Pediatric Hypoplastic Left Heart Syndrome Medication

  • Author: P Syamasundar Rao; Chief Editor: Stuart Berger, MD   more...
 
Updated: Nov 10, 2011
 

Medication Summary

Before the Norwood procedure or cardiac transplantation in patients with hypoplastic left heart syndrome (HLHS), treat infants with prostaglandin E1 infusion, diuretics, inotropes, and afterload reduction. Drug management after cardiac transplantation is not discussed in this article.

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Prostaglandins

Class Summary

Prostaglandin E1 promotes dilatation of the ductus arteriosus in infants with ductal-dependent cardiac abnormalities.

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Alprostadil (Prostaglandin E1, Prostin)

 

Causes relaxation of smooth muscle, primarily within the ductus arteriosus. Used in infants with ductal-dependent congenital heart disease due to restricted systemic blood flow. The drug is also useful in neonates with ductal dependent pulmonary circulation.

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Diuretic agents

Class Summary

These agents decrease preload by increasing free-water excretion. Decreasing preload may improve systolic ventricular function.

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Furosemide (Lasix)

 

Loop diuretic that blocks sodium reabsorption in the ascending limb of loop of Henle.

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Spironolactone (Aldactone)

 

This drug is a potassium-sparing loop diuretic.

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Cardiac glycosides

Class Summary

These medications improve ventricular systolic function by increasing the calcium supply available for myocyte contraction.

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Digoxin (Lanoxin)

 

This form inhibits the sodium-potassium ATPase pump in cardiac myocytes.

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Inotropic agents

Class Summary

These agents stimulate alpha-adrenergic and beta-adrenergic and beta-dopaminergic receptors in the heart and vascular bed.

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Dopamine (Intropin)

 

At lower doses, stimulation of beta1-adrenergic and beta1-dopaminergic receptors results in positive inotropism and renal vasodilatation; at higher doses, stimulation of alpha-adrenergic receptors results in peripheral and renal vasoconstriction.

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Dobutamine (Dobutrex)

 

This drug primarily stimulates the beta1-adrenergic receptor and has less alpha-adrenergic stimulation, leading primarily to increased myocardial contractility.

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Afterload-reducing agents

Class Summary

Afterload reduction improves myocardial performance and theoretically reduces atrioventricular and semilunar valve insufficiency.

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Captopril (Capoten)

 

ACE inhibitor, which decreases the production of angiotensin II, a potent vasoconstrictor, resulting in peripheral vasodilatation and afterload reduction, improving myocardial performance and theoretically reducing AV and semilunar valve insufficiency.

Administer a test dose of 0.1 mg PO to assess initial response

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Antiplatelet agents

Class Summary

These agents are used in the treatment or prevention of thrombo-occlusive disease mediated by the action of platelets. They inhibit platelet function by blocking cyclooxygenase and subsequent aggregation.

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Aspirin (Anacin, Ascriptin, Bayer Aspirin)

 

Inhibits the enzyme cyclooxygenase that reduces production of thromboxane A2, which is a potent vasoconstrictor and platelet-aggregating agent.

Antiplatelet effects of aspirin last the entire life of the platelet (6-10 d) and are not reversible.

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Contributor Information and Disclosures
Author

P Syamasundar Rao  MD, Professor of Pediatrics, Division of Cardiology, University of Texas Medical School at Houston; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas

P Syamasundar Rao is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Turner, MD  Associate Professor of Pediatrics, Wayne State University School of Medicine; Consulting Staff, Carman and Ann Adams Department of Pediatrics, Division of Cardiology, Children's Hospital of Michigan

Daniel R Turner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Thomas J Forbes, MD, FACC, FSCAI  Associate Professor (Clinical-Educator), Director of Catheterization Laboratory, Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University

Thomas J Forbes, MD, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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.

Julian M Stewart, MD, PhD  Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College

Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics

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. Noonan JA, Nadas AS. The hypoplastic left heart syndrome. Pediat Clinics N Amer. 1958;5:1029.

  2. Lev M, Arcilla RA, Rimoldi HJA et al. Premature narrowing or closure of foramen ovale. Am Hear J. 1963;65:638.

  3. Norwood WI, Kirklin JK, Sanders SP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol. Jan 1980;45(1):87-91. [Medline].

  4. Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. Jan 6 1983;308(1):23-6. [Medline].

  5. Bailey L, Concepcion W, Shattuck H, Huang L. Method of heart transplantation for treatment of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. Jul 1986;92(1):1-5. [Medline].

