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Hypoplastic Left Heart Syndrome: Differential Diagnoses & Workup

Author: P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
Coauthor(s): Daniel R Turner, MD, Associate Professor of Pediatric Cardiology, Wayne State University School of Medicine; Consulting Staff, Carman and Ann Adams Department of Pediatrics, Division of Cardiology, Children's Hospital of Michigan; 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
Contributor Information and Disclosures

Updated: Sep 22, 2009

Differential Diagnoses

Aortic Stenosis, Valvar
Myocarditis, Viral
Atrioventricular Septal Defect, Unbalanced
Total Anomalous Pulmonary Venous Connection
Cardiac Tumors
Coarctation of the Aorta
Interrupted Aortic Arch

Other Problems to Be Considered

  • Associated cardiac abnormalities
    • Anomalous pulmonary venous connection
    • Coarctation of the aorta
    • Complete atrioventricular canal
    • Coronary artery abnormalities (especially in patients with aortic atresia and mitral stenosis)
    • Persistent left superior vena cava
    • Endocardial fibroelastosis (especially in patients with aortic atresia and mitral stenosis)
  • Associated noncardiac abnormalities - Genetic disorders
  • Significant noncardiac abnormalities
    • CNS malformation
    • Diaphragmatic hernia
    • Necrotizing enterocolitis

Workup

Laboratory Studies

The following studies are indicated in hypoplastic left heart syndrome (HLHS)

  • CBC count
    • Measure hemoglobin levels, because severe neonatal anemia can cause high-output congestive heart failure (CHF) and cardiogenic shock. The hemoglobin level is usually normal in hypoplastic left heart syndrome.
    • Obtain a total WBC count with differential. Sepsis can cause symptoms of shock. The WBC count is typically normal.
  • Electrolytes
    • Electrolyte abnormalities may be present in infants with poor oral intake secondary to CHF. Use carbon dioxide and bicarbonate to assess acid-base status.
    • Electrolyte levels are usually normal. The carbon dioxide level may be low if a metabolic acidosis is present. The carbon dioxide level may be high if respiratory failure sets in.
  • BUN/creatinine
    • Infants with critical illness and significantly reduced systemic perfusion may show evidence of renal failure.
    • The creatinine level may be transiently elevated.
  • Liver function tests
    • Infants with critical illness and significantly reduced systemic perfusion and CHF may show evidence of hepatocellular damage.
    • Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels may be transiently elevated.
  • ABG and lactic acid
    • Assessing acid-base status is paramount, especially to rule out metabolic acidosis. Most infants have some evidence of metabolic acidosis, which should be immediately corrected. This is usually secondary to decreased systemic perfusion. Appropriate intervention to augment systemic flow should be promptly undertaken. Elevated levels of serum lactic acid generally precede a fall in pH, as acidosis develops.
    • Assessment of PaO2 and PaCO2 is important for respiratory management and manipulation of pulmonary vascular resistance by mechanical ventilation and the addition of supplemental inhaled nitrogen. The PaO2 is optimally 30-45 mm Hg, and the PaCO2 is ideally 45-50 mm Hg.
  • Karyotype
    • Chromosomal analysis is indicated for infants with dysmorphic features.
    • Nearly 25% of infants have chromosomal abnormalities.

Imaging Studies

  • Chest radiography 
    • Cardiomegaly and increased pulmonary vascular markings are typically present.
    • Marked pulmonary edema may be noted in infants with obstructed pulmonary venous return, usually due to markedly restrictive patent foramen ovale.
    • Although the radiography findings are not specific for the condition, the presence of a large heart with increased pulmonary vascular markings in a mildly cyanotic neonate should always prompt inclusion of hypoplastic left heart syndrome in the differential diagnosis. 
  • Echocardiography
    • Echocardiography is the test of choice for diagnosing hypoplastic left heart syndrome. Two-dimensional imaging clearly shows the hypoplastic left ventricle and ascending aorta. The right atrium, tricuspid valve, right ventricle, and main pulmonary artery are larger than usual.

      This echocardiographic still frame shows a long-a...

      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.

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      This echocardiographic still frame shows a long-a...

      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-cham...

      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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      This echocardiographic still frame shows a 4-cham...

      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.

