Ectopic origin of coronary arteries
Introduction
Introduction to the origin of coronary artery ectopic Common origins of coronary ectopic origin are from the pulmonary artery and aorta. The origin of the common carotid artery and the innominate artery is extremely rare and often accompanied by severe cardiac malformation. In 1885, Brooks first reported two patients. The most common origin of the ectopic origin was the left coronary artery. In addition, the rare right coronary artery originated from the pulmonary artery. Both coronary arteries originated from the pulmonary artery. The origin of the left coronary artery circumflex artery The pulmonary artery and the coronal artery or conical coronary artery originate in the pulmonary artery. Bilateral coronary arteries originate from the pulmonary arteries and die within a few days after birth due to severe myocardial ischemia and hypoxia, and are rarely diagnosed clinically. Left coronary artery ectopic originates from the pulmonary artery. The left coronary artery originated in the pulmonary artery, but its branch distribution and path were normal. The incidence rate was only 1 in every 300,000 live births and 0.26% in congenital heart disease. basic knowledge The proportion of illness: the incidence rate is about 0.0001% - 0.0002% Susceptible people: good for newborns Mode of infection: non-infectious Complications: myocardial ischemia, heart failure
Cause
Origin of coronary ectopic origin
The disease is a congenital heart disease. Under normal circumstances, the left and right coronary arteries are emitted from the aorta and provide the blood needed by the heart. Their openings are deep in the aortic roots, respectively. In the sinus of the aneurysm, if the aortic valve attachment line is bounded, the aortic sinus can be divided into the sinus and the sinus. The vast majority (80%-91%) of the openings are in the sinus, and the other openings are in the sinus. Outside the sinus or sinus line, and in the case of abnormalities, it is more common that the coronary artery originates from the pulmonary artery and the aorta, and a few can also originate from the common carotid artery and the innominate artery, often accompanied by other severe deformities.
Prevention
Prevention of coronary ectopic origin
The disease is a congenital disease, so there is no effective preventive measures, the natural prognosis is worse, most of them die within 2 months after birth, and the cases with rich collateral circulation can survive into adulthood, but often Left ventricular ischemic lesions gradually worsen and die from chronic congestive heart failure or sudden death, so once the diagnosis is clear, active surgical treatment is the only effective measure to prevent further deterioration of the disease.
Complication
Coronary artery ectopic origin complications Complications, myocardial ischemia, heart failure
The mortality rate of this disease is high (it is reported that the first year of the disease has a mortality rate of 90%). When the left coronary artery originates from the pulmonary artery, it is more likely to cause myocardial ischemia and myocardial infarction in children. Patients often show acute, severe heart rate. Depletion.
Symptom
Coronary artery ectopic origin symptoms common symptoms fatigue irritability palpitations pale collateral circulation development non-heart rate increased left ventricular hypertrophy angina pectoris hepatomegaly heart failure
First, clinical manifestations
The infant can have no abnormal performance within 1 month after birth. It can start to show myocardial ischemia and hypoxia after 2 to 3 months of birth. When feeding or crying, it induces shortness of breath, irritability, pale lips or cyanosis, sweating and fatigue. Increased heart rate, cough, wheezing, etc. may be caused by angina pectoris and heart failure. A few cases with abundant left and right coronary collateral circulation can delay angina and chronic congestive heart failure until about 20 years old. Symptoms, continuous murmurs are often heard in the anterior region of the patient, and mitral regurgitation is also severe.
Second, physical examination
Poor growth, small size, weight loss, increased breathing, heart rhythm expansion, heart rate growth, hepatomegaly, jugular vein filling, lung field rales and other signs of heart failure, apical mitral can be heard Close the incomplete systolic murmur, the coronary collateral circulation is rich, and the soft continuation murmur can be heard in the anterior region.
Third, pathological anatomy
Coronary artery ectopic originates from the pulmonary artery
1. The left coronary artery originates from the pulmonary artery;
2. The left coronary artery originates from the pulmonary artery;
3. The origin of the left and right coronary arteries originates from the pulmonary artery;
4. The accessory coronary artery originates from the pulmonary artery;
5. The circumflex coronary artery originates from the pulmonary artery.
The left coronary artery originates from the left or posterior valvular sinus of the pulmonary artery. It is divided into the anterior descending branch and the circumflex branch from 5 to 6 mm from the initial part. There are collateral circulation branches between the left and right coronary arteries, but the number is small. In different cases, the origin and branch of the right coronary artery are normal, the left ventricular hypertrophy is highly enlarged, the left ventricle apical area is more obvious, the left ventricle is extensively fibrotic, and the subendocardial area is most prominent, often with myocardial infarction lesions. Fibrotic lesions sometimes present with focal calcification, and there are different degrees of fibroelastic tissue hyperplasia under the endocardium. Due to extensive fibrosis or even calcification of the papillary muscles, the papillary muscles are dysfunctional, and the endocardial fibroelastic tissue is caused by hyperplasia. Mitral regurgitation is often present due to fusion and shortening and left ventricular fibrosis leading to enlargement of the left ventricle and mitral annulus.
