Infants and children with surgically corrected or palliated congenital heart disease (CHD) are at risk for chronic respiratory failure, necessitating home mechanical ventilation (HMV) via tracheostomy. However, very little data exists on this population or their outcomes. We conducted a retrospective chart review of all children with CHD enrolled in the Childrens Hospital Los Angeles HMV program between 1994 and 2009. Data were collected on type of heart lesion, surgeries performed, number of failed extubations, timing of tracheostomy, mortality, length of time on HMV, weaning status, associated co-morbidities, and Risk Adjusted classification for Congenital Heart Surgery (RACHS-1) category. Thirty-five children were identified; six with single ventricle anatomy, who received palliative procedures. Twenty-three (66%) patients are alive; 8 (23%) living patients have been weaned off HMV. Twelve (34%) patients are deceased. The incidence of mortality for single ventricle patients was 50%, and only one of the surviving children has received final palliation and weaned off HMV. Eight of nine patients (89%) with a RACHS score > or =4 died, and none have been weaned off of HMV. The 5-year survival for all CHD HMV patients was 68%; 90% for patients with RACHS < or =3; and 12% for patients with score > or =4. Children with more complex lesions, as demonstrated by single ventricle physiology or greater RACHS scores, had higher mortality rates and less success weaning off HMV. This case series suggests that caregivers should give serious consideration to the type of heart defect as they advise families considering HMV in children with CHD.