Follow-up

Author: Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Associate Professor of Pediatrics, Wayne State University School of Medicine
Coauthor(s): Aparna Kulkarni, MBBS, MD, Fellow, Department of Cardiology, Children's Hospital of Michigan
Contributor Information and Disclosures

Updated: Nov 24, 2008

Follow-up

Further Inpatient Care

  • Admit patients with tetralogy of Fallot with pulmonary atresia (TOF-PA) for testing and surgical intervention.
  • 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. Transcatheter placement of a pulmonic valve is currently under development.

Further Outpatient Care

  • 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 and Doppler evaluation of hemodynamic adequacy should be performed. After complete repair, the patient needs to be evaluated for the development of right ventricle–to–pulmonary artery conduit stenosis as well as pulmonic regurgitation.
  • A few patients may never reach the stage of complete repair because of very hypoplastic pulmonary arteries. These patients often are hypoxemic and polycythemic and may require oxygen supplementation.

Transfer

  • Transfer to a tertiary care center is indicated for complete diagnostic evaluation and surgical intervention.

Complications

  • Residual right ventricular dysfunction from hypoplastic pulmonary arteries or conduit stenosis
  • Cyanosis, hypoxemia, and polycythemia
  • Atrioventricular conduction abnormalities, right bundle branch block, ventricular arrhythmias in the postoperative patients
  • Significant pulmonic valve regurgitation

Prognosis

  • The prognosis depends on the specific anatomy and type of intervention. Surgical morbidity and mortality and long-term survival have steadily improved into the current era, with most of these patients now surviving into adulthood.17
  • Long-term follow up data are not widely available; however, recent outcome does seem to be more favorable. Most patients who undergo placement of a right ventricle to pulmonary conduit require one or more conduit replacements, secondary to progressive conduit stenosis or insufficiency.18,19

Patient Education

  • Educate patients and their families about anatomic details and long-term prognosis, the potential need for multiple surgeries and catheterizations, and postoperative complications.
  • At all patient care visits, emphasize the need for bacterial endocarditis prophylaxis.
  • For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Tetralogy of Fallot.

Miscellaneous

Medicolegal Pitfalls

  • Failure to consider the diagnosis, especially in a newborn with cyanosis

Special Concerns

  • Genetic counseling is strongly recommended in patients of childbearing age; the chance that patients with tetralogy of Fallot (TOF) could have an offspring with congestive heart disease (CHD) is as high as 15%.
  • Patients with residual right ventricular dysfunction or pulmonary hypertension are advised to avoid pregnancy because it carries significant mortality risk.
  • All patients with tetralogy of Fallot with pulmonary atresia are required to take appropriate antibiotic bacterial endocarditis prophylaxis.
  • Exercise recommendations must be tailored to individual patients by considering the presence of cyanosis, right ventricular hypertension, right ventricular dysfunction, or dysrhythmias.
 


More on Tetralogy of Fallot With Pulmonary Atresia

Overview: Tetralogy of Fallot With Pulmonary Atresia
Differential Diagnoses & Workup: Tetralogy of Fallot With Pulmonary Atresia
Treatment & Medication: Tetralogy of Fallot With Pulmonary Atresia
Follow-up: Tetralogy of Fallot With Pulmonary Atresia
Multimedia: Tetralogy of Fallot With Pulmonary Atresia
References
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References

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

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

  3. Perry LW, Neill CA, Ferencz C. Infants with congenital heart disease: the cases. In: Ferencz C, Rubin JD, Loffredo CA, et al. Perspectives in Pediatric Cardiology: Epidemiology of Congenital Heart Disease. Armonk, NY: The Baltimore Washington Infant study; 1997:59-102.

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

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

  6. Marino B, Digilio MC, Toscano A, et al. Anatomic patterns of conotruncal defects associated with deletion 22q11. Genet Med. Jan-Feb 2001;3(1):45-8. [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. Jun 15 1996;77(15):1375-6. [Medline].

  8. 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. Mar 5 2003;41(5):852-7. [Medline].

  9. 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.

  10. 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. Jul 23 2002;106(4):473-8. [Medline]. [Full Text].

  11. 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. Jun 2006;16(3):281-8. [Medline].

  12. 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. Sep 2003;126(3):694-702. [Medline].

  13. 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. Aug 2007;84(2):606-10; discussion 610-1. [Medline].

  14. 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. May 2008;3(3):176-84. [Medline].

  15. 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. Oct 2000;18(4):480-6. [Medline].

  16. 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. May 1997;113(5):858-66; discussion 866-8. [Medline].

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

  18. 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. Feb 2003;75(2):399-410; discussion 410-1. [Medline].

  19. 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].

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Further Reading

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Keywords

tetralogy of Fallot, TOF, tetralogy of Fallot with pulmonary atresia, TOF-PA, pulmonary atresia with ventricular septal defect, VSD, end-stage tetralogy of Fallot, Fallot tetralogy, Fallot's tetralogy, Fallot tetrad, Fallot's tetrad, CATCH22 syndrome, cardiac defect, abnormal face, thymic hypoplasia, cleft palate, hypocalcemia, microdeletion of band 22q11, vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, renal and radial anomalies, VATER syndrome, coloboma, heart disease, atresia choanae, retarded growth, retarded development, CNS anomalies, genital hypoplasia, ear anomalies, deafness, CHARGE syndrome, Alagille syndrome, cat's eye syndrome, de Lange syndrome, Klippel-Feil syndrome, trisomy 21, maternal diabetes mellitus, maternal phenylketonuria

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Contributor Information and Disclosures

Author

Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Associate Professor of Pediatrics, Wayne State University School of Medicine
Michael Pettersen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Society of Echocardiography
Disclosure: Nothing to disclose.

Coauthor(s)

Aparna Kulkarni, MBBS, MD, Fellow, Department of Cardiology, Children's Hospital of Michigan
Aparna Kulkarni, MBBS, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology
Disclosure: Nothing to disclose.

Medical Editor

Ira H Gessner, MD, Professor Emeritus, Pediatric Cardiology
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, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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.

 
 
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