  6. Rao PS, Striepe V, Merrill WH. Hypoplastic left heart syndrome. In: Kambam J (ed.). Cardiac Anesthesia for Infants and Children. St. Louis, MO: Mosby-Year Book,; 1994:296-309.

  7. Freedom RM, Williams WG, Dische MR, Rowe RD. Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution. Br Heart J. Aug 1976;38(8):821-6. [Medline].

  8. Balfour IC, Covitz W, Davis H, Rao PS, Strong WB, Alpert BS. Cardiac size and function in children with sickle cell anemia. Am Heart J. Aug 1984;108(2):345-50. [Medline].

  9. Bharati S, Lev M. The surgical anatomy of hypoplasia of aortic tract complex. J Thorac Cardiovasc Surg. Jul 1984;88(1):97-101. [Medline].

  10. Von Rueden TJ, Knight L, Moller JH, Ewards JE. Coarctation of the aorta associated with aortic valvular atresia. Circulation. Nov 1975;52(5):951-4.

  11. Jonas RA, Lang P, Hansen D, Hickey P, Castaneda AR. First-stage palliation of hypoplastic left heart syndrome. The importance of coarctation and shunt size. J Thorac Cardiovasc Surg. Jul 1986;92(1):6-13. [Medline].

  12. Norwood WI Jr. Hypoplastic left heart syndrome. Ann Thorac Surg. Sep 1991;52(3):688-95. [Medline].

  13. Rudolph AM. Congenital Diseases of the Heart. Chicago: Year Book Medical; 1974.

  14. Rao PS. Fetal and Neonatal Circulation. In: Kambam J (ed.). Cardiac Anesthesia for Infants and Children. St. Louis, MO: Mosby-Year Book; 1994:Chapter 2; pp. 10-19.

  15. Sihha SN, Rusnak SL, Sommers HM, et al. Hypoplastic left ventricle syndrome:analysis of 30 autopsy cases in infants with surgical considerations. Am J Cardio. 1968;21:166.

  16. Fyler DC. Report of the New England Regional Infant Cardiac Program. Pediatrics. Feb 1980;65(2 Pt 2):375-461.

  17. Freedom RM. Aortic atresia. In: Keith JD, Rowe RD, Vlad P, eds. Heart Disease in Infants and Children. 3rd ed. New York: McMillian; 1978.

  18. Fyler DC. Prevalence trends. In: Fyler DC, ed. Nadas' Pediatric Cardiology Hanley & Belfus. Philadelphia: 1992.

  19. Hornik CP, He X, Jacobs JP, et al. Complications after the norwood operation: an analysis of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg. Nov 2011;92(5):1734-40. [Medline].

  20. Galindo A, Nieto O, Villagra S, Graneras A, Herraiz I, Mendoza A. Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery. Ultrasound Obstet Gynecol. May 2009;33(5):560-6. [Medline].

  21. Nora JJ. Multifactorial inheritance hypothesis for etiology of congenital heart disease: the genetic-environmental interaction. Circulation. 1968;38:604-17.

  22. Lev M, Arcilla R, Rimoldi HJ, Licata RH, Gasul BM. Premature narrowing or closure of the foramen ovale. Am Heart J. May 1963;65:638-47. [Medline].

  23. Weinberg PM, Chin AJ, Murphy JD, Pigott JD, Norwood WI. Postmortem echocardiography and tomographic anatomy of hypoplastic left heart syndrome after palliative surgery. Am J Cardiol. Dec 1 1986;58(13):1228-32. [Medline].

  24. Hinton RB, Martin LJ, Rame-Gowda S, Tabangin ME, Cripe LH, Benson DW. Hypoplastic left heart syndrome links to chromosomes 10q and 6q and is genetically related to bicuspid aortic valve. J Am Coll Cardiol. Mar 24 2009;53(12):1065-71. [Medline].

  25. Grossfeld P, Ye M, Harvey R. Hypoplastic left heart syndrome: new genetic insights. J Am Coll Cardiol. Mar 24 2009;53(12):1072-4. [Medline].

  26. Glauser TA, Rorke LB, Weinberg PM, Clancy RR. Congenital brain anomalies associated with the hypoplastic left heart syndrome. Pediatrics. Jun 1990;85(6):984-90. [Medline].

  27. Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoracotomy. J Am Med Assoc. 1966;196:991-992.

  28. Rao PS. Role of Interventional Cardiology In Neonates: Part I. Non-Surgical Atrial Septostomy. Congenital Cardiol Today. 2007;5(12):1-12.

