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

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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

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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.
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.

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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


Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case

  1. Hemodynamics change dramatically during pregnancy. Cardiac output increases by 30-50% during pregnancy due to: ↑ SV, ↑HR, ↓SVR.  
  2. 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. 
  3. 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.  
  4. 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.  
  5. 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 <30% or NYHA III-IV) 
    • Systemic RV with moderate or severely decreased ventricular function 
    • Previous peripartum cardiomyopathy with residual ventricular impairment 
    • Severe mitral stenosis or severe symptomatic aortic stenosis 
    • Severe aortic dilation (>45mm in Marfan, >50mm a/w bicuspid aortic valve) 
    • Severe (re)coarctation 
    • Vascular Ehlers-Danlos syndrome 
    •  Fontan with any complication  

Educational Video

Produced by Dr. Karan Desai

References


CardioNerds Case Reports: Recruitment Edition Series Production Team

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