Korean J Thorac Cardiovasc Surg.  2001 Jul;34(7):552-555.

Corrective Surgery of Congenital Cardiac Anomalies in the Noonan syndrome: Report of two cases

• Lee SH
• Lee JH
• Sim SB
• Park JK
• Kwack MS
• Kim SW
• Oh YS
• Youn HJ
• Chung WS
• Hong SJ

Abstract

Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovale. The second 28-year-old male patient had valvular and subvalvular pulmonary stenosis with typical Noonan's face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovale was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

Keyword

Noonan syndrome; Pulmonary artery, stenosis

MeSH Terms

Adult
Congenital Abnormalities
Follow-Up Studies
Foramen Ovale, Patent
Hair
Humans
Hypertelorism
Intellectual Disability
Karyotype
Male
Neck
Noonan Syndrome*
Phenotype
Pulmonary Subvalvular Stenosis
Pulmonary Valve Stenosis