[Third degree atrioventricular block in a young woman with congenital central hypoventilation syndrome].

Congenital central hypoventilation syndrome (CCHS) is a rare multisystem disorder characterized by autonomic nervous system dysfunction, which often manifests as failure to maintain ventilatory homeostasis during sleep. We present a case with a third degree atrioventricular block in a young woman with CCHS, which to our knowledge has not been seen before. As sudden death is known to occur among patients with CCHS, and it is under consideration to offer regularly monitoring of patients throughout childhood and adolescence.