Topsy-Turvy Heart: Multimodality Prenatal and Postnatal Imaging for Clinical Management

Topsy-turvy heart is an extremely rare congenital heart disease characterized by an axial rotation of the heart owing to a strict supero-inferior orientation of the ventricles and to an elongation of the airways and neck vessels.

We report a case diagnosed prenatally with topsy-turvy heart and aortopulmonary window, with high suspicion of associated airway abnormalities, using combined fetal echocardiography and fetal cardiac magnetic resonance. Immediately after birth, the newborn developed severe respiratory failure requiring mechanical ventilation.

Multimodality imaging provided comprehensive assessment of the complex intracardiac anatomy and the severe associated vascular and airway anomalies, including severe bronchomalacia and parenchymal lung disease. Despite maximal intensive care support, the clinical condition of the newborn deteriorated rapidly. Considering the clinical course and all imaging findings, the interdisciplinary experts team, based on the available literature, estimated the prognosis as dismal and recommended redirection of care.

Multimodality imaging used pre- and postnatally in newborns with complex heart disease helps for timely clinical decision making.