A Case Study of Acute Aortic Dissection, Which Occurred in a Mother and Daughter with Marfan Syndrome on the Same Day

(Mitsubishi-Kyoto Hospital, Kyoto, Japan)

Hiromasa Nakamura Hiroyuki Nakajima Atsushi Nagasawa
Atsushi Shimizu
Case 1 was a 48-year-old mother who was under observation for Marfan syndrome and thoracic aortic dilation. She was brought to the hospital with chest pain, and a CT scan revealed acute aortic dissection(Stanford A). Aortic incompetence was also observed, and an emergency Bentall procedure was performed. Case 2, her daughter, was a 26 years old and 39 weeks pregnant. She did not meet the diagnostic criteria for Marfan syndrome. She experienced severe back pain on the same day that her mother was admitted for aortic dissection. Because the patient did not agree to the use of a contrast agent due to concern about its effect on the fetus, emergency cesarean section was performed. Subsequently, a CT scan performed on the patient showed acute aortic dissection(Stanford B). Accordingly, antihypertensive therapy was commenced. In both cases, the patients were discharged after they recovered. Although case 2 did not meet criteria for Marfan syndrome, because of the hereditary disposition, we strongly suspect this was a Marfan syndrome pregnancy. This type of case is included in the case literature on cesarean and vigilant perinatal care is thought to be necessary.
  Jpn. J. Cardiovasc. Surg. 38:151-155(2009)