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Outflow Tract Anomalies

  • Leo LopezEmail author
  • Roque Ventura
  • Nadine F. Choueiter
Chapter

Abstract

Anomalies of the aortic and pulmonary outflow tracts are usually associated with obstruction, regurgitation, and/or aneurysmal dilation of the proximal great arteries, and they represent some of the conditions most frequently encountered by congenital heart disease specialists. In most instances, a full preoperative diagnosis is performed by standard transthoracic echocardiography and other imaging modalities such as cardiac catheterization and magnetic resonance imaging. However, transesophageal echocardiography (TEE) serves an important role in the perioperative management of these patients. Preoperative TEE can provide information regarding the morphology of the outflow tracts and the degree of obstruction and regurgitation; postoperative TEE can evaluate the success of a surgical procedure and exclude residual obstruction, regurgitation, or other potential complications. In addition, TEE is sometimes necessary outside of the operating room setting for older patients with poor transthoracic echocardiographic windows, particularly if the patient has undergone prior surgery. This chapter discusses the use of TEE for the evaluation of both right and left sided outflow tract anomalies.

Keywords

Aortic stenosis, valvar Aortic stenosis, subvalvar Aortic stenosis, supravalvar Aortic regurgitation Sinus of Valsalva aneurysm Pulmonary stenosis, valvar Double-chambered right ventricle Pulmonary regurgitation 

Supplementary material

Video 11.1a

Deep transgastric long axis view at 0° showing the left ventricular outflow tract (AVI 4028 kb)

Video 11.1b

Deep transgastric sagittal view at 90° showing the right ventricular outflow tract (AVI 4850 kb)

Video 11.2

Three-dimension mid esophageal aortic valve short axis view depicting the three-dimensional nature of a normal tri-leaflet aortic valve (AVI 7151 kb)

Video 11.3

Mid esophageal aortic valve long axis view at 120° showing the LV outflow tract (AVI 6532 kb)

Video 11.4

Mid esophageal aortic valve short axis view at ~30° shows a true bicuspid aortic valve with only two leaflets (the right and left coronary leaflets) and absent non-coronary leaflet, giving a “fishmouth” appearance to the valve orifice. Color flow Doppler shows turbulence across the valve, as well as the origin of the left coronary artery seen posteriorly, and a brief glimpse of the right coronary artery seen anteriorly (MPG 4520 kb)

Video 11.5

Mid esophageal aortic valve short axis view at 30° showing a bicuspid aortic valve with fusion or underdevelopment of the intercoronary commissure (AVI 7165 kb)

Video 11.6

Mid esophageal aortic valve short axis view at 30° showing a bicuspid aortic valve with fusion or underdevelopment of the commissure between the right and non-coronary leaflets (AVI 2827 kb)

Video 11.7

Mid esophageal aortic valve short axis view at 30° of a dysplastic aortic valve with thickened right and non coronary leaflets (AVI 9750 kb)

Video 11.8

Mid esophageal aortic valve long axis view at 120° showing a dysplastic aortic valve associated with valvar aortic stenosis (AVI 8222 kb)

Video 11.9

Transcatheter aortic valve replacement/implantation (TAVR/TAVI). This video, obtained from a mid esophageal aortic valve long axis view at 120–130° first shows the abnormal aortic, which is thickened and has restricted motion. A catheter and then the balloon-mounted valve are seen, with the balloon shown as it is expanded and the valve implanted in the aortic position. During balloon dilation, rapid ventricular pacing is performed to reduce ventricular ejection, thereby stabilizing the valve for placement. Following valve implantation, leaflet motion is seen and there rare two jets of regurgitation seen—one central (transvalvular), one peripheral (paravalvular). LM left main coronary artery (Video provided courtesy of Siemens Medical Systems USA, Inc. © 2012–13 Siemens Medical Solutions USA, Inc. All rights reserved) (MPG 39428 kb)

Video 11.10

Mid esophageal four chamber view with probe anteflexion to visualize the left ventricular outflow tract. This video shows a subaortic fibromuscular ridge associated with subvalvar aortic stenosis, depicting a steep aorto-septal angle and extension of the fibromuscular ridge to the anterior mitral leaflet. Color Doppler demonstrates flow aliasing originating at the region of the ridge or membrane (AVI 11046 kb)

