Objective To discuss key points of prevention and treatment of perioperative pulmonary hypertensive crisis in patients with serious pulmonary arterial hypertension associated with ventricular septal defect. Methods Retrospectively analyzed the nursing experience of perioperative pulmonary hypertensive crisis on 31 patients with serious pulmonary arterial hypertension associated with ventricular septal defect from March to December during 2016.Among these patients,7 patients occurred pulmonary hypertensive crisis.The prevention contained avoiding oxygen lack,keeping pH alkaloid in the body, application of pulmonary vasodilator, deep sedation. Results A total of 30 cases survived the perioperative period, and were discharged from the hospital, one died. Conclusions The patients with serious pulmonary arterial hypertension had more risks during the perioperative period,the main cause of death was pulmonary hypertensive crisis during this time.So prevention of pulmonary hypertension crisis is the key point of postoperative nursing.