Corrected aortic malposition
Introduction
Introduction to corrective aortic dislocation The curvature of the normal primitive heart tube protrudes to the right, hence the name of the right wing (D-Loop), so that the right ventricle is located on the right side, and the left ventricle is located on the left side, as in the development process, the tube is not bent to the right but To the left, called L-Loop, the anatomical right ventricle is located at the left rear and becomes the ventricle of the arterial system, while the anatomical left ventricle is located at the right front, becoming the ventricle of the venous system. At this time, the arterial trunk is still separated. Rotate, but the position is reversed, so that the main pulmonary artery is located at the right rear and the ascending aorta is located at the left front. Although the position of the large blood vessel is reversed, the pulmonary artery is still connected to the venous ventricle, and the aorta is connected to the ventricle of the artery, and the blood direction is physiologically Correction, it is called corrective aortic dislocation. The left and right branches of the atrioventricular conduction beam are still distributed to the corresponding anatomical ventricle, but the circumferential ventricle is opposite in position, so the conduction beam is also opposite. basic knowledge The proportion of sickness: 0.00001% Susceptible people: no special people Mode of infection: non-infectious Complications: atrial septal defect tricuspid regurgitation heart failure heart failure
Cause
Corrective aortic dislocation
The disease is a rare congenital heart disease, because during the process of embryonic development, the central tube is not bent to the right but to the left, resulting in the anatomy of the right ventricle at the left rear, becoming the ventricle of the arterial system, and anatomy The left ventricle is located in the right front and becomes the ventricle of the venous system. At this time, the arterial trunk is still separated and rotated, but the position is reversed, and the blood direction is still physiologically normal, although the left and right branches of the atrioventricular conduction beam are still distributed to Corresponding anatomical ventricles, but the circumferential ventricle position is opposite, so the conduction beam is also opposite.
Prevention
Correction of large artery dislocation prevention
This disease is a rare congenital heart disease. There is no preventive measure at present. Because this disease often combines with other cardiac malformations, it is mainly clinically to diagnose the combined diseases and take appropriate surgical treatment.
Complication
Corrective aortic dislocation complications Complications, septal defect, tricuspid regurgitation, heart failure, heart failure
Corrected aortic dislocation (CTGA) often combined with intracardiac malformation, mainly ventricular septal defect, pulmonary stenosis, including atrial septal defect, right ventricular double exit and other lesions, the previous treatment of CTGA with intracardiac malformation, mostly using the Restelli method To achieve the purpose of physiological correction, the shortcoming of the operation is that the left ventricle still bears the pulmonary circulation blood pump, and the anatomy of the right ventricle long-term burden system circulation, due to the anatomical features of the right ventricle, it is difficult to bear the long-term high pressure, high resistance system circulation Late-stage tricuspid regurgitation and right heart failure (functional left heart failure), the 10-year and 20-year survival rates were 74% and 46%, respectively. The most common cause of death was a pulmonary infection. Heart failure, tricuspid regurgitation and complete heart block, which are the most common complications of this disease.
Symptom
Corrected aortic dislocation symptoms common symptoms aortic dislocation arrhythmia tachycardia conduction block pulmonary infection cardiac malformation ventricular septal defect pulmonary stenosis heart failure cyanosis
Mainly depends on the combined cardiac malformation, according to different malformations, the clinical common is:
1. Left to right shunt group: with ventricular septal defect, often with shortness of breath, decreased activity, weight loss, lung infection and heart failure, signs similar to ventricular septal defect.
2. Right to left shunt group: with pulmonary stenosis, common purpura and hypoxic episodes.
3. Left atrioventricular valve insufficiency group: the ventricular septum is complete, the murmur is located in the fourth intercostal space adjacent to the sternum, but not in the apical region, often with arrhythmia.
Upper and lower vena cava blood returns to the normal right atrium, enters the right ventricle through the atrioventricular valve of the second leaf, and then enters the pulmonary artery. The blood of the pulmonary vein returns to the normal left atrium and enters the left through the atrioventricular valve of the three leaves. The ventricle of the position is then sent to the aorta. The structure of the right ventricle is the same as that of the normal left ventricle, while the structure of the left ventricle is the same as that of the normal right ventricle. Although the left and right ventricles are reversed, the aorta is also dislocated. Therefore, the direction of blood flow is still in line with the normal body, the requirements of the pulmonary circulation, without other malformations, can be asymptomatic, but most of the clinical cases are combined with other malformations, common ventricular septal defect, pulmonary stenosis, left atrioventricular valve Insufficient closure, aortic stenosis, pre-excitation syndrome, atrioventricular block and paroxysmal tachycardia.
Examine
Correction of aortic dislocation
The disease has the following methods:
(1) Chest radiography: There is a slightly oblique ascending aorta shadow on the left upper edge of the heart, lacking common lung artery shadow, abnormal heart position, such as right heart and normal internal organs or median heart or left heart with visceral transposition .
(2) Electrocardiogram examination: atrial arrhythmia and abnormal ventricular depolarization are the main features. Atrioventricular block accounts for about 1/3 of cases, and atrioventricular septum, arrhythmia at junction, paroxysmal tachycardia And ventricular premature beats.
(3) Echocardiography: the posterior atrioventricular valve (tricuspid valve) and the semilunar valve are not connected. The semilunar valve is located in a plane on the left front and a right posterior direction. The contraction time interval may indicate that the front aorta is connected to the systemic circulation. The posterior aorta flows into the lungs.
(4) right heart catheterization and cardiovascular angiography: can show the contour of the ventricle, aorta, location and relationship, diversion and clear diagnosis.
Diagnosis
Diagnosis and correction of corrective aortic dislocation
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
The clinical identification of this disease is mainly related to the identification of ventricular septal defect and tetralogy of Fallot:
1. The left-to-right shunt group is confused with the large ventricular septal defect. The clinical symptoms are difficult to distinguish, mainly determined by echocardiography and right heart catheterization, and cardiovascular angiography.
2. The right-to-left shunt group is similar to the tetralogy of Fallot. The quadriplecosis is obvious, the heart is shoe-shaped, right heart catheter and angiography, and Doppler echocardiography is a clear diagnosis.