    • Other structural abnormalities should be excluded.
    • Doppler and color Doppler imaging are also important.4
    • Evaluate tricuspid regurgitation, which is a preoperative risk factor for the Norwood procedure, and blood flow across the atrial septum. High-velocity Doppler jet across the atrial septum indicates restrictive interatrial communication. Observe retrograde blood flow from the ductus arteriosus into the transverse aortic arch and ascending aorta.
    • Evaluate the aortic arch and thoracic aorta for evidence of coarctation.
    • Two-dimensional and Doppler echocardiographic features are sufficiently characteristic of hypoplastic left heart syndrome such that cardiac catheterization and angiography are no longer necessary for diagnosis of this anomaly.
    • Variability of the chamber and vessel size is seen in hypoplastic left heart syndrome.
  • Ultrasonography of the head and abdomen
    • Ultrasonography of the head was thought to be necessary only if the infant has had a significantly long period in shock with potentially poor cerebral perfusion. However, at most institutions, routine head ultrasonography to exclude central nervous system abnormality and abdominal ultrasonography to evaluate kidneys are performed prior to the Norwood procedure or cardiac transplantation.
    • A significant prevalence of abnormalities is observed using ultrasonography of the head25 and abdomen.

Other Tests

  • Electrocardiography
    • Electrocardiography typically reveals sinus tachycardia, right-axis deviation, right atrial enlargement, and right ventricular hypertrophy with a qR configuration in the right precordial leads.
    • A paucity of left ventricular forces is noted in the left precordial leads.
    • ST-T wave changes suggestive of myocardial ischemia may be present in some patients.

Procedures

  • Cardiac catheterization (pre–Norwood procedure)
    • Routine diagnostic catheterization is not necessary because 2-dimensional and Doppler echocardiography can provide the necessary anatomic and hemodynamic data. However, a focused catheterization may become necessary to resolve any echocardiographic discrepancies that may be deemed important in surgical management.
    • If catheterization is performed, the features reflect the pathophysiology described above.
    • Oxygen saturation data indicate moderate-to-severe systemic venous desaturation, with a step-up at the level of right atrium secondary to left to right shunt across the atrial septum. The oxygen saturations in the right ventricle, pulmonary artery and aorta are similar reflecting common mixing in the right atrium. Mild systemic arterial desaturation is usually present unless severe pulmonary edema is noted.
    • The right atrial pressure is mildly elevated, and the left atrial pressure is moderate to severely elevated, unless a large atrial septal defect is present. The right ventricular and pulmonary arterial systolic pressures are at systemic level. If the ductus arteriosus is constricted, these pressures are higher than those in the aorta.
    • Angiography, although not necessary in all cases, reveals hypoplasia of the mitral valve, left ventricle and aorta. The ascending aorta is perfused in a retrograde fashion and serves as a common coronary artery, supplying both the right and left coronary arteries.
    • Perform interventional catheterization with blade/balloon atrial septostomy or static dilatation of the atrial septum to relieve pulmonary venous hypertension if blood flow from left atrium to right atrium is severely restricted at the atrial septum.26,27 Because of marked hypoplasia of the left atrium, conventional Rashkind balloon or Park blade atrial septostomy may not be possible. In such situations, static dilatation of the atrial septum may be performed.28,27 If the atrial septum is extremely thick with a markedly restrictive atrial septum, stent implantation to keep the atrial septum open may become necesssary.27
    • When hybrid procedures are contemplated, stenting of the ductus, atrial septum, or both may become necessary.
  • Cardiac catheterization (pre–bidirectional Glenn [stage II] procedure)
    • Perform routine catheterization before the operation to obtain hemodynamic data and several important angiograms.
    • Calculate pulmonary vascular resistance to ensure the patient's suitability for the stage II procedure.
    • Perform angiography in the right ventricle to show ventricular function and tricuspid regurgitation.
    • Perform angiography in the transverse aortic arch near the shunt to show pulmonary artery size and distribution and to rule out recurrent aortic coarctation or significant aortopulmonary collateral vessels.
    • If collateral vessels are found, they may be occluded with coils at the same catheterization.
  • Pre-Fontan (stage III) procedure
    • Routine catheterization before completing the operation is generally recommended.
    • Measure pulmonary artery pressure and calculate pulmonary vascular resistance and perform right ventricular angiography.
    • Delineate pulmonary artery anatomy by performing angiography at the superior vena cava–pulmonary artery anastomosis via an internal jugular approach.
    • Recurrent coarctation of the aorta and significant collateral vessels are excluded again.
    • Descending aortography and selective right and left subclavian artery angiography to identify any collateral vessels to lungs is recommended.
    • Postcatheterization complications include hemorrhage, vascular disruption after balloon dilation, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm but are rare.
  • Catheter intervention
    • In the neonate, obstruction at the level of the patent foramen ovale (atrial septum) may be treated with conventional Rashkind balloon atrial septostomy. However, because the left atrium is small, Rashkind septostomy26 may not be feasible. In addition, the septum may be too thick to be torn by balloon septostomy; therefore, Park blade septostomy may be necessary and should precede the Rashkind procedure.29,30 However, hypoplastic left atrium may preclude blade septostomy. Static dilatation of the atrial septum with a balloon angioplasty catheter may be used and may not only relieve the obstruction but also keep some restriction, such that no rapid fall in the pulmonary vascular resistance occurs. Static balloon dilatation is preferred by the senior author.28,27
    • In some patients, the atrial septum may be intact or have a tight patent foramen ovale that may not even allow passage of a catheter. In such situations, puncture of the atrial septum by a Brockenbrough technique or radiofrequency perforation of the atrial septum followed by static balloon atrial septal dilatation or, preferably, stent implantation may become necessary.27,31
    • If progressive cyanosis develops after a previous Blalock-Taussig shunt, and if the hypoxemia is due to a stenotic shunt, balloon dilatation may be used to improve oxygen saturation.32 However, if the patient is of sufficient size and age to undergo a bidirectional Glenn procedure, this procedure should be performed instead of balloon angioplasty of a narrowed Blalock-Taussig shunt.
    • If severe aortic coarctation is present, particularly after Norwood procedure, balloon angioplasty may be useful in relieving aortic obstruction and may help achieve reduce right ventricular afterload.33
    • If significant branch pulmonary artery stenosis is present before a bidirectional Glenn or Fontan conversion or after a Fontan procedure, balloon angioplasty or placement of intravascular stents is recommended.34,35
    • Development of aortopulmonary collateral vessels has been increasingly observed in recent studies. Before the final Fontan conversion, occlusion of these vessels in the catheterization laboratory, usually by means of coil embolization,36,37 is recommended to reduce left ventricular volume overloading and, probably, the duration of chest tube drainage following surgery.
    • Following completion of Fontan procedure, some patients may develop recurrent pleural effusion, liver dysfunction, plastic bronchitis or protein-losing enteropathy.38 In these patients, following exclusion of obstructive lesion in the Fontan circuit, puncture of the atrial septum by a Brockenbrough technique followed by static balloon atrial septal dilatation or stent implantation may be beneficial.
    • Patients who have undergone a fenestrated Fontan operation or have a residual atrial defect, despite correction, may have significant right-to-left shunting causing severe hypoxemia. These residual atrial defects may be closed using transcatheter techniques.39,40

