Aortic origin of coronary artery abnormality

Introduction to the origin of coronary artery abnormalities The left or right coronary artery abnormalities of either the two or single coronary arteries originate from the corresponding aortic sinus, that is, the left coronary artery originates from the right sinus of the aorta, and the right coronary artery originates from the left sinus, of which 1/2 One third of the cases form a tangential or acute angle with the aortic wall in the proximal segment, and travel between the aorta and the pulmonary artery, which can produce symptoms of myocardial ischemia and sudden death, requiring surgical treatment. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: myocardial infarction

Coronary artery abnormal origin aortic etiology

(1) Causes of the disease

The pathogenesis of this malformation is unclear, probably due to the connection of the left or right coronary plexus with the corresponding aortic sinus. The left coronary artery plexus and the right aortic sinus bud connect to produce a left coronary artery abnormality originating from the aorta. Right sinus; the right coronary plexus is connected to the left aortic sinus bud to form the left aortic sinus of the right coronary artery.

(two) pathogenesis

Pathological anatomy

Ogden et al found that coronary artery abnormalities originated in the aorta in 1/3 of the total coronary artery malformation, most of which were benign, and the patient could survive to 80-90 years old. However, the left coronary artery originated from the right aortic sinus, right Coronary artery abnormalities originate in the left sinus (Fig. 1). The proximal end of the aorta and pulmonary artery are at risk of potential myocardial ischemia and sudden death. The abnormal coronary opening is mostly crack-like, with a diameter of about 1.0 mm. The end of the heart is tangential or acute to the wall of the aorta (Fig. 2), or there is a longer sneak in the wall of the aorta called the coronary artery in the aortic wall, perpendicular to the origin of the normal coronary artery and its proximal end perpendicular to the wall of the aorta different.

(1) Left coronary artery abnormality originates from right aortic sinus: The left coronary artery is divided into four categories: 1 in front of the right ventricular outflow tract, and then divided into left anterior descending coronary artery and convoluted coronary artery (Fig. 3); Between the two major arteries; 3 lines through the supraorbital iliac muscle; 4 around the aorta and posterior branches, Robert reported 17 cases of left coronary artery abnormalities originating from the aortic right sinus, 9 cases (53%) left coronary The arteries passed between the two arteries, 8 cases showed crack-like openings, 7 of 9 cases died of coronary artery malformations; 6 cases died of sudden death outside the hospital, and another died of severe heart failure after myocardial infarction. The other 3 types were all Benign, died of other causes unrelated to the heart, but there are very few cases of myocardial ischemia and sudden death in classification 34. Cheitlin reported that 33 cases of left coronary artery abnormalities originated from the right aortic sinus, of which 9 (27.3%) were Unexplained death, 8 cases, age 13 to 22 years old, left coronary artery between the aorta and pulmonary artery, sudden death after exercise; another case, 36 years old, left coronary artery buried in the supraorbital muscle, due to ventricular tachycardia Death, Murphy 1 case, 12 years old, had a history of repeated chest pain and syncope, and had an acute myocardial infarction on the electrocardiogram. The left coronary artery originated from the right aortic sinus and the left coronary artery bypassed the posterior aorta. The breast artery coronary artery bypass graft was cured and the electrocardiogram returned to normal.

(2) Right coronary artery abnormality originates from the left aortic sinus: Kragel and Robert divide the right coronary artery from the left aortic sinus according to the position of the right coronary artery and divide the two major arteries into four categories: 1 right coronary artery opening is located Behind the left sinus (Fig. 4); 2 in the left sinus; 3 in the left border; 4 right coronary artery and left coronary artery open together and ride across the left sinus and right-left valve junction. In 1994, Rinaldi reported 8 cases. The right coronary artery originated from the left aortic sinus and was a slit-like opening. The right coronary artery opening was classified according to Kragel and Robert. Each of the 2 cases was confirmed by surgery.

Kragel reported 25 cases of right coronary artery abnormalities originating from the left aortic sinus, 8 cases of right coronary artery opening behind the left sinus, 5 cases above the left sinus, 10 cases above the right-left border, and 2 cases of right and left coronary artery The opening was located at the right-left junction and the left sinus, and 8 of them were confirmed by autopsy to be deformed and died.

A group of reports reported that approximately 80% of autopsy showed left ventricular and/or right ventricular endocardial scars, and very few had myocardial infarction, ventricular aneurysm and wall thrombus, and a few patients had left ventricular hypertrophy and bicuspid aorta The flap or valve junction was partially fused, and the other group reported that the vast majority of patients died suddenly and no changes in the heart were found in the autopsy.

