Pediatric Cardiology

, Volume 34, Issue 8, pp 1945–1948 | Cite as

Atherosclerosis Causing Recurrent Catastrophic Aortopulmonary Shunt Dehiscence in a Patient with Alagille Syndrome

  • L. May
  • F. L. Hanley
  • A. J. Connolly
  • S. Reddy
Case Report


Alagille syndrome (ALGS) is an autosomal dominant disorder associated with cholestatic liver disease, pulmonary valvar stenosis or atresia, vasculopathy, and renal disease. Although the liver and cardiac manifestations contribute to overall morbidity and mortality during their life span, these patients also carry a burden of important but often underappreciated vascular abnormalities. This report describes a 3 year-old girl with Alagille syndrome, hepatic cholestasis, systemic hypertension, hypercholesterolemia, hypertriglyceridemia, and tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs). She presented for bilateral pulmonary artery plasty and central shunt upsizing. She then experienced three shunt dehiscence episodes, necessitating emergent intervention. Autopsy showed diffuse atherosclerosis and significant atherosclerotic plaque at the site of shunt dehiscence. This is the first reported case of ALGS with TOF/PA/MAPCAs and catastrophic shunt dehiscence due to significant generalized vasculopathy caused by dyslipidemia and atherosclerosis. Dyslipidemia, a known comorbidity in ALGS, is one of few modifiable risk factors that should be screened for and treated, particularly before cardiac surgery.


Alagille syndrome Atherosclerosis Dyslipidemia Vasculopathy 


  1. 1.
    Agarwal V, Ramnarine I, Corno AF, Pozzi M (2005) Recurrent coarctation in a patient with Alagille syndrome. Interact Cardiovasc Thorac Surg 4:514–516CrossRefPubMedGoogle Scholar
  2. 2.
    Crippin J, Lindor K, Jorgensen R, Kottke B, Harrison J, Murtaugh P (1992) Hypercholesterolemia and atherosclerosis in primary biliary cirrhosis: what is the risk? Hepatology 15:858–862CrossRefPubMedGoogle Scholar
  3. 3.
    Davit-Spraul A, Pourci M, Atger V et al (1996) Abnormal lipoprotein pattern in patients with Alagille syndrome depends on icterus severity. Gastroenterology 111:1023–1032CrossRefPubMedGoogle Scholar
  4. 4.
    Garcia M, Ramonet M, Ciocca M et al (2005) Alagille syndrome: cutaneous manifestations in 38 children. Pediatr Dermatol 22:11–14CrossRefPubMedGoogle Scholar
  5. 5.
    Kamath B, Spinner N, Emerick K et al (2004) Vascular anomalies in Alagille syndrome: a significant cause of morbidity and mortality. Circulation 109:1354–1358CrossRefPubMedGoogle Scholar
  6. 6.
    LaBrecque D, Mitros F, Nathan R (1982) Four generations of arteriohepatic dysplasia. Hepatology 2:467–474CrossRefPubMedGoogle Scholar
  7. 7.
    Levy E, Bendayan M, Thibault L, Lambert M, Paradis K (1995) Lipoprotein abnormalities in two children with minimal biliary excretion. J Pediatr Gastroenterol Nutr 20:432–439CrossRefPubMedGoogle Scholar
  8. 8.
    Niessen K, Karsan A (2007) Notch signaling in the developing cardiovascular system. Am J Physiol Cell Physiol 293:1–11CrossRefGoogle Scholar
  9. 9.
    Quek S, Tan L, Quek S, Yip W, Aw W, Quak S (2000) Abdominal coarctation and Alagille syndrome. Pediatrics 106:E9CrossRefPubMedGoogle Scholar
  10. 10.
    Shefler A, Chan M, Ostman-Smith I (1997) Middle aortic syndrome in a boy with arteriohepatic dysplasia (Alagille syndrome). Pediatr Cardiol 18:232–234CrossRefPubMedGoogle Scholar
  11. 11.
    Subramaniam P, Knisely A, Portmann B et al (2011) Diagnosis of Alagille syndrome: 25 years of experience at King’s College Hospital. J Pediatr Gastroenterol Nutr 52:84–89CrossRefPubMedGoogle Scholar
  12. 12.
    Woolfenden A, Albers G, Steinberg G, Hahn J, Johnston D, Farrell K (1999) Moyamoya syndrome in children with Alagille syndrome: additional evidence of a vasculopathy. Pediatrics 103:505–508CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • L. May
    • 1
  • F. L. Hanley
    • 2
  • A. J. Connolly
    • 3
  • S. Reddy
    • 1
  1. 1.Department of PediatricsStanford University School of MedicineStanfordUSA
  2. 2.Department of Cardiothoracic SurgeryStanford University School of MedicineStanfordUSA
  3. 3.Department of PathologyStanford University School of MedicineStanfordUSA

Personalised recommendations