Key result
Uhl's anomaly presents with isolated RV failure and high mortality lacking effective therapy.
This case report demonstrates that a 10-year survival is possible in an unoperated patient with Uhl's anomaly, a rare condition typically characterized by high mortality.
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May allow 10-year survival without surgery in Uhl's anomaly; leaves optimal management and natural history open.
Faria et al. (2020) studied this question. Uhl's anomaly is an extremely rare disease characterized by isolated RV dilatation and failure, carrying a poor prognosis and high mortality due to the lack of an ideal treatment.
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