The ECHS project aims to optimize health care to patients with CHS in Europe. Central hypoventilation syndromes (CHS) are extremely rare disorders of central autonomic respiratory control and global dysfunction of autonomous nervous system. CHS include a heterogeneous group of not fully characterized diseases. Severe chronic central hypoventilation is the hallmark and the most life-threatening feature. Treatment requires advanced techniques of ventilation support (positive pressure ventilation, chest shell ventilation, phrenic nerve pacing, diaphragm pacing) during lifetime. However, currently large disparities exist in access to diagnosis and treatment across Europe while long-term outcomes are unknown.
The European CHS community will benefit from better understanding of physiopathology of the extremely rare CHS.
• Implementation of a European CHS register (ECHS register), as a critical mass of patients is required for high-quality epidemiological and clinical studies. Patients with CHS will be identified in the 14 participating European countries. More partners from more countries are to be recruited in order to expand the network towards Eastern Europe and Northern Europe. Data will be collected through a secured web-based register.
• European standards and guidelines for diagnosis and treatment of CHS will be determined.
• The state of existing services for CHS in Europe will be provided for.
• A multi-lingual website will facilitate information dissemination and communication between patients and health professionals.
The ECHS Network project has a potentially high impact in Europe. Patients with CHS will benefit from the awareness of services provided in Europe and high-quality care based on European standards and guidelines. European health professionals will benefit from the dissemination of up-to-date information and agreed guidelines for management of CHS. European home ventilation services and ventilator industries will benefit from the feedback on the performance of their services or products. Communication between all stakeholders will be enhanced. The project will help mobilizing a critical mass of resources and expertise devoted to CHS. The ECHS Network project has potentially high multiplier effects beyond Europe and beyond CHS. Indeed, international communities of CHS and of rare diseases in general would benefit from the project methods and results.