Korean J Thorac Cardiovasc Surg.  2000 May;33(5):419-421.

Noonan Syndrome with Double-Chambered Right Ventricle and Atrial Septal Defect: 1 Case Report

• Park YW
• Lee SY
• Jeong YS
• Yum W

Abstract

Noonan syndrome is characterized by typical facies, congenital heart defect, and some clinical features similar to Turner syndrome, but with normal chromosomes. The most commonly associated cardiac defects are pulmonary valvular stenosis and strial septal defect. We experienced a case of Nonan syndrome associated with pulmonay valve stenosis with double-chambered right ventricle and atrial septal defect and cryptorchidism. Pulmonary valvotomy was done through transannular incision. Hypertrophied muscle bundles were excised. Atrial septal defect was closed directly. RVOT was reconstructed with pericardial transannular patch. Orchiopexy was performed simultaneously without any problem.

Keyword

Noonan syndrome; Double chambered right ventricle

MeSH Terms

Constriction, Pathologic
Cryptorchidism
Facies
Heart Defects, Congenital
Heart Septal Defects, Atrial*
Heart Ventricles*
Male
Noonan Syndrome*
Orchiopexy
Turner Syndrome