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Shone's anomaly: report of one case
1Department of Pediatrics, National Cheng-Kung University Hospital, Tainan, Taiwan, R.O.C.
Zhonghua Minguo Xiao Er Ke Yi Xue Hui Za Zhi [Journal]. Zhonghua Minguo Xiao Er Ke Yi Xue Hui
|January 1, 1993
Summary
Shone's anomaly, a rare congenital heart defect, involves multiple left-sided obstructive lesions. This case highlights the severe presentation and challenges in managing complex congenital heart disease in infants.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Shone's anomaly is a complex congenital heart condition characterized by multiple left-sided obstructive lesions.
- Early diagnosis and intervention are critical for managing infants with Shone's anomaly.
Observation:
- A two-month-old infant presented with severe congestive heart failure and cyanosis from birth.
- Diagnostic imaging revealed a parachute mitral valve, aortic/subaortic stenosis, ventricular septal defect, aortic coarctation, and patent ductus arteriosus.
Findings:
- The patient underwent surgical angioplasty for aortic coarctation.
- Despite intervention, the infant unfortunately expired two days post-surgery.
- Autopsy confirmed a typical presentation of Shone's anomaly.
Implications:
- This case underscores the critical nature and poor prognosis of severe Shone's anomaly.
- It highlights the significant challenges in surgical management and the need for comprehensive treatment strategies.
- Further research into novel therapeutic approaches for complex congenital heart defects is warranted.