Journal of Cardiothoracic and Vascular Anesthesia 37 (2023) 832836

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Journal of Cardiothoracic and Vascular Anesthesia

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E-Challenges & Clinical Decisions

An Unusual Cause of Aortic Regurgitation in a Patient

With Bicuspid Aortic Valve
Jiaqin Ren, MD*,y, Mingjing
1
Chen, MDz, Li Tang, MD*,
*, * ,1
Lei Du, PhD , Haibo Song, PhD
*
Department of Anesthesiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
y
Department of Anesthesiology, Guiqian International General Hospital, Guiyang, Guizhou, China
z
Department of Epidemiology, College of Public Health & Health Professions and College of Medicine, Uni-
versity of Florida, Gainesville, FL

Aortic fibrous strands are considered residual tissue from aortic valve development. Rupture of these strands is an important albeit uncommon
cause of aortic regurgitation (AR). The authors describe a 67-year-old man who was admitted to the authors’ hospital with sudden onset shortness
of breath and diagnosed with severe AR. The patient was scheduled for Bentall surgery. The transesophageal echocardiogram (TEE) found mul-
tiple fibrous strands that were present in multiple locations of the aortic valve, some of which were ruptured. Ruptured fibrous strands are in the
differential diagnosis in patients presenting with acute AR without a more conventional explanation, and TEE is instrumental in securing
the diagnosis.
Ó 2022 Published by Elsevier Inc.

Key Words: Bicuspid Aortic Valve; Aortic Regurgitation; TEE; Fibrous strand; Aortic Valve

BICUSPID AORTIC VALVE (BAV) is present in 0.9%-to-
2% of the population.1 A study from the Mayo Clinic reported
that 13% of BAV patients had pure aortic regurgitation (AR). 2
The main causes of AR in this population are prolapse of
the larger cusp and cusp stress fenestration caused by aortic
root dilatation, infective endocarditis, aortic dissection, and
trauma.1,3 Herein, the authors report an unusual case of AR
explored by transesophageal echocardiography (TEE) and dis-
cuss how to recognize it and participate in surgical decision-
making.
Case Presentation
A 67-year-old male patient came to the authors’ emergency
department due to sudden shortness of breath, dizziness, and
edema of both lower limbs. On transthoracic echocardiography
1
Address correspondence to Haibo Song, PhD and Lei Du, PhD, Department
of Anesthesiology, West China Hospital, Sichuan University, No. 37 Guo Xue
Alley, Chengdu 610041, Sichuan, China.
E-mail addresses: dulei@scu.edu.cn (L. Du), pdasonghaibo@163.com
(H. Song).
(TTE), he was found to have severe AR and dilation of the
ascending aorta (Fig 1A), A BAV was also suspected with
slightly thickened leaflets and a linear attachment of the AV
(Fig 1B) to the aortic lumen (Fig 1C). He had no fever and
infective endocarditis was not suspected. He was scheduled
for an urgent Bentall procedure under general anesthesia with
cardiopulmonary bypass and TEE monitoring. Moreover, the
TEE demonstrated mobile echodensities in the aortic lumen
(Video 1 and 2).
E-Challenge
Based on the images and videos provided, what is the cause
of AR?
Clinical Course
Intraoperative TEE examination before cardiopulmonary
bypass demonstrated a BAV with a raphe between the left and
right coronary cusps (Fig 2). Multiple fibrous strands attached
to different locations of the BAV could be observed clearly by

https://doi.org/10.1053/j.jvca.2022.12.024
1053-0770/Ó 2022 Published by Elsevier Inc.
 J. Ren et al. / Journal of Cardiothoracic and Vascular Anesthesia 37 (2023) 832836 833

Fig 1. Preoperative TTE image. A,Color Doppler showed severe AR; B,A short linear object was attached to the AV (white arrow); C and D,This is an abnormal
linear fibrous object in the aortic lumen (white arrow). AO, aortic opening; LA, left atrium; LV, left ventricle.

