Congenital ruptured aortic sinus aneurysm

Introduction to the rupture of congenital aortic sinus aneurysm Aortic sinus aneurysm rupture is a rare congenital heart disease. During the development of the embryo, due to the dysplasia of the aortic valve sinus, the middle layer of the sinus wall is weak or missing, making the middle layer of the aortic wall Lack of continuity between the aortic valve annulus, causing a weak point in the base of the aortic valve sinus, post-natal aortic blood flow pressure will gradually push out the weak area of the aortic valve sinus to form aortic aneurysm-like protrusion, Finally, rupture occurs with or without physical labor or trauma, ie, aortic sinus aneurysm rupture is formed. The aortic sinus aneurysm is often windy, with a rupture at the apex. The rupture of the sinus tumor occurs in the right coronary sinus, followed by the non-coronary sinus, and the left coronary sinus is rare. Due to the anatomical relationship, the right coronary artery sinus aneurysm mostly broke into the right ventricular cavity (about 70/100), a few broke into the right atrial cavity, and most of the non-coronary sinus aneurysms broke into the right atrium cavity. Accounted for 70/100), a small amount broke into the right ventricular cavity. basic knowledge Sickness ratio: 0.0004% Susceptible people: no special people Mode of infection: non-infectious Complications: aortic regurgitation, pulmonary stenosis, patent ductus arteriosus

Causes of congenital aortic sinus aneurysm rupture

During embryonic development, due to the dysplasia of the aortic valve sinus, the middle layer of the sinus wall is weak or missing, which causes a lack of continuity between the aortic wall and the aortic valve annulus, resulting in aortic valve sinus The weak point of the basement, the post-natal aortic blood flow pressure gradually pushes out the weak area of the aortic valve sinus to form an aortic aneurysm-like protrusion, and finally ruptures with or without physical labor or trauma. , that is, the formation of aortic sinus aneurysm rupture.

Prevention of congenital aortic sinus aneurysm rupture

According to reports, the incidence rate is higher in Western countries than in Western countries. The incidence rate in China is 1/100~2/100 of congenital heart disease. Hope first described the disease in 1839. In 1958, McGoon, Kirklin et al reported that it was performed under cardiopulmonary bypass. The repairing success was successful. The age of onset of the disease was mostly between 20 and 40 years old. About one-third of the patients had a rapid onset of illness. The domestic surgical mortality rate was about 5-10%, and the long-term operation was satisfactory.

Complications of congenital aortic sinus aneurysm rupture Complications aortic regurgitation pulmonary stenosis patent ductus arteriosus

Aortic sinus aneurysms can often be combined with other cardiac malformations, the most common of which are concurrent ventricular septal defects (about 40 to 50%), which is more important for left and right ventricular load, often accompanied by aortic regurgitation, pulmonary artery Stenosis, aortic coarctation and patent ductus arteriosus.

When the aortic sinus tumor is not ruptured, in addition to severe obstruction of the right ventricle, generally asymptomatic, more common in men, about 40% have a history of sudden precordial pain, often rupture during intense activity, then palpitations, shortness of breath. It can rapidly deteriorate to heart failure, and more patients have slow onset. After exertion, their anger, palpitations, and fatigue are gradually worsening, resulting in loss of mobility.

Congenital aortic sinus aneurysm rupture symptoms Common symptoms Ventricular septal defect severe pain Cardiac malformation conduction block ventricular hypertrophy Heart angina angina chest tightness dyspnea right heart failure

For the diagnosis of aortic sinus aneurysm, based on medical history, physical examination and cardiac continuity murmur, combined with X-ray film and echocardiography, it is generally not difficult to be clear, for some patients with diagnostic suspicion, need to make a differential diagnosis, Right heart catheterization or retrograde aortic angiography is required.

After aortic sinus aneurysm rupture, it usually ruptures into the right hypotensive heart chamber, and blood flows from the high pressure aorta into the low-pressure right ventricle. Due to the obvious pressure gradient between the two, a large number of left-to-right shunts are generated. The hemodynamic changes are similar to patent ductus arteriosus, increased pulmonary blood flow, increased right ventricular load, resulting in right ventricular enlargement, pulmonary hypertension and right heart failure. Aortic sinus aneurysm breaks into the right atrium cavity and causes right atrial pressure. Significantly increased, the right atrium was significantly enlarged, the blood flow in the superior and inferior vena cava was blocked, and the symptoms of right heart failure occurred. The rupture of the aortic sinus aneurysm into the pericardial cavity caused acute cardiac tamponade.

