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.