호흡부전을 야기한 주산기 심근병증을 동반한 근긴장성 이영양증

Myotonic dystrophy is an autosomal-dominant inherited neuromuscular disorder that`s characterized by slowly progressive muscular dystrophy, muscle weakness and myotonia. The clinical features may vary from just cataracts to involvement of multiple organ systems such as various muscles, the heart, lung and intestine. Its most common complication is postoperative respiratory failure. We encountered a patient who developed sudden unexpected peripartum cardiomyopathy (PPCM) and respiratory failure due to presumed myotonic dystrophy after cesarean section. We report here on our clinical experience with this malady and we include a brief review of the medical literature on myotonic dystrophy. (Korean J Anesthesiol 2009;57:376∼80)