  29. Rao PS. Static balloon dilatation of the atrial septum. Am Heart J. 1993;125:1826.

  30. Park SC, Neches WH, Zuberbuhler JR, Lenox CC, Mathews RA, Fricker FJ. Clinical use of blade atrial septostomy. Circulation. Oct 1978;58(4):600-6. [Medline].

  31. Rao PS. Transcatheter Blade Atrial Septostomy. Cath Cardiovasc Dgn. 1984;10:335-342.

  32. Atz AM, Feinstein JA, Jonas RA, Perry SB, Wessel DL. Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect. Am J Cardiol. Apr 15 1999;83(8):1224-8. [Medline].

  33. Rao PS, Levy JM, Chopra PS. Balloon angioplasty of stenosed Blalock-Taussig anastomosis: role of balloon-on-a-wire in dilating occluded shunts. Am Heart J. Nov 1990;120(5):1173-8. [Medline].

  34. Siblini G, Rao PS, Nouri S, Ferdman B, Jureidini SB, Wilson AD. Long-term follow-up results of balloon angioplasty of postoperative aortic recoarctation. Am J Cardiol. Jan 1 1998;81(1):61-7. [Medline].

  35. Rao PS, Balfour IC, Singh GK, Jureidini SB, Chen S. Bridge stents in the management of obstructive vascular lesions in children. Am J Cardiol. Sep 15 2001;88(6):699-702. [Medline].

  36. Rao PS. Stents in the management of congenital heart disease in pediatric and adult patients. Indian Heart J. Nov-Dec 2001;53(6):714-30. [Medline].

  37. Siblini G, Rao PS. Coil Embolization in the Management of Cardiac Problems in Children. J Invasive Cardiol. Sep 1996;8(7):332-340. [Medline].

  38. Rao PS. Transcatheter Embolization of Unwanted Blood Vessels in Children. In: Rao PS, Kern MJ. (editors). Catheter Based Devices for Treatment of Noncoronary Cardiovascular Disease in Adults and Children. Philadelphia, PA: Lippincott, Williams & Wilkins; 2003.

  39. Rao PS. Protein-Losing Enteropathy following the Fontan Operation (Editorial). J Invasive Cardiol. 2007;19:447-448.

  40. Rao PS, Chandar JS, Sideris EB. Role of inverted buttoned device in transcatheter occlusion of atrial septal defects or patent foramen ovale with right-to-left shunting associated with previously operated complex congenital cardiac anomalies. Am J Cardiol. Oct 1 1997;80(7):914-21. [Medline].

  41. Rao PS. Transcatheter Closure of Atrial septal Defects with Right-to-left Shunt,. In: Rao PS, Kern MJ, eds. Catheter Based Devices for Treatment of Noncoronary Cardiovascular Disease in Adults and Children. Philadelphia, PA: Lippincott, Williams & Wilkins; 2003.

  42. Peterson AL, Quartermain MD, Ades A, Khalek N, Johnson MP, Rychik J. Impact of mode of delivery on markers of perinatal hemodynamics in infants with hypoplastic left heart syndrome. J Pediatr. Jul 2011;159(1):64-9. [Medline].

  43. Baba K, Ohtsuki S, Kamada M, Kataoka K, Ohno N, Okamoto Y. Preoperative management for tricuspid regurgitation in hypoplastic left heart syndrome. Pediatr Int. Jun 2009;51(3):399-404. [Medline].

  44. Day RW, Barton AJ, Pysher TJ, Shaddy RE. Pulmonary vascular resistance of children treated with nitrogen during early infancy. Ann Thorac Surg. May 1998;65(5):1400-4. [Medline].

  45. Caylor GC, Smeloff EA, Miller GE. Surgical palliation of hypoplastic left side of the heart. New Engl J Med. 1970;282:780.

  46. Dotty DB, Knott HW. Hypoplastic left heart syndrome:experience with an operation to establish functionally normal circulation. J Thorac Cardiovasc Surg. 1977;74:624.

  47. Fontan F, Baudet E:. Surgical repair of tricuspid atresia. Thorax. May 1971;26(3):240-8.

  48. Kreutzer G, Bono H, Galindez E:. Una operacion para la correccion de la atresia tricuspidea. Ninth Argentine Congress of Cardiology, Oct. 31-Nov. 6. Buenos Aires, Argentina:. 1971.

  49. de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg. Nov 1988;96(5):682-95. [Medline].

  50. Sano S, Ishino K, Kawada M et al:. Right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:504-510.

  51. Sano S, Ishino K, Kado H, Shiokawa Y, Sakamoto K, Yokota M. Outcome of right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome: a multi-institutional study. Ann Thorac Surg. Dec 2004;78(6):1951-7; discussion 1957-8. [Medline].

