126. Cardio-Obstetrics: Pregnancy and Aortic Disorders with Dr. Nupoor Narula

CardioNerd Amit Goyal, Cardio-OB series co-chair and University of Texas Southwestern Cardiology Fellow, Dr. Sonia Shah, and episode lead and Johns Hopkins University Cardiology Fellow, Dr. Anum Minhas, discuss pregnancy and aortic disorders with Dr. Nupoor Narula of Weill Cornell Medical College. Special introduction by Sukrit Narula. In this episode we discuss the presentation and management of aortopathies during pregnancy. We begin by examining the pathophysiology of aortic disease during pregnancy, followed by a review of the heritable aortopathies and their risk of dissection. We then discuss preconception evaluation and antepartum care of women with aortopathies. We end with addressing management at the time of labor and delivery. Claim free CME for enjoying this episode! Abstract • Pearls • Quotables • Notes • References • Guest Profiles • Production Team CardioNerds Cardio-Obstetrics Series PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Episode Abstract - Pregnancy and Aortic Disorders In this episode we discuss the presentation and management of aortopathies during pregnancy. We begin by examining the pathophysiology of aortic disease during pregnancy, followed by a review of the heritable aortopathies and their risk of dissection. We then discuss preconception evaluation and antepartum care of women with aortopathies. We end with addressing management at the time of labor and delivery. Pearls - Pregnancy and Aortic Disorders 1. Assessment of aortic root and ascending aortic measurements should be performed prior to conception in women with known aortopathies, connective tissue diseases with high risk for aortopathies, bicuspid aortic valve or familial thoracic aortic syndromes. Dimensions should always be verified with multi-modality imaging prior to decision-making. 2. It is important to recognize that the immediate postpartum period is a high risk period for aortic dissection in women with aortopathies. 3. Goal systolic blood pressure is 4.0 cm.The 2018 ESC guidelines advise against pregnancy for aortic root diameter > 4.5 cm.The decision to intervene prophylactically during pregnancy is highly individualized and complex, and we must weight patient factors including gestational age and rapidity of growth while balancing risk/benefits to the fetus and mother. Ehlers-Danlos syndrome (EDS)Vascular EDS patients are at high risk of arterial and uterine rupture, and the 2018 ESC Guidelines recommend against pregnancy. If after shared-decision making a patient decides to proceed with pregnancy, the patient should be cared for in a specialized center with a multi-disciplinary Cardio-Ob team.Loeys-Dietz syndromeThere is limited data is available, and it is insufficient to make any clear recommendations regarding pregnancy. Most centers will generally approach prophylactic aortic root surgery in Loeys-Dietz syndrome similar to Marfan disease and consider elective repair at 4.0 to 4.5 cm.More frequent intrapartum echocardiograms may be considered given some suggestion of increased risk of dilatation during pregnancy.Turner SyndromeThere is a strong association with bicuspid aortic valve (present in 15-30% of patients). Generally, we consider elective surgery in patients with an aortic size index (ASI) (ascending aortic root size indexed to body surface area) of > 2.5 cm/m2 and that pregnancy should be avoided at this ASI cutoff. During pregnancy, prophylactic aortic surgery can be considered with a dilated aorta (> 2.5 cm/m2 or rapid enlargement > 3mm)Many women with Turner syndrome require assisted reproductive therapy (ART) and assisted reproductive therapy may increase the risk of cardiovascular complications.Imaging of the thoracic aorta and heart are recommended within two years prior to pregnancy or assisted reproductive therapy.Imaging should be performed at least once per 20 weeks if there is no increased ASI or other cardiovascular risk factors (e.g., family history of dissection or sudden death, bicuspid aortic valve, coarctation, elongation of the transverse arch).Imaging should be performed every 4-8 weeks for those with ASI > 2.0 cm/m2 or additional risk factors during pregnancy, and once during the first month after delivery. Subsequent imaging intervals depend on the severity of the aortic enlargement.Bicuspid aortic valve (BAV)Up to 50% of patients with BAV also have ascending aortic dilatationESC guidelines recommend elective aortic surgery if the ascending aorta is > 5.0 cm prior to pregnancy.Familial Thoracic aortic aneurysmsFamily history is present in 20% of patients.A series in 53 women with ACTA2 mutations and total of 137 pregnancies showed 8 pregnancy-associated dissections (6 were type A) .Evidence is limited, however, and no clear recommendations for pregnancy are available. What are some delivery considerations for women with aortopathies?The overall goal is to decrease the cardiovascular stress of the delivery process, and risk for both the mother and baby need to be considered jointly with the Maternal Fetal Medicine team.For Marfan Syndrome and aortic root between 4-4.5 cm,