Aortic sinus aneurysms can often be combined with other cardiac malformations, the most common of which are concurrent ventricular septal defects (about 40 to 50%), which is more important for left and right ventricular load, often accompanied by aortic regurgitation, pulmonary artery Stenosis, aortic coarctation and patent ductus arteriosus.

The progress of the disease varies with the size of the rupture. The greater the rupture, the more the left-to-right flow, the early symptoms appear and the disease progresses rapidly.

Unruptured aortic sinus aneurysms do not present clinical symptoms, and symptoms appear after rupture. The majority of the onset age is between 20 and 40 years old. About one-third of the patients have a rapid onset, and suddenly feel the heart before severe labor. Area or upper abdomen severe pain, chest tightness and difficulty breathing, the condition is similar to angina pectoris, the condition deteriorates rapidly, and can die of right heart failure within a few days after the onset. Most cases have a small breach. After the onset, there may be several weeks, months or After several years of remission, the symptoms of right heart failure are present. A small number of patients have very few ruptures. Only a small amount of left-to-right shunts can occur. For a long time, patients can have no symptoms. These patients are often accidentally discovered due to heart murmurs. Diagnosis by echocardiography, right heart catheterization, and aortic angiography.

Physical examination: diastolic blood pressure decreased significantly, pulse pressure difference increased, water rushing pulse, capillary pulsation and femoral artery gunshot sound, tremor could be heard between the 3rd and 4th ribs on the left sternal border during cardiac examination. Typical round or continuous rough noise, the nature of the noise is similar to patent ductus arteriosus, but the location of the noise is low, the case of aortic sinus aneurysm breaking into the right atrium often presents jugular vein engorgement, electrocardiogram: showing left ventricular hypertrophy Or double ventricular hypertrophy, myocardial damage and right bundle branch block, X-ray chest X-ray: see the heart is enlarged, the pulmonary artery segment is prominent, the lung field is hyperemia, the lung texture is increased, echocardiography: showing the middle segment of the aortic sinus wall, diastolic Period of the aortic sinus wall into the right ventricular outflow tract, right heart catheterization: can confirm the increase in blood oxygen saturation in the right atrium, right ventricle or pulmonary artery, suggesting that there is left to right shunt in this part, retrograde aortic angiography: Identify the location of the aortic sinus tumor and the broken heart chamber.

Examination of rupture of congenital aortic sinus aneurysm

Electrocardiogram: showing left ventricular hypertrophy or biventricular hypertrophy, myocardial damage and right bundle branch block.

X-ray chest X-ray: See the obvious enlargement of the heart, the pulmonary artery segment is prominent, and the lung and blood-filled lung texture is increased.

Echocardiography: shows the middle segment of the aortic sinus wall, and the diastolic aortic sinus wall is dislodged into the right ventricular outflow tract.

Right heart catheterization: It can be confirmed that the blood oxygen saturation in the right atrium, right ventricle or pulmonary artery is elevated, suggesting that there is a left-to-right shunt in the department.

Retrograde aortic angiography: the location of the aortic sinus tumor and the broken heart chamber can be identified.

Diagnosis and diagnosis of congenital aortic sinus aneurysm rupture

The following diseases should be differentiated from aortic sinus aneurysms:

(1) patent ductus arteriosus, main pulmonary artery septal defect, such as left-to-right shunt in the aorta, no sudden medical history, machine-like continuous murmur position in the left second intercostal space, noise often toward both lungs and ipsilateral In the subclavian region, echocardiography has left-to-right shunt in the descending aorta and left pulmonary artery. If necessary, right heart catheterization or retrograde aortic angiography can confirm the diagnosis.

(B) ventricular septal defect coexisting aortic valve regurgitation this disease has no sudden history, systolic and diastolic back and forth murmur parts in the left 2,3 intercostal, echocardiography showed ventricular septal echo segment loss and ventricle There is a left-to-right shunt in the cavity, and the aortic valve can show signs of valve insufficiency.

(3) There is abnormal traffic in the left and right coronary arteries and the heart chamber or coronary veins in the coronary arteries, and continuous murmurs can be heard in the anterior region of the heart. The diastolic phase is predominant, and echocardiography or retrograde aortic angiography is seen. The coronary artery is enlarged and the contrast agent is seen to flow from the coronary artery into the heart chamber.

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