Coronary artery anomaly (anomalous left coronary artery from the pulmonary artery (ALCAPA) or White-Garland syndrome, Bland-White-Garland syndrome – congenital heart disease, which is found mainly in infants in approximately 0.5% of all congenital heart defects.
The disease occurs early in pregnancy during the formation of the baby’s heart. In this case, the developing blood vessels of the heart are connected in the wrong way.
In a normal heart the left coronary artery begins in the aorta, the main blood vessel and through which oxygenated blood flows from the heart to the rest of the body. When abnormal discharge of the left coronary artery from the pulmonary artery, the blood from the left coronary artery abnormal delivered to the pulmonary artery and not to the heart, so heart does not receive enough oxygen (oxygen deficiency), whereby the cardiac muscle tissue begin to die that can lead to a heart attack in a child.
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Timely diagnosed and properly corrected the defect is of great importance and gives a good prognosis of life. Therefore, it is necessary to treat the disease in its early stages.
Properly executed 3D echocardiography in Israel allows us to estimate as accurately as heart defect, and modern surgical techniques allow to fix the problem and prevent heart attacks in children.
Usually ALCAPA is an isolated anomaly of the heart, but in rare cases, it occurs along with other congenital defects. Among them: patent ductus arteriosus, ventricular septal defect (VSD), Tetralogy of Fallot (TOF), aortic stenosis.
Originally myocardial ischemia is temporary and occurs only during the period of increased activity of the myocardium, for example, when a child eats or crying. Further increases in myocardial oxygen consumption lead to dysfunction of the papillary muscles and mitral valve varying degrees of mitral insufficiency.
Proper circulation between the right and left coronary blood flow system is necessary for normal functioning. Due to impaired blood flow of the left coronary artery, the blood enters the pulmonary trunk because of low pulmonary vascular resistance, there is the so-called leakage, and the heart does not receive enough oxygen. Therefore, the combination of left ventricular dysfunction and significant mitral regurgitation results in chronic heart failure (CHF), the symptoms of which are tachypnea, poor nutrition, irritability, sweating. Inadequate myocardial perfusion causes severe pain in the chest, and the symptoms of myocardial ischemia can sometimes be misinterpreted as normal infantile colic.
Symptoms: abnormal heart rhythm, enlarged heart, heart murmurs, rapid pulse, crying or sweating during feeding, pale skin, poor appetite, shortness of breath, sweating.
These symptoms may occur within the first 2 months of life.
Abnormal discharge of the left coronary artery of the lung may also be diagnosed in an adult.
Diagnostics
Electrocardiogram. Test of the electrical activity of the heart.
Angiography. Evaluation of the structure and location of the blood vessels of the heart using a contrast agent, under the control of CT or MRI.
Diagnostic coronary angiography. Procedure in which a thin tube (catheter) is inserted into a blood vessel of the heart to measure blood pressure and oxygen levels.
MRI (cardiac magnetic resonance imaging).
Chest x-ray.
ECHO. Ultrasound of the heart.
ALCAPA Treatment
In most cases, the abnormal coronary artery requires surgical treatment. The volume of surgery depends on the child’s condition and size of the involved blood vessels.
If due to lack of oxygen severely damaged mitral valve, the child may also need surgery to repair or replace the valve. The mitral valve regulates blood flow between the left chambers of the heart.
If the heart of a child is seriously damaged due to lack of oxygen, may be considered a heart transplantation.
In some cases, our specialists prescribe medication to support the child’s body for surgery. It may be drugs such as diuretics, inotropic agents (medicines which strengthen the heart muscle), beta-blockers, ACE inhibitors (drugs that reduce stress on the heart).
If a child does not receive proper treatment, it can not survive during their first year of life. Mortality in the first year of life is 90%. However, those children who survive can have serious complications or die suddenly during the following years. Sudden death can occur due to insufficient collateral circulation between the left and right coronary arteries.
Most children do well with timely treatment as well as continue to live a normal life. Nevertheless, they must undergo regular inspection pediatric cardiologist.
