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Mesenteric ischemia in hypoplastic left heart syndrome
A Hebra1, M F Brown, R B Hirschl
1Department of Surgery, Children's Hospital of Philadelphia, PA 19104.
Journal of Pediatric Surgery
|April 1, 1993
Summary
Mesenteric ischemia, often misdiagnosed as NEC in infants with hypoplastic left heart syndrome (HLHS), has a 97% mortality rate. Early recognition and management are critical for these complex congenital heart disease patients.
Area of Science:
- Pediatric Cardiology
- Gastrointestinal Surgery
- Neonatology
Background:
- Hypoplastic left heart syndrome (HLHS) is often considered uniformly fatal.
- Mesenteric ischemia is a serious complication in infants, frequently misdiagnosed as necrotizing enterocolitis (NEC).
Purpose of the Study:
- To investigate the incidence, clinical presentation, and outcomes of mesenteric ischemia in patients with HLHS.
- To differentiate mesenteric ischemia from NEC in HLHS patients and assess its impact on mortality.
Main Methods:
- Retrospective review of 387 HLHS patients treated between January 1984 and December 1990.
- Analysis of clinical data, pathology reports, and surgical findings for patients diagnosed with mesenteric ischemia.
- Comparison with existing literature on NEC in congenital heart disease.
Main Results:
- Mesenteric ischemia occurred in 8% (31/387) of HLHS patients, with onset at a mean age of 17.5 weeks.
- 80% of patients had documented low perfusion and hypotension prior to diagnosis.
- Mortality for HLHS patients with mesenteric ischemia was 97%, regardless of management.
Conclusions:
- Mesenteric ischemia in HLHS patients is a distinct clinical entity with extremely high mortality.
- It is often misdiagnosed as NEC, highlighting the need for improved diagnostic criteria and management strategies.
- Aggressive medical and surgical interventions had limited impact on survival in this cohort.