Bland-White-Garland syndrome is a malformation of the coronary arteries. The condition exists from birth. Bland-White-Garland syndrome is typically characterized by the left artery originating from the pulmonary artery. As a result of the disease, myocardial ischemia usually forms in affected patients in infancy. Bland-White-Garland syndrome is treatable only by surgical intervention.
What is Bland-White-Garland syndrome?
Bland-White-Garland syndrome got its name from Bland, a specialist in cardiology, and White and Garland. Basically, Bland-White-Garland syndrome is a very rare condition. The incidence is only 0.5 percent of all defects of the heart that are congenital. Thus, the prevalence is approximately 1 in 25,000 individuals. Bland-White-Garland syndrome is sometimes referred to by the international abbreviation ALCAPA. In the context of the disease, there is a defective origin of the left coronary artery. This artery erroneously arises from the pulmonary artery. Due to the malformation in the structure of the heart, many patients suffering from Bland-White-Garland syndrome develop myocardial ischemia at a young age. In addition, the majority of affected individuals suffer from weakness of the heart muscle, which also develops relatively early. As a result of the malformation, blood flows from the right coronary artery into the left coronary artery and eventually enters the pulmonary artery. Surgical intervention is usually considered to correct Bland-White-Garland syndrome.
Causes
Bland-White-Garland syndrome represents 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 on genes that trigger the disease are a possibility. The corresponding artery connects to the pulmonary artery, which transports deoxygenated blood. As a result, in the long term, the organism is undersupplied with oxygen due to Bland-White-Garland syndrome. The myocardium also requires a high oxygen saturation in the blood. However, since this is not achieved, health problems arise. If adequate therapy for Bland-White-Garland syndrome fails to occur, the disease is fatal for nearly 80 percent of all affected patients within the first year of life.
Symptoms, complaints, and signs
Bland-White-Garland syndrome includes several disease signs and symptoms that may pose a significant threat to patients’ lives. First, individuals affected by Bland-White-Garland syndrome are characterized by suffering from infectious diseases more frequently than healthy individuals. Another typical sign of Bland-White-Garland syndrome is cyanosis, which develops as a result of the undersupply of oxygen to the body. In the majority of cases, patients with Bland-White-Garland syndrome develop cardiac insufficiency. This insufficiency manifests itself, for example, in a reduction in physical capacity, excessive sweat production and dyspnea. Bland-White-Garland syndrome may also cause disturbances of the heart rhythm. In addition, some of the affected patients have problems with blood circulation. This is accompanied, for example, by infarctions or ischemia of the myocardium. Often, the typical symptoms of Bland-White-Garland syndrome occur within the first six months after the patient’s birth.
Diagnosis and Course
The diagnosis of Bland-White-Garland syndrome is based primarily on the clinical symptoms of the condition. First, a thorough history is taken with the affected patient or the responsible caregiver. In some cases, some symptoms become apparent shortly after the birth of the affected baby and arouse suspicion of a heart defect. There is a wide range of investigative methods for diagnosing Bland-White-Garland syndrome. Usually, several procedures are used to confirm the diagnosis. First, an ECG examination is performed, and left ventricular ischemia of the myocardium can be detected. In addition, the malformations of Bland-White-Garland syndrome are detected during this examination.In addition, X-rays of the thorax are taken, revealing what is known as cardiomegaly. There is also evidence of vascular congestion in the lungs. The pumping function of the left hand is reduced, and patients also suffer from a typical insufficiency of the mitral valves. In some cases, an examination using a cardiac catheter is also ordered to confirm the diagnosis of Bland-White-Garland syndrome.
