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Specialists in the Field of Coarctation (ISTA)
Information About the Field of Coarctation (ISTA)
What is coarctation of the aorta?
Coarctation of the aorta refers to a stricture of the aorta, right after it leaves the heart. Due to the stricture of the blood vessel, called stenosis, the lower body half is supplied with less blood than the upper half, as the vessels responsible for the upper half branch off before the stenotic part of the aorta and therefore are not affected the stricture directly. However, high blood pressure in the upper and low blood pressure in the lower body half can frequently be measured.
Coarctation of the aorta is a congenital heart defect that makes up approximately 5% of all inborn heart defects. It affects boys twice as much as girls.
Which forms are there?
There are two main forms of coarctation of the aortic isthmus. First, there is the non-critical form, that often appears during adolescence or adulthood and frequently shows a low-grade stenosis. Secondly, there is the critical form, which appears in infants within days after birth and must be treated immediately as it is so severe that it can be fatal.
Coarctation of the aorta in infants
Babies with high-grade coarctation of the aorta often present with poor drinking, fast heart rate and breathing and a grayish skin color for the first few days after birth. Usually, these symptoms do not appear until a few days later, as right after birth the the two large vessels leaving the heart are still connected. It is through this connection, known as the ductus arteriosus, that the constriction can be bypassed to deliver sufficient blood to the lower half of the body. However, this bridge between blood vessels closes during the first few days of life leading to reduced blood flow to lower parts of the body. Consequently, not only the heart but also other organs like the kidneys begin to fail and these babies require urgent treatment.
Coarctation of the aorta in adolescents and adults
During later stages of life, coarctation of the aorta usually draws attention due to hypertension of the upper limbs. Other symptoms may appear as well, including headaches, cold pale legs and recurring pain in the calves called " claudication".
Over the course of life, stenosis stresses the heart severely, particularly the left ventricle which must continue to pump against a greater resistance. This strain of pressure can cause damage to the heart. Also, the constant high blood pressure of the upper body can bring about dangerous complications. For instance, it is associated with an increased risk of brain bleeding .
How is coarctation of the aorta diagnosed?
If coarctation of the aorta is suspected, the first step is a blood pressure measurement of the upper and lower limbs. This often reveals significant differences. However, these differences between upper and lower extremities may not show in many patients, as multiple circulations to bypass the stenosis will have formed over the course of a person’s life. This way, the lower body half can still be supplied with enough blood.
The standard for infants is an echocardiography , which is an ultrasound examination of the heart, is performed. Adolescents and adults mostly undergo CT or MRI examinations aimed to illustrate the path of blood vessels. This is referred to as CT or MRI angiography.
Therapy of coarctation of the aorta
The most important goal of therapy is to eliminate the stenosis and establish a normal diameter of the aorta which can be reached in one of two ways. One option is surgery, which involves removing the stenotic part and replacing it with a vascular prosthesis. The other way is carrying out an interventional procedure which is less invasive and aims to widen the coarctation of the aorta and using a balloon catheter and subsequently introducing a stent.
Neonates with critical stage coarctation of the aorta are preferably treated with surgery. Until their surgery, this group of patients is also administered “prostaglandin”, a medication that keeps the connection between the great vessels open to ensure adequate blood supply to the lower body half.
Also teenagers and adults can be treated with surgery, however in most cases their stenotic part is opened using the balloon catheter and stent. This method can also be carried out following a surgery, in case there is a recurrence of the stenosis. Older children benefit from this method in one more way. They can receive a stent that can be further dilated later on in order to adjust it to the growth of the child. As a result, repeated surgery may be delayed and in the best case scenario be completely avoided.
Life expectancy and prognosis of coarctation of the aorta
If left untreated or if inadequately treated, life expectancy is reduced and physical performance is limited.
After successful therapy, life expectancy and resilience for example in sports is not limited compared to healthy people. Control check-ups are still mandatory, though, as they serve to monitor the success of treatment. These examinations include blood pressure measurements, echocardiography, stress tests and potentially MRI and CT exams.
These control check-ups are used as a tool to monitor potential complications or secondary diseases that may arise.
For example, the aorta can become constricted again or problems with heart valves or chambers may be the result. There is also an increased risk of coronary heart disease , damage to the eyes or heart attacks . The increased blood pressure can persist even after the therapy and can then be managed with medications such as beta-blockers.
Which doctors and clinics are specialists for coarctation of the aorta?
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