More on Hypoplastic Left Heart Syndrome

Overview: Hypoplastic Left Heart Syndrome
Differential Diagnoses & Workup: Hypoplastic Left Heart Syndrome
Treatment & Medication: Hypoplastic Left Heart Syndrome
Follow-up: Hypoplastic Left Heart Syndrome
Multimedia: Hypoplastic Left Heart Syndrome
References
Further Reading
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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. 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].

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

  21. 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].

  22. 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].

  23. 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].

  24. 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].

  25. 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].

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

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

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

  29. 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].

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

  31. 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].

  32. 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].

  33. 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].

  34. 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].

  35. 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].

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

  37. 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.

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

  39. 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].

  40. 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.

  41. 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].

  42. 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].

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

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

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

  46. 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.

  47. 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].

  48. 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.

  49. 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].

  50. 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.

  51. 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.

  52. 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].

  53. 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].

  54. 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].

  55. 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].

  56. 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].

  57. 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].

  58. 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].

  59. 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.

  60. 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.

  61. 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].

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

  63. 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].

  64. 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].

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

  66. 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].

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

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Keywords

hypoplastic left heart syndrome, HLHS, prostaglandin, PGE, prostaglandin E1, PGE1, Fontan, hemi-Fontan, pre-Fontan, Norwood, Sano, hybrid, hypoplasia of the aortic tract complex, patent foramen ovale, atrial septal defect, patent ductus arteriosus, pulmonary stenosis, endocardial fibroelastosis, metabolic acidosis, oliguria, tricuspid regurgitation, hypothermia, tachycardia, respiratory distress, hepatosplenomegaly, treatment, diagnosis

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Contributor Information and Disclosures

Author

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

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

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.

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

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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