Fig 2. TEE showed that the AV was bicuspid. A raphe was between the right
cusp and left cusp (white arrow), and the commissures were at 4 and 10
o’clock (blue arrow). LA, left atrium; RA, right atrium; RV, right ventricle.
TEE (Video 1 and 2), which were numbered as “i, ii, iii”,
respectively. Strands i and ii were the ruptured fibrous strands
(Fig 3, A): strand i was attached to the free edge of the fused
cusp and fluttered into the aorta in systole and into the left ven-
tricular outflow tract (LVOT) in diastole (Fig 3B and 3C); and
strand ii was attached to the root of the fused cusp and pre-
sented in the LVOT in diastole (Fig 3D). Strand iii was the lin-
ear stripe located between the nonfused cusp and the aortic
wall demonstrated by 2-dimensional TEE (Fig 4). Under direct
surgical visualization, strand i was a peculiar ruptured fenes-
trated fibrous strand on the free edge of the middle part of the
fused cusp. The ruptured fibrous strand ii was on the fused
cusp at the right noncoronary cusp commissure (Figs 5 and 6).
Strand iii contained iiia and iiib and was noted to have a pair of
unruptured fibrous strands connected to the nonfused cusp and
the aortic wall (Fig 6). Other echocardiographic findings included
dilation of the left atrium (47 mm), right atrium (55 mm), and left
ventricle (76 mm), severe aneurysmal dilation of the aortic root
(54 mm), and a dilated ascending aorta (63 mm). The fused cusp
prolapsed into the LVOT in diastole (Fig 4), leaving a coaptation
gap resulting in severe AV regurgitation.
The patient underwent a successful Bentall procedure to
replace the AV, aortic root, and ascending aorta. Postoperative
TTE indicated that the biologic aortic valve and ascending
aorta were functional. His postoperative course was unevent-
ful, and he was discharged without significant complications.

Fig 3. Ruptured fibrous strands. A, The ruptured fibrous strands i and ii attached to the fused cusp (white and yellow arrows); B and C, Ruptured fibrous strand i
connected to the free edge of the fused cusp (white arrow); It presented in the LVOT in diastole (B), and presented in the aorta in systole (C); D, A string of waving
tissue(ii) is attached to the fused cusp and presented in the LVOT in diastole (white arrow). AO, aortic opening; LA, left atrium; LV, left ventricle; LVOT, left ven-
tricular outflow tract.
834 J. Ren et al. / Journal of Cardiothoracic and Vascular Anesthesia 37 (2023) 832836

Fig 6. Removed aortic valve. The aortic valve was a fused BAV, the raphe was
between LCC and RCC. i was a ruptured fenestrated fibrous strand attached to the
free edge of the fused cusp, and ii was another ruptured fibrous strand located on
the RCC; iiia and iiib were a pair of unruptured fibrous strands located on both
sides of the nonfused cusp. BAV, bicuspid aortic valve; LCC, left coronary cusp;
NCC, noncoronary cusp; RCC, right coronary cusp.

Fig 4. Prolapsed fused cusp and unruptured fibrous strand iii. The fused cusp
prolapsed into the LVOT during diastole (white arrow); the linear fibrous stripe
(iii) anchored between the nonfused cusp and the aortic wall (yellow arrow). AO,
aortic opening; LA, left atrium; LVOT, left ventricular outflow tract.
Discussion
Fibrous strands might represent embryonic remnants of the
aortic cusp formation process, which could leave fibrous tissue
between the cusps and the aorta wall or LVOT.4-8 When both
ends of the fibrous strand are attached to the same leaflet, this
may lead to a fenestrated cusp.9 Most cases of fibrous strands
on aortic valves have been reported in Asian countries, particu-
larly Japan, and are found commonly in patients with tricuspid
aortic valves.5 Interestingly, a similar pathology has been
described previously in dogs.10 The cause of fibrous strand for-
mation is unclear, but risk factors may be related to race or
genetics.
Physiologically, these fibrous strands are supportive tissues
that retain AV coaptation.5 They can also cause acute AR once
their state is modified, such as rupture, increased stress,
decreased stress, perforation and other factors. Previous stud-
ies have suggested fibrous strand-associated AR is caused by 2

