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PERIPARTUM CARDIOVASCULAR DISEASE MINI-FOCUS ISSUE
CASE REPORT: CLINICAL CASE
Bicuspid Aortic Valve and Ascending
Aortic Aneurysm in a Twin Pregnancy
Eryn Bryant, MSN, NP-C,
a
Sandra Tsai, MD, MPH,
a,b
Eleanor Levin, MD,
a
Dominic Fleischman, MD,
c
Jessica Ansari, MD,
d
Michael Fischbein, MD,
e
Katherine Bianco, MD,
f
Abha Khandelwal, MD, MS
a
ABSTRACT
Bicuspid aortic valve with ascending aortic aneurysm is a common condition encountered in pregnancy. There are limited
data on how to manage these patients. To our knowledge, we report the only case of a bicuspid aortic valve and aortic
aneurysm with twin gestations. (Level of Difficulty: Intermediate.) (J Am Coll Cardiol Case Rep 2020;2:96–100) © 2020
The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access
article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
HISTORY OF PRESENTATION
A 34-year-old G1P0 Caucasian woman with a bicuspid
aortic valve (BAV) and a dilated ascending aorta of
4.9 cm presented to a women’s heart health clinic at
12 weeks of a twin gestation for evaluation and
management of her high-risk pregnancy.
Two months prior, at an outside facility, a positive
human chorionic gonadotropin urine pregnancy test
led to an ultrasound, which revealed an ovarian mass
concerning for cyst versus malignancy. She was too
early in the pregnancy for the ultrasound to detect a
fetalheartbeat.Shethenunderwent a right ovarian
cystectomy for the 9.2-cm right ovarian cyst with
pathology demonstrating a conventional type
borderline serous tumor. She was also in the process
of infertility workup because she had been trying to
become pregnant for 12 months prior. It was then
recommended that she undergo an oophorectomy;
however, when her human chorionic gonadotropin
remained positive, she discovered she was pregnant
and decided to defer until after pregnancy.
During the workup for her ovarian cystectomy, she
underwent an echocardiogram, which revealed a BAV
and an ascending aortic aneurysm of 4.9 cm (Figure 1).
She reported she had a known BAV since the age of
7 years with periodic echocardiograms and clinical
monitoring until approximately age 18, at which time
she was reportedly told no intervention was required
and the BAV could be monitored in the future. She
was active all throughout her childhood and
remained so at the time of the initial consultation.
From a cardiac standpoint, she was asymptomatic
and denied chest pain, back pain, palpitations, exer-
tional dyspnea, orthopnea, postural nocturnal dys-
pnea, dizziness, and syncope (New York Heart
Association functional class I).
LEARNING OBJECTIVES
Monitoring of a bicuspid aortic valve with a
dilated aorta during a twin pregnancy in a
nonsyndromic patient is discussed.
Anesthetic and delivery management in a
bicuspid aortic valve and a dilated aorta is
examined.
ISSN 2666-0849 https://doi.org/10.1016/j.jaccas.2019.12.012
From the
a
Division of Cardiovascular Medicine, Stanford School of Medicine, Stanford, California;
b
Department of Primary Care
and Population Health, Stanford School of Medicine, Stanford, California;
c
Department of Radiology, Stanford School of Medicine,
Stanford, California;
d
Division of Obstetric Anesthesia, Stanford School of Medicine, Stanford, California;
e
Division of Cardio-
thoracic Surgery, Stanford School of Medicine, Stanford, California; and the
f
Department of Obstetrics and Gynecology, Stanford
School of Medicine, Stanford, California. The authors have reported that they have no relationships relevant to the contents of this
paper to disclose.
Informed consent was obtained for this case.
Manuscript received November 15, 2019; revised manuscript received December 4, 2019, accepted December 4, 2019.
JACC: CASE REPORTS VOL. 2, NO. 1, 2020
ª2020 THE AUTHORS. PUBLISHED BY ELSEVIER ON BEHALF OF THE AMERICAN
COLLEGE OF CARDIOLOGY FOUNDATION. THIS IS AN OPEN ACCESS ARTICLE UNDER
THE CC BY-NC-ND LICENSE (http://creativecommons.org/licenses/by-nc-nd/4.0/).
