Patients with Blanda-White-Garland syndrome need a consultation with a cardiac surgeon and a thorough examination to determine the indications and contraindications for surgical treatment. Various surgical methods for the correction of Blanda-White-Garland syndrome are aimed at ensuring adequate blood supply to the myocardium in the basin of the left coronary artery.
In the case of a well-developed collateral circulation, the mouth of the abnormal coronary artery is ligated (ligation of the coronary artery fistula). In this case, the normal right coronary artery assumes the entire load on the myocardial blood supply.
Another option for the correction of Blanda-White-Garland syndrome is bypassing the anomalously diverging left coronary artery with the help of the intrathoracic arteries (mammarocoronary bypass). In Blanda-White-Garland syndrome, it is possible to translocate the left coronary artery into the aorta, connect the left coronary artery and the aorta using a tunnel inside the pulmonary trunk.
Blanda-White-Garland syndrome (SBUG, BWG syndrome) is an abnormal branch of the coronary arteries supplying the heart muscle from the pulmonary trunk. In cardiology, Blanda-White-Garland syndrome accounts for 0.5% of all congenital heart defects. The clinical signs of the disease were studied and described in 1933 by the American cardiologists E. Bland, P. White, J. Garland, in whose honor this anomaly was called “Bland-White-Garland syndrome”. Blanda-White-Garland syndrome is 2 times more common in girls than in boys.
There are four anatomical variants of Blanda-White-Garland syndrome: abnormal discharge of the left, right, both, or additional coronary arteries from the trunk of the pulmonary artery. Statistically, an abnormal branching of the left coronary artery is more common. It is also customary to distinguish two types of the syndrome that differ in the clinical picture: infantile (with poorly developed collateral blood supply) and adult (with well-developed collateral blood supply).
Blanda-White-Garland syndrome is usually found in an isolated form, but can be combined with other defects: atrial septal defect, ventricular septal defect, Fallot’s notebook, coarctation of the aorta, open arterial duct, transposition of the great vessels.
Blanda-White-Garland syndrome is a congenital anomaly of the coronary bed characterized by the discharge of the coronary arteries (usually the left) from the pulmonary artery, as a result of which a significant portion of the myocardium receives venous blood supply. The clinical manifestations of Blanda-White-Garland syndrome include tachypnea, fatigue, suffocation, pallor, sweating. Blanda-White-Garland syndrome is diagnosed using auscultation data, ECG, cardiac ultrasound, x-ray, aortography, coronary angiography, ventriculography, MRI and CT. For the correction of Blanda-White-Garland syndrome, several types of operations have been proposed – reimplantation of the left coronary artery into the aorta, artery bypass surgery, ligation of the mouth of the left coronary artery, etc.
Causes of Blanda-White-Garland Syndrome
Blanda-White-Garland syndrome is formed due to abnormal embryonic coronary arteries.
Several hypotheses have been proposed to explain the embryogenesis of the defect:
- the discharge of coronary vessels begins from the pulmonary part of the arterial trunk due to improper formation of the aortic pulmonary septum;
- the germ of the left coronary artery from the very beginning forms in the region of the pulmonary artery;
- coronary artery discharge is formed in the aortic and pulmonary valves. Normally, in the future, the beginnings of the pulmonary artery regress, and only two aortic primordia develop. In some cases, the coronary bud of the pulmonary artery is preserved and expands, causing an abnormal discharge of the coronary vessels from the pulmonary trunk.
Risk factors predisposing to the development of Blanda-White-Garland syndrome are currently unknown; a clear connection with any genetic disorders was not detected.
Features of the coronary circulation in Blanda-White-Garland syndrome
Normally, the left coronary artery originates from the left sinus of Valsalva, and in Blanda-White-Garland syndrome, it departs from the root of the pulmonary trunk. Its further branching, as a rule, remains normal. The features of the intracardiac circulation in Blanda-White-Garland syndrome are primarily determined by the ratio of pressures in the aorta and the pulmonary artery.
In the prenatal period, the pressure in the pulmonary trunk and oxygenation of the blood are comparable with the aorta, therefore, the abnormal left coronary artery ensures normal blood flow and adequate blood supply to the heart muscle. At birth, there is a decrease in pressure in the pulmonary artery, as a result of which the part of the myocardium supplied by the anomalously located coronary artery receives an insufficient amount of weakly oxygenated blood. A reduction in coronary perfusion is accompanied by severe myocardial ischemia, dysfunction, and progressive damage to the area of the heart muscle.
At the same time, the intercoronary anastomoses gradually form, causing blood to flow into the abnormal vessel not only from the pulmonary artery, but also from the right coronary artery extending from the aorta. As the number of intercoronary anastomoses increases, the direction of blood flow in the abnormal artery changes, a discharge from the coronary bed into the pulmonary artery is formed, which leads to the development of the “stealing phenomenon”, i.e., deterioration of myocardial blood supply in the pool of a normally located right coronary artery. This is accompanied by further ischemic damage to the myocardium and aggravation of its dysfunction.
