End-stage or advanced heart failure (HF) is defined by persistent and worsening symptoms, which intrude with patient’s quality of life, despite any attempts of maximal medical treatment. The prognosis of patients affected by end-stage HF is poor, with an overall survival of 70% at one year and 50% at two years. In patients with end-stage HF, three alternative treatments are available: heart transplantation is the gold standard, while left ventricle-assisted device and total artificial heart are long-term mechanical circulation support considered to bridge. The cornerstone of the treatment of patients affected by end-stage HF with clinical symptoms of respiratory distress is noninvasive positive ventilation (NIV); when this option fails, endotracheal intubation should be considered. Medical therapy (diuretics, intravenous inotropes and vasodilator drugs) may have a synergic effect to improve the respiratory function. NIV ventilation modalities include continuous positive airway pressure (CPAP) or bi-level positive airway pressure (Bi-PAP), every treatment needs to be individualized, with a strict monitoring of the patient’s compliance. Furthermore, noninvasive pressure support ventilation (NIPSV) constitutes a real help on inspiration, alternating with high-flow nasal cannula (HFNC). Respiratory management of patients with end stage HF is a multimodal approach, as bridge to heart transplantation if feasible, including noninvasive ventilation strategies, medical therapies and invasive maneuvers when NIV fails.