Article type: Images
Title: Very Severe aortic stenosis with a mean gradient of 134 mmHg in a 17-year-old female with bicuspid aortic valve
Authors
  1. Aman Tejas Patel, MD, Resident physician, Department of Cardiology, Smt NHL Municipal Medical College, Ahmedabad, India
  2. Subrahmanya Murti Velamakanni, MD, Resident physician, Department of Cardiology, Smt NHL Municipal Medical College, Ahmedabad, India
  3. Gajanan Khadkikar, MD, Resident physician, Department of Cardiology, Smt NHL Municipal Medical College, Ahmedabad, India
  4. Tejas Patel, DM, Professor and head, Department of Cardiology, Smt NHL Municipal Medical College, Ahmedabad, India
Corresponding author: Subrahmanya Murti Velamakanni, Room 12060, SVP Hospital, Ellisbridge, Ahmedabad, India. Email id: subrahmanyamurti@gmail.com
Keywords: bicuspid aortic valve, Aortic stenosis
Abstract
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.
Article text
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.1 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.2 This represents a rare occurrence of severe symptomatic AS in bicuspid AV manifesting at a young age with an astonishingly high gradient.
References
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.
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.
Legends to figures
Figure 1: Showing a parasternal long axis view demonstrating concentric left ventricle hypertrophy. LA – Left atrium, LV – Left Ventricle, Ao – Aorta
Figure 2: Parasternal short axis view showing a bicuspid aortic valve. LA – Left atrium, RA – Right atrium, RV – Right ventricle
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
Figure 4: Modified parasternal view showing dilated ascending aorta
Supplementary material: Movie clip for figure 2, Movie clip for figure 3