Complications
Because Bland-White-Garland syndrome occurs in infancy, it can lead to dangerous situations. If the syndrome is not treated, death usually results. In most cases, patients are very susceptible to infectious diseases. For this reason, life expectancy is reduced for those suffering from Bland-White-Garland syndrome. Sufferers also suffer from cardiac insufficiency, so that certain heavy work or stress cannot be performed. As a result, the patient is restricted in his or her daily activities and is also unable to perform any special sporting activities. Heavy physical exertion often leads to sweating. Since the affected person suffers from heart problems, the risk of a heart attack is greatly increased. In this case, the infarction can already occur in childhood and, in the worst case, lead to death. Treatment must be carried out immediately and is aimed primarily at correcting the heart defect. If this is not done, death will result from Bland-White-Garland syndrome. It cannot be predicted whether other complications will occur in the further course of the patient. Usually, the parents also suffer greatly from depression and other psychological ailments due to Bland-White-Garland syndrome.
When should you see a 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 diagnosis. Usually, Bland-White-Garland syndrome is treated immediately after birth as well. However, a doctor should always be consulted if the affected person experiences an undersupply of oxygen due to the condition. A medical examination is also necessary if the patient experiences decreased exercise tolerance or persistent fatigue. In general, due to Bland-White-Garland syndrome, complaints of blood flow should always be checked by a physician. Likewise, cardiac complaints and increased sweat production may be indicative of this condition. The diagnosis can be made by a cardiologist or in a hospital. However, the treatment itself is performed by surgery. The sooner surgery is initiated, the higher the chance of survival of the affected person due to Bland-White-Garland syndrome.
Treatment and therapy
The options for treating Bland-White-Garland syndrome are limited. In numerous cases, affected individuals are unable to survive without timely and successful therapy. The only option is to correct Bland-White-Garland syndrome and the associated malformations in the structure of the heart by surgical intervention. In view of the poor chances of survival without therapeutic intervention, such operations are often almost without alternative. As part of the surgical procedure, patients are connected to a heart-lung machine. In the first step, the corresponding coronary artery coming from the pulmonary artery is severed. The pulmonary artery is sealed with the help of a special pericardial tissue. After that, the coronary artery is attached to a part of the ascending aorta. If the affected patient suffers from mitral valve insufficiency, correction is usually performed during the same operation.
Outlook and prognosis
The prognosis of Bland-White-Garland syndrome is considered unfavorable. The mortality rate is very high and, in addition, numerous complaints occur due to the disease, which cannot be cured. The cause of the syndrome is currently considered uncertain. Various research findings suggest that the disease is genetic. Since interference with the genetics of humans is prohibited for legal reasons, it makes it difficult for scientists and researchers to take appropriate measures. The treatment plan involves alleviating existing symptoms. At the same time, an attempt is 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, a surgical intervention is performed. In this, corrections of the malformations of the heart take place. The intervention requires tact and is considered a challenge. This procedure 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 on an individual basis depending on the degree of Bland-White-Garland syndrome. The organism’s undersupply of oxygen is then corrected in various treatment steps. In regular control examinations, the heart activity as well as possible circulatory disturbances are checked and treated. In some patients, the heart must be monitored permanently.
Prevention
Bland-White-Garland syndrome is a congenital disease associated with malformations of the heart. Therefore, there are no known ways to prevent it.
What you can do yourself
People diagnosed with Bland-White-Garland syndrome should, above all, take it easy on themselves. This is because there is no conservative treatment for Bland-White-Garland syndrome. Surgical intervention is necessary to connect the left coronary artery to the aorta. Patients should avoid physical exertion. However, exercise should not be completely avoided. Light regular exercise and control of health parameters by a cardiologist are necessary and useful. After all, once the diagnosis has been made, the aim is to at least maintain, if not improve, the state of health until the surgical intervention. 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 lead to maintaining or even improving the current health condition. For this reason, foods such as fresh fruits, vegetables, fish and high-quality oils belong on the menu. Fats should be kept relatively low, and meat should also be on the menu no more than once or twice a week. In addition, a low-salt diet is important, as is drinking enough water and unsweetened teas. The amounts of the individual components should be discussed in advance with the attending physician. Those who then get enough sleep will remain as healthy as possible until the final treatment.
