Right ventricular double outlet
Introduction
Introduction to the right ventricle double exit The meaning of the right ventricle double outlet originates from the right ventricle in both the aorta and the pulmonary artery, or the majority of one aorta and the other large artery originate in the right ventricle, and the ventricular septal defect is the only outlet of the left ventricle. Ventricular septal defect is usually larger than the aortic diameter. In only 10% of cases, the ventricular septal defect is smaller than the aortic opening. About 60% of the ventricular septal defect is located under the aortic valve, 30% is below the pulmonary valve, and a few cases have ventricular septal defect. The location is in the middle of the aorta and the pulmonary artery opening. In a few cases, the ventricular septal defect is located in the middle and lower part of the ventricular septum and is far from the aortic opening. basic knowledge Sickness ratio: 0.05% Susceptible people: no specific population Mode of infection: non-infectious Complications: acute respiratory distress syndrome, pneumothorax, emphysema, atelectasis, pneumonia, heart failure, arrhythmia, cerebral infarction, chylothorax
Cause
Right ventricular double exit cause
Cause:
The hemodynamic changes in the right ventricle double outlet are mainly determined by the location and size of the ventricular septal defect, and whether or not the pulmonary stenosis and its extent are combined. When the ventricular septal defect is located under the aortic valve without pulmonary stenosis, the left ventricular blood flow is large. Part of the defect directly enters the aorta, while the right ventricle mainly enters the pulmonary artery, and the pulmonary blood flow increases. The clinical and simple ventricular septal defect are similar to pulmonary pulmonary hypertension. When the ventricular septal defect is located under the pulmonary valve without pulmonary stenosis, the left ventricle The blood mainly enters the pulmonary artery through the defect, while the right ventricle mainly enters the aorta. The clinical and complete aortic dislocations are similar to the ventricular septal defect. There is pulmonary congestion and severe purpura. The ventricular septal defect is large, and the left ventricle is unobstructed. Left, The right ventricular pressure is equal, the ventricular septal defect is small, the left ventricle is blocked, the left and right ventricles have a pressure gradient, the left ventricular pressure is higher than the right ventricle, regardless of the location and size of the ventricular septal defect, if there is pulmonary stenosis, clinical Similar to the serious tetralogy of Fallot, there is lung ischemia and severe purpura.
Prevention
Right ventricle double exit prevention
The right ventricular double-extension surgery still has a high mortality rate. The main problem is that the severe pulmonary vascular obstructive disease, unsatisfactory pulmonary artery stenosis, and the combined malformation with severe hemodynamic effects have not been satisfactorily corrected or corrected. Poor, and complicated with low-cardiac syndrome caused by complete atrioventricular block and other factors, it is considered that the pulmonary vascular resistance of patients with right ventricle double exit exceeds 800dyn, S, cm-5 and pulmonary circulation and systemic blood flow The comparison is less than 1:3, and in patients with pulmonary stenosis, the ratio of right ventricular pressure to left ventricular pressure is measured at the end of surgery. If the ratio of the pressure is greater than 0.75, the operative mortality is high, and the common cause of death is heart. Failure, low cardiac output syndrome, hemorrhagic pulmonary edema, arrhythmia, complete atrioventricular block, respiratory failure and infection.
Complication
Right ventricular double outlet complications Complications, acute respiratory distress syndrome, pneumothorax, emphysema, atelectasis, heart failure, arrhythmia, cerebral infarction, chylothorax
The disease often combined with other malformations, including pulmonary valve or funnel stenosis, aortic stenosis, atrioventricular valve malformation, ventricular dysplasia, atrial septal defect, abnormal coronary opening.
The complications of this disease are as follows:
1. Severe pulmonary complications with acute respiratory distress syndrome
Acute respiratory distress syndrome: In addition to the primary symptoms such as trauma, infection, poisoning and other symptoms and signs, mainly manifested as sudden, progressive respiratory distress, shortness of breath, cyanosis, often accompanied by irritability, anxious expression, sweating Etc., the characteristics of respiratory distress can not be improved by the usual therapy, nor can it be explained by other primary cardiopulmonary diseases (such as pneumothorax, emphysema, atelectasis, pneumonia, heart failure).
2, systemic infection: systemic or pulmonary infection secondary to prolonged artificial respiration.
3, arrhythmia.
4, cerebral infarction.
5. VSD residual leakage.
6, body pulmonary artery shunt.
