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\begin{document}

\title{Bicuspid Aortic Valve: Progression of Stenosis and Clinical Relevance}



\author[1]{Saqib Masroor}%
\affil[1]{University of Toledo Medical Center}%


\vspace{-1em}



  
  \date{March 14, 2025}


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\textbf{Title:} Bicuspid Aortic Valve: Progression of Stenosis and
Clinical Relevance

\textbf{Running Head:} Progression of Stenosis in Bicuspid Aortic Valve

\textbf{Authors:} Saqib Masroor, MD, MBA, MHS

University of Toledo College of Medicine and Life Sciences, Department
of Surgery, Division of Cardiothoracic Surgery

\textbf{Meeting Presentation:} None

\textbf{Disclosure:} None

\textbf{Word Count:} 717

\textbf{Corresponding Author:}

Saqib Masroor, MD, MHS

Chief, Division of Cardiothoracic Surgery

University of Toledo College of Medicine and Life Sciences

3000 Arlington Avenue, Toledo, Ohio 43614

Phone: 419-383-5150

Fax: 419-383-3149

Bicuspid aortic valve (BAV) is the most common congenital anomaly and
nearly 50\% of people with BAV require aortic valve replacement (AVR)
about 10-20 years earlier than those with tricuspid aortic valves
(TAV)1. It is however not clearly known if aortic stenosis (AS)
progresses at a faster pace in BAV vs TAV. Studies so far reported in
the literature have been inconclusive and marked by relatively small
number of patients and short to mid-term follow-up (2-6
years)\textsuperscript{1-2}.

In this issue of the Journal of Cardiac Surgery, Shang et
al\textsuperscript{3} report on 59 patients with BAV, matched 1:3 with
TAV patients with mild or moderate aortic stenosis, and conclude that
the progression to severe AS in both groups is similar. However, BAV was
associated with an increased risk of AVR. This is an elegant study,
however there are some issues with its methodology and design of the
study. The authors write in the introduction that ``\ldots{}older age,
male sex, coronary artery disease, plasma level of oxidized
phospholipids,\ldots{} have been associated with more rapid prognosis''.
Yet, the study did not investigate the incidence of coronary artery
disease, dyslipidemia or metabolic syndrome in the two groups. Moreover,
the BAV group is 20 years younger than the TAV group. Hence the
influence of these unmatched variables on the outcomes cannot be ruled
out.

Another possible source of error is inconsistent echocardiographic
assessment of AS. Earliest echocardiograms are from 2005 and the latest
one is from 2020. Echocardiography was not done for the purposes of the
study and hence there may not have been a consistency in the assessment
of the AS severity. One of the three hemodynamic parameters necessary
for AS severity assessment is peak jet velocity. Accurate measurement of
peak jet velocity requires using multiple acoustic windows to determine
the highest velocity and it requires particular attention to patient
positioning and adjustment of ultrasound transducer position and angle.
Misalignment of ultrasound beam and AS jet results in underestimation of
jet velocity and even greater underestimation of the pressure
difference.\textsuperscript{4} The reason I raise this issue is the fact
that while patients with trace or no AS were excluded from the study,
12\% of BAV and 5\% of TAV patients ended up with no AS at the study
end.

A more consistent and accurate way of following progression of AS would
be desirable.

And one candidate for this is contrast-enhanced CT imaging to assess
aortic valve calcification, which has been shown to have strong
correlation with AS. \textsuperscript{5} However others have shown that
this correlation is strong in TAV patients of all ages, but in BAV
patients, it only holds strong in patients older than 51 years of age.
Younger patients with BAV can have significant AS without an associated
increase in AV calcification. \textsuperscript{6}

Finally, no account of BAV is complete without a mention of associated
aortopathy. Shang does not include data on the size of the ascending
aorta in the patients. In fact, despite similar progression AS, BAV
patients exhibit a higher incidence of surgery. This could very well be
due to the progressive enlargement of aorta requiring aortic surgery and
concomitant AVR even with moderate AS. Indeed Song et
al\textsuperscript{7} have shown that in BAV patients followed up for 6
years, while 12\% patients underwent AVR, another 10\% patients
underwent replacement of aorta.

Overall, Shang et al should be complimented on putting together a
valuable series of patients with BAV and highlighting their progression
of AS. It is becoming increasingly clear that the rate of progression of
AS is the same in BAV and TAV patients, but they still require more
surgical intervention, whether it be AVR or aortic replacement or a
combination thereof. While academically we may continue to question
whether to perform closer surveillance of BAV patients (with mild AS)
than TAV or not, the more important point for us clinicians is to look
at BAV and aorta as one entity. It is preferable that surgeons follow
these asymptomatic BAV patients, as they are better trained and equipped
to treat both the valvular disease and aortopathy. These patients do
require more frequent and earlier interventions. And because failure of
timely intervention in these patients can be catastrophic. While BAV
occurs in 1\% of the population, it comprises 10-15\% of patients with
aortic dissection!\textsuperscript{8}

REFERENCES

1. Shen M, Tastet L, Capoulade R, et al. Effect of bicuspid aortic valve
phenotype on progression of aortic stenosis. European Heart J --
Cardiovasc Imaging 2020:21;727-34

2. Ryu DR, Park SJ, Han H, et al. Progression rate of aortic valve
stenosis in Korean patients. J Cardiovasc Ultrasound 2010:18:127-33

3. Shang M, Kahler-Quesada A, Mori M, et al. Progression of aortic
stenosis in patients with bicuspid aortic valve. ----------------

4. Baumgartner H, Hung J, Bermego J, et al. Recommendations on the
echocardiographic assessment of aortic valve stenosis: A focused update
from the European Association of Cardiovascular imaging and the American
Society of Echocadiograpny. J Am Soc Echocardiogr 2017;30:372-92

5. Pawade T, Sheth T, Guzzetti E, Dweck MR and Clavel M-A. Why and how
to measure aortic valve calcification in patients with aortic stenosis.
J Am Coll Cardiol Img 2019;12:1835-48

6. Shen M, Tastet L, Capoulade R, et al. Effect of age and aortic valve
anatomy on calcification and haemodynamic severity of aortic stenosis.
Heart 2017;103(1):32-9

7. Song S, Seo J, Cho I, et al. Progression and outcomes of
non-dysfunctional bicuspid aortic valve: Longitudinal data from a large
Korean bicuspid aortic valve registry. Front Cardiovasc Med
2020;7:603323 doi: 10.3389/fcvm.2020.603323

8. Edwards WD, Leaf DS and Edwards JE. Dissecting aortic aneurysm
associated with congenital bicuspid aortic valve. Circulation
1978;57(5):1022-5

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