Functional univentricular heart is a rare and complex
congenital anomaly. The prognosis is usually poor without
any surgical intervention and long-term survival is rare.
In this article, we report a 57-year-old female case with a
double inlet left ventricle with valvular pulmonary stenosis,
left anterior rudimentary right ventricle, ventricular septal
defect, discordant ventriculoatrial connection, patent
foramen ovale and Glenn shunt who was followed for 20
years. Cardiac catheterization revealed a classic Glenn
shunt anatomy. The patient is currently under follow-up
with medical treatment