Abnormal left coronary artery branching from the pulmonary artery: symptoms, diagnosis, treatment

Anomalous origin of the left coronary artery from the pulmonary artery accounts for 0.22% of all congenital heart defects. The left coronary artery originates from the left, less often from the right sinus of the pulmonary artery, its further course and branches are the same as in the norm. In case of pronounced intercoronary anastomoses, it is dilated and tortuous. The mouth of the right coronary artery is located in the right coronary sinus of the aorta. A wide network of anastomoses is visible on the anterior surface of the heart. With a sufficient number of anastomoses, clinical signs of the defect appear at a later date, often in the second decade of life; with insufficient anastomoses, and therefore with insufficient blood supply to the myocardium, signs of the defect are detected early, sometimes already in the neonatal period. Infantile and adult types of anomalous origin of the left coronary artery are distinguished. Myocardial ischemia may be secondary to low perfusion pressure as a result of blood flow through collaterals from the right to the left coronary artery, and then to the pulmonary artery. In severe "steal syndrome", subendocardial blood flow is particularly affected. This is one of the reasons for the development of secondary fibroelastosis of the endo- and myocardium in this defect.

Symptoms of abnormal origin of the left coronary artery from the pulmonary artery can manifest at any age. The first signs: deterioration of the general condition, lethargy, sweating. Half of the patients may have attacks of sudden anxiety, dyspnea. Against this background, an increase in body temperature, the appearance of loose stools (has a reflex, as in angina pectoris and myocardial infarction, character) are possible. Many children in the decompensation stage lag behind in physical development, left-sided cardiac hump develops early. The apical impulse is diffuse, weakened. The boundaries of relative cardiac dullness are expanded mainly to the left. Heart sounds are muffled, systolic murmur can be heard. Heart failure develops to a greater extent according to the left ventricular type. As follows from the above, based on the nature of the complaints and clinical signs, it is quite difficult to suspect an abnormal origin of the left coronary artery from the pulmonary artery, therefore the diagnosis is based on fairly specific results of an instrumental examination.

Anomalous origin of the left coronary artery from the pulmonary artery may be suspected for the first time by ECG. A pronounced deviation of the electrical axis of the heart to the left, a deep Q wave (more than 4 mm or more than 1/4 of its R) in I, aVL and left chest leads (maximum in lead aVL) are detected. In the same leads (especially diagnostically important in leads I and aVL), a negative T wave is detected. In case of insufficiency of intercoronary anastomoses, signs of a previous myocardial infarction appear on the ECG.

Chest X-rays reveal cardiomegaly predominantly in the left sections.

Echocardiographic examination reveals dilatation of the left ventricle with signs of hypokinesia, increased echogenicity of the papillary muscles. When examining the aortic root, a normally originating right coronary artery and the absence of a left coronary artery are found. Abnormal origin of the coronary artery is often accompanied by mitral regurgitation.

Cardiac catheterization and angiocardiography are performed to exclude other coronary anomalies and defects.

Treatment of anomalous origin of the left coronary artery from the pulmonary artery

There is no specific drug treatment. If there are signs of heart failure, appropriate drug therapy is indicated. Surgical treatment is performed as early as possible. The operation of choice is direct reimplantation of the left coronary artery into the aorta, which restores the system of two coronary arteries.

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