Circular coarctation of ascending aortic root
Introduction
Introduction Its main lesion is aortic limited short-segmental stenosis or occlusion leading to aortic blood flow disorders. The vast majority (more than 95%) of the lesions in the aortic constriction are at the junction of the distal aortic arch and the descending thoracic aorta, that is, the aortic isthmus, adjacent to the arterial catheter or arterial ligament. However, in a very small number of cases, the narrowing segment can be located in the aortic arch, the descending thoracic aorta or even the abdominal aorta. Sometimes the aorta can be narrowed in two places. Very few patients have a family history. The disease is more common in men, the ratio of male to female is 3 to 5:1.
Cause
Cause
The most common lesion is a diaphragm-like stenosis above the coronary valve sinus. There is a small hole in the central part of the diaphragm, sometimes the diaphragm is connected with the left coronary valve, and the left coronary artery blood flow is obstructed, and the aortic valve leaf may be thickened. The ascending aorta has a normal appearance and is not accompanied by a stenosis and enlargement. Another type of localized stenosis of the stenosis, the ascending aorta is narrow in the stenosis, showing a sand-absorbent or "8" shape, where the aortic wall is thickened, the intima is thickened, and the tissue is thickened. The examination of the lesion is similar to aortic constriction. Extensive aortic stenosis is rare, with stenosis extending from the ascending aorta above the ascending aorta and the origin of the innominate artery, and even invading the aortic arch. Aortic stenosis is often accompanied by coronary artery tortuosity and coronary sinus enlargement, which can have multiple stenosis of the surrounding pulmonary artery, such as pulmonary stenosis, common pulmonary artery dysplasia, aortic arch branch stenosis, aortic coarctation or ventricle Interval defect.
Examine
an examination
Related inspection
Cardiovascular electrocardiogram
First, most cases show symptoms of aortic stenosis in childhood. Because coronary atherosclerotic lesions occur earlier, angina is more common, and some patients have a family history. Aortic stenosis, especially in cases with mental retardation, special face and extensive stenosis of the pulmonary artery. Often in the early years due to severe obstruction of the left ventricular outflow tract and coronary artery disease, sudden death, rarely treated, rarely grow into adulthood.
Second, the signs are similar to other types of aortic outlet stenosis, but the systolic snoring is not heard, the location of the heart murmur and tremor is higher than the stenosis of the valve, and the aortic diastolic murmur is rare. Some patients have poor growth and development, short stature, low intelligence, many words, and have a special face: mandibular retraction, nostril anteversion, low nose bridge, thick lip, forehead width, large eye distance, poor tooth occlusion, about 5% of patients with blood Calcium is increased.
Third, X-ray examination and ECG examination showed signs similar to other types of aortic outlet stenosis.
Fourth, cardiac catheterization: left heart catheter examination and continuous recording of the pressure curve may find that the pressure waveform changes in the upper part of the aorta.
5. Selective left ventricular angiography can show the location, length and severity of the stenosis on the valve. The same can be seen in the shape and function of the aortic valve, as well as the coronary sinus and coronary artery. Right heart angiography shows whether the common pulmonary artery and its branches also have lesions.
Sixth, section echocardiography: can directly display the location and length of the stenosis on the valve.
Diagnosis
Differential diagnosis
The need to identify the annular atrophy of the ascending aorta is as follows:
First, multiple arteritis: the typical clinical manifestations of multiple arteritis are not difficult to diagnose, but atypical can be identified with other diseases. Any young person, especially a woman who has more than one of the following manifestations, should be suspected or diagnosed.
1. Ischemic symptoms appear in unilateral or bilateral limbs, accompanied by weakened or disappeared arterial pulsations, decreased blood pressure or undetectable or bilateral limb pulse pressure difference greater than 1.33 kPa (10 mmHg) or lower limb systolic blood pressure lower than upper limb systolic blood pressure less than 2.67 kPa (20mmHg) (same width cuff).
2. Cerebral ischemia symptoms, accompanied by unilateral or bilateral carotid pulsation weakened or disappeared and neck vascular murmur. However, some people, due to increased pulse pressure or increased heart rate, can hear mild vascular murmurs in the right neck, and should be differentiated from pathological murmurs according to the following characteristics: 40 years old, especially female, and appear Typical symptoms and signs for more than a month, limbs or brain.
3. Recent high blood pressure or refractory hypertension, accompanied by high-level vascular murmurs above the upper abdomen.
4. Not obvious low fever, rapid blood sedimentation, accompanied by abnormal changes in vascular tone, limb pulse or blood pressure. Can involve the pulmonary artery or coronary artery caused by the corresponding clinical manifestations.
5. There is no change in the fundus.
Second, aortic stenosis: Most patients with aortic stenosis are adults, with no history of rheumatism, often found heart murmur during physical examination. Because of the strong compensatory capacity of the left ventricle, there may be no symptoms in the clinic or only complaints are prone to fatigue. These patients are referred to as asymptomatic aortic stenosis. As the disease progresses, symptoms gradually appear. When the left ventricular end-diastolic pressure rises, breathing difficulties occur during exercise, and the head is dizzy. However, during the period of time, the left atrial systolic blood pressure is increased due to the increase of heart rate after exercise. The heart discharges blood, so the above symptoms are relatively stable. Once the symptoms such as fainting and angina after exercise appear, it indicates that the condition has deteriorated.
3. Patent ductus arteriosus: The arterial catheter is the blood vessel of the fetal blood circulation communicating the pulmonary artery and descending aorta. It is located between the left pulmonary artery root and the descending aortic isthmus. The normal state is more closed than the short-term after birth. If it fails to close, it is called patent ductus arteriosus. The symptoms of patent ductus arteriosus depend on the thickness of the catheter, the size of the sub-flow, the level of pulmonary vascular resistance, the age of the patient, and the combined intracardiac malformations. In full-term infants, although the catheter is large, it takes 6-8 weeks after birth, and symptoms appear after the pulmonary vascular resistance decreases. Premature babies have fewer pulmonary arteriolar smooth muscles and lower vascular resistance, so they can have symptoms in the first week, often with shortness of breath, tachycardia and acute dyspnea. It is more obvious when breastfeeding, and is prone to colds, upper respiratory tract infections, pneumonia, etc. Since then, the child has been compensated, rarely have self-conscious symptoms, but poor development and thin body. Some children are prone to fatigue and guilt after only being tired. Patients with moderately sized catheters are generally asymptomatic, and symptoms of decompensation of heart function such as shortness of breath and palpitations do not occur until after intense activity in their 20s. Pulmonary hypertension can occur below 2 years of age, but the obvious signs of pulmonary hypertension are mostly older, showing dizziness, shortness of breath, and hemoptysis. After the activity, the hairpin (more than half of the body is obvious). If complicated with subacute endocarditis, there are systemic symptoms such as fever, loss of appetite, and sweating. Endocarditis rarely occurs in childhood, but is more common in adolescence.
