Uhl’s anomaly, a rare disease. Case report
DOI:
https://doi.org/10.35366/109509Keywords:
Uhl’s anomaly, congenital heart disease, right ventricular dysfunction, dilated heart diseaseAbstract
Uhl’s anomaly is a rare heart disease characterized by a dilated and thin-walled right ventricle due to
the absence of myocardium. The hypothesis is an alteration in embryonic development and apoptosis
of the myocardium in the right ventricle. It presents with heart failure. The definitive diagnosis is
determined by pathological anatomy; however, the echocardiographic and magnetic resonance
findings allow us to approach the diagnosis. There is no standard treatment, so it is initially medical
and symptomatic. Surgical options range from palliative surgery to heart transplant, but the prognosis
is uncertain. We present the case of an infant with dyspnea and diaphoresis.
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Copyright (c) 2023 Instituto Nacional de Rehabilitación Luis Guillermo Ibarra Ibarra
This work is licensed under a Creative Commons Attribution 4.0 International License.
© Instituto Nacional de Rehabilitación Luis Guillermo Ibarra Ibarra under a Creative Commons Attribution 4.0 International (CC BY 4.0) license which allows to reproduce and modify the content if appropiate recognition to the original source is given.