PAST MEDICAL HISTORY
She also had documented elevated blood pressure
readings at clinic visits without a diagnosis of hy-
pertension (but was diagnosed with chronic hyper-
tension), obesity (BMI 35 kg/m
2
), and gestational
diabetes in her first trimester. She had no family
history of valvular heart disease or aortopathies. Her
examination demonstrated no syndromic features.
INVESTIGATIONS
She was offered elective termination versus carrying
a high-risk pregnancy and pre-term delivery. If she
elected to carry, the timing and mode of delivery
were discussed, as was anesthesia management. The
risks and benefits based on limited data extrapolation
were discussed with her, and it was elected by the
patient to continue.
MANAGEMENT
She was counseled on the risk of pre-term delivery,
fetal demise, cardiovascular death, and morbidity
related to an aortic dissection. A multidisciplinary
maternal-fetal medicine, anesthesia, and cardiac
team deemed her pregnancy high risk for cardiovas-
cular morbidity and/or mortality. She and her family
were counseled on these risks, expressed under-
standing, and elected to proceed with the pregnancy.
To avoid radiation in pregnancy, magnetic resonance
imaging of the aorta (Figure 2) was performed to
accurately assess aortic dimensions. She met with the
cardiothoracic surgery team as a precautionary mea-
sure should earlier intervention be required. She was
started on a beta-blocker at the initial consultation,
given exercise restrictions of avoiding heavy lifting,
and monitored with transthoracic echocardiograms
every 4 weeks until delivery (Table 1). Throughout her
pregnancy, she remained clinically asymptomatic,
normotensive, and New York Heart Association
functional class I.
DISCUSSION
To our knowledge, this is the first published case
report of a successful pregnancy in a patient with BAV
and a dilated aorta with twin gestation. BAV is the
most common congenital cardiac abnormality with
coexistent dilatation of the ascending aorta (1,2). In
addition to the complications of aortic stenosis or
regurgitation, there are potential risks of aortic
enlargement or dissection in pregnancy. Prior reports
suggest that these risks may be slightly higher in the
presence of aortic regurgitation. The risk of aortic
dissection is thought to be low at 3.1% per
10,000 patient-years; however, this repre-
sents an 8-fold increase over the risk to the
general population (3,4).
BAV is considered to have a strong genetic
component with an inheritance pattern that may be
autosomal dominant with variable penetrance or
sporadic. It can be isolated or part of other heritable
congenital syndromes and aortopathies (1,5). The
2018 American College of Cardiology/American Heart
Association guidelines for the management of adults
with congenital heart disease and the American As-
sociation of Thoracic Surgery consensus guidelines
recommend fetal ultrasound screening when the
mother has BAV (1,5,6).
Aortic aneurysms are diagnosed when the diameter
is >2 SDs above the mean or referenced aortic
segment or using z-scores. However, current guide-
lines still use absolute values for intervention rec-
ommendations (7).Lastly,muchofthedataon
aneurysms in pregnancy come from extrapolation
from Marfan syndrome patients (2,8).
In pregnancy, the increases in heart rate, stroke
volume, and blood pressure, along with hormonal
shifts, result in increased stress and changes in the
FIGURE 1 MRI of Thoracic Aorta
Magnetic resonance imaging: the oblique sagittal view of the thoracic aorta showing an
ascending aortic aneurysm.
ABBREVIATIONS
AND ACRONYMS
BAV =bicuspid aortic valve
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JANUARY 2020:96–100 Bicuspid Aortic Valve and Ascending Aortic Aneurysm
97
aortic media (1,3,9,10). The increased risk of dissec-
tion is greatest in the third trimester or postpartum.
Recent studies using monthly to every 6 weeks serial
echocardiographic monitoring during pregnancy
indicate a 0% maternal mortality.
The American Association of Thoracic Surgery
guidelines recommend that in the case of an aortic
root diameter >4.5 cm, pregnancy should be
discouraged (5). The 2018 European Society of Car-
diology guidelines for the management of cardiovas-
cular diseases during pregnancy recommend the
avoidance of pregnancy when the aorta diameter is
>5.0 cm (6). Because of the increased risk of
dissectioninthecaseofBAV,somerecommenda
lower threshold for prophylactic repair before plan-
ned pregnancy of 4.0 cm, especially in the context of
Marfan syndrome or other collagen vascular disor-
ders. The European Society of Cardiology guidelines
recommend considering surgery in this case with an
aortic diameter >5.0 cm (6).
