Anomalous left coronary artery from the pulmonary artery
What is Bland-White-Garland syndrome?Typical of Bland-White-Garland syndrome is that the left artery, unlike the one shown here, emanates from the pulmonary artery.
© Sebastian Kaulitzki - stock.adobe.com
Bland-White-Garland syndrome was named after Bland, a specialist in cardiology, as well as White and Garland. Basically, Bland-White-Garland syndrome is a very rare disease. The incidence amounts to only 0.5 percent of all heart defects that are innate. Thus, the prevalence is about 1: 25, 000 people.
The Bland-White-Garland syndrome may also be referred to by the internationally recognized abbreviation ALCAPA. The disease has a defective origin of the left coronary artery. This is erroneously caused by the pulmonary artery. Due to the malformation in the structure of the heart, patients suffering from Bland-White-Garland syndrome often develop so-called myocardial ischaemia at an early age.
In addition, the vast majority of affected persons suffers from a weakness of the heart muscle, which also already results relatively early. As a result of the malformation, the blood flows from the right into the left coronary artery and finally into the pulmonary artery. As a rule, surgical intervention may be considered to correct the Bland-White-Garland syndrome.
Bland-White-Garland Syndrome is a congenital defect in the anatomy of the heart. For this reason, genetic causes may be involved in the development of Bland-White-Garland syndrome. Here are mutations on genes in question that trigger the disease.
The corresponding artery connects to the pulmonary artery, which carries low-oxygen blood. As a result, there is a long-term undersupply of the organism with oxygen by the Bland-White-Garland syndrome. The myocardium also requires a high level of oxygen saturation in the blood.
However, as this is not achieved, there are health problems. If there is no adequate treatment for Bland-White-Garland syndrome, the disease will be fatal for nearly 80 percent of all patients within the first year of life.
Symptoms, complaints & signs
The Bland-White-Garland syndrome includes several signs of disease and discomfort that may be a significant threat to the lives of patients. On the one hand, people affected by Bland-White-Garland syndrome are more likely to suffer from infectious diseases than healthy people. Another typical sign of Bland-White-Garland syndrome is cyanosis, which occurs as a result of oxygen deficiency in the body.
In the vast majority of cases, patients develop heart failure as part of Bland-White-Garland syndrome. This insufficiency manifests itself, for example, in a reduction in physical capacity, excessive sweat production and dyspnea. The Bland-White-Garland syndrome may also cause heart rhythm disorders.
In addition, some of the affected patients have problems with the blood circulation. Infarcts or ischemia of the myocardium, for example, are associated with this. Often, the typical complaints of Bland-White-Garland syndrome occur within the first half year after the birth of the patients.
Diagnosis & History
The diagnosis of Bland-White-Garland syndrome relies primarily on the clinical symptoms of the disease. First of all, a thorough medical history is taken with the patient or responsible guardian. In some cases, some symptoms appear shortly after the birth of the affected baby and raise the suspicion of a heart defect.
The range of diagnostic methods for the diagnosis of Bland-White-Garland syndrome is large. As a rule, several methods are used to ensure the diagnosis. First, an ECG scan is performed to detect myocardial left ventricular ischemia. In addition, the malformations of Bland-White-Garland syndrome are detected in this study.
In addition, X-ray images of the thorax are made, whereby a so-called cardiomegaly is visible. There are also indications of vascular congestion in the lungs. The pumping function on the left is reduced and patients also suffer from typical mitral valve insufficiency. In some cases, cardiac catheterization is also used to help diagnose Bland-White-Garland syndrome.
Since Bland-White-Garland Syndrome already occurs in infancy, it can lead to dangerous situations. If the syndrome is not treated, it usually comes to death. In most cases, patients are very susceptible to infectious diseases. Because of this, life expectancy is reduced for patients with Bland-White-Garland syndrome.
Those affected also suffer from heart failure, so certain heavy work or stress can not be performed. The patient is thereby restricted in his daily activities and can perform no special sports activities. Often it comes by a heavy physical stress to sweats.
