Aims Duchenne muscular dystroph y (DMD) is characterized by respiratory and heart involvements. In the context ofpermanently wheelchair bound and on mechanical ventilation (MV) patients, the clinical presentation of acute heart failure(AHF) syndrome may be atypical. We sought to describe clinical and genetic profiles and to determine prognosis of DMDand Becker muscular dystrophy (BMD) patients on home MV (HMV), hospitalized for AHF.Methods and results We included genetically proven DMD and BMD patients on HMV admitted for AHF. A total of 13 patients(11 DMD and 2 BMD) fulfilled the inclusion criteria. Median age was 34.0 [interquartile range (IQR) 26.0; 40.0] years. Medianpulmonary vital capacity was 9.0% (6.0; 15.0) of predicted value. Long-term invasive ventilation was performed in 69% ofpatients. All the 11 DMD patients carried out-of-frame DMD gen e mutations. At admission, dyspnoea was present in 46%,lipothymia in 23%, and abdominal discomfort in 38.4% of patients. A total of 53.8% of patients showed anasarca. Cardiogenicshock presentation was found in six patients (46%). Ejection fraction was severely altered [median 25% (IQR 20; 30)]. Intra-hospital mortality rate was 30%, reaching 53.8 % after 1 year. Previous episodes of AHF ≥ 2 were associated with intra-hospitalmortality (P = 0.025). In patients with cardiogenic shock, intra-hospital mortality rate was 66.6%, reaching 83.3% after 1 year.Conclusions In adult DMD and BMD patients with severe ejection fraction alteration and on HMV, admitted for AHF, rightcardiac signs are frequent. The intra-hospital and 1 year mortality rate was high and was associated with previous episodes ofAHF ≥ 2.