Skip to main content

Advertisement

SpringerLink
Log in

Quadrivalvar Replacement in Infantile Marfan Syndrome

  • CASE REPORT
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Marfan syndrome (MS) is a connective tissue disease involving the cardiovascular, ocular, and the musculoskeletal systems. MS has variable phenotypic expression and is most often diagnosed in adult life. Infantile-onset MS is rare and is associated with severe cardiovascular manifestations; there is an extremely high mortality during the first 2 years of life. We present a case of a child with severe infantile MS who, during the course of infancy and early childhood, developed aortic root dilatation and polyvalvar insufficiency requiring subsequent successful replacement of the aortic root and of all cardiac valves. To our knowledge, this is the first reported case of quadrivalvar replacement in the pediatric age group.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Booms P, Cisler J, Mathews KR, et al. (1999) Novel exon skipping mutation in the fibrillin I gene: two “hotspots” for the neonatal Marfans syndrome. Clin Genet 55:110–117

    Article  CAS  PubMed  Google Scholar 

  2. Caldarone CA, Raghuveer G, Hills CB, et al. (2001) Long-term survival after mitral valve replacement in children aged <5 years: A multi-institutional study. Circulation 104(Suppl I):I143–I147

    CAS  PubMed  Google Scholar 

  3. Cataneo SM, Bethea BT, Alejo DE, et al. (2004) Surgery for aortic root aneurysms in children: a 21-year experience in 50 patients. Ann Thorac Surg 77:168–176

    Article  Google Scholar 

  4. Dunn JM (1981) Porcine valve durability in children. Ann Thorac Surg 32:357–368

    Article  CAS  PubMed  Google Scholar 

  5. Geva T, Sanders SP, Diogenes MS, Rockenmacher S, van Praagh R (1990) Two-dimensional and Doppler echocardiographic and pathologic characteristics of the infantile Marfan syndrome. Am J Cardiol 65:1230–1237

    Article  CAS  PubMed  Google Scholar 

  6. Gott VL, Cameron D, Alejo DE, et al. (1999) Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med 340:1307–1313

    Article  CAS  PubMed  Google Scholar 

  7. Gray JR, Bridges AB, Faed MJW, et al. (1994) Ascertainment and severity of Marfan syndrome in a Scottish population. J Med Genet 31:51–54

    Article  CAS  PubMed  Google Scholar 

  8. Heide HT, Schrander-Stumpel C, Pals G, Delhaas T (2005) Neonatal Marfan syndrome: clinical report and review of the literature. Clin Dysmorph 14:81–84

    Article  PubMed  Google Scholar 

  9. Kamikubo Y, Murashita T, Yasuda K, Matano J, Sakai K (2000) Mitral valve replacement and subsequent composite graft replacement of the aortic root for infantile Marfan syndrome. Jpn J Thorac Surg 48:366–369

    CAS  Google Scholar 

  10. Kodolitsch Y, Raghunath M, Nienaber CA (1988) Das Marfan Syndrom: Prävalenz und natürlicher Verlauf der kardiovaskulären Manifestation. Z Kardiol 87:150–160

    Google Scholar 

  11. Kraseman T, Kotthoff S, Kehl HG, et al. (2005) Cardiac transplantation in neonatal Marfan syndrome—a life-saving approach. Thorac Cardiovasc Surg 53(Suppl 2):146–148

    Article  Google Scholar 

  12. Pasque M, Williams WG, Coles JG, et al. (1987) Tricuspid valve replacement in children. Ann Thorac Surg 44:164–168

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Pediatric Cardiology, Children’s Hospital of New York, Columbia University College of Physicians and Surgeons, 3959 Broadway BH-2N, New York, NY, 10032, USA

    S. Strigl & W. M. Gersony

  2. Division of Cardiothoracic Surgery, Children’s Hospital of New York, Columbia University College of Physicians and Surgeons, 3959 Broadway BH-2N, New York, NY, 10032, USA

    J. M. Quagebeur

Authors
  1. S. Strigl
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J. M. Quagebeur
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. W. M. Gersony
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to W. M. Gersony.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Strigl, S., Quagebeur, J. & Gersony, W. Quadrivalvar Replacement in Infantile Marfan Syndrome. Pediatr Cardiol 28, 403–405 (2007). https://doi.org/10.1007/s00246-006-0066-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-006-0066-4

Keywords

Search

Navigation