6 ERS Practical Handbook Noninvasive Ventilation Introduction predominantly in neuromuscular conditions. Many of these children now survive to adolescence or adulthood, as shown in the section entitled “Chronic NIV in heredi- tary neuromuscular disorders”. Furthermore, characterisation of the genotypes and phenotypes of some congenital disorders (e.g. congenital myasthenia and congenital muscular dystrophies) has clarified the natural history of these conditions, thereby facilitating anticipatory care plans and enabling personalised ventilatory care. Present trends There is also growing interest in NIV in cardiology. There is no doubt that patients with heart disease and OSA benefit from treatment of the OSA. By contrast, Cheyne-Stokes respiration is a form of central sleep apnoea that has been rec- ognised for centuries in chronic heart failure. It was previously thought to be simply a marker of severe disease and an epiphenomenon, but recently, the link to the progression of disease has been explored and it has been found to be more prevalent in milder cases. This is important, as heart failure is common and the majority of those affected have mild cardiac impairment. Recent work shows that around half of patients with mild heart failure have sleep disordered breathing too. CPAP can be used to treat OSA but does not work in central sleep apnoea or Cheyne–Stokes respiration. The new ventilatory concept of ASV aims to smooth out the Cheyne–Stokes pattern and, in doing so, may reduce associated sym- pathetic stimulation and arousals from sleep (see the section entitled “Chronic NIV in heart failure patients: ASV, NIV and CPAP”). Several large international multicentre trials of ASV in heart failure patients with predominant central sleep apnoea are now in progress, with cardiac and all-cause mortality or unplanned admissions as major outcome measures. However early results suggest ASV may not improve outcome contrary to expectations, and may cause harm in severe heart failure patients with central sleep apnoea. Palliative care NIV is now being used in some situations to palliate symptoms without the aim of prolonging survival or substantially modifying arterial blood gas tensions. Here, goals such as reduction in dyspnoea and control of symptoms of nocturnal hypoventilation should be set pre-emptively so that if these are not met, NIV can be discontinued and palliative efforts directed elsewhere. Nava et al. (2013) have shown that in oncology patients with solid tumours complicated by ARF and an expected life expectancy of 6 months, NIV reduced dyspnoea more rapidly than oxygen therapy alone and patients required less morphine. The benefit was most marked in hypercapnic patients and within the first hour of therapy, sug- gesting that responses can be rapidly gauged. NIV combined with cough-assist devices can also be used to manage severely ill type 1 spinal muscular atrophy infants with the aim of discharging the patient to their home and managing breathlessness. However, these approaches have been used in units familiar with NIV, and wide translation to oncology units and other palliative care centres needs to be carefully considered and managed. The use of NIV to palliate symptoms is discussed further in the section entitled “NIV in palliative care and at the end of life”.