Acute respiratory distress syndrome (ARDS) is a severe respiratory condition that is characterized by rapidly progressive hypoxemia with noncardiogenic pulmonary edema. Despite the improvement of therapeutic methods, the mortality of ARDS is in the range of 40%-50% all over the world. Some studies have shown that a significant number of patients with ARDS had acute cor pulmonale (ACP), and ACP is independently associated with the mortality of patients with ARDS, which has attracted wide attention in recent years. This paper reviewed recent related studies, summarized the prevalence, pathogenesis and diagnostic approaches of ACP in ARDS, especially echocardiography which was considered as a cornerstone for ACP diagnosis, and elucidated the beneficial effects of right ventricular protective ventilatory strategy and prone-positioning on the pulmonary vasculature and right heart, in order to provide a novel idea for the therapy of ACP in ARDS.