Fourth, pathophysiology
The pathophysiological effects of left coronary artery ectopic origin from pulmonary artery depend on the pressure difference between systemic circulation and pulmonary circulation, and the extent and extent of collateral circulation between left and right coronary arteries. In the fetal and neonatal period, left and right ventricular pressure The blood oxygen level is equal, the pulmonary circulation resistance is similar to the systemic circulation resistance, so the left coronary artery originating from the pulmonary artery can obtain the same perfusion pressure and oxygen supply as the aorta, and has no effect on the development and growth of the fetus. On the 10th day, the pulmonary circulation resistance decreased, the pulmonary artery pressure dropped to normal, and at the same time, the blood oxygen saturation of the pulmonary artery also decreased to about 70%, and the hematocrit of blood decreased from 70 to 80% before birth to about 3 months after birth. About 40%, the hemoglobin content decreased, so the ectopic origin of the left coronary artery not only decreased the perfusion pressure to 2.7 ~ 6.7KPa (20 ~ 50mmHg), but also significantly reduced the oxygen content of the perfused blood, leading to the left coronary artery Insufficient myocardial oxygen supply in the donor site, hypertrophic lesions in the left ventricle, and subendocardial myocardium due to ischemia and hypoxia Fibrosis, the survival of infants after birth depends on the development of the collateral circulation between the left and right coronary arteries. If the collateral circulation is fully developed, the baby can survive; if the collateral circulation is not fully developed, it will lead to myocardial infarction and death. Excessive collateral circulation is derived from the right coronary artery of the aorta to transport blood flow, through the collateral circulation into the left coronary artery and pulmonary artery, resulting in left to right shunt, resulting in congestive heart failure and coronary vascular steal syndrome, about 80 ~90% of patients die of congestive heart failure or myocardial infarction within 1 year after birth. Only a small number of patients have abundant collateral circulation between left and right coronary arteries, and most of the myocardium includes left ventricular palpebral and most ventricular septum. And the blood supply to the left ventricular wall from the right coronary artery may survive into adulthood.
Examine
Examination of the origin of coronary ectopic
The auxiliary examination methods that are beneficial to this disease are:
(1) Chest X-ray examination: the heart shadow is significantly enlarged, the left heart is full and bulging, the apex is round and blunt, and protrudes outward to the left iliac crest, and covers the spine backwards. The blood vessels in the lungs are congested, but the pulsation is weak.
(2) Electrocardiogram examination: often showed signs of myocardial infarction in the anterior external wall, L1 and AVL leads showed Qr and T wave inversion, V5V6 showed deep Q wave, often accompanied by T wave inversion, and left anterior ventricular lead showed ST Segment elevation, and often signs of left ventricular hypertrophy.
(3) Right heart catheterization: Coronary collateral circulation is rich in cases. The blood from the right coronary artery from the aorta enters the left coronary artery and then flows into the pulmonary artery through the collateral circulation. Therefore, the blood oxygen in the pulmonary artery is increased, which can be displayed at the pulmonary artery level. Left to right shunt, pulmonary artery pressure can also increase.
(4) Echocardiography: showing left ventricular enlargement, myocardial contractility is significantly weakened, and sectioned echocardiography and ultrasound pulse Doppler examination can show that left coronary artery ectopic originated from the pulmonary artery.
(5) Serum enzyme assay can be found to increase creatine phosphokinase, lactate dehydrogenase and aspartate aminotransferase.
(6) Radionuclide myocardial imaging: myocardial imaging with 201TI can show that the myocardium is not developed in the lesion area of the anterior and posterior parts of the heart.
(7) Selective cardiac angiography: Cardiovascular angiography is a reliable method for the diagnosis of coronary artery ectopic origin. Aortic angiography and selective right coronary angiography show that only one right coronary artery originates from the aorta, right coronary The arteries were significantly thickened, the contrast agent filled the left coronary artery in the opposite direction, and then returned to the pulmonary artery. Selective left ventricular angiography often showed enlargement of the left ventricular cavity, significant reduction of left ventricular contractility and left ventricular anterior wall dysfunction, selective left ventricular angiography. It is helpful to diagnose mitral regurgitation. In some cases, the left coronary artery may be developed when the contrast agent is injected into the pulmonary artery.
Diagnosis
Diagnosis and diagnosis of the origin of coronary artery ectopic
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
This disease manifests as congestive heart failure in children with onset, and needs to be differentiated from endocardial fibroelastosis, and also with primary dilated cardiomyopathy, coronary aneurysm, Kawasaki disease, pre-cardiac region continuity. Identification such as noise.
