Broken aortic arch
| Classification according to ICD-10 | |
|---|---|
| Q25.2 | Atresia of the aorta |
| ICD-10 online (WHO version 2019) | |
The interrupted aortic arch is one of the rarest congenital heart or vascular malformations . This is a special form of coarctation of the aorta . There is no connection between the initial part of the aorta and the part that supplies the lower half of the body, called the aortic arch . As a rule, this heart defect does not appear as the only malformation , but is associated with other malformations, e.g. B. a ventricular septal defect (VSD = hole in the heart septum) or a more open ductus arteriosus Botalli combined.
Effects
Due to the interruption of the blood flow in the aorta, the lower half of the body is not supplied with blood. Another heart defect must also exist so that the body, in particular the liver , kidneys and gastrointestinal tract can be supplied . The left ventricle pumps against the interruption with enormous pressure and the outlet is then e.g. B. the VSD. The lungs are flooded and the blood quickly returns to the left ventricle. Both the lungs and the heart are in a constant state of stress and these organs eventually become overloaded.
If there is no VSD, but the circulation only depends on the ductus arteriosus, which is necessary prenatally, the children will go into shock when this connection closes in the first hours, days or weeks of life. They can then die quickly if not acted on in time.
Diagnosis
- The clinical examination is unspecific and ambiguous. The blood pressure in the legs may be lower than that in the arms.
- The echocardiography normally shows all malformations.
- When X-ray you can see the increased blood flow through the lungs and usually an enlarged heart.
- A cardiac catheter examination can be dispensed with in clear cases. Nevertheless, because of the other malformations and in view of the operation , the indication should be given generously.
therapy
The therapy consists initially of the administration of prostaglandin , a drug that can keep the ductus arteriosus open or reopen it. The operation must then be done as soon as possible.
- Two-stage procedure during the operation : The connection of the interrupted aortic arch is created, depending on the severity of the interruption, either by connecting the existing aortic parts or using a plastic prosthesis ( patch ). In addition, the pulmonary artery is limited in its outflow with a ribbon ( pulmonary banding ). As with coarctation of the aorta, this operation can becarried outwithout the use of the heart-lung machine , and the child can be stabilized through this relatively "small intervention". In a second operation, the remaining heart defects must be operated on at a later point in time. The plastic prosthesis does not grow with the child and must be replaced as the child develops.
- A one -step procedure for the operation is now also carried out in view of improved intensive medical care and technology. With the help of the heart-lung machine, all malformations are corrected during an operation.
Results
Overall, the results are equally good after both the one-step and the two-step approach. Due to the complex heart defect, there is still a worldwide mortality rate of around 30 to 50% (2004). After the correction (as with coarctation of the aorta ), constrictions can form again at the surgical sites, which necessitate renewed operations or balloon dilatations with the cardiac catheter or stent implantation . Follow-up treatment can be expected in around 50% of patients over the next 10 years. Lifelong follow-up care is required, as is compliance with endocarditis prophylaxis .
Adults with a disrupted aortic arch
There are teenagers and adults with this malformation. In them, the ductus arteriosus has closed so slowly that bypass circuits could form. The affected people have the same symptoms as those with high-grade coarctation of the aorta .
