Introduction:

Hypoplastic left heart syndrome is a rare abnormality that occurs in the prenatal period. Its clinical manifestations are related to the presence of hypoxemia at the systemic level.

Objective:

To describe the clinical manifestations and diagnostic elements of hypoplastic left heart syndrome.

Clinical case:

A male newborn, at term, who presented clinical and echographic manifestations that permitted the diagnosis of hypoplastic left heart syndrome.

Conclusions:

Despite advances in science and technology, hypoplastic left heart syndrome presents a high mortality. Proper planning for pregnancy, accurate pregnancy monitoring, proper genetic counseling, and the use of surgical techniques to correct the resulting deformities seem to be the most effective tools to deal with this deadly condition.

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