The nature of coronary hemodynamics can significantly change in the presence of concomitant CHD occurring with pulmonary hypertension (DMPP, DMZHP, etc.). This is to some extent difficult for the lifetime diagnosis of Blanda-White-Garland syndrome.
Thus, during the Blanda-White-Garland syndrome, there are 3 pathophysiological phases. The first phase is characterized by adequate blood filling of the left coronary artery, due to the high pressure in the pulmonary trunk. The second phase (critical) is caused by a drop in pressure in the pulmonary artery and the development of anastomoses between the right and left coronary arteries. The third phase begins with the development of retrograde blood flow from the left coronary artery to the pulmonary trunk and leads to myocardial ischemia.
At autopsy, patients with Blanda-White-Garland syndrome show an enlarged spherical heart, severe left ventricular dilatation, marked myocardial fibroelastosis, hypertrophy and deformity of papillary muscles, left ventricular aneurysms, and often transmural or subendocardial cardiopathy;
Symptoms of Blanda-White-Garland syndrome
The clinical manifestations of Blanda-White-Garland syndrome primarily depend on its variant. Infantile type usually declares itself at the age of 2-3 months. At the same time, there are difficulties in feeding the child (the so-called angina feeding): rapid snoring breathing, shortness of breath, sweating, pale skin, cyanosis of the lips, cough, regurgitation, vomiting. Attacks can also be provoked by defecation, cry, any physical effort, intercurrent infections.
Children are lethargic, quickly tired, slowly increase body weight. Objectively determined by the expansion of the boundaries of the heart, the deafness of heart tones, tachycardia, systolic murmur and canter rhythm, liver enlargement, moist rales in the lungs. Later, hypoidal edema, hydrothorax, and ascites join.
Blanda-White-Garland syndrome is the most common cause of myocardial infarction in children. More than 90% of children with infantile type die during the first year of life from severe heart failure, rhythm disturbances and cardiac conduction.
In the adult-type syndrome, inter-arterial collaterals provide adequate coronary blood supply and relatively favorable progression of the disease. For a long time, the condition of patients can be kept stable. Clinical manifestations can develop at the age of 3-25 years and even later, characterized by exertional angina and rest angina, signs of heart failure, severe arrhythmias and blockades. Sometimes the first manifestation of Blanda-White-Garland syndrome is sudden coronary death. A rare exception is the poor symptom course of the defect and its accidental detection during elective electrocardiography or coronary angiography due to myocardial infarction.
Diagnosis of Blanda-White-Garland syndrome
Diagnosis of Blanda-White-Garland syndrome is performed using electrocardiography, echocardiography, chest X-ray, coronary angiography, aortography, left ventriculography, cardiac sounding, MRI and MSCT of the heart.
On radiographs in patients with Blanda-White-Garland syndrome, cardiomegaly, congestion of blood in the pulmonary circle of blood circulation is detected. Characteristic ECG signs are left ventricular hypertrophy of the myocardium and its ischemic lesion, blockade of the left leg of the bundle of His. EchoCG detects systolic dysfunction of the left ventricular myocardium with zones of hypo-and akinesia. Doppler echocardiography often allows to record a turbulent blood flow from the abnormal coronary artery into the pulmonary trunk.
Aortography and selective coronary angiography (CT-coronary angiography, MSCT-coronary angiography) provide the most accurate information regarding Blanda-White-Garland syndrome. At the same time on the angiograms visualized branched right and abnormally located left coronary arteries, retrograde discharge of contrast into the pulmonary trunk.
In the final phase of the development of Blanda-White-Garland syndrome, cardiac catheterization reveals an increased arterialization of blood at the level of the pulmonary trunk, caused by a pronounced left-right discharge of blood. Left ventriculography reveals dilatation of the ventricular cavity, signs of mitral insufficiency. With the help of MRI and MSCT of the heart, the location of the coronary arteries is determined, and concomitant pathology of the heart and vessels is detected.
Blanda-White-Garland syndrome prognosis
The natural course of Blanda-White-Garland syndrome is associated with a high risk of death in early childhood (infantile type) or young age (adult type).
The discharge of the right coronary artery from the trunk of the pulmonary artery is extremely rare, does not lead to any clinically significant lesions of the heart and is compatible with normal life. Cases of discharge of both coronary arteries from the pulmonary trunk are prognostically unfavorable and lead to the death of patients during the first 2 weeks of life.
Significantly improved prognosis with timely detection and adequate cardiac surgery correction of Blanda-White-Garland syndrome.