Small aorta and pulmonary arteries
Introduction
Introduction The tricuspid valve and/or posterior valve occasionally attach to the proximal apical wall of the proximal apex, which accounts for 0.5% to 1.0% of congenital heart disease. Pulmonary blood is less and heart shadow is enlarged, aortic and pulmonary artery shadows are small, and heart shadow is like a balloon.
Cause
Cause
The main pathological anatomical features of this malformation are right ventricular atrialization and functional right ventricular stenosis. The position of the right atrioventricular ring is normal (often enlarged). The anterior lobes of the tricuspid valve are normally attached to the annulus fibrosus, while the attachment points of the temporal and posterior lobes are obviously moved down and down the endocardium of the right ventricular wall. The extent of the movement and the manner of attachment vary from person to person, even if the attachment point of the valvular valve is adjacent to the annulus fibrosus, but because the leaflets are too long, they can often adhere to the right ventricular wall at different distal ends of the annulus.
Examine
an examination
Related inspection
Cardiovascular angiography two-dimensional echocardiography electrocardiogram electron beam CT
The following points have a reference value when diagnosing this disease:
1. The symptoms of bruising in the neonatal period are obviously reduced or completely disappeared, and the cyanosis reappears after the elderly.
2. Cyanosis symptoms combined with tachyarrhythmia should first consider the disease.
3. Right-to-left shunt congenital heart disease, less pulmonary blood and no right ventricular hypertrophy.
4. The heart is enlarged, but the pulsation in the anterior region is very weak. There is a "multi-temporal" heart sound during auscultation.
5. Pulmonary blood is less and the heart is enlarged. The aorta and pulmonary artery are small, and the heart is like a balloon.
6.P-port high, but no right ventricular hypertrophy.
7. The right heart lead shows a complete right bundle branch block and a small multi-phase wide QRS wave.
8. There are blue-purple with B-type pre-excitation syndrome, and the pulmonary blood is less.
9. There are QR wave and T wave inversion on the V1~4 lead.
10. Echocardiography shows the tricuspid attachment point moving down.
Ebstein syndrome can be classified into 3 types based on clinical manifestations and hemodynamic changes during diagnosis:
1 light type: no or mild cyanosis, cardiac function I ~ II grade heart light ~ moderate increase, intracardiac shunt mainly left to right, no difference between right atrium and functional right ventricle angiography without ball The levy does not require surgery or only closes the intracardiac defect, and the prognosis is good.
2 stenosis type: cyanosis is obvious, heart function is above grade II, heart is mild to moderately increased, pulmonary circulation blood flow is reduced, there is pressure difference between enlarged right atrium and functional right ventricle, intracardiac shunt is right to left, cardiovascular The angiography sees the double ball sign and requires surgery.
3 insufficiency type: no or mild cyanosis, heart function level II or above, heart severity increased, no pressure difference between right atrium and functional right ventricle, intracardiac shunt can be left to right or right to left, angiography visible The right atrium is extremely large, with a double ball sign and requires surgery.
Diagnosis
Differential diagnosis
The following points have a reference value when diagnosing this disease:
1. The symptoms of bruising in the neonatal period are obviously reduced or completely disappeared, and the cyanosis reappears after the elderly.
2. Cyanosis symptoms combined with tachyarrhythmia should first consider the disease.
3. Right-to-left shunt congenital heart disease, less pulmonary blood and no right ventricular hypertrophy.
4. The heart is enlarged, but the pulsation in the anterior region is very weak. There is a "multi-temporal" heart sound during auscultation.
5. Pulmonary blood is less and the heart is enlarged. The aorta and pulmonary artery are small, and the heart is like a balloon.
6.P-port high, but no right ventricular hypertrophy.
7. The right heart lead shows a complete right bundle branch block and a small multi-phase wide QRS wave.
8. There are blue-purple with B-type pre-excitation syndrome, and the pulmonary blood is less.
9. There are QR wave and T wave inversion on the V1~4 lead.
10. Echocardiography shows the tricuspid attachment point moving down.
Ebstein syndrome can be classified into 3 types based on clinical manifestations and hemodynamic changes during diagnosis:
1 light type: no or mild cyanosis, cardiac function I ~ II grade heart light ~ moderate increase, intracardiac shunt mainly left to right, no difference between right atrium and functional right ventricle angiography without ball The levy does not require surgery or only closes the intracardiac defect, and the prognosis is good.
2 stenosis type: cyanosis is obvious, heart function is above grade II, heart is mild to moderately increased, pulmonary circulation blood flow is reduced, there is pressure difference between enlarged right atrium and functional right ventricle, intracardiac shunt is right to left, cardiovascular The angiography sees the double ball sign and requires surgery.
3 insufficiency type: no or mild cyanosis, heart function level II or above, heart severity increased, no pressure difference between right atrium and functional right ventricle, intracardiac shunt can be left to right or right to left, angiography visible The right atrium is extremely large, with a double ball sign and requires surgery.
