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Septal hypertrabeculation/noncompaction: cardiac and neurologic implications
Claudia Stöllberger1, Josef Finsterer
12nd Medical Dept, Krankenanstalt Rudolfstiftung, Austria. claudia.stoellberger@chello.at
Septal affection in left ventricular hypertrabeculation/noncompaction (LVHT/NC) is a rare congenital finding, predominantly in children and females, often leading to a poor prognosis. Its association with neuromuscular disorders remains unclear.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiovascular Imaging
Background:
- Left ventricular hypertrabeculation/noncompaction (LVHT/NC) is a congenital heart condition.
- It is frequently associated with neuromuscular and other systemic disorders.
- LVHT/NC typically affects the apex and walls, but rarely the interventricular septum.
Purpose of the Study:
- To review and summarize current knowledge on septal involvement in LVHT/NC.
- To elucidate the characteristics and implications of septal LVHT/NC.
Main Methods:
- Literature review focusing on studies reporting septal LVHT/NC.
- Analysis of patient demographics, clinical associations, and outcomes.
Main Results:
- Septal LVHT/NC is more prevalent in children and adolescents, particularly females.
- Patients with septal LVHT/NC exhibit a poor prognosis.
- The congenital origin is strongly suggested.
Conclusions:
- Septal LVHT/NC is a distinct entity with specific demographic features.
- The link between septal LVHT/NC and neuromuscular disorders is not well-established.
- Septal LVHT/NC is likely a primary cardiac abnormality, not a manifestation of neuromuscular disease.
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