hypoplastic left heart syndrome

Summary

Summary: A condition caused by underdevelopment of the whole left half of the heart. It is characterized by hypoplasia of the left cardiac chambers (HEART ATRIUM; HEART VENTRICLE), the AORTA, the AORTIC VALVE, and the MITRAL VALVE. Severe symptoms appear in early infancy when DUCTUS ARTERIOSUS closes.

Top Publications

  1. Hinton R, Martin L, Tabangin M, Mazwi M, Cripe L, Benson D. Hypoplastic left heart syndrome is heritable. J Am Coll Cardiol. 2007;50:1590-5 pubmed
    ..study sought to determine the size of the genetic effect (heritability) in families identified by a hypoplastic left heart syndrome (HLHS) proband...
  2. Han R, Gurofsky R, Lee K, Dipchand A, Williams W, Smallhorn J, et al. Outcome and growth potential of left heart structures after neonatal intervention for aortic valve stenosis. J Am Coll Cardiol. 2007;50:2406-14 pubmed
    ..However, the continued hypoplasia of the mitral valve warrants further consideration in the long-term management of these patients. ..
  3. Qian Y, Liu J, Itatani K, Miyaji K, Umezu M. Computational hemodynamic analysis in congenital heart disease: simulation of the Norwood procedure. Ann Biomed Eng. 2010;38:2302-13 pubmed publisher
    b>Hypoplastic left heart syndrome (HLHS) is a congenital heart disease which should be treated at neonate. Even now, its operation is one of the greatest challenges...
  4. Emani S, Bacha E, McElhinney D, Marx G, Tworetzky W, Pigula F, et al. Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart. J Thorac Cardiovasc Surg. 2009;138:1276-82 pubmed publisher
    ..This approach may provide an alternative to single-ventricle management in this difficult patient group. ..
  5. Petko C, Moller P, Hoffmann U, Kramer H, Uebing A. Comprehensive evaluation of right ventricular function in children with different anatomical subtypes of hypoplastic left heart syndrome after Fontan surgery. Int J Cardiol. 2011;150:45-9 pubmed publisher
    There is some evidence that hypoplastic left heart syndrome (HLHS) survivors with a larger left ventricular (LV) cavity may have poorer long-term outcome than those with mitral and aortic valve atresia (MA/AA) and negligible LV...
  6. Naguib A, Winch P, Schwartz L, Isaacs J, Rodeman R, Cheatham J, et al. Anesthetic management of the hybrid stage 1 procedure for hypoplastic left heart syndrome (HLHS). Paediatr Anaesth. 2010;20:38-46 pubmed publisher
    Despite advances in the surgical and perioperative management of patients with hypoplastic left heart syndrome (HLHS), outcomes for this high-risk group of patients remains suboptimal...
  7. Ong C, Hia C, Lim T, Teo L. Isolated left-ventricular apical hypoplasia presenting as a left-ventricular mass on echocardiography. Pediatr Cardiol. 2012;33:1456-7 pubmed publisher
    ..This report describes an asymptomatic pediatric patient who initially presented with a left-ventricular mass on echocardiography. ..
  8. Feinstein J, Benson D, Dubin A, Cohen M, Maxey D, Mahle W, et al. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012;59:S1-42 pubmed publisher
    ..defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS)...
  9. Petko C, Voges I, Schlangen J, Scheewe J, Kramer H, Uebing A. Comparison of right ventricular deformation and dyssynchrony in patients with different subtypes of hypoplastic left heart syndrome after Fontan surgery using two-dimensional speckle tracking. Cardiol Young. 2011;21:677-83 pubmed publisher
    The left ventricle in patients with hypoplastic left heart syndrome may influence right ventricular function and outcome...

More Information

Publications62

  1. Iascone M, Ciccone R, Galletti L, Marchetti D, Seddio F, Lincesso A, et al. Identification of de novo mutations and rare variants in hypoplastic left heart syndrome. Clin Genet. 2012;81:542-54 pubmed publisher
    b>Hypoplastic left heart syndrome (HLHS) is one of the most severe congenital heart malformations, characterized by underdevelopment of the structures in the left heart-aorta complex. The majority of cases are sporadic...
  2. Tumabiene K, Magpali A, Chiong L, Jara R, Abarquez R, Abelardo N. A plump and fatty heart: isolated left ventricular apical hypoplasia. Echocardiography. 2012;29:E193-6 pubmed publisher
    ..To our knowledge, this is the first reported adult case in Asia. ..
  3. Mahtab E, Gittenberger de Groot A, Vicente Steijn R, Lie Venema H, Rijlaarsdam M, Hazekamp M, et al. Disturbed myocardial connexin 43 and N-cadherin expressions in hypoplastic left heart syndrome and borderline left ventricle. J Thorac Cardiovasc Surg. 2012;144:1315-22 pubmed publisher
    Borderline left ventricle is the left ventricular morphology at the favorable end of the hypoplastic left heart syndrome. In contrast to the severe end, it is suitable for biventricular repair...
  4. Jones B, Ditchfield M, Cahoon G, Hardy P, D Udekem Y, Brizard C, et al. Cardiac magnetic resonance imaging prior to bidirectional cavopulmonary connection in hypoplastic left heart syndrome. Heart Lung Circ. 2010;19:535-40 pubmed publisher
    ..obtained from cardiac catheterisation is not essential in pre-operative assessment of children with hypoplastic left heart syndrome (HLHS) undergoing Bidirectional Cavopulmonary Connection (BCPC)...
  5. Porras D, Brown D, Marshall A, del Nido P, Bacha E, McElhinney D. Factors associated with subsequent arch reintervention after initial balloon aortoplasty in patients with Norwood procedure and arch obstruction. J Am Coll Cardiol. 2011;58:868-76 pubmed publisher
    ..The risk for arch reintervention is highest in patients with proximal arch obstruction, those age <3 months at the time of BD, and those with less successful acute results. ..
  6. Ricci M, Mohapatra B, Urbiztondo A, Birusingh R, Morgado M, Rodriguez M, et al. Differential changes in TGF-?/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome. J Card Fail. 2010;16:628-34 pubmed publisher
    b>Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the left ventricle (LV) and increased biomechanical stress on the right ventricle (RV) from single ventricle physiology...
  7. Prakash A, Satiroglu E, Porras D, McElhinney D, Keane J, Lock J, et al. Risk factors for profuse systemic-to-pulmonary artery collateral burden in hypoplastic left heart syndrome. Am J Cardiol. 2013;112:400-4 pubmed publisher
    Risk factors for developing systemic-to-pulmonary artery collaterals (SPCs) in hypoplastic left heart syndrome (HLHS) are unknown...
  8. McElhinney D, Marshall A, Wilkins Haug L, Brown D, Benson C, Silva V, et al. Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome. Circulation. 2009;120:1482-90 pubmed publisher
    ..in the midgestation fetus with a normal-sized or dilated left ventricle predictably progresses to hypoplastic left heart syndrome when associated with certain physiological findings...
  9. Hickey E, Caldarone C, McCrindle B. Left ventricular hypoplasia: a spectrum of disease involving the left ventricular outflow tract, aortic valve, and aorta. J Am Coll Cardiol. 2012;59:S43-54 pubmed publisher
    quot;Hypoplastic left heart syndrome" is an unsatisfactory term describing lethal underdevelopment of the left ventricle (LV)...
  10. Haffajee J, Finley J, Brooks E, Kuvin J, Patel A. Echocardiographic characterization of left ventricular apical hypoplasia accompanied by a patent ductus arteriosus. Eur J Echocardiogr. 2011;12:E17 pubmed publisher
    ..As more cases are recognized and patients are followed over time, the natural history and optimal treatment for this cardiomyopathy may be further elucidated. ..
  11. Gelehrter S, Fifer C, Armstrong A, Hirsch J, Gajarski R. Outcomes of hypoplastic left heart syndrome in low-birth-weight patients. Pediatr Cardiol. 2011;32:1175-81 pubmed publisher
    The objective of this study was to assess outcomes of hypoplastic left heart syndrome (HLHS) patients weighing ? 2.5 kg throughout staged palliation. We performed a single-center retrospective review...
  12. Voges I, Jerosch Herold M, Hedderich J, Westphal C, Hart C, Helle M, et al. Maladaptive aortic properties in children after palliation of hypoplastic left heart syndrome assessed by cardiovascular magnetic resonance imaging. Circulation. 2010;122:1068-76 pubmed publisher
    The status of the reconstructed aorta in hypoplastic left heart syndrome is considered an important determinant of long-term prognosis...
  13. Rychik J, Szwast A, Natarajan S, Quartermain M, Donaghue D, Combs J, et al. Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience. Ultrasound Obstet Gynecol. 2010;36:465-70 pubmed publisher
    To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS)...
  14. Khoo N, Smallhorn J, Kaneko S, Myers K, Kutty S, Tham E. Novel insights into RV adaptation and function in hypoplastic left heart syndrome between the first 2 stages of surgical palliation. JACC Cardiovasc Imaging. 2011;4:128-37 pubmed publisher
    This study sought to examine the changes in ventricular function of hypoplastic left heart syndrome (HLHS) between the first 2 stages of surgical palliation...