Because the sufferers suffer from heart problems, the risk of a heart attack is greatly increased. The infarction can occur even in childhood and in the worst case lead to death. Treatment must be carried out promptly and is aimed primarily at correcting the heart defects. Failure to do so will result in Bland-White-Garland syndrome death.
It can not be predicted whether there will be other complications as the patient progresses. As a rule, parents also suffer greatly from Bland-White-Garland syndrome from depression and other mental health problems.
When should you go to the doctor?
In most cases, Bland-White-Garland syndrome is detected and diagnosed before birth or immediately after birth. For this reason, in most cases no visit to the doctor is necessary for the diagnosis. As a rule, treatment for Bland-White-Garland syndrome also takes place directly after birth. However, a doctor should always be consulted when it comes to the sufferer due to the disease to a shortage of oxygen.
Even with a reduced load capacity or with a permanent fatigue of the patient a medical examination is necessary. In general, due to Bland-White-Garland syndrome, circulation problems should always be checked by a physician. Similarly, heart problems and increased perspiration may indicate this condition.
The diagnosis can be made by a cardiologist or in a hospital. However, the treatment itself is performed through surgery. The earlier the operation is initiated, the higher the chance of survival of the person affected due to the Bland-White-Garland syndrome.
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Treatment & Therapy
The options for treating Bland-White-Garland syndrome are limited. In many cases, the ill people without a timely and successful therapy are unable to survive. It is only possible to correct the Bland-White-Garland syndrome and the associated malformations in the structure of the heart by surgical interventions.
In view of the poor chances of survival without therapeutic intervention, such operations are often almost without alternative. As part of the surgical procedure, the patients are connected to a heart-lung machine. In the first step, the corresponding coronary artery, which comes from the pulmonary artery, is separated.
The pulmonary artery is sealed using a special pericardial tissue. Thereafter, the coronary artery is applied to a portion of the ascending aorta. If the affected patient suffers from mitral valve deficiency, a correction usually occurs during the same operation.
Outlook & Forecast
The prognosis of Bland-White-Garland syndrome is considered unfavorable. The mortality rate is very high and also due to the disease numerous complaints that can not be cured. The cause of the syndrome is currently considered uncertain. Various research results indicate that the disease is genetically determined. Since an intrusion into human genetics is prohibited for legal reasons, it makes it difficult for scientists and researchers to take appropriate measures.
The treatment plan provides relief of the existing symptoms. At the same time an attempt is made to extend the life expectancy of the patient. Without medical care, the newborns die within a short time in almost all cases.
Once the patient is in a stable state of health, surgery is performed. This is where corrections of malformations of the heart take place. The procedure requires intuition and is considered a challenge. This measure increases the chance of survival to a considerable extent.
In particular, slight malformations can be corrected during surgery and allow a significant improvement in health. Nevertheless, a prognosis is only possible individually depending on the degree of manifestation of the Bland-White-Garland syndrome. The undersupply of the organism to oxygen is then remedied in various treatment steps.
In regular check-ups the heart activity as well as possible circulatory disturbances are checked and treated. In some patients, the heart must be monitored permanently.
Bland-White-Garland syndrome is a congenital disorder that accompanies heart malformations. Therefore, no possibilities for prevention are known.
You can do that yourself
People with the diagnosis of Bland-White-Garland syndrome should be especially careful. Because for the Bland-White-Garland syndrome, there is no conservative therapy. An operative procedure is necessary to connect the left coronary artery with the aorta. For patients, avoid physical exertion.
Nevertheless, one should not completely give up movement. Easy regular exercise and control of health parameters by a cardiologist are necessary and useful. Because as soon as the diagnosis is made, it is necessary to maintain the state of health at least until the surgical procedure, if not to improve it.
Therefore, patients should pay attention to their diet. An optimal supply of all nutrients and a healthy diet are very important. It can cause to maintain or even improve the current state of health. For this reason, foods such as fresh fruits, vegetables, fish and high quality oils are on the menu. The fats should be kept relatively low and meat should not be more often than once or twice a week on the menu.
In addition, a low-salt diet is important and adequate drinking of water and unsweetened teas. The amounts of the individual components should be discussed in advance with the attending physician. Those who still sleep enough stay as healthy as possible until the final treatment.