Hypoplastic Left Heart Syndrome (HLHS)

  • In Sook Park
  • Soo-Jin Kim
  • Hyun Woo Goo


This chapter includes types, morphology, clinical manifestations, diagnosis, natural and unnatural course, treatment, and fetal diagnosis of hypoplastic left heart syndrome, including hybrid procedure, Fontan operation, and heart transplantation.


Aortic atresia Mitral atresia PAGOD syndrome Norwood operation Sano operation Heart transplantation Hybrid palliation Fontan operation 

Suggested Reading

  1. 1.
    Bernstein D, Naftel D, Chin C, et al. Pediatric heart transplant study. Outcome of listing for cardiac transplantation for failed Fontan: a multi-institutional study. Circulation. 2006;114:273–80.CrossRefGoogle Scholar
  2. 2.
    Forbess JM, Cook N, Serraf A, et al. An institutional experience with second- and third-stage palliative procedures for hypoplastic left heart syndrome: the impact of the bidirectional cavopulmonary shunt. J Am Coll Cardiol. 1997;29:665–70.CrossRefGoogle Scholar
  3. 3.
    Ghanayem NS, Jaquiss RD, Cava JR, et al. Right ventricle-to-pulmonary artery conduit versus Blalock-Taussig shunt: a hemodynamic comparison. Ann Thorac Surg. 2006;82:1603–10.CrossRefGoogle Scholar
  4. 4.
    Kim JB, Park JJ, Ko JK, Goo HW, Kim YH, Park IS, Yun TJ, Seo DM. A case of PAGOD syndrome with hypoplastic left heart syndrome. Int J Cardiol. 2007;114:270–1.CrossRefGoogle Scholar
  5. 5.
    Lamour JM, Kanter KR, Naftel DC, et al. Cardiac Transplant Registry Database; Pediatric Heart Transplant Study. The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease. J Am Coll Cardiol. 2009;54:160–5.CrossRefGoogle Scholar
  6. 6.
    Luce WA, Schwartz RM, Beauseau W, et al. Necrotizing enterocolitis in neonates undergoing the hybrid approach to complex congenital heart disease. Pediatr Crit Care Med. 2011;12:46–51.CrossRefGoogle Scholar
  7. 7.
    Mitchell MB, Campbell DN, Ivy D, et al. Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure. Thorac Cardiovasc Surg. 2004;128:693–702.CrossRefGoogle Scholar
  8. 8.
    Mosca RS, Kulik TJ, Goldberg CS, et al. Early results of the Fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2000;119:1110–8.CrossRefGoogle Scholar
  9. 9.
    Reemtsen BL, Pike NA, Starnes VA. Stage I palliation for hypoplastic left heart syndrome: Norwood versus Sano modification. Curr Opin Cardiol. 2007;22:60–5.CrossRefGoogle Scholar
  10. 10.
    Sano S, Ishino K, Kawada M, et al. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:504–10.CrossRefGoogle Scholar
  11. 11.
    Yun TJ, Cho WC, Jung SH, Seo DM, Goo HW, Kim YH. Reverse Blalock-Taussig shunt facilitates the growth of the ascending aorta after hybrid palliation. Ann Thorac Surg. 2007;83:1886–8.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • In Sook Park
    • 1
  • Soo-Jin Kim
    • 2
  • Hyun Woo Goo
    • 3
  1. 1.Department of Pediatric CardiologyUniversity of Ulsan College of Medicine, Asan Medical CenterSeoulSouth Korea
  2. 2.Department of PediatricsKonkuk University Medical CenterSeoulSouth Korea
  3. 3.Department of RadiologyUniversity of Ulsan College of Medicine, Asan Medical CenterSeoulSouth Korea

Personalised recommendations