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Archives of cardiovascular diseases
Volume 106, n° 3
pages 180-181 (mars 2013)
Doi : 10.1016/j.acvd.2011.09.006
Received : 11 August 2011 ;  accepted : 15 September 2011
Unusual operation for tetralogy of Fallot
Opération inhabituelle pour la tétralogie de Fallot
 

Sherif Moustafa a, Naeem Merchant b, Timothy Prieur a,
a Adult Congenital Heart Clinic, Department of Cardiac Sciences, University of Calgary, Peter Lougheed Hospital, 3500 26, avenue Northeast, Calgary, AB T1Y 6J4, Canada 
b Department of Diagnostic Imaging, University of Calgary, Foothills Medical Center, Calgary, AB, Canada 

Corresponding author. Fax: +1 403 250 9539.

Keywords : Tetralogy of Fallot, Extracardiac conduit, Computed tomography

Mots clés : Tétralogie de Fallot, Conduit extracardiaque ;Tomodensitométrie

Abbreviations : CCT, PA, RVOT


A 21-year-old woman presented with recurrent palpitations in the setting of previously documented Tetralogy of Fallot. Primary repair had been performed at the age of 9years, with requirement for dual-chamber pacemaker implantation in the early postoperative period. Unfortunately, no operative details were obtainable. Transthoracic and transoesophageal echocardiography confirmed the presence of severe RVOT obstruction with peak and mean gradients of 100 and 63mmHg, respectively, together with trivial pulmonary regurgitation. In addition, moderate right ventricular dilatation with moderate/severe systolic dysfunction and elevated right ventricular systolic pressure (90mmHg) were found. However, echocardiography was unable to localize the site of the RVOT/PA obstruction (Figure 1, Appendix A). CCT was carried out with a 64-row multidetector scanner after peripheral vein injection of 90 cc of the non-ionic contrast medium Ioversol (Optiray 320mg/mL; Tyco Healthcare Canada, Inc., Pointe Claire, QC, Canada) as a bolus dose at the rate of 5mL/s with retrospective electrocardiogram gating. CCT demonstrated a thickened small RVOT (9mm) with a dynamic obstruction secondary to a hypertrophied muscle bundle. Additionally, there was a conduit placed between the upper right ventricular body and the normal-calibre right PA, with severe calcification and stenosis. The main PA had an unusual shape and was narrowed at the bifurcation (12mm), with an enlarged origin of the left PA (25mm) connected to the native RVOT (Figure 2, Figure 3). The origin and course of the coronary arteries were normal. The patient underwent successful conduit excision, resection of the native RVOT muscle bundles, RVOT/proximal main PA pericardial patch, arterioplasty of the distal main PA and the proximal portions of both PA branches and pulmonary valve replacement with a 27mm mosaic bioprosthetic valve. Surgery was uneventful.



Figure 1


Figure 1. 

Transthoracic Doppler echocardiogram through the right ventricular outflow tract (RVOT), showing severe stenosis with peak and mean gradients of 100 and 63mmHg, respectively.

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Figure 2


Figure 2. 

A curved multiplanar reconstruction showing a conduit between the body of the upper right ventricle (RV) and the right pulmonary artery (PA), with severe calcification and proximal stenosis of the conduit (white arrows).

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Figure 3


Figure 3. 

Three-dimensional volume-rendered reconstruction demonstrating the right ventricle to right pulmonary artery (RPA) conduit (white arrow) and an enlarged left pulmonary artery (LPA) connected to the native right ventricular outflow tract (RVOT) (yellow arrow). The main pulmonary artery (PA) had an unusual shape and was narrowed at the bifurcation.

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In the presence of a contraindication to cardiac magnetic resonance imaging, such as pacemaker implantation, CCT provides superb image quality, permitting accurate, detailed evaluation of native RVOT and PA anatomy.

Disclosure of interest

The authors declare that they have no conflicts of interest concerning this article.


Appendix A. Supplementary data

Click here to download the file (3.9 Mo)
 Video 1 
Video 1. 

Transthoracic colour Doppler echocardiogram modified short-axis view, showing significant turbulence across the distal right ventricular outflow tract, pulmonary valve and main pulmonary artery, without delineation of the actual site of obstruction.



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