Home mechanical ventilation (HMV) is increasingly used as a therapeutic option to patients with symptomatic chronic hypoventilation. There is, however, a paucity of solid data on factors that could affect prognosis in patients on home ventilation. In the present study, our aim was to study several factors in these patients with potential influence on survival. We examined 1526 adult patients from a nationwide HMV register to which data had been reported prospectively for 10 years. The patients constituted a broad diagnostic spectrum and the primary outcome in this study was death. We found by far the poorest survival rate in the ALS patients with only 5% alive after 5 years. Among the other patient groups the survival pattern was more uniform and the scoliosis, polio and Pickwick patients presented the best survival rate, after 5 years being around 75%. No factors were associated with a greater hazard for death in the ALS patients; in the non-ALS patients, however, negative predictors for survival were age, concomitant use of oxygen therapy, tracheostomy ventilation and start of ventilatory support in an acute clinical setting. Center size or county specific home ventilation treatment prevalence did not affect survival. In conclusion, in a large material of patients on HMV we found by far the poorest survival in the ALS patients. In the non-ALS patients a number of patient-related factors affected survival, while the size of the treating center or the regional treatment prevalence did not.