Fig 5. In the midesophageal aortic valve short axis. A, a fibrous strand is between NCC and the aortic wall (white arrow). B,the fenestrated fibrous strand is on the
fused cusp (yellow arrow). LCC, left coronary cusp; NCC. noncoronary cusp; RCC, right coronary cusp.
 J. Ren et al. / Journal of Cardiothoracic and Vascular Anesthesia 37 (2023) 832836
mechanisms: one is rupture of the fibrous strands between the
aortic cusp and the aortic wall5,6 or on the fenestrated cusp,9
the other one is fibrous strands restricting closure of the aortic
cusp.4 Approximately 53% of reported cases of AR caused by
fibrous strands are related to the ruptured strands,5 and are gen-
erally attributable to rupture of a single strand. A patient with
AR caused by multiple ruptured fibrous strands is highly rare.
Herein, the authors described a case of AR in a patient with a
fenestrated BAV, which was caused by multiple fibrous
strands at different locations. There are 3 possible causes of
AR: (1) the rupture of the fibrous strands i and ii attached to
the fused cusp cause the fused cusp to lose support and pro-
lapse into LVOT in diastole (Fig 4); (2) due to a severe aneu-
rysmal dilation of the aortic root, increased stress on the
unruptured fibrous strand iii prevented the closure of the non-
fused cusp; (3) the enlargement of the aortic root caused the
aortic ring to dilate, resulting in aortic insufficiency, which led
to a coaptation gap, resulting in a severe AR.
A thorough assessment of the aortic valve and aortic root is
necessary in cases of poorly defined AR or in patients in
whom there is fluttering tissue noted (Video 1). This examina-
tion should aim to exclude other causes of AR such as rheu-
matic disease, infection, and severe calcification,
degeneration, Lambl’s excrescences, other neoplasms, dissec-
tion flap, or fibroelastoma. A ruptured strand usually causes
AR, and the causes of strand rupture often include aging degra-
dation, myxomatous degeneration, infection, tumor, hyperten-
sion, infectious endocarditis, or stress.6,11-13 Compared to
TTE,12 TEE is more sensitive in recognizing the location,
number, and state of fibrous strands. In the authors’ patient,
TEE clearly revealed that the classification of BAV was
Siever’s Type I, which is right/left cusp fusion with raphe14 in
the view of the midesophageal aortic valve short-axis (Fig 2),
and disclosed multiple fibrous strands, including ruptured and
unruptured fibrous strands, which were at different locations
on the aortic valve.
For treatment, the authors’ patient received the Bentall pro-
cedure due to severe AR with an ascending aorta diameter
>45 mm, recommended by the current guidelines,15 and sub-
sequently successfully discharged. Clarifying the mechanism
of AR may have implications for potential treatment options.
Some valves with ruptured strands may be amenable to repair
with a suspension of the cusp, plication, or reinforcement with
geometric annuloplasty.16,17
In the case of AR with aortic dilation, it may be per-
formed by central plication suture of the prolapsed cusp
with plication of the aortic wall or suture annuloplasty. In
addition, BAV repair has been better than conventional
AVR in terms of hemodynamics and survival over the past
20 years.18 Minimally invasive surgery is also a good
option, Wei et al.19 reported a patient with AR who under-
went a successful transcatheter aortic valve replacement
due to a confined noncoronary cusp caused by fibrous
835
strand between the noncoronary cusp and aortic root. In
general, the most appropriate surgical method should be
selected according to the patient’s condition.
Conclusion
Fibrous strands are residual tissue left behind after aortic
valve development. Echocardiographers must be familiar with
this pathology, and ruptured fibrous strands should be on the
differential diagnosis for poorly defined aortic valve disease.
Furthermore, understanding the mechanisms by which fibrous
strands impact aortic valve function may change the surgical
approach to management.
Conflict of Interest
None.
Supplementary materials
Supplementary material associated with this article can be
found in the online version at doi:10.1053/j.jvca.2022.12.024.
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