  15. Petko C, Uebing A, Furck A, Rickers C, Scheewe J, Kramer H. Changes of right ventricular function and longitudinal deformation in children with hypoplastic left heart syndrome before and after the Norwood operation. J Am Soc Echocardiogr. 2011;24:1226-32 pubmed publisher
    ..and deformation parameters before and at steady state after the Norwood operation in neonates with hypoplastic left heart syndrome. A further aim was to delineate factors that affected these changes...
  16. Vogel M, Wilkins Haug L, McElhinney D, Marshall A, Benson C, Silva V, et al. Reversible ductus arteriosus constriction due to maternal indomethacin after fetal intervention for hypoplastic left heart syndrome with intact/restrictive atrial septum. Fetal Diagn Ther. 2010;27:40-5 pubmed publisher
    ..Indomethacin promotes closure of the DA in newborns and in fetal life, a potentially life threatening complication in fetuses with ductus-dependent congenital heart disease...
  17. Galantowicz M, Cheatham J, Phillips A, Cua C, Hoffman T, Hill S, et al. Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg. 2008;85:2063-70; discussion 2070-1 pubmed publisher
    ..of a novel hybrid approach have resulted in a reliable, reproducible alternative treatment for hypoplastic left heart syndrome (HLHS). Herein we report our results using this hybrid approach in a uniform risk cohort...
  18. Larrazabal L, Selamet Tierney E, Brown D, Gauvreau K, Vida V, Bergersen L, et al. Ventricular function deteriorates with recurrent coarctation in hypoplastic left heart syndrome. Ann Thorac Surg. 2008;86:869-74; discussion 869-74 pubmed publisher
    Recurrent coarctation (re-CoA) after stage I palliation in hypoplastic left heart syndrome (HLHS) is deleterious. We studied whether re-CoA had an effect on ventricular systolic function...
  19. Szwast A, Tian Z, McCann M, Donaghue D, Rychik J. Right ventricular performance in the fetus with hypoplastic left heart syndrome. Ann Thorac Surg. 2009;87:1214-9 pubmed publisher
    In the fetus with hypoplastic left heart syndrome (HLHS), the single right ventricle (RV) pumps the entire cardiac output in utero...
  20. Holzer R, Wood A, Chisolm J, Hill S, Phillips A, Galantowicz M, et al. Atrial septal interventions in patients with hypoplastic left heart syndrome. Catheter Cardiovasc Interv. 2008;72:696-704 pubmed publisher
    ..an institutional experience performing percutaneous atrial septal interventions in patients with hypoplastic left heart syndrome (HLHS)...
  21. Bove E, de Leval M, Migliavacca F, Guadagni G, Dubini G. Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the Norwood procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:1040-7 pubmed
    ..The superior hydraulic performance of the lateral tunnel Fontan operation after the hemi-Fontan procedure relative to any other method may be due to closer to optimal caval offset achieved in the surgical reconstruction. ..
  22. Andrews R, Tulloh R. Hypoplastic left heart syndrome: diagnosis and management. Hosp Med. 2002;63:24-7 pubmed
    The prognosis of hypoplastic left heart syndrome has improved dramatically in recent years with advances in reconstructive surgery...
  23. Lim D, Peeler B, Matherne G, Kron I, Gutgesell H. Risk-stratified approach to hybrid transcatheter-surgical palliation of hypoplastic left heart syndrome. Pediatr Cardiol. 2006;27:91-95 pubmed publisher
    We prospectively employed a risk-stratified approach to first-stage palliation of hypoplastic left heart syndrome. High-risk features included severe tricuspid insufficiency, severe right ventricular dysfunction, a severely restrictive ..
  24. Chang R, Chen A, Klitzner T. Clinical management of infants with hypoplastic left heart syndrome in the United States, 1988-1997. Pediatrics. 2002;110:292-8 pubmed
    To evaluate changes in the clinical management of infants with hypoplastic left heart syndrome (HLHS) over a 10-year period...
  25. Bautista Hernandez V, Marx G, Gauvreau K, Pigula F, Bacha E, Mayer J, et al. Coarctectomy reduces neoaortic arch obstruction in hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2007;133:1540-6 pubmed
    ..after stage I palliation is an important risk factor affecting interstage mortality in patients with hypoplastic left heart syndrome, with no accepted standard surgical approach...
