<div>Article type: Images</div><div>Title: Very Severe aortic stenosis with a mean gradient of 134 mmHg in a
17-year-old female with bicuspid aortic valve</div><div>Authors</div><ol><li>Aman Tejas Patel, MD, Resident physician, Department of Cardiology,
Smt NHL Municipal Medical College, Ahmedabad, India</li><li>Subrahmanya Murti Velamakanni, MD, Resident physician, Department of
Cardiology, Smt NHL Municipal Medical College, Ahmedabad, India</li><li>Gajanan Khadkikar, MD, Resident physician, Department of Cardiology,
Smt NHL Municipal Medical College, Ahmedabad, India</li><li>Tejas Patel, DM, Professor and head, Department of Cardiology, Smt NHL
Municipal Medical College, Ahmedabad, India</li></ol><div>Corresponding author: Subrahmanya Murti Velamakanni, Room 12060, SVP
Hospital, Ellisbridge, Ahmedabad, India. Email id:
subrahmanyamurti@gmail.com</div><div>Keywords: bicuspid aortic valve, Aortic stenosis</div><div>Abstract</div><div>Bicuspid aortic valve is the most common congenital anomaly and is
associated with the early onset of severe aortic stenosis by the fifth
decade. Here, we present the echocardiographic images of a 17-year-old
with bicuspid aortic valve who presented with symptomatic severe aortic
stenosis with a very high mean transvalvular gradient of 134 mmHg.</div><div>Article text</div><div>A 17-year-old female presented
with a history of New York Heart Association class III breathlessness.
On examination, a grade 4/6 systolic murmur was present in the aortic
area. On transthoracic echocardiography, there was concentric left
ventricle (LV) hypertrophy with normal size (Figure 1). The aortic valve
(AV) was bicuspid in morphology with fusion of left and right coronary
cusps with a median raphe (Figure 2). This was consistent with a type 1
bicuspid AV as per the Sievers classification.<sup>1</sup> There
was severe aortic stenosis (AS). On suprasternal view, by the AV
velocity time integral (VTI), the maximum pressure gradient (PG) across
the valve of 219 mmHg, aortic valve maximum velocity (AVvmax) was 7.4
m/s and mean PG was 134 mmHg (Figure 3). The aortic annulus and root
were normal sized. The ascending aorta (maximum diameter – 39 mm) and
the arch of aorta (maximum diameter - 39 mm) were dilated, likely as a
part of bicuspid aortopathy (Figure 4). The largest systematic review of
the natural history of bicuspid AV with 11502 patients from 13 studies
had a male to female ratio of 3:1 with the mean age of patients with
symptomatic severe AS being the fifth decade.<sup>2</sup> This
represents a rare occurrence of severe symptomatic AS in bicuspid AV
manifesting at a young age with an astonishingly high gradient.</div><div>References</div><div>Sievers HH, Schmidtke C. A classification system for the bicuspid
aortic valve from 304 surgical specimens. The Journal of thoracic and
cardiovascular surgery. 2007 May 1;133(5):1226-33.</div><div>Masri A, Svensson LG, Griffin BP, Desai MY. Contemporary natural
history of bicuspid aortic valve disease: a systematic review. Heart.
2017 Sep 1;103(17):1323-30.</div><div>Legends to figures</div><div>Figure 1: Showing a parasternal long axis view demonstrating concentric
left ventricle hypertrophy. LA – Left atrium, LV – Left Ventricle, Ao
– Aorta</div><div>Figure 2: Parasternal short axis view showing a bicuspid aortic valve.
LA – Left atrium, RA – Right atrium, RV – Right ventricle</div><div>Figure 3: Showing continuous wave (CW) doppler jet across the aortic
valve in the suprasternal view. AV – Aortic valve, vmax – Maximum
velocity, PG – Pressure gradient</div><div>Figure 4: Modified parasternal view showing dilated ascending aorta</div><div>Supplementary material: Movie clip for figure 2, Movie clip for figure 3</div>