Pregnancy in women with heart disease leads to worse maternal perinatal outcomes. Therefore, women with significant heart disease benefit from a control by a multidisciplinary team, in centers with experiences that cover the preconception period, pregnancy and postpartum. The clinical case of a 26-year-old with a complex heart congenital disease due to mitral hypoplasia, malposition of large vessel, only ventricle, with Fontan-Kreutzer surgical correction, in her first pregnancy is presented. In 2018, the Guidelines of the European Society of Cardiology classified pregnancies in women with Fontan circulation as moderate to high risk of maternal morbidity and mortality (WHO class III or IV risk), highlighting that the risk increases if the Fontan is not normally functional. The patient was controlled by a multidisciplinary team at the High Obstetric Risk Polyclinic of the "Manuel Quintela" Clinic Hospital - Montevideo, Uruguay; with good maternal perinatal results.

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