48. 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,

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 Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support 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 <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

https://youtu.be/GzqFaNJseDc
Produced by Dr. Karan Desai

References

ACCSAP – AS 
VHD in Pregnancy 
CardioObstetrics – JACC 2020 
JACC – High-Risk Cardiac Diseaes in Pregnancy – Part 1 
JACC 2016 – Risk of Pregnancy in Moderate to Severe Aortic Stenosis 
ESC 2018 Guidelines for the mgmt of cvd in pregnancy (includes WHO classification) 
Ross procedure 

CardioNerds Case Reports: Recruitment Edition Series Production Team

Bibin Varghese, MD

Rick Ferraro, MD

Tommy Das, MD

Eunice Dugan, MD

Evelyn Song, MD

Colin Blumenthal, MD

Karan Desai, MD

Amit Goyal, MD

Daniel Ambinder, MD