48. Case Report: Critical Bicuspid Aortic Valve Stenosis Complicating Pregnancy – Vanderbilt University

CardioNerds (Amit & Dan) join Vanderbilt University cardiology fellows (Tara Holder, Majd El-Harasis, and Amar Parikh) for a Sunday morning brunch, Nashville style! They discuss an enthralling case of bicuspid aortic valve with critical aortic stenosis complicating pregnancy. Program director Dr. Julie Damp provides the E-CPR and a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai. Jump to: Patient summary - Case figures & media - Case teaching - Educational video - References - Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A 22yo transgender F2M man (G1P0000) at 32w5d was found to have a late-peaking systolic ejection murmur on a regularly scheduled OB visit. He reported recent left-sided exertional chest pain and intermittent lightheadedness, as well as a history of a childhood heart murmur. TTE showed a bicuspid aortic valve (LCC-NCC fusion) with severe aortic stenosis (peak velocity 4.83 m/s, mean gradient of 56 mmHg, AVA at 0.5 cm2 and Dimensionless Index at 0.15). Furthermore, there was preserved ejection fraction and no associated aortopathy. Following a syncopal episode, the patient was admitted for cardiac optimization prior to delivery. With shared decision making, he ultimately delivered via cesarean section prior to valvular intervention. Post-partum, he underwent balloon aortic valvuloplasty with improvement in mean aortic gradient to 27 mmHg and trace aortic insufficiency. He was asymptomatic at 5 months post-procedure with similar gradients across the aortic valve on TTE. Case Media Chest - X ray Final gradients: Peak velocity 4.83 m/s Mean >50 (56) mmHg AVA 0.5 (AVAi=0.27) DOI=0.15 https://youtu.be/YF8TrNmsGh4 Episode Schematics & Teaching Click to enlarge ??? The CardioNerds 5! – 5 major takeaways from the #CNCR case Hemodynamics change dramatically during pregnancy. Cardiac output increases by 30-50% during pregnancy due to: ↑ SV, ↑HR, ↓SVR. Immediately post-partum, patients with existing valvular lesions are at high risk of heart failure! Hemodynamic changes peripartum are unpredictable. ↑Preload: relief of IVC compression, auto-transfusion of 300-500mL blood from placenta with each uterine contraction, and intravenous fluids and/or blood products. ↓Preload: hemorrhage ↑Afterload: SBP & DBP increase with each uterine contraction ↓Afterload: systemic vasodilation from epidural and spinal analgesia ↑CO: by up to 30% in the first stage of labor and up to 80% in the immediate post-partum period. 2/2 ↑SV. Most pregnant patients with symptomatic AS can be managed medically, with balloon aortic valvuloplasty reserved for patients with refractory symptoms. TAVR and SAVR may be considered, ideally reserved for the 2nd trimester. Spontaneous vaginal delivery carries a lower risk than c-section due to smaller shifts in blood volume, decreased bleeding, and avoidance of preload-shifting anesthetic agents. In a woman of child-bearing age with severe symptomatic AS who is planned for AVR, shared decision making is key in choice of valve! A mechanical valve is more durable than a bioprosthetic valve, but requires anticoagulation that could complicate a future pregnancy. Alternatives include a Ross procedure (replacing the aortic valve using the patient's own pulmonic valve - "pulmonary autograft" - and a cadaveric pulmonic valve is placed in the pulmonic position - "pulmonary allograft"), Ozaki procedure (recreating a new aortic valve from the patient's pericardium), performing a bioprosthetic AVR with risk of earlier degeneration, and performing a TAVR with plans for a surgical AVR later. Decision making is complex and warrants a multidisciplinary team accounting for patient preferences. The modified World Health Organization (WHO) classification stratifies the risk of pregnancy in women with cardiovascular disease. Pregnancy is contraindicated in women with WHO group IV lesions: Pulmonary artery hypertension Severe systemic ventricular dysfunction (EF 45mm in Marfan, >50mm a/w bicuspid aortic valve) Severe (re)coarctation Vascular Ehlers-Danlos syndrome Fontan with any complication Educational Video https://youtu.be/GzqFaNJseDc Produced by Dr. Karan Desai https://twitter.com/ThomasMDas/status/1297266319650435078?s=20 References Nishimura, R. A., Otto, C. M., Bonow, R. O. et al. (2017). 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation, 135(25), e1159–e1195. Sharma, G., Lindley, K., & Grodzinsky, A. (2020). Cardio-Obstetrics: Developing a Niche in Maternal Cardiovascular Health. Journal of the American College of Cardiology, 75(11), 1355–1359. Elkayam, U., Goland, S., Pieper, P. G. et al. (2016). High-Risk Cardiac Disease in Pregnancy: Part I. Journal of the American College of Cardiology, 68(4), 396–410. Orwat, S., Diller, G. P., van Hagen, I. M. et al (2016). Risk of Pregnancy in Moderate and Severe Aortic Stenosis: From the Multinational ROPAC Registry. Journal of the American College of Cardiology, 68(16), 1727–1737. Mazine, A., El-Hamamsy, I., Verma, S. et al. (2018). Ross Procedure in Adults for Cardiologists and Cardiac Surgeons: JACC State-of-the-Art Review. Journal of the American College of Cardiology, 72(22), 2761–2777. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), Regitz-Zagrosek, V., Blomstrom Lundqvist, C., Borghi, C. et al. (2011). ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). European heart journal, 32(24), 3147–3197. CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MDRick Ferraro, MDTommy Das, MDEunice Dugan, MDEvelyn Song, MDColin Blumenthal, MDKaran Desai, MDAmit Goyal, MDDaniel Ambinder, MD