Corrective Surgery of Congenital Cardiac Anomalies in the Noonan syndrome: Report of two cases / 대한흉부외과학회지
The Korean Journal of Thoracic and Cardiovascular Surgery
;
: 552-555, 2001.
Article
in Korean
| WPRIM
| ID: wpr-30080
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Phenotype
/
Pulmonary Subvalvular Stenosis
/
Pulmonary Valve Stenosis
/
Congenital Abnormalities
/
Follow-Up Studies
/
Foramen Ovale, Patent
/
Karyotype
/
Hair
/
Hypertelorism
/
Intellectual Disability
Type of study:
Observational study
/
Prognostic study
Limits:
Adult
/
Humans
/
Male
Language:
Korean
Journal:
The Korean Journal of Thoracic and Cardiovascular Surgery
Year:
2001
Type:
Article
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