PMC4367175_01
Chest X-ray with prominent right cardiac border secondary to dilatation of the ascending aorta (double arrow) and absent aortic knob (arrow).
fped-03-00019-g001.jpg
PMC4367175_01_fped-03-00019-g001.jpg
F1
The chest radiography (Figure 1) showed mild cardiomegaly (ICT 0.64) and an absent aortic knob, compatible with the diagnosis of coarctation.
Long axis parasternal view showing doming of the aortic valve, aliaising of flow secondary to stenosis, and left ventricular hypertrophy. Short axis view showing bicuspid aortic valve. Abbreviations: AoV, aortic valve; LA, left atrium; LV, left ventricle; RV, right ventricle.
fped-03-00019-g002.jpg
PMC4367175_01_fped-03-00019-g002.jpg
F2
The echocardiography showed a bicuspid aortic valve (Figure 2) with severe stenosis (peak systolic gradient of 60 mmHg and mean of 30 mmHg), left ventricular hypertrophy (Z-score +3.3), preserved systolic and diastolic function (mitral E/A ratio 1.6; mitral A deceleration time 0.10 s, E/E' 22 secondary to increased left heart filling pressure), and tortuous aortic arch with narrowing and acceleration of flow at the aortic isthmus (Figure 3).
Suprasternal view of the aortic arch with severe narrowing of the aortic isthmus and aliasing of flow. Abbreviations: Asc Ao, ascending aorta; Desc Ao, descending aorta.
fped-03-00019-g003.jpg
PMC4367175_01_fped-03-00019-g003.jpg
F3
The echocardiography showed a bicuspid aortic valve (Figure 2) with severe stenosis (peak systolic gradient of 60 mmHg and mean of 30 mmHg), left ventricular hypertrophy (Z-score +3.3), preserved systolic and diastolic function (mitral E/A ratio 1.6; mitral A deceleration time 0.10 s, E/E' 22 secondary to increased left heart filling pressure), and tortuous aortic arch with narrowing and acceleration of flow at the aortic isthmus (Figure 3).
MR angiography, volume rendering 3D reconstruction. The arrow indicates the atretic aortic arch, diverticulums on either side of the zone of discontinuity suggests the presence of a fibrous strand at that position, compatible with the diagnosis of atresia. There are numerous collaterals such as both mammary arteries, intercostals arteries, and posterior mediastinal arteries. There was hypoplasia of the horizontal aorta and gothic aspect of the ascending aorta.
fped-03-00019-g004.jpg
PMC4367175_01_fped-03-00019-g004.jpg
F4
There was no patent ductus arteriosus (PDA), no associated endofibroelastosis and the descending aorta was fed exclusively by massive systemic collateral circulation (Figures 4-6).
MR angiography with MIP reconstruction of the atretic aortic arch (5 mm) indicated by the arrow with dilatation (33 mm) of the ascending aorta, secondary to bicuspid aortic valve.
fped-03-00019-g005.jpg
PMC4367175_01_fped-03-00019-g005.jpg
F5
There was no patent ductus arteriosus (PDA), no associated endofibroelastosis and the descending aorta was fed exclusively by massive systemic collateral circulation (Figures 4-6).
MR angiography, volume rendering 3D reconstruction. Posterior view of the aortic arch before surgery.
fped-03-00019-g006.jpg
PMC4367175_01_fped-03-00019-g006.jpg
F6
There was no patent ductus arteriosus (PDA), no associated endofibroelastosis and the descending aorta was fed exclusively by massive systemic collateral circulation (Figures 4-6).
MR angiography, volume rendering 3D reconstruction. Posterior view of the aortic arch after surgery.
fped-03-00019-g007.jpg
PMC4367175_01_fped-03-00019-g007.jpg
F7
The post-operative CMR 1 month after surgery showed good surgical result with patency of the aortic isthmus (Figure 7), and there was no differential in blood pressure between the upper and lower limbs.