Patients with congenital central hypoventilation syndrome (CCHS) (Ondine's curse syndrome) have impaired autonomic control of ventilation with intact voluntary control of respiration. Autonomic dysfunction and cardiac abnormalities are common in CCHS. Bradyarrhythmias are life-threatening and often require pacemaker insertion. We presented a case of a patient with CCHS suffering from long sinus pauses requiring cardiac pacemaker insertion. Patients with CCHS are at risk for pulmonary hypertension and cor pulmonale secondary to chronic hypoxia. Diaphragmatic pacing has been beneficial in some patients with CCHS. In this article, we review concomitant cardiac abnormalities and the occurrence of bradyarrhythmias in patients with CCHS.
- Cardiac abnormalities
- Congenital central hypoventilation syndrome
- Ondine's curse
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine