Complex Management of Unsuspected Aortic Dissection at Term Pregnancy in Undiagnosed Marfan’s Syndrome

P0087 

Case Video Submission 

Sara Hazaveh (1), Vladimir Joseph (2), Elie Elmann (2), Marian Vandyck-Acquah (2)

(1) Hackensack Meridian University Medical Center, Hackensack, NJ, (2) Hackensack Meridian University Medical Center, Hackensack , NJ

Sara Hazaveh    -  Contact Me
Hackensack Meridian University Medical Center

Vladimir Joseph    -  Contact Me
Hackensack Meridian University Medical Center

Elie Elmann    -  Contact Me
Hackensack Meridian University Medical Center

Marian Vandyck-Acquah    -  Contact Me
Hackensack Meridian University Medical Center

Sara Hazaveh    -  Contact Me
N/A

Background: Aortic dissection (AD) is a potential complication of pregnancy in Marfan Syndrome (MFS) which is life-threatening for both the pregnant woman and the baby, survival rate is reported to be 20-30%. Aortic root dilation in MFS and peripartum hormonal and hemodynamic changes of pregnancy, which peak at the third trimester, are risk factors for AD. We present a case of a patient with MFS that highlights the need for more research into MFS and pregnancy and improvement aortic root screening guidelines to reduce risk of AD.
Case: A 42-year-old female with history of bilateral lens dislocation, aortic root dilation (4cm), and family history of MFS but normal body habitus presented at 37-weeks gestation after in-vitro-fertilization (IVF) with hours of constant mid back pain. Blood pressure was 160/80, decelerations noted on fetal monitoring prompted an emergent cesarean section for suspected abruption causing the pain. Patient with continued back pain after delivery, a post-op echocardiogram revealed type-A dissection with aortic regurgitation.
Decision‐making: CT angio confirmed AD from the right coronary artery ostium, involving the arch, and extending down the descending aorta to the proximal left common iliac artery. After stabilization with IV labetalol, a multidisciplinary team proceeded with urgent median sternotomy with modified Bentall procedure with mechanical valve, single coronary artery bypass graft and descending thoracic aortic stent. She was discharged on warfarin and metoprolol.
Conclusion: A careful history is crucial to make a diagnosis of MFS as women may not have typical skeletal features. It is crucial to have a high index of suspicion at presentation when MFS characteristics are present and to have pre-pregnancy counseling, use a multidisciplinary team, and have early prophylactic intervention when needed to avoid catastrophe. We suggest research to determine the role of fertility drugs and age in AD risk, and the consideration of the use of aortic root Z-scores and of moving the cut off for pre pregnancy prophylactic aortic replacement, which is currently at 4.5cm, to 4.0 cm in MFS.

Dissection