Abstract: To summarize the perioperative nursing experience of a pregnancy patient with Ⅵ mucopolysaccharidoses. In view of the problems of emotional anxiety,worry about fetal outcome,short stature,abnormal spine development,large tongue,small thoracic size,intercostal diaphragm impaired movements,limited muscle strength and cardiac function grade Ⅱ in this patient,key points of nursing included preoperative psychological supportive care,comfort improvement guidance,activity safety management,heart failure dynamic protection,maternal and fetal multidisciplinary monitoring,postoperative fluid precision balance,close airway monitoring,complication prevention,maternal and infant intimacy building measures. After careful treatment and nursing,the patient was discharged on the sixth day after cesarean section. The newborn was discharged from the Neonatal Department on the 43rd day after birth. Both of the maternal and fetal outcome were well.

Key words: Mucopolysaccharidoses, Pregnancy, Heart Failure, Perioperative Nursing, Critical Care Nursing