"Fatal" complete atrioventricular septal defect
Hospital Name: Suzhou Hospital Affiliated to Nanjing Medical University
Model: S60
Patient information: Ruan, 25 years old, pregnant 1, gave birth 0; after 24 weeks of menopause, he found heart abnormality and came to our hospital for consultation for echocardiography, and other parts were normal.
Pregnancy examination: fetal NT examination and Down's screening in early pregnancy were normal. No fever, cold or family history.
Ultrasound findings:
1. Normal cardiothoracic proportion, fetal heart rate 135 times / min, no obvious abnormal heart rate.
2. Most of the fetal heart is located in the left thoracic cavity, the apex of the heart points to the left, and the heart accounts for about 1 / 3 of the thoracic cavity. The inner diameter of right atrium and right ventricle was larger than that of left atrium and left ventricle. The "ten" cross structure disappeared in the heart, and a large segment of echo was interrupted in the lower and upper parts of the atrial septum. Color Doppler showed the blood flow of four chambers. A large blood flow bundle in diastole entered the ventricle through the common atrioventricular valve, and the atrioventricular regurgitation was seen in systole. Only the common atrioventricular valve opens to the left and right ventricles.
3. It can be seen that the two major arteries originate from the right ventricle in parallel. The aorta is in front of the right, the aortic diameter is obviously widened, the inner diameter is about 4.6mm, and the valve opening and closing activities are acceptable; the pulmonary artery is in the back of the left, the inner diameter is obviously thinned, and the inner diameter of the valve ring is about 1.7mm. Color blood flow can be seen in pulmonary artery, and the maximum flow velocity of pulmonary artery is 137cm / s by continuous Doppler.
4. The three vessel trachea section showed that the aortic arch was on the right side of the trachea, and the long axis of the active arch was visible.
5. The superior and inferior vena cava flow into the right atrium, and at least one pulmonary vein is connected to the left atrium.
Ultrasound prompts:
Complex congenital malformation of fetus
1. Complete atrioventricular septal defect (right ventricular dominant type)
2. Right ventricular double outlet
3. Pulmonary artery stenosis
4. Right aortic arch
The fetal four chamber section showed that the left ventricle was significantly reduced, the right ventricle was enlarged, and the left atrium was smaller than the right atrium; the continuity of the middle and lower part of the atrial septum and the upper part of the ventricular septum was interrupted, and the cross image of "ten" disappeared, only a group of common atrioventricular valves were seen, the common valves crossed the defect of the atrioventricular septum, and the attachment point of the atrioventricular valves on the ventricular septum could not be shown, and the four chambers communicated with each other.
Color Doppler showed the blood flow of four chambers. A large blood