Accuracy of two-dimensional echocardiography in the diagnosis of aortic arch obstruction

Abstract

To evaluate the predictive accuracy of two-dimensional echocardiography in the diagnosis of aortic arch obstruction, 540 consecutive patients aged 2 days to 15 years (mean 2 months) who underwent subsequent cardiac catheterization and angiography were prospectively studied. At angiography, 51 patients had aortic arch obstruction; of these, 35 had juxtaductal coarctation, 15 isthmic hypoplasia and 1 a type B interrupted aortic arch. The presence of arch obstruction was correctly identified with two-dimensional echocardiography in 45 of 51 patients with this condition (overall sensitivity 88%). Two-dimensional echocardiography clearly defined a juxtaductal coarctation in 33 of 35 patients and isthmic hypoplasia in 13 of 15 patients (sensitivity 94% and 73%, respectively). The form and type of interrupted aortic arch were clearly distinguished from other forms and types of arch obstruction. Among the 489 patients without aortic arch obstruction, two-dimensional echocardiography wrongly diagnosed the presence of such obstruction in 9 patients (overall specificity 98%). Forty-six (92%) of the 51 patients had at least one associated intracardiac abnormality. Twenty-two (44%) had a ventricular septal defect, 21 (42%) a bicuspid aortic valve and 4 (18%) subaortic stenosis. Five patients had complex congenital cardiac malformations. All associated abnormalities were prospectively identified with two-dimensional echocardiography. Thus, two-dimensional echocardiography is highly specific in diagnosing aortic arch obstruction. It is less sensitive for the diagnosis of isthmic hypoplasia in the neonatal period.

An unexpected finding late after repair of coarctation of the aorta

“…3,4 This malformation occurs up to five times more frequently in males than females and is associated with ventricular septal defect (30 to 45%), bicuspid aortic valve (30 to 85%), patent ductus arteriosus, mitral stenosis and aortic stenosis (valvular, subvalvular, or supravalvular). 5,6 Intracerebral aneurysms and Turner's syndrome are extracardiac defects associated with coarctation of the aorta. 2 Significant CoA is defined as a gradient of more than 20 mmHg with or without proximal hypertension or the presence of (any degree of) coarctation associated with proximal hypertension.…”

Validation of two echocardiographic indexes to improve the diagnosis of complex coarctations

“…Associated anomalies of the aortic arch with isolated coarctation, in particular displacement of the left subclavian artery and narrowing of the distal transverse arch, have been widely described [4][5][6][7][8][9][10]. Their presence, independent of haemodynamic repercussions of the coarctation, has not been used for the diagnosis until recently.…”

Multislice computed tomography and X-ray correlation of the classical secondary vascular adaptive features associated with aortic coarctation

“…There are a variety of cardiac abnormalities which present more commonly in patients with coarctation, such as bicuspid aortic valve, ventricular septal defect and patent ductus arteriosus [4] . In the setting of aortic coarctation there are secondary or noncardiac vascular abnormalities that are present especially in cases of long standing disease in the absence of any treatment.…”