When anomalous left coronary artery from the pulmonary artery is a malformation of the coronary arteries. The disease exists from birth. Typical of Bland-White-Garland syndrome is that the left artery starts from the pulmonary artery. As a result of the disease, the affected patients usually develop myocardial ischemia as early as infancy. The Bland-White-Garland syndrome can only be treated with surgery.
What is Bland-White-Garland Syndrome?
According to theinternetfaqs.com, the Bland-White-Garland syndrome got its name after Bland, a specialist in cardiology, as well as White and Garland. Basically, Bland-White-Garland syndrome is a very rare disease. The incidence is only 0.5 percent of all heart defects that are innate. The prevalence is thus around 1: 25,000 people.
The Bland-White-Garland Syndrome is sometimes also referred to by medical professionals with the internationally accepted abbreviation ALCAPA. As part of the disease, there is an incorrect origin of the left coronary artery. This erroneously arises from the pulmonary artery. Due to the deformity in the structure of the heart, so-called myocardial ischemia develops in numerous cases in patients with Bland-White-Garland syndrome at a young age.
In addition, the majority of those affected suffer from a weakness of the heart muscle, which also occurs relatively early. As a result of the malformation, the blood flows from the right to the left coronary artery and finally flows into the pulmonary artery. Surgical interventions are usually considered to correct Bland-White-Garland syndrome.
Causes
Bland-White-Garland syndrome is a congenital defect in the anatomy of the heart. For this reason, there is a possibility that genetic causes are involved in the development of Bland-White-Garland syndrome. Mutations in genes that trigger the disease come into question.
The corresponding artery connects to the pulmonary artery, which transports deoxygenated blood. As a result, in the long term, the organism is insufficiently supplied with oxygen due to the Bland-White-Garland syndrome. The myocardium also needs a high level of oxygen saturation in the blood.
However, since this is not achieved, health problems arise. If there is no adequate therapy for Bland-White-Garland syndrome, the disease ends fatally for almost 80 percent of all sick patients within the first year of life.
Symptoms, ailments & signs
Bland-White-Garland syndrome comprises several signs and symptoms that can pose a significant risk to the life of the patient. On the one hand, people affected by Bland-White-Garland syndrome are characterized by the fact that they suffer from infectious diseases more often than healthy people. Another typical sign of Bland-White-Garland syndrome is cyanosis, which occurs as a result of the body being insufficiently supplied with oxygen.
In the majority of cases, patients develop heart failure as part of Bland-White-Garland syndrome. This insufficiency manifests itself, for example, in a decrease in physical resilience, excessive sweat production and dyspnea. Bland-White-Garland syndrome may also cause cardiac rhythm disorders.
In addition, some of the affected patients have problems with their blood circulation. This is associated, for example, with infarcts or ischemia of the myocardium. The typical symptoms of Bland-White-Garland syndrome often appear within the first six months of the patient’s birth.
Diagnosis & course
The diagnosis of Bland-White-Garland syndrome is based primarily on the clinical symptoms of the disease. First, a thorough anamnesis is carried out with the sick patient or the responsible custodian. In some cases, some symptoms show up shortly after the baby is born, suggesting a heart defect.
There is a large selection of diagnostic methods for diagnosing Bland-White-Garland syndrome. Usually several procedures are used to secure the diagnosis. First, an EKG examination is carried out, whereby the left ventricular ischemia of the myocardium can be detected. In addition, the malformations of the Bland-White-Garland syndrome are identified as part of this examination.
In addition, X-rays are made of the thorax, whereby a so-called cardiomegaly becomes visible. There are also indications of vascular congestion in the lungs. The pumping function on the left hand is reduced, and the patients also suffer from typical insufficiency of the mitral valves. In some cases, a cardiac catheter examination is also ordered to confirm the diagnosis of Bland-White-Garland syndrome.
Complications
Since Bland-White-Garland syndrome occurs in infancy, it can lead to dangerous situations. If left untreated, the syndrome usually results in death. In most cases, the patients are very susceptible to infectious diseases. For this reason, life expectancy is reduced for people with Bland-White-Garland syndrome.
Those affected also suffer from a weak heart, so that certain heavy work or loads cannot be carried out. The patient is thereby restricted in his daily activities and cannot carry out any particular sporting activities. Often times, heavy physical exertion leads to sweating.
