Successful management of aortic dissection in a patient with Marfan syndrome with total placenta previa

Postpartum hemorrhage presents a difficult situation for obstetricians, and several methods have been developed to reduce mortality and morbidity. We recently read the review of uterine compression sutures for postpartum hemorrhage in this journal, which suggested that compression sutures offer improved hemostasis and preserve future fertility 1. Placenta previa can cause postpartum hemorrhage, and we here report successful management of aortic dissection in a patient with Marfan syndrome with total placenta previa. A 27-year-old woman (gravida 2, para 1) at 33+6 weeks of gestation with acute back pain was referred to the emergency department. She appeared to have characteristics of Marfan syndrome, including above-average height and long, slender limbs with long fingers and toes. Regular uterine contraction and vaginal bleeding were noted. Ultrasonographic findings revealed total placenta previa. Contrast-enhanced chest computed tomography showed the intimal flap within the descending thoracic aorta, suggesting aortic dissection. Aortic dissection from the ascending aorta to upper aortic bifurcation, Stanford type A, extended to both common carotid arteries, with an intramural hematoma in the left common carotid artery. We performed an emergency cesarean delivery, and a 1.73-kg female infant was born. During the operation, we performed “Hysterectomy prevention using the uterine hollow obliterations” (HYUNHO) procedure for total placenta previa and placenta accreta 2. Packed red blood cells were transfused for the postpartum hemorrhage. She was intentionally sedated during intubation, and we maintained low blood pressure in the intensive care unit for 12 h, at which time cardiothoracic surgeons performed a total arch replacement. While 12 h is not the optimal time frame, we delayed the procedure to lower the bleeding tendency with heparin injections. We developed the HYUNHO procedure as a uterine compression method for placenta previa and anticipate further study of this procedure for postpartum hemorrhage. We believe this case provides guidance for other obstetricians in rare cases of Marfan syndrome with aortic dissection and total placenta previa. Postpartum hemorrhage is a delicate situation for any obstetrician, and in this case, a variety of uterine compression sutures were possible, even in a patient with Marfan syndrome being treated with continuous heparin before undergoing total arch replacement. It is unfortunate that the local clinic obstetrician did not diagnosis Marfan syndrome during antenatal care of this patient. Obstetricians should be aware of both medical and surgical management of aortic dissection and check the morphology as part of a routine examination. Cesarean section with cardiopulmonary bypass in Marfan syndrome patients increases the risk of bleeding; however, placing an intrauterine balloon compresses the uterus to minimize bleeding 3. Successful management of total placenta previa in Marfan syndrome has not been reported. We believe this case will provide guidance to other obstetricians performing uterine compression suturing.