7, superior vena cava obstruction.
Other important complications include tracheotomy, delayed chest closure, delayed wound healing, chylothorax, and hemolysis.
Symptom
Right ventricular double exit symptoms Common symptoms Cardiac malformation Dyspnea purpura finger (toe) Large artery dislocation Pulmonary artery stenosis Ventricular septal defect Heart failure Ventricular hypertrophy
The clinical manifestations of the right ventricle double outlet vary, depending on the type of lesion, the size of the ventricular septal defect and its relationship with the aorta, pulmonary artery, through the ventricular septal defect, left ventricular blood flow direction, pulmonary circulation blood flow and whether it is accompanied by other cardiac malformations. However, regardless of the type of lesion, the sick child presents symptoms in the early postnatal period (average 2 months, ranging from 1 day to 4 years old), the most common being purpura and congestive heart failure, and the severely ill newborns are not. After treatment, early death, radical surgery within 2 months after birth, the surgical mortality rate was as high as 50%, so it is often necessary to perform palliative surgery, such as pulmonary artery bypass or body-pulmonary bypass to prolong life, in recent years 2 The operative mortality rate of radical surgery for children aged around 10 years has been reduced to about 10%. Chest X-ray, ECG and cardiac catheterization vary widely according to different types of lesions. Segmental echocardiography and cardiac angiography are the most reliable. Diagnostic methods, both of which can show that the aortic advance and the pulmonary artery originate in the right ventricle, the anterior valvular lobes and the aorta May not connected between the lobes, and can display each position of the aorta, pulmonary artery opening, ventricular septal defect position and size, and with cardiac malformations such as pulmonary stenosis, mitral valve cleft like.
(1) Right ventricle double outlet, uniform atrioventricular, right aorta with aortic ventricular septal defect without pulmonary stenosis is the most common type, clinical manifestations and large ventricular septal defect with pulmonary hypertension are similar, often showing pulmonary blood flow Increased recurrent respiratory infections, pneumonia, stunting and cardiac insufficiency, chest radiographs: increased heart shadow and pulmonary congestion, electrocardiogram: right ventricular hypertrophy, common indoor conduction block, right heart catheterization: left and right ventricles Pressure and main, pulmonary artery pressure is similar, pulmonary vascular resistance increased, due to left ventricular ejection through the ventricular septal defect into the right ventricle and aortic artery oxygen saturation increased, selective right ventriculography: visible main, pulmonary artery at the same time development.
(2) Right ventricle double outlet, uniform atrioventricular, right aorta, aortic ventricular septal defect, clinical manifestations of pulmonary stenosis similar to severe tetralogy of Fallot, with purpura, sputum, clubbing (toe And hypoxic episodes, chest radiographs: showing lung ischemia, electrocardiogram: left, right atrium, right ventricular hypertrophy, right heart catheterization: characterized by left ventricular ejection through the ventricular septal defect to the right ventricle and then re-entry The aorta, so the right ventricular oxygen saturation is higher than the right atrium, selective right ventriculography: see the right ventricle, aorta, pulmonary artery development at the same time, and see the right ventricular funnel and / or pulmonary stenosis.
(3) Right ventricle double outlet, uniform atrioventricular, right aorta with pulmonary valvular ventricular septal defect, with or without pulmonary stenosis, clinical manifestations of purpura in babies, dyspnea and congestive heart failure, growth retardation, Clubbing (toe), chest radiograph: showing pulmonary congestion and enlarged heart, ECG: right axis deviation, right ventricular hypertrophy, right heart catheterization: left and right ventricular pressure similar to the main, pulmonary artery pressure, right atrium, Right ventricular and pulmonary arterial oxygen saturation increased, selective right ventriculography: right ventricle, aorta, pulmonary artery development at the same time.
(D) right ventricle double outlet, the chamber is consistent with the two aortic openings related to the ventricular septal defect aorta and pulmonary artery opening, the ventricular septal defect is larger, located under the opening of the two aorta, clinical manifestations and aortic valve Ventricular septal defect is similar, large flow rate, mild purpura or heart failure, chest radiograph: showing increased pulmonary blood flow, enlarged heart shadow, electrocardiogram examination: double ventricular hypertrophy, right heart catheterization: right ventricular pressure is similar to systemic arterial pressure Increased blood oxygen saturation in the right ventricle, selective right ventriculography: simultaneous development of the aorta and pulmonary artery, ventricular septal defect under the two large arteries.