The current clinical standard of management
would suggest serial monitoring based on aortic
dimension and growth during pregnancy. The
consideration of >5.0 mm dilation through the preg-
nancy should trigger a personalized plan for inter-
vention with elective termination, delivery once fetal
lung maturity is established, or surgical repair early in
the postpartum period (6).
ForpatientswithisolatedBAVandadilated
aorta $5.0 cm, consideration for prophylactic surgery
should be pursued. Prolonged pushing is not advised
FIGURE 2 CTA Thoracic Aorta
Computed tomography angiography: the oblique sagittal maximum intensity projection
of the thoracic aorta showing the ascending aortic aneurysm (postpartum).
FIGURE 3 CT Volume-Rendered Images of Aortic Valve
(A) Volume-rendered images of the aortic valve viewed from above in systole shows an incomplete valve opening due to fusion of the left and right aortic cusps by a
raphe (Sievers type I, R-L). (B) Volume-rendered images of the aortic valve viewed from above in diastole.
TABLE 1 Aortic Measurements
Gestational Week
Imaging
Modality
Proximal
Ascending Aorta
Mean AV
Gradient
4 TTE 4.9 cm 14 mm Hg
12 TTE 4.9 cm 12 mm Hg
16 TTE 4.9 cm 17 mm Hg
18 MRI 4.9 4.8 cm NA
22 TTE 5.0 cm 20 mm Hg
26 TTE 5.0 cm 22 mm Hg
30 TTE 4.9 cm 21 mm Hg
6 weeks postpartum TTE 4.9 cm 17 mm Hg
10 months postpartum CT 5.1 cm NA
CT ¼computed tomography; MRI ¼magnetic resonance imaging; NA ¼not
applicable; TTE ¼transthoracic echocardiography.
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Bicuspid Aortic Valve and Ascending Aortic Aneurysm JANUARY 2020:96–100
98
in those with an aorta >5.0 cm or growth >5.0 mm in
pregnancy (1,6). Anesthetic management based on
European guidelines suggests if aortic diameters are
4.0 to 4.5 cm, vaginal delivery with regional anes-
thesia and invasive arterial pressure monitoring is
needed.Acesareansectionshouldbeconsideredin
aortas larger than this (3,4,6).
Obtaining an accurate and consistent aortic mea-
surement can be challenging whether based on
echocardiography or magnetic resonance imaging
because techniques for measuring differ slightly.
Medical management includes beta-blockers in preg-
nancy rather than angiotensin-converting enzyme
inhibitors or angiotensin receptor blockers, which are
contraindicated in pregnancy. They should be used
to prevent hypertension with systolic pressure
maintained <140 mm Hg and diastolic pressure
<90 mm Hg. Some studies advocate a decrease in
heart rate by 20 beats/min. Some early reports indi-
cated atenolol could cause fetal growth restriction;
therefore, it should be avoided (3,4).
Delivery planning should be done in a multidisci-
plinary and personalized fashion to include a high-
risk obstetrician, anesthesiologist, and cardiac team.
It should integrate parity status, gestation status,
patient age, family history, and concomitant collagen
vascular disorders and syndromes, in addition to the
aortic dimensions, aortic valve function, and aortic
growth rate.
FOLLOW-UP
She underwent elective cesarean section at 34 weeks’
gestation and delivered 2 healthy baby girls. Her aorta
continued to dilate postpartum (Figures 3 and 4);
therefore, a planned elective repair was scheduled
after 11 months.
CONCLUSIONS
Pregnancy in a patient with BAV with a dilated aorta
has traditionally been associated with high risk;
however, when not associated with syndromic aor-
topathies and with careful monitoring and multidis-
ciplinary management, it can be carried out
successfully.
ADDRESS FOR CORRESPONDENCE: Dr. Abha Khan-
delwal, Division of Cardiovascular Medicine, Stanford
School of Medicine, 300 Pasteur Drive, H2156 MC
5319, Stanford, California 94305. E-mail: akhandel@
stanford.edu.
FIGURE 4 CTA Post-Partum
Computed tomographic images (left) 2 months’and (right) 10 months’postpartum showing the size of the ascending aortic aneurysm
at 51 mm.
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KEY WORDS aorta, bicuspid aortic valve,
pregnancy
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