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Hypoplastic left heart syndrome in the second trimester
1Department of Pathology, University of Cambridge, England, UK.
Summary
Hypoplastic left heart syndrome may arise from impaired left ventricular function, not initial developmental failure. This finding suggests an early-stage evolution of the condition with variable progression.
Area of Science:
- Pediatric Cardiology
- Fetal Echocardiography
- Congenital Heart Disease
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect with high mortality.
- Current understanding often points to primary embryogenesis failure.
Observation:
- A 19-week gestation fetus presented with normal cardiac dimensions but abnormal left ventricular (LV) myocardial echoes.
- Impaired LV shortening and absent forward flow in the LV outflow tract were noted.
- Autopsy revealed LV subendocardial calcification.
Findings:
- The observed features suggest an early, evolving stage of HLHS.
- Abnormal LV myocardial performance and low output appear to impede LV growth.
- This challenges the notion of a primary embryogenesis defect.
Implications:
- This case provides insight into the variable temporal course of HLHS development.
- Understanding early functional deficits may inform prenatal diagnosis and management strategies.
- The findings suggest HLHS may result from progressive myocardial dysfunction rather than an initial malformation.