(5) Right ventricle double outlet, the chamber is consistent with the ventricular septal defect and the aortic opening of the aorta and the pulmonary artery opening. The ventricular septal defect is located under the cone, and the atrioventricular common channel type under the tricuspid valve or Located in the apex of the apex, clinical manifestations of large ventricular septal defect and pulmonary hypertension symptoms, chest X-ray: pulmonary congestion, increased heart shadow, electrocardiogram: left and right ventricular hypertrophy, right heart catheterization: right ventricular oxygen saturation Increased, selective right ventricular angiography: shows the location of the two large arteries simultaneously developing and displaying ventricular septal defects.
(6) Right ventricle double outlet, inconsistent atrioventricular, often with pulmonary stenosis and right heart, ventricular septal defect mostly located under the pulmonary valve clinical manifestations of purpura in the infancy, hypoxia, chest X-ray: showing the heart and internal organs Position or inverse position, electrocardiogram examination: left and right ventricular hypertrophy, right heart catheterization and right ventricle angiography: left and right ventricular pressure are similar, catheter is not easy to insert into the pulmonary artery, but pulmonary oxygen saturation is increased and pressure is reduced, angiography sees two The aorta originates from the right ventricle, and the ventricular septal defect is located below the supraorbital sac and has a narrowing of the pulmonary valve.
Pathological change
The hemodynamic changes in the right ventricle double outlet are mainly determined by the location and size of the ventricular septal defect, and whether or not the pulmonary stenosis and its extent are combined. When the ventricular septal defect is located under the aortic valve without pulmonary stenosis, the left ventricular blood flow is large. Part of the defect directly enters the aorta, while the right ventricle mainly enters the pulmonary artery, and the pulmonary blood flow increases. The clinical and simple ventricular septal defect are similar to pulmonary pulmonary hypertension. When the ventricular septal defect is located under the pulmonary valve without pulmonary stenosis, the left ventricle The blood mainly enters the pulmonary artery through the defect, while the right ventricle mainly enters the aorta. The clinical and complete aortic dislocations are similar to the ventricular septal defect. There is pulmonary congestion and severe purpura. The ventricular septal defect is large, and the left ventricle is unobstructed. Left, The right ventricular pressure is equal, the ventricular septal defect is small, the left ventricle is blocked, the left and right ventricles have a pressure gradient, the left ventricular pressure is higher than the right ventricle, regardless of the location and size of the ventricular septal defect, if there is pulmonary stenosis, clinical Similar to the serious tetralogy of Fallot, there is lung ischemia and severe purpura.
The right ventricle double outlet typing method Many Lev, Kirklin et al. from the surgical point of view, according to the relationship between the anatomical position of the ventricular septal defect and the arterial trunk are as follows:
(1) aortic ventricular septal defect; (2) ventricular septal defect of the pulmonary valve; (3) ventricular septal defect associated with the opening of the two arteries; (4) ventricular septal defect unrelated to the opening of the two major arteries.
I right ventricle double exit, the room relationship is consistent
Aortic ventricular septal defect
With pulmonary stenosis;
No pulmonary artery stenosis.
2. Pulmonary ventricular septal defect
With pulmonary stenosis;
No pulmonary artery stenosis.
3. Ventricular septal defect associated with two major arterial openings
Pulmonary artery stenosis
No pulmonary stenosis
4. Ventricular septal defect unrelated to the opening of the two major arteries
With pulmonary stenosis;
No pulmonary artery stenosis.
II right ventricle double exit, inconsistent relationship between rooms
Aortic ventricular septal defect
No or pulmonary stenosis.
2. Pulmonary ventricular septal defect
No or pulmonary stenosis.
3. Ventricular septal defect associated with two major arterial openings
No or pulmonary stenosis
4. Ventricular septal defect unrelated to the opening of the two major arteries
No or pulmonary stenosis.
III Other complex types
Complete venous venous drainage, complete common atrioventricular access, mitral atresia or stenosis, aortic stenosis or hypoplasia.
Examine
Right ventricular double exit check
History
Clinical manifestations depend on the relationship between aortic and pulmonary opening and ventricular septal defect, pay attention to the presence or absence of pulmonary stenosis, funnel and pulmonary stenosis, similar to tetralogy of Fallot, may have hypoxic fainting, convulsions and symptoms Finger toe, no pulmonary artery stenosis, increased pulmonary blood, prone to pulmonary hypertension, similar to large ventricular septal defect.