Because people have heart problems, the risk of a heart attack is greatly increased. The infarct can occur in childhood and in the worst case lead to death. Treatment must be carried out immediately and is primarily aimed at correcting the heart defects. Failure to do this will result in death from Bland-White-Garland syndrome.
It cannot be predicted whether there will be other complications in the further course of the patient. As a rule, the parents also suffer a lot from depression and other psychological complaints due to Bland-White-Garland syndrome.
When should you go to the doctor?
In most cases, Bland-White-Garland syndrome is recognized and diagnosed before birth or immediately after birth. For this reason, in most cases a visit to the doctor is not necessary for the diagnosis. As a rule, Bland-White-Garland syndrome is treated immediately after the birth. However, a doctor should always be consulted if the patient suffers from an insufficient supply of oxygen due to the disease.
A medical examination is also necessary in the event of reduced resilience or if the patient is permanently exhausted. In general, due to Bland-White-Garland syndrome, circulatory discomfort should always be checked by a doctor. Heart problems and increased sweat production can also indicate this condition.
Diagnosis can be made by a cardiologist or in a hospital. The treatment itself, however, is carried out through a surgical procedure. The earlier the operation is started, the higher the chances of survival for the person with Bland-White-Garland syndrome.
Treatment & Therapy
Options for treating Bland-White-Garland syndrome are limited. In numerous cases, the sick people are unable to survive without timely and successful therapy. There is only the possibility of correcting the Bland-White-Garland syndrome and the associated malformations in the structure of the heart through surgical interventions.
In view of the poor chances of survival without therapeutic intervention, there are often almost no alternatives to such operations. As part of the surgical procedure, the patients are connected to a heart-lung machine. In the first step, the corresponding coronary artery that comes from the pulmonary artery is cut off.
The pulmonary artery is sealed with the help of a special pericardial tissue. The coronary artery is then attached to part of the ascending aorta. If the affected patient suffers from mitral valve insufficiency, correction is usually carried out during the same operation.
Outlook & forecast
The prognosis for Bland-White-Garland syndrome is considered unfavorable. The death rate is very high and the disease also causes numerous complaints that cannot be cured. The cause of the syndrome is currently uncertain. Various research results suggest that the disease is genetic. Since interference with human genetics is prohibited for legal reasons, it makes it difficult for scientists and researchers to take appropriate measures.
The treatment plan provides relief from the symptoms that are present. At the same time, attempts are made to extend the patient’s life expectancy. Without medical care, the newborns die within a short time in almost all cases.
As soon as the patient is in a stable state of health, an operation is carried out. This is where the malformations in the heart are corrected. The procedure requires tact and is considered a challenge. This measure increases the chance of survival to a considerable extent.
In particular, slight malformations can be corrected during an operation and enable a significant improvement in health. Nevertheless, a prognosis is only possible individually depending on the severity of the Bland-White-Garland syndrome. The insufficient supply of oxygen to the organism is then remedied in various treatment steps.
The heart’s activity and possible circulatory disorders are checked and treated in regular check-ups. In some patients, the heart needs constant monitoring.
Prevention
Bland-White-Garland syndrome is a congenital disease that is associated with deformities of the heart. Therefore, there are no known ways to prevent it.
You can do that yourself
Most of all, people diagnosed with Bland-White-Garland Syndrome should take it easy. There is no conservative therapy for Bland-White-Garland syndrome. Surgical intervention is necessary to connect the left coronary artery to the aorta. Patients should avoid physical exertion.
Nevertheless, one should not completely do without exercise. Light regular exercise and the control of health parameters by a cardiologist are necessary and sensible. Because as soon as the diagnosis is made, it is important to at least maintain the state of health until the surgical intervention, if not to improve it.
Therefore, patients should also pay attention to their diet. An optimal supply of all nutrients and a heart-healthy diet are very important. It can help maintain or even improve your current state of health. For this reason, foods such as fresh fruit, vegetables, fish and high-quality oils should be on the menu. The fats should be kept relatively low and meat should not be on the menu more than once or twice a week.
In addition, it is important to have a low-salt diet and drink enough water and unsweetened teas. The quantities of the individual components should be discussed in advance with the attending physician. Those who get enough sleep will stay as healthy as possible until the final treatment.