  26. Azakie T, Merklinger S, McCrindle B, Van Arsdell G, Lee K, Benson L, et al. Evolving strategies and improving outcomes of the modified norwood procedure: a 10-year single-institution experience. Ann Thorac Surg. 2001;72:1349-53 pubmed
    ..Outcomes and risk factors for mortality were sought. Hypoplastic left heart syndrome or a variant was the primary diagnosis in 118 infants (69%). The overall 5-year survival rate was 43%...
  27. Andrews R, Tulloh R, Sharland G, Simpson J, Rollings S, Baker E, et al. Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis. Arch Dis Child. 2001;85:474-7 pubmed
    Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options...
  28. Vasquez J, Rabah R, Delius R, Walters H. Hypoplastic left heart syndrome with intact atrial septum associated with deletion of the short arm of chromosome 18. Cardiovasc Pathol. 2003;12:102-4 pubmed
    We report on a female newborn with deletion of the short arm of the chromosome 18 (del 18p) and hypoplastic left heart syndrome (HLHS) with intact atrial septum...
  29. Hinton R, Martin L, Rame Gowda S, Tabangin M, Cripe L, Benson D. Hypoplastic left heart syndrome links to chromosomes 10q and 6q and is genetically related to bicuspid aortic valve. J Am Coll Cardiol. 2009;53:1065-71 pubmed publisher
    This study was designed to identify disease loci for hypoplastic left heart syndrome (HLHS) and evaluate the genetic relationship between HLHS and bicuspid aortic valve (BAV)...
  30. Stasik C, Gelehrter S, Goldberg C, Bove E, Devaney E, Ohye R. Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg. 2006;131:412-7 pubmed
    Tremendous strides have been made in the outcomes for hypoplastic left heart syndrome and other functional single-ventricle malformations over the past 25 years...
  31. Marin C, Sanchez M, Maroto E, Ossaba S, Ruiz Y, Zabala J. MR imaging of isolated left ventricular apical hypoplasia. Pediatr Radiol. 2007;37:703-5 pubmed
    ..To our knowledge, isolated left ventricular apical hypoplasia has not been reported in a child. ..
  32. Dasgupta C, Martinez A, Zuppan C, Shah M, Bailey L, Fletcher W. Identification of connexin43 (alpha1) gap junction gene mutations in patients with hypoplastic left heart syndrome by denaturing gradient gel electrophoresis (DGGE). Mutat Res. 2001;479:173-86 pubmed
    ..In the latter group, 14 children had hypoplastic left heart syndrome (HLHS) in which connexin43 gene defects were detected in eight...
  33. Tabbutt S, Dominguez T, Ravishankar C, Marino B, Gruber P, Wernovsky G, et al. Outcomes after the stage I reconstruction comparing the right ventricular to pulmonary artery conduit with the modified Blalock Taussig shunt. Ann Thorac Surg. 2005;80:1582-90; discussion 1590-1 pubmed
    ..In all, 149 infants underwent a stage I reconstruction (95 mBTS, 54 RV-PA) for hypoplastic left heart syndrome (HLHS) or variants...
  34. Brown S, Boshoff D, Eyskens B, Gewillig M. Hybrid approach as bridge to biventricular repair in a neonate with critical aortic stenosis and borderline left ventricle. Eur J Cardiothorac Surg. 2009;35:1080-2 pubmed publisher
    ..The hybrid approach allowed adequate time (months) for careful consideration and acted as a bridge to biventricular repair in this infant. ..
  35. McBride K, Zender G, Fitzgerald Butt S, Koehler D, Menesses Diaz A, Fernbach S, et al. Linkage analysis of left ventricular outflow tract malformations (aortic valve stenosis, coarctation of the aorta, and hypoplastic left heart syndrome). Eur J Hum Genet. 2009;17:811-9 pubmed publisher
    ..outflow tract (LVOT) malformations aortic valve stenosis (AVS), coarctation of the aorta (CoA), and hypoplastic left heart syndrome (HLHS) are significant causes of infant mortality...
  36. Tchervenkov C, Jacobs M, Tahta S. Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome. Ann Thorac Surg. 2000;69:S170-9 pubmed
    b>Hypoplastic left heart syndrome (HLHS) encompasses a spectrum of structural cardiac malformations that are characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity ..
  37. Friedberg M, Silverman N. The systolic to diastolic duration ratio in children with hypoplastic left heart syndrome: a novel Doppler index of right ventricular function. J Am Soc Echocardiogr. 2007;20:749-55 pubmed
    Right ventricular (RV) function is an important determinant of clinical status in children with hypoplastic left heart syndrome (HLHS). However, assessment of RV function remains challenging because of its complex morphology...