2. Physical examination
The anterior region of the anterior region, the left sternal border 3,4 intercostal rough systolic murmur, accompanied by tremor, the second sound of the pulmonary artery reduced or disappeared.
3. ECG
Patients with no pulmonary stenosis, right ventricular hypertrophy, left ventricular hypertrophy, prolonged PR interval, common indoor conduction block, with pulmonary stenosis, right atrial hypertrophy and right ventricular hypertrophy and indoor conduction block, also There is left ventricular hypertrophy and prolonged PR interval.
4. X-ray inspection
Generally, the heart shadow is enlarged, the left 4th arch is prolonged, and the pulmonary artery stenosis is reduced. The pulmonary blood flow is reduced similar to the tetralogy of Fallot. In patients without pulmonary stenosis, the pulmonary blood flow is increased, similar to the giant ventricular septal defect with pulmonary hypertension.
5. Cardiac catheterization and right heart catheterization
In patients without pulmonary stenosis, the left and right ventricular pressures are equal, but the ventricular septal defect is small, the left ventricular pressure is higher than the right ventricle and pulmonary artery pressure, the pulmonary artery stenosis, the pulmonary artery pressure is reduced, and the right ventricular oxygen saturation is higher than the right atrium. Right ventricular angiography, pulmonary artery development at the same time.
6. Echocardiography
The aorta is shown, the pulmonary artery originates from the right ventricle, or one aorta originates from the right ventricle, and the other aorta 90% originates from the right ventricle. The aorta is in the same plane as the pulmonary artery. The aortic valve and the mitral valve have no fiber connection.
Diagnosis
Diagnosis of right ventricle double exit diagnosis
Differential diagnosis
In terms of pathogenesis, DORV is derived from abnormal development of the articular cone. It is a transitional disease between tetralogy of Fallot and complete transposition of the great arteries. Therefore, it should be clinically associated with tetralogy of Fallot and complete transposition of the great arteries. Identification:
1, tetralogy of Fallot
The diagnosis of tetralogy of Fallot is limited to the four pathological anatomy proposed by Fallot:
1 pulmonary artery stenosis.
2 high ventricular septal defect.
3 aorta rides across.
4 right ventricular hypertrophy, after many experts studied, the connotation of pathological anatomy was deepened, and the pathological anatomy of the quadruple syndrome was defined as a cardiac malformation composed of characteristic ventricular septal defect and pulmonary stenosis, and the ventricular septal defect was huge, about equal to Aortic opening, located under the aorta, more common simple ventricular septal defect, pulmonary stenosis has funnel stenosis, there may be other parts of the stenosis, the aortic part originates from the right ventricle, but the mitral valve and aorta There is fiber continuity between the petals, as well as right ventricular hypertrophy.
Generally manifested as early cyanosis, poor respiratory endurance, sputum; systolic ejection murmur on the left sternal border and second heart sound in the pulmonary artery area, red blood cell count, increased hemoglobin and hematocrit, decreased arterial oxygen saturation, heart It has a boot-like shape, a small vascular texture in the lungs, and an echocardiogram showing aortic straddle and ventricular septal defect.
2, complete transposition of the great arteries
Complete aortic dislocation refers to the misplacement of the two aorta, the aorta receives venous blood from the right ventricle, and the pulmonary artery receives oxygen from the pulmonary veins of the left ventricle, thus forming two isolated circulatory systems, the right atrium. right ventricle aorta whole body body vein right atrium is a cycle; left atrium left ventricle pulmonary artery lung pulmonary vein left atrium is another circulatory system, most patients die in the neonatal period, surviving patients All are associated with other malformations, including: atrial septal defect, ventricular septal defect, patent ductus arteriosus, pulmonary stenosis, aortic coarctation, etc., clinical manifestations of dyspnea, purpura, progressive heart enlargement and early heart failure, Due to the different malformations, the degree of pulmonary congestion and blood circulation of the pulmonary circulation are different, and the symptoms and time of occurrence are different. Chest X-ray examination shows obvious heart enlargement and pulmonary blood increase, mainly relying on echocardiographic diagnosis, cardiovascular Contrast imaging is only used when there is a suspected multiple ventricular dysplasia or when the anatomy of the aortic arch needs to be clarified.
