We report a case of vagal hypertonia syndrome in a newborn infant, developed after surgical repair of an aortic coarctation combined with banding of the pulmonary artery trunk. The parasympathetic activity had adverse repercussions on haemodynamics. The diagnosis was confirmed by prolonged asystole on the oculocardiac reflex and by concomitant arrhythmia and disorders of conduction demonstrated by Holter recordings. To our knowledge, no other case of vagal hypertonia associated with a congenital cardiopathy has yet been reported. Infants with this syndrome are at a high risk of sudden death. Treatment with vagolytic drugs is of questionable value, and prolonged supervision of the patient is mandatory.

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