I. Endocardial fibroelastosis
Endocardial fibroelastosis (EFE), also known as endocardial sclerosis, is not known for its etiology. It is a common type of primary cardiomyopathy in children. It is also called primary endocardial elastic fiber. Hyperplasia, congenital heart disease such as aortic coarctation, aortic stenosis, aortic valve atresia and other endocardial fibroelastosis, called secondary endocardial fibroelastosis, its clinical significance depends on the original Cardiac malformations, clinical manifestations of congestive heart failure, often occur after respiratory infections.
1. General symptoms can be classified into three types according to the severity of symptoms:
(1) fulminant: sudden onset of illness, sudden dyspnea, vomiting, refusal to eat, perioral cyanosis, pale, irritability, tachycardia, scattered wheezing or dry rales in the lungs, liver enlargement, also Visible edema, both signs of congestive heart failure, a small number of children presented with cardiogenic shock, visible irritability, pale complexion, cold limbs and accelerated pulse and weak symptoms, the age of this type of disease is more than 6 months Can cause sudden death.
(2) acute type: onset is also faster, but the development of congestive heart failure is not as sharp as the fulminant, often complicated by pneumonia, accompanied by fever, wet rales in the lungs, some children develop brain due to the fall of the wall thrombus Embolism, etc., most died of heart failure, a small number of treatment can be alleviated.
(3) Chronic type: the disease is slightly slow, the age is more than 6 months, the symptoms are acute, but the progress is slow, some children's growth and development are affected, can be relieved after treatment, live to adulthood, but also due to recurrent Death in heart failure.
Second, primary dilated cardiomyopathy
This type is characterized by left or ventricular or bilateral ventricular enlargement, accompanied by cardiac hypertrophy, ventricular systolic dysfunction, with or without congestive heart failure, ventricular or atrial arrhythmia is more common, the condition is progressively aggravated Death can occur at any stage of the disease, and can occur at all ages, but it is mostly in middle age, and the onset is slow. The heart is enlarged at the initial examination, and the heart function is compensated without conscious discomfort. After a period of time, the symptoms gradually appear. This period can sometimes be more than 10 years. Symptoms are mainly congestive heart failure. Among them, shortness of breath and edema are the most common. They are initially anxious after labor or exhaustion, and then have shortness of breath during mild activities or rest. Paroxysmal irritability at night, due to low cardiac output, patients often feel weak, physical examination see heart rate acceleration, apical beat to the left and down, there may be lifting pulsation, heart sounds to the left to expand, often can hear the third sound Or the fourth sound, when the heart rate is fast, it is a galloping horse. Due to the enlargement of the heart chamber, there may be a systolic murmur caused by the relative mitral or tricuspid regurgitation. This kind of murmur is in the heart function. After the relief, the blood pressure is mostly normal, but the blood pressure is lower in the advanced case, the pulse pressure is small, and the diastolic blood pressure can be slightly increased in the presence of heart failure. The appearance of alternating pulse suggests left heart failure, the pulse is often weak, and the base of the second pulse is in heart failure. There may be rales, liver enlargement in right heart failure, edema from the lower limbs, pleural effusion and ascites are not common in advanced patients, various arrhythmias can occur, as the first or main performance, and a variety of heart rhythms The combination of abnormalities and the formation of a more complex heart rhythm can occur repeatedly, sometimes very stubborn, high atrioventricular block, ventricular fibrillation, sinus block or pause can lead to A-S syndrome, which is one of the causes of death, in addition, There are still embolisms in the brain, kidneys, lungs, etc.
Third, Kawasaki disease
The disease is an acute systemic vasculitis, which is inflammation of the blood vessel wall and can progress to vasodilation (aneurysm), which mainly affects the coronary artery (the blood vessels supplying the heart), but not all patients develop an aneurysm. Most patients present with acute symptoms without complications.
The disease begins to manifest as unexplained hyperthermia, lasting at least 5 days, the child is often very irritated, fever can be accompanied by or subsequent conjunctivitis (eye congestion), no rash with secretions or secretions can be more Variety, such as measles or scarlet fever-like rash, urticaria, papules, etc., the rash is mainly distributed in the trunk and limbs, and often occurs in the anal perineal area.
Oral changes appear as lip color bright red, chapped, tongue congestion, often referred to as "yangmei tongue" and pharyngeal congestion can be affected by the hands and feet, manifested as palm and plantar congestion and swelling, after these performances (about two to three weeks) Characteristic peeling of the finger and toe ends occurs.
More than half of the patients present with enlarged lymph nodes in the neck, usually a single lymph node with a diameter of at least 1.5 cm.
Sometimes, other symptoms such as joint pain and / or joint swelling, abdominal pain, diarrhea, irritability and headache can also be seen that heart involvement is the most serious manifestation of Kawasaki disease, because it may lead to long-term complications, you can find heart murmur, arrhythmia Or echocardiographic abnormalities, the layers of the heart can have a certain degree of inflammatory response, namely pericarditis (inflammation of the membrane surrounding the heart), myocarditis (inflammation of the myocardium) and valve involvement, however, the main feature of this disease is The formation of coronary aneurysms.