2. Pathophysiology

There are four different hypotheses for this malformation that produce myocardial ischemia, myocardial infarction, and sudden death:

1Cohn and Benson et al believe that abnormal coronary arteries pass through the narrow transverse sinus of the aorta and pulmonary artery. After intense exercise, the pressure of the two arteries rises and expands, and the abnormal origin of the coronary artery from the outside can cause the blood flow to slow and decrease, due to Pulmonary arterial pressure is low and it is difficult to support this hypothesis.

2 Benson and Jokl believe that left coronary artery dysplasia has been reported in several cases.

3Jokl also believes that due to the left or right coronary artery in the proximal part of the abnormal origin and the aortic wall forming a tangent, acute angle and distortion, hairpin bending in the path, obstructing blood flow.

The hypothesis of 4Cheirlin and Sack is accepted by most scholars. It is believed that the mechanism of myocardial ischemia and sudden death caused by this malformation is multifactorial. First, in most patients, the proximal part of the left and right coronary arteries is adjacent to the wall of the aorta. Tangent or acute angle and crack-like opening, resulting in coronary flow disorder during strenuous exercise, followed by abnormal left or right coronary artery proximal to the aortic wall, no vascular adventitia and in the same layer as the aorta, When the arterial pressure is particularly high in diastolic blood pressure during vigorous exercise, the expansion and elongation of the ascending aorta with elastic fibers causes partial collapse and blockage of the coronary artery in the aortic wall (Fig. 5), and finally during vigorous exercise. Arterial dilatation may cause the left coronary artery to form a flap and block.

Coronary artery abnormal origin aorta prevention

Primary prevention

Congenital heart disease is caused by environmental factors, genetic factors and the interaction between the two. The prevention of genetic factors mainly focuses on premarital examination, avoiding close relatives' marriage, and accepting genetic counseling. More importantly, it is to find ways to avoid and prevent it. Environmental factors such as viral infections, drugs, ethanol and maternal diseases that may cause adverse changes in genetic predisposition during pregnancy to break the interaction between environmental factors and genetic factors are the key to primary prevention.

2. Secondary prevention

(1) Early diagnosis: Early diagnosis of congenital heart disease can be divided into two steps.

1 fetal diagnosis: in the 16 to 20 weeks of pregnancy, amniotic fluid cell culture, chromosome analysis, genetic diagnosis and enzyme activity determination, amniotic fluid metabolites, special protein and enzyme activity, etc. The above examinations were performed by women's vaginal aspiration of villus at 8 to 12 weeks of gestation, which is of great value for congenital heart disease caused by single gene mutations and chromosomal aberrations.

2 Infancy diagnosis: A comprehensive physical examination should be performed on the babies born, especially the cardiovascular system should be carefully auscultated and found to be further examined by cardiac ultrasound.

(2) Early treatment: Once the fetal diagnosis of congenital cardiovascular malformation is confirmed in the fetal period, the pregnancy should be terminated in time. For some hereditary enzymes or metabolic deficiency diseases, relevant replacement therapy should be carried out early after birth, and the conditional hospital Gene therapy can be performed to prevent the occurrence of the corresponding disease.

3. Three levels of prevention

Once the congenital heart disease is clearly diagnosed, the fundamental method of treatment is to perform surgery to completely correct the cardiac vascular malformation, thereby eliminating the pathophysiological changes caused by the deformity. Those who have not been operated or temporarily unable to operate should avoid overwork according to the condition. In order to avoid heart failure, if heart failure occurs, anti-heart failure treatment, prevention and treatment of complications, patients with congenital heart disease in the implementation of invasive examination or treatment, including cardiac catheterization, extraction, tonsillectomy, etc., should be routinely applied antibiotics to prevent Infective endocarditis.

Coronary artery abnormal origin aortic complications Complications, myocardial infarction, sudden death

It is prone to myocardial ischemia, myocardial infarction and sudden death of unknown cause.

Coronary artery abnormal origin aortic symptoms common symptoms sudden death coma angina syncope

Some patients, especially large children and young people, have symptoms of angina, syncope and coma; but other patients have no clinical symptoms, sudden death after strenuous exercise, and generally no abnormal signs.

A small number of patients have been diagnosed by echocardiography or electron beam tomography. The most reliable diagnostic method is selective coronary angiography, which can show the origin of coronary artery abnormalities originating from the location of the aorta and the path of abnormal coronary arteries. Waiting between.

Coronary artery abnormal origin aortic examination

Coronary angiography can show that coronary artery abnormalities originate from the location of the aorta and the straight path.

Diagnostic diagnosis of aortic anomalies of coronary artery origin

A small number of patients have been diagnosed by echocardiography or electron beam tomography. The most reliable diagnostic method is selective coronary angiography, which can show the origin of coronary artery abnormalities originating from the location of the aorta and the path of the abnormal coronary artery, whether it passes between the two major arteries, etc.

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