Congenital central hypoventilation syndrome (CCHS) patients may have heart rhythm disorders related to autonomic nervous system dysfunction such as, sinus bradycardia and transient asystole with potentially dramatic syncopal events. The diagnosis of these cardiac alterations may be challenging with conventional periodical Holter monitoring. The case shows how continuous heart rhythm monitoring provided by the implantable cardiac monitor (ICM) may play a crucial role for their early diagnosis and the choice of the appropriate therapeutic intervention.
Keywords: cardiac pauses; congenital central hypoventilation syndrome; diaphragm pacing system; implantable cardiac monitor; implantable cardiac pacemaker.
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