Video 11.11

Systolic anterior motion (SAM) of the mitral valve, in a patient with pronounced ventricular septal hypertrophy. This video, obtained from the mid esophageal four chamber, two chamber and long axis views, shows significant systolic displacement of the anterior mitral leaflet into the left ventricular outflow tract. SAM appears to be caused by a “drag” effect resulting from a hyperdynamic, underfilled left ventricle. The anterior leaflet is pulled into the left ventricular outflow tract and contributes to dynamic outflow tract obstruction. The SAM prevents the mitral valve from effective coaptation and produces significant mitral regurgitation, as noted by the color flow Doppler jet. The left ventricular outflow tract gradient can be very high; in this patient the peak velocity was measured at nearly 8 m/s, or 256 mmHg. Note the dagger-shaped spectral Doppler tracing (MPG 20424 kb)

Video 11.12

Mid esophageal aortic valve long axis view at 120° showing a subaortic fibromuscular ridge (black asterisk) and a dysplastic aortic valve resulting in combined subvalvar and valvar aortic stenosis in addition to a small membranous ventricular septal defect (the fibromuscular ridge is located at the crest of the muscular septum) (AVI 20924 kb)

Video 11.13

Mid esophageal aortic valve long axis view at 120° showing discrete narrowing at the sino-tubular junction resulting in supravalvar aortic stenosis (AVI 9697 kb)

Video 11.14

Deep transgastric long axis view at 30° showing discrete narrowing of the sino-tubular junction resulting in supravalvar aortic stenosis without and with color mapping (there is associated aortic regurgitation) (AVI 4953 kb)

Video 11.15

Quadricuspid aortic valve as seen from the mid esophageal aortic valve short axis view (MOV 2037 kb)

Video 11.16a

Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in a mid esophageal aortic valve short-axis view at 45° (AVI 9125 kb)

Video 11.16b

Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in a modified mid esophageal right ventricular inflow-outflow view at 90° (AVI 11555 kb)

Video 11.17

Mid esophageal aortic valve long axis view at 120° showing a prosthetic aortic valve with motion of the prosthetic hemidisc leaflets (AVI 5826 kb)

Video 11.18

Mid esophageal ascending aortic long axis view at 120° showing a markedly dilated aortic root and ascending aorta with associated aortic dissection along the anterior wall of the ascending aorta in a patient with Marfan syndrome (AVI 11790 kb)

Video 11.19

Markedly dilated aortic root as seen from the mid esophageal four chamber and aortic valve short and long axis views, showing central aortic regurgitation (MPG 13788 kb)

Video 11.20

Mid esophageal aortic valve long axis with counterclockwise probe rotation to demonstrate the right ventricular outflow tract followed by upper esophageal aortic arch short axis views at 90°. This video shows valvar pulmonary stenosis with thin but doming pulmonary valve leaflets, and post-stenotic dilation of the main pulmonary artery (along with swirling of flow) in association with a moderate secundum atrial septal defect (not shown). This study was done in the catheterization laboratory during closure of the defect; a catheter is seen in the left atrium (AVI 19514 kb)

Video 11.21

Mid esophageal ascending aortic short axis at 0° showing the main pulmonary artery in the setting of mild valvar pulmonary stenosis, and minimal flow acceleration across the valve. In this video the bifurcation of the branch pulmonary arteries can also be seen (AVI 6695 kb)

Video 11.22

Mid esophageal right ventricular inflow-outflow view at 60° showing a double-chambered right ventricle with prominent muscle bundles at the infundibular os separating the proximal right ventricular chamber from the well-developed right ventricular infundibular chamber and resulting in subvalvar pulmonary stenosis (the pulmonary annulus is within normal limits in size) (AVI 12855 kb)

Video 11.23

Deep transgastric sagittal view at 90° showing a double-chambered right ventricle with prominent muscle bundles at the infundibular os separating the proximal right ventricular chamber from the well-developed right ventricular infundibular chamber and resulting in subvalvar pulmonary stenosis (the pulmonary annulus is within normal limits in size); this is an ideal view to measure the gradient along the right ventricular outflow tract (AVI 9265 kb)

Video 11.24

Mid esophageal right ventricular inflow-outflow view at 60 showing free pulmonary regurgitation in the setting of tetralogy of Fallot repair with a transannular right ventricular outflow tract patch (AVI 8032 kb)

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Copyright information

© Springer-Verlag London 2014

Authors and Affiliations

  • Leo Lopez
    • 1
    Email author
  • Roque Ventura
    • 2
  • Nadine F. Choueiter
    • 1
  1. 1.Department of PediatricsAlbert Einstein College of Medicine, Children’s Hospital at MontefioreBronxUSA
  2. 2.Department of PediatricsMiami Children’s HospitalMiamiUSA

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