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Ivemark syndrome. A case with successful surgical intervention
T Tkebuchava1, L K von Segesser, M Lachat
1Department of Cardiovascular Surgery, University Hospital, Zürich, Switzerland.
Scandinavian Cardiovascular Journal : SCJ
|January 1, 1997
Insights
Early palliative surgery, including Glenn and hemi-Fontan procedures, offers a viable treatment for Ivemark syndrome in infants with a single ventricle, promoting survival and well-being.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
Background:
- Ivemark syndrome, characterized by splenic agenesis, complex cardiac malformations, and visceral abnormalities, presents significant challenges in pediatric care.
- Single ventricle physiology requires specialized surgical palliation to improve hemodynamics and long-term outcomes.
Observation:
- A 19-day-old infant diagnosed with Ivemark syndrome underwent staged palliative surgical interventions.
- The surgical procedures included the Glenn procedure and a hemi-Fontan operation.
Findings:
- The infant survived the palliative surgeries and demonstrated positive clinical progress.
- At five months post-operation, the child remains alive and in good health, indicating successful palliation.
Implications:
- Early surgical intervention in Ivemark syndrome with single ventricle physiology can be a beneficial and life-sustaining approach.
- This case supports the consideration of early palliative surgery as a worthwhile strategy for improving outcomes in complex congenital heart defects.
Abstract:
A 19-day-old boy with Ivemark syndrome (splenic agenesis associated with complex cardiac malformations and visceral abnormality) underwent palliative surgery including Glenn and hemi-Fontan procedures. Five months later the child is alive and well. We believe that early palliative surgery is worthwhile in Ivemark syndrome with a single ventricle.