Brief overview
- What is aortic coarctation? Congenital narrowing of the main artery (aorta)
- Course of the disease and prognosis: After successful treatment of the malformation, the prognosis is very good.
- Causes: Maldevelopment of the aorta in the first weeks of embryonic development
- Risk factors: In some cases, aortic isthmus stenosis occurs in families. Sometimes in combination with other syndromes such as Turner syndrome.
- Diagnostics: Typical symptoms, cardiac ultrasound, X-ray if necessary, computer tomography, magnetic resonance imaging
- Treatment: Surgery (removal of the dilated section of the aorta and “end-to-end anastomosis”), bridging with a vascular graft or a plastic prosthesis, widening of the narrowed section of the vessel with a balloon and insertion of a stent (vascular support)
- Prevention: No preventive measures possible
What is aortic coarctation?
The constriction in the artery is an obstacle to blood flow: Sufficient blood no longer reaches the lower half of the body – abdominal organs and legs are insufficiently supplied with oxygen. Blood builds up in the left ventricle in front of the constriction and the heart has to pump harder to fight against the resistance. This leads to an enormous pressure load in the ventricle. As a result, it enlarges and thickens. Eventually, the first symptoms of heart failure appear. In addition, the blood pressure rises in the vessels that lie above the constriction and supply the head and arms.
Which symptoms occur depends on how far the aorta is narrowed and where exactly the narrowing is located. Some patients show no symptoms, while in others the aorta is so severely narrowed that a life-threatening condition quickly develops.
What is the ductus arteriosus botalli?
Before birth, the unborn child does not breathe through the lungs, but receives the oxygen it needs via the umbilical cord. As the lungs are not yet working, the blood largely bypasses the pulmonary circulation (which begins in the right heart, passes through the lungs and ends in the left atrium).
Forms of aortic coarctation
There are different forms of aortic coarctation stenosis. Doctors distinguish between “critical” and “non-critical” stenoses.
Non-critical aortic coarctation: In this form, the stenosis is located where the ductus arteriosus botalli opens into the aorta. The unborn child’s heart has already adapted to the increased resistance in the womb and blood flow to the legs is restricted. In order to maintain the blood supply, blood vessels are formed that bypass the constriction (collateral vessels). If the duct closes after birth, there are usually only mild symptoms. These may not develop until adulthood.
Frequency
Three to five percent of all congenital heart defects are aortic coarctation stenoses. Aortic coarctation affects around one in 3,000 to 4,000 newborns, boys are affected twice as often as girls.
In 70 percent of cases, aortic coarctation occurs as the only malformation of the heart, in 30 percent together with other heart defects such as a ventricular septal defect or a non-closing ductus arteriosus Botalli (persistent ductus arteriosus Botalli).
What is the life expectancy with aortic coarctation?
If aortic coarctation is recognized and treated in time, the prognosis is very good. After successful correction, life expectancy is comparable to that of the normal population. However, it is important to have regular check-ups: In some cases, the aorta narrows again despite successful treatment. Sometimes so-called aneurysms form on the aorta over time: the aorta expands like a balloon and, in the worst case, threatens to rupture.
In some cases, the increased blood pressure persists even after the operation. These patients receive lifelong antihypertensive medication.
What are the symptoms of aortic coarctation?
The symptoms of aortic coarctation depend on how severely and at what point the aorta is narrowed.
Symptoms of non-critical aortic coarctation stenosis
In non-critical aortic coarctation, the body has already adapted to the increased resistance in the vascular system in the womb. There are different symptoms depending on how narrow the aorta is:
Patients with only slight narrowing may show no or only mild symptoms. For example, they become tired very quickly.
If the narrowing is more pronounced, the following symptoms typically occur:
- High blood pressure in the upper half of the body: headaches, dizziness, nosebleeds, tinnitus
- Low or normal blood pressure in the lower half of the body: weakened pulse in the legs and groin, abdominal pain, limping, pain in the legs, cold feet
- Chronic pressure in the left ventricle: Affected children show signs of cardiac insufficiency one to two days after birth and then usually stabilize again.
Symptoms of critical aortic coarctation
Cause and risk factors
The cause of aortic coarctation is a maldevelopment of the aorta in the first weeks of embryonic development. It is not known how this occurs. In most cases, the malformation develops spontaneously.
Risk factors
Aortic isthmus stenosis occurs more frequently in some families. A genetic predisposition is probable, but has not yet been conclusively proven. The risk of aortic isthmus stenosis is slightly increased if it runs in families: If the mother herself is affected, the risk for direct offspring increases to five to seven percent, while the risk of recurrence for siblings is two to three percent.
In some cases, aortic isthmus stenosis occurs in combination with other congenital syndromes: for example, around 30 percent of all girls born with Turner syndrome suffer from a narrowing of the aortic isthmus. Patients with other diseases such as Williams-Beuren syndrome or neurofibromatosis are less frequently affected.
Examination and diagnosis
Physical examination
During the physical examination, the doctor looks for typical changes such as a heart murmur (often not audible until the end of the fourth week of life), bluish discoloration of the skin, rapid breathing or different blood pressure values in the arms and legs.
Heart ultrasound
Further examinations
If necessary, the doctor will carry out further examinations. These include, for example, an X-ray examination, computer tomography (CT) or magnetic resonance imaging (MRI).
Treatment
The treatment of aortic coarctation depends on its severity. Critical aortic coarctation always requires intensive medical treatment. Affected newborns are ventilated by machine and given medication to maintain vital functions. Prostaglandin E (PGE) keeps the ductus open and restores blood flow in a similar way to before birth. In addition, cardiac drugs such as dopamine help to strengthen the heart. The aim of drug treatment is to stabilize the small patient to such an extent that surgery can be performed within the first 28 days of life.
Surgery for aortic coarctation
Various procedures are available for the surgical correction of aortic coarctation. In newborns and infants, the doctor most frequently performs an end-to-end anastomosis: This involves cutting the aorta, removing the narrow section (resection) and reconnecting the two ends of the aorta (end-to-end anastomosis).
In adults, a so-called prosthetic interposition is the treatment of choice: the narrowed area of the aorta is bridged by a vascular graft or a plastic prosthesis.
In the interventional treatment of aortic coarctation, the aorta is not accessed via an operation, but via a cardiac catheter that is inserted into the aorta via the groin vein. After the doctor has dilated the narrowed area of the aorta using a balloon (balloon angioplasty), he places a small metal mesh tube (stent implantation). The stent keeps the vessel permanently open.
Prevention
Aortic coarctation is a congenital malformation of the aorta. There are no measures to prevent the disease. If aortic coarctation occurs frequently in the family, it is advisable to inform the doctors treating you in the event of pregnancy. They will examine the newborn to detect aortic coarctation and treat it at an early stage. Prenatal diagnosis of aortic coarctation (prenatal diagnostics) is difficult but also possible.
Author & source information
This text complies with the requirements of medical literature, medical guidelines and current studies and has been reviewed by medical professionals.
