Introduction:

the single ventricle is a rare pathology within adult congenital heart disease. This is a group of severe abnormalities of the heart structure that follow developmental errors, resulting in a single ventricular chamber. In this population, pharmacological measures are ineffective in preventing progression to end-stage heart failure. However, surgical procedures help to extend survival and quality of life.

Objective:

to report the case of congenital heart disease with low prevalence.

Clinical case:

the case of a 28-year-old man with a history of cyanogenic congenital heart disease, univentricular heart type with undetermined morphology and double outflow tract is presented; the patient was diagnosed since his first year of life, which has only received medical management.

Conclusions:

unfortunately, being a rare congenital heart disease in adults, there are few references to medical management. If they are not submitted to surgical intervention at an early age, which offers a greater quality of life and survival, some patients die during infancy due to heart failure.