  52. Pizarro C, Malec E, Maher KO, et al. Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome. Circulation. Sep 9 2003;108(10 Suppl 1):II155-II160.

  53. Choussat A, Fontan F, Besse P. Selection criteria for Fontan procedure. In: Shinebourne EA, Anderson RH, eds. Pediatric Cardiology, 1977. Harcourt Health Sciences Group. 1978:559.

  54. Laks H, Pearl JM, Haas GS, Drinkwater DC, Milgalter E, Jarmakani JM. Partial Fontan: advantages of an adjustable interatrial communication. Ann Thorac Surg. Nov 1991;52(5):1084-94; discussion 1094-5. [Medline].

  55. Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. Nov 1990;82(5):1681-9. [Medline].

  56. Thompson LD, Petrossian E, McElhinney DB, Abrikosova NA, Moore P, Reddy VM. Is it necessary to routinely fenestrate an extracardiac fontan?. J Am Coll Cardiol. Aug 1999;34(2):539-44. [Medline].

  57. Konertz W, Schneider M, Herwig V, Kampmann C, Waldenberger F, Hausdorf G. Modified hemi-Fontan operation and subsequent nonsurgical Fontan completion. J Thorac Cardiovasc Surg. Sep 1995;110(3):865-7. [Medline].

  58. Hausdorf G, Schneider M, Konertz W. Surgical preconditioning and completion of total cavopulmonary connection by interventional cardiac catheterisation: a new concept. Heart. Apr 1996;75(4):403-9. [Medline].

  59. Sidiropoulos A, Ritter J, Schneider M, Konertz W. Fontan modification for subsequent non-surgical Fontan completion. Eur J Cardiothorac Surg. May 1998;13(5):509-12; discussion 512-3. [Medline].

  60. Galantowicz M, Cheatham JP. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol. May-Jun 2005;26(3):190-9. [Medline].

  61. Bacha EA, Daves S, Hardin J, et al:. Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy. J Thorac Cardiovasc Surg. Jan 2006;131(1):163-171.e2. Epub 2005 Dec 5.

  62. Tworetzky W, Wilkins-Haug L, Jennings RW, et al. Balloon dilation of severe aortic stenosis in the fetus: potential for prevention of hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention. Circulation. Oct 12 2004;110(15):2125-31. Epub 2004 Oct 4.

  63. Braudis NJ, Curley MA, Beaupre K, et al. Enteral feeding algorithm for infants with hypoplastic left heart syndrome poststage I palliation. Pediatr Crit Care Med. Jul 2009;10(4):460-6. [Medline].

  64. Bove EL. Current status of staged reconstruction for hypoplastic left heart syndrome. Pediatr Cardiol. Jul-Aug 1998;19(4):308-15. [Medline].

  65. Sinzobahamvya N, Photiadis J, Kumpikaite D, Fink C, Blaschczok HC, Brecher AM. Comprehensive Aristotle score: implications for the Norwood procedure. Ann Thorac Surg. May 2006;81(5):1794-800. [Medline].

  66. Phelps HM, Mahle WT, Kim D, et al. Postoperative cerebral oxygenation in hypoplastic left heart syndrome after the Norwood procedure. Ann Thorac Surg. May 2009;87(5):1490-4. [Medline].

  67. Kon AA. Ethics of Cardiac Transplantation in Hypoplastic Left Heart Syndrome. Pediatr Cardiol. Apr 25 2009;[Medline].

  68. Elzenga NJ, Gittenberger-de Groot AC, Oppenheimer-Dekker A. Coarctation and other obstructive aortic arch anomalies: their relationship to the ductus arteriosus. Int J Cardiol. Dec 1986;13(3):289-308. [Medline].

  69. Rao PS. Perinatal circulatory physiology. Indian J Pediatr. Jul-Aug 1991;58(4):441-51. [Medline].

 
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This echocardiographic still frame shows a long-axis view of the aortic arch in a patient with hypoplastic left heart syndrome (HLHS). The ascending aorta is markedly hypoplastic, serving only to deliver blood in a retrograde fashion to the coronary arteries. An echo-bright coarctation shelf is seen at the insertion of the ductus arteriosus.
This echocardiographic still frame shows a 4-chamber view of the heart in a patient with hypoplastic left heart syndrome (HLHS). A large right ventricle (RV) and hypoplastic left ventricle (star) are seen. Right atrium = RA. Left atrium = LA.
 
 
 
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