This report describes five cases of double outlet right ventricle (DORV) in four dogs (aged 3-18 months, two males and two females) and a domestic shorthair cat (aged 6 months, female) who presented with various clinical signs including tachypnea (n = 5), exercise intolerance (n = 5), mucous cyanosis (n = 3), delayed growth (n = 2), and/or lethargy (n = 2). The represented canine breeds were poodle, Yorkshire terrier, Samoyed, and Shetland sheepdog. For all animals, echocardiography revealed marked aortic dextroposition with both arterial trunks totally arising from the right ventricle, associated with a ventricular septal defect and various other congenital abnormalities, including subvalvular aortic stenosis (n = 2), minor aortic insufficiency (n = 5), subvalvular pulmonic stenosis with pulmonary trunk hypoplasia (n = 1), patent ductus arteriosus (n = 1), minor mitral and/or tricuspid dysplasia (n = 3). Subsequent cardiac remodeling was characterized by marked right ventricular hypertrophy for all patients, associated with right ventricular and right atrial dilation for most of them (4/5). Two dogs died soon after the initial DORV diagnosis (i.e. after 24 h and two months). A surgical correction attempted for another dog confirmed the presence of a DORV associated with patent ductus arteriosus, but the animal died during the procedure from sudden cardiac arrest. The fourth dog underwent a contrast-enhanced retrospective electrocardiogram-gated multidetector computed tomography angiography under general anesthesia, which confirmed the conotruncal malformation. Despite episodes of exercise intolerance, this dog is still alive, at the age of 53 months, as is the cat at the age of 21 months.