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Tetralogy of Fallot With Pulmonary Atresia Medication

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

Medication Summary

Newborns with tetralogy of Fallot with pulmonary atresia (TOF-PA) may require the ductus arteriosus (DA) as the main source of pulmonary blood flow. A prostaglandin E1 (PGE1) (Alprostadil) infusion maintains patency of the ductus.

Infants with multiple systemic pulmonary collaterals may develop symptomatic heart failure requiring medical therapy.

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Prostaglandins

Class Summary

Prostaglandin E1 (PGE1) (Alprostadil) is a vasodilating agent that also promotes dilatation of the ductus arteriosus (DA) in infants with ductal-dependent cardiac abnormalities.

Alprostadil IV (Prostin VR Pediatric Injection)

 

Alprostadil is first-line palliative therapy to temporarily maintain patency of the ductus arteriosus (DA) before surgery. This agent is beneficial in infants who have congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent DA for adequate oxygenation and lower body perfusion. Alprostadil produces vasodilation and increases cardiac output. Each 1-mL ampule contains 500 mcg/mL.

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Diuretic agents

Class Summary

Diuretic agents promote excretion of water and electrolytes by the kidneys. These drugs are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention results in edema or ascites. Children who have congestive heart failure (CHF) symptoms often require multiple diuretics for effective control.

Furosemide (Lasix)

 

Furosemide increases excretion of water by interfering with the chloride-binding cotransport system, which in turn inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule. Individualize the drug dose to the patient. Depending on the clinical response, administer adult doses at increments of 20-40 mg, no sooner than 6-8 hours after the previous dose, until the desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments, until a satisfactory effect is achieved.

Spironolactone (Aldactone)

 

Spironolactone is used for management of edema resulting from excessive aldosterone excretion. This agent competes with aldosterone for receptor sites in the distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

Hydrochlorothiazide (Microzide)

 

Hydrochlorothiazide inhibits the reabsorption of sodium in the distal tubules, causing an increased excretion of sodium and water, as well as potassium and hydrogen ions.

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Inotropic agents

Class Summary

Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic congestive heart failure (CHF). Poor ventricular function may necessitate the use of inotropic medications.

Digoxin (Lanoxin)

 

Digoxin is a cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. This agent acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

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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].

 
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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.
 
 
 
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