Skip to main content
SpringerLink
Log in

Noonan syndrome with double-chambered right ventricle

  • Case Reports
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

The association of Noonan syndrome and a double-chambered right ventricle has not been reported previously in the medical literature. We report two patients with Noonan syndrome associated with pulmonary valve stenosis and double-chambered right ventricle due to anomalous hypertrophied muscle bundles. Pulmonary valve stenosis was operated on. However, hypertrophied muscle bundles were not resected, as they caused no obstruction to blood passage. Postoperative follow-up examinations revealed no symptoms. In conclusion, in those with Noonan syndrome there may be anomalous muscle bundles in the right ventricular cavity. It may not be necessary to resect them if they do not obstruct the right ventricular outflow tract.

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

Similar content being viewed by others

References

  1. Fellows KE, Martin EC, Rosenthal A (1977) Angiocardiography of obstructing muscular bands of the right ventricle.AJR Am J Roentgenol 128:249–256

    PubMed  CAS  Google Scholar 

  2. Goens MB, Campbell D, Wiggins JW (1992) Spontaneous chylothorax in Noonan syndrome.Am J Dis Child 146:1453–1456

    PubMed  CAS  Google Scholar 

  3. Hindle WV, Engle MA, Hagstrom JWC (1968) Anomalous right ventricular muscles: a clinicopathologic study.Am J Cardiol 21: 487–495

    Article  PubMed  Google Scholar 

  4. Mendez HMM, Opitz JM (1985) Noonan syndrome: a review.Am J Med Genet 21:493–506

    Article  PubMed  CAS  Google Scholar 

  5. Nora JJ, Nora AH, Sinha AK, Spangler RD, Lubs HA (1974) The Ullrich-Noonan syndrome (Turner phenotype).Am J Dis Child 127: 48–55

    PubMed  CAS  Google Scholar 

  6. Ranke MB, Heidemann P, Knupfer C, et al (1988) Noonan syndrome: growth and clinical manifestations in 144 cases.Eur J Pediatr 148:220–227

    Article  PubMed  CAS  Google Scholar 

  7. Rowland TW, Rosenthal A, Castenada AR (1975) Double chamber right ventricle: experience with 17 casesAm Heart J 89:455–462

    Article  PubMed  CAS  Google Scholar 

  8. Sharland M, Burch M, McKenna WM, Paton MA (1992) A clinical study of Noonan syndrome.Arch Dis Child 67:178–183

    Article  PubMed  CAS  Google Scholar 

  9. Tinker A, Uren N, Schofield J (1989) Severe pulmonary hypertension in Ullrich-Noonan syndrome.Br Heart J 62:74–77

    PubMed  CAS  Google Scholar 

  10. Von Doenhoff LJ, Nanda NC (1983) Obstruction within the right ventricular body: two-dimensional echocardiographic features.Am J Cardiol 51:1498–1501

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Cardiology, Hacettepe University, Faculty of Medicine, 06100, Ankara, Turkey

    S. Özkutlu, E. Çil & M. Saraçlar

  2. Department of Thoracic and Cardiovascular Surgery, Hacettepe University, Faculty of Medicine, 06100, Ankara, Turkey

    I. Paşaoğlu

Authors
  1. S. Özkutlu
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. E. Çil
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. I. Paşaoğlu
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. Saraçlar
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Özkutlu, S., Çil, E., Paşaoğlu, I. et al. Noonan syndrome with double-chambered right ventricle. Pediatr Cardiol 17, 251–253 (1996). https://doi.org/10.1007/BF02524803

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02524803

Key words

Search

Navigation