Discussion
Pseudocoarctation of the aorta is a very rare congenital anomaly of the
aorta, that causes elongation, stenosis, twisting, and kinking of the
aorta at the isthmus level as a result of compression of the 3rd and 7th
dorsal aortic segments during the embryological period1. The mean age at diagnosis is 43, and it is observed
with equal frequency in men and women 1. The etiology
of aortic pseudocoarctation is unknown This anomaly has also been
associated with familial conditions like chromosomal abnormalities
(18p−/18q+, Turner, Noonan, and Hurler) and congenital heart diseases
such as the bicuspid aorta 2. There are very few cases
reported worldwide of aortic pseudocoarctation. The last systematic
review published in 2015 ffound that there have been a at least 18
instances during the previous twenty years 1. It has
been reported that approximately half of these patients present with
hypertension, the rest with symptoms such as dyspnea and dysphagia due
to lung and esophageal compressions, and abdominal pain due to aortic
dissection 1.
Gay and Young defined diagnostic criteria in 1969 and include the
abnormal posteroanterior chest roentgenogram, the absence of an
upper/lower extremity pressure difference, no signs of increased
collateral circulation, and definitive aortogram images3. Nowadays, the initial test for diagnosis is usually
echocardiography due to its easy accessibility and important in
evaluation of descending aorta and associated congenital defects. Also,
chest X-ray is a simple test that can help in the diagnosis. The ’3
sign’ typical for aortic coarctation and notching of the ribs are not
seen in pseudocoarctation patients 4. Although there
is no clear definition of chest X-ray findings in the literature, It has
been reported in some case reports that the ’pseudo 3 sign’ and buckling
of the aorta may be seen 4,5. CT angiography and MRI
angiography are important imaging modalities to evaluate the narrowed
segment of the aorta and to rule out associated aortic aneurysm or
aortic dissection 1. Cardiac catheterization is the
gold standard for accurate measurement of pressure gradient if
diagnostic uncertainty exists.
Aortic pseudocoarctation and coarctation are in the same disease
spectrum, and their clinical presentations differ depending on whether
there is hemodynamically significant stenosis in the descending aorta or
not. While there are significant gradient-related hemodynamic results in
the aortic segment, such as the difference in blood pressure between the
upper and lower extremities, inability to palpate lower extremity
peripheral pulses, and delayed radiofemoral pulse in true coarctation,
these findings are not observed in pseudocoarctation. The clinical
findings in our patient, especially the absence of pulse delay and blood
pressure difference between the four extremities, suggested that king
and elongated aortic segment not caused significant blood flow
obstruction in desendan aorta. This hypothesis was supported by the
absence of collateral circulation in thoracic CT angiography and the
absence of a significant gradient in the aortic catheterization study.
In conclusion, we diagnosed our patient with aortic pseudocoarctation.
Specific guidelines on the management of pseudocoarctation are lacking.
Conservative management of pseudocoarctation, which does not cause
significant hemodynamic stenosis and aneurysm formation, is recommended1,6. Unnecessary surgical interventions should be
avoided in these patients. However, the presence of a pseudoaneurysm or
aortic aneurysm of aorta adjacent to pseudocoarctation, which carries a
high risk of rupture, requires prompt intervention1,7. Our patient’s aortic diameters were within normal
limits, there was no aneurysmal enlargement. Antihypertensive treatment
of our patient with hypertension, bicuspid aortic valve and
pseudocoarctation was adjusted to a blood pressure target of
<130/80 mmHg. Avoidance of isometric exercise with high static
load, annual follow-up, and bicuspid aortic valve screening for
first-degree relatives were recommended.