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Hemolysis following correction of double-outlet right ventricle
Insights
Severe intravascular hemolysis occurred in a child after double-outlet right ventricle repair. This hemolysis, likely caused by surgical patch turbulence, resolved as the patch endothelialized.
Area of Science:
- Cardiology
- Pediatric Surgery
- Hematology
Background:
- Double-outlet right ventricle (DORV) is a complex congenital heart defect.
- Surgical correction often involves prosthetic materials like Dacron patches.
- Intraventricular repair aims to redirect blood flow within the ventricles.
Observation:
- A 4.5-year-old child experienced rapid-onset severe intravascular hemolysis post-DORV repair.
- Clinical signs included jaundice (bilirubin 4.3 mg/dL), a significant hematocrit drop (47% to 25%), and elevated reticulocyte count (22%).
Findings:
- Hemolysis onset within 24 hours of surgery suggests an acute complication.
- The presumed cause was turbulent blood flow from a long, curved Dacron patch.
- Hemolysis gradually resolved over 6 weeks, coinciding with patch endothelialization.
Implications:
- This case highlights a potential complication of Dacron patches in pediatric cardiac surgery.
- Turbulence-induced hemolysis may be mitigated by patch design and endothelialization.
- Long-term outcomes suggest successful resolution and recovery are possible.
Abstract:
A 41/2-year-old child developed a severe degree of intravascular hemolysis within 24 hours after intraventricular correction of double-outlet right ventricle with a Dacron patch. The child developed jaundice, with a serum bilirubin of 4.3 mg. per cent. The hematocrit value fell from 47 to 25 per cent and the reticulocyte count rose to 22 per cent. The hemolysis diminished after 3 weeks and completely disappeared after 6 weeks. We presume that the hemolysis was due to turbulence caused by a long, curved patch and that its disappearance coincided with the endothelialization of the patch. The child is well 3 years after the operation.