  38. Salih C, Sheppard M, Ho S. Morphometry of coronary capillaries in hypoplastic left heart syndrome. Ann Thorac Surg. 2004;77:903-7; discussion 907 pubmed
    b>Hypoplastic left heart syndrome is a condition characterized by a constellation of morphological malformations affecting the left side of the heart...
  39. Flett A, Elliott P, Moon J. Images in cardiovascular medicine. Cardiovascular magnetic resonance of isolated left ventricular apical hypoplasia. Circulation. 2008;117:e504-5 pubmed publisher
  40. Marshall A, Levine J, Morash D, Silva V, Lock J, Benson C, et al. Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome. Prenat Diagn. 2008;28:1023-8 pubmed publisher
    Neonates with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have a high rate of mortality...
  41. Maeda K, Yamaki S, Kado H, Asou T, Murakami A, Takamoto S. Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect. Circulation. 2004;110:II139-46 pubmed
    ..intact atrial septum [IAS]) has been reported to be a risk factor that negatively impacts survival in hypoplastic left heart syndrome (HLHS)...
  42. Kon A, Ackerson L, Lo B. Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome. Am J Cardiol. 2003;91:1506-9, A9 pubmed
  43. Caldarone C, Benson L, Holtby H, Van Arsdell G. Main pulmonary artery to innominate artery shunt during hybrid palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2005;130:e1-2 pubmed
  44. Zeltser I, Menteer J, Gaynor J, Spray T, Clark B, Kreutzer J, et al. Impact of re-coarctation following the Norwood operation on survival in the balloon angioplasty era. J Am Coll Cardiol. 2005;45:1844-8 pubmed
    ..Recurrent coarctation following BA occurred in 17% of patients, usually within the first year after BA. ..
  45. Fernández Valls M, Srichai M, Stillman A, White R. Isolated left ventricular apical hypoplasia: a new congenital anomaly described with cardiac tomography. Heart. 2004;90:552-5 pubmed
    ..Isolated LV apical hypoplasia is a unique, presumably congenital, cardiac anomaly that is an important condition to recognise. ..
  46. Guzzetta N. Phenoxybenzamine in the treatment of hypoplastic left heart syndrome: a core review. Anesth Analg. 2007;105:312-5 pubmed
    ..The following review provides an overview of the pharmacology of phenoxybenzamine, the surgical and physiologic implications of the Norwood procedure, and a discussion of the pros and cons of phenoxybenzamine administration...
  47. Pizarro C, Murdison K, Derby C, Radtke W. Stage II reconstruction after hybrid palliation for high-risk patients with a single ventricle. Ann Thorac Surg. 2008;85:1382-8 pubmed publisher
    ..our experience with stage II reconstruction after hybrid palliation in high-risk newborns with hypoplastic left heart syndrome and variants, focusing on the hemodynamic, reintervention, and operative data...
  48. Irving C, Chaudhari M. Fatal presentation of congenital isolated left ventricular apical hypoplasia. Eur J Cardiothorac Surg. 2009;35:368-9 pubmed publisher
    ..We describe a sudden and fatal presentation of this anomaly in a previously well 19-year-old male, confirming the fact that this is not a benign condition but a spectrum with the potential for significant complications. ..
  49. Muthurangu V, Taylor A, Hegde S, Johnson R, Tulloh R, Simpson J, et al. Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. Circulation. 2005;112:3256-63 pubmed
    ..Cardiovascular MR and echocardiography were performed in 37 infants with hypoplastic left heart syndrome before BCPC, and the findings were compared with surgical findings...
  50. Dealmeida A, McQuinn T, Sedmera D. Increased ventricular preload is compensated by myocyte proliferation in normal and hypoplastic fetal chick left ventricle. Circ Res. 2007;100:1363-70 pubmed
    ..the ability of fetal myocardium to respond to increased preload by myocyte hyperplasia and support the rationale for prenatal surgical interventions in certain cases of congenital heart disease such as hypoplastic left heart syndrome.
  51. Tweddell J, Hoffman G, Mussatto K, Fedderly R, Berger S, Jaquiss R, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation. 2002;106:I82-9 pubmed
    Outcome of stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS) has improved coincident with application of treatment strategies including continuous superior vena cava oximetry (SvO2), phenoxybenzamine (POB), strategies ..
  52. Galantowicz M, Cheatham J. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol. 2005;26:190-9 pubmed
    ..techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal...
  53. Haseyama K, Satomi G, Yasukochi S, Matsui H, Harada Y, Uchita S. Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2006;132:1232-3 pubmed