Corrective Surgery of Congenital Cardiac Anomalies in the Noonan syndrome: Report of two cases.
- Author:
Sun Hee LEE
1
;
Ju Hyun LEE
;
Sung Bo SIM
;
Jae Kil PARK
;
Moon Sub KWACK
;
Se Wha KIM
;
Yong Seog OH
;
Ho Joong YOUN
;
Wook Sung CHUNG
;
Soon Jo HONG
Author Information
1. Department of Thoracic and Cardiovascular Surgery. St. Mary' s Hospital, The Catholic University of Korea, Korea.
- Publication Type:Case Report
- Keywords:
Noonan syndrome;
Pulmonary artery, stenosis
- MeSH:
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
- From:The Korean Journal of Thoracic and Cardiovascular Surgery
2001;34(7):552-555
- CountryRepublic of Korea
- Language:Korean
-
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.
