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Timing of delivery and aortic replacement in a pregnant patient with Marfan syndrome and rapidly advancing aortic dilation
Abstract Number: SA-78
Abstract Type: Case Report/Case Series
Introduction: Patients with Marfan syndrome are at risk of aortic dissection during pregnancy, and risk increases with degree of aortic dilation. Pregnancy may be contraindicated, pre-pregnancy aortic repair may be required, or more emergent repair may be needed for precipitous aortic enlargement or dissection. We present a patient with Marfan syndrome complicated by rapid aortic dilation, and discuss management dilemmas including timing of cesarean delivery and aortic replacement.
Case: A 25-year-old with Marfan syndrome and prior mitral valve replacement for infectious endocarditis presented to prenatal clinic at 11 weeks gestational age (GA). Echocardiogram, delayed until 26 weeks GA due to poor compliance, showed an aortic diameter of 44 mm consistent with dimensions obtained 3 years prior. Cardiac MRI at 34 weeks GA revealed rapid advancement of dilation to 50 mm, and the patient was admitted for cardiology and cardiac surgery consultation, antenatal steroids, and delivery planning. Cesarean delivery was performed under epidural anesthesia. Epidural blockade was incomplete despite large volumes of local anesthetic, and supplemental propofol infusion was needed for sedation. She was hemodynamically stable throughout and a healthy infant was delivered without complication. Due to concern for unacceptably high risk of intraabdominal bleeding from the heparin dose required for cardiopulmonary bypass (CPB), aortic replacement was scheduled for 6 weeks postpartum (PP). Due to surgical cancellations, the patient ultimately underwent aortic root replacement 3 months PP.
Discussion: Pregnancy may accelerate aortic root dilation of Marfan syndrome due to cellular changes in the elastic wall of the aorta as well as hemodynamic changes. Patients with a genetically mediated aortopathy such as Marfan’s should undergo replacement when aortic diameter is 50 mm or growth is >5 mm/year (class I recommendation); prophylactic replacement should be considered pre-conception when aortic diameter >40 mm (class IIa).1 Rapid aortic dilation became apparent at 34 weeks GA in our patient, and it was necessary to weigh the risk of dissection with continuation of pregnancy against the risks of prematurity. Cesarean delivery is recommended to avoid increases in aortic intraluminal pressure and shear force across the aorta, which are common during the second stage of vaginal delivery. Neuraxial anesthesia was desirable to help maintain even hemodynamics, though effective blockade was challenging, as is common in Marfan patients due to a high prevalence of scoliosis and dural ectasia.2 Following cesarean section, there are no guidelines for the safe timing of CPB-dose anticoagulation in a case that is neither emergent nor elective, as was the case in our patient. The risk of life-threatening post-operative hemorrhage must be weighed against the risk of aortic dissection.
1. Circulation 2010;121:266-369
2. Int J Obstet Anesth 2014;23:287-8