Aorto-pulmonary window
| Classification according to ICD-10 | |
|---|---|
| Q21.4 | Aortopulmonary septal defect. Aortopulmonary window |
| ICD-10 online (WHO version 2019) | |
The aorto-pulmonary window or aortopulmonary window is one of the rarest congenital malformations of the cardiovascular or vascular system. It is also called "aorto-pulmonary septal defect". It is a defective separation between the top portion of the large body artery ( aorta ascendens) and the pulmonary trunk (the trunk of the pulmonary artery or pulmonary artery ). Based on the exact location and extent of the defect, a basic distinction is made between four different types of aorto-pulmonary window. The fact that the aorta and the pulmonary artery have two different valves distinguishes this heart defect from the common arterial trunk .
Associated malformations
The more common heart malformations that can occur with an aorto-pulmonary window include an interrupted aortic arch , the ventricular septal defect , the atrial septal defect , a persistent ductus arteriosus , a failure of the right pulmonary artery from the aorta, or a Fallot tetralogy . More rarely, the aorto-pulmonary window is associated with a transposition of the large arteries , coronary anomalies, or a single ventricle .
consequences
The incorrect separation of the vessels and the approximately five times higher pressure in the body circulation than in the pulmonary circulation result in a considerable increase in blood flow to the pulmonary vessels. Clinically, the children then suffer from dyspnea (shortness of breath) and breathe quickly (tachypnea). You are conspicuous by bronchitis or asthma-like symptoms and the stress on the heart is noticeable through sweating, rapid fatigue and a lack of weight gain. If the aorto-pulmonary window remains in place for too long, there is a risk of pulmonary hypertension (high pulmonary pressure).
Diagnosis
- Echocardiography as the non-invasive examination of first choice
- X-rays show the increased blood flow through the lungs and most often an enlarged heart
- EKG does not give any clear indications for the heart defect
- Cardiac catheters with a clear clinical and echocardiographic finding could be dispensed with. However, since the heart defect is often associated with other malformations, the indication should be given generously, especially with regard to the operation.
therapy
The operation with the help of the heart-lung machine should be performed quickly after the diagnosis. The basic procedure is to separate the vessels and seal them with a patch . Depending on the severity of the malformation, there are several surgical options that must be decided individually. Overall, the operative results after an operation are very good. If there are other malformations, the state of health results from the summary of all heart defects.
However, since the heart defect is very rare, no center can look back on large numbers of cases.
Long-term expectations
The results after a correction are usually very good, but of course depend on whether there are other malformations. Lifelong check-ups are necessary, as is compliance with endocarditis prophylaxis .
Adults with aorto-pulmonary window
Amazingly, an unoperated aorto-pulmonary window also occurs in adolescents or adults. Either the heart defect was not diagnosed if the symptoms were initially mild or an early operation was not performed. Since curative treatment is not possible due to the fixed pulmonary hypertension, the possibility of a lung transplant must be discussed. Overall, however, this group of patients is the very big exception.
literature
- Charlotte Pia Kaiser: The fetal atrioventricular septal defect - associated findings and course in 246 cases, © Universitäts- und Landesbibliothek Bonn July 8, 2010, DNB 1008227293 , OCLC 669794963 (Dissertation University of Bonn, Medical Faculty, February 19, 2010, 40 pages, 1 Reviewer: Christoph Berg, 2nd reviewer: Peter Bartmann, full text - online PDF, free of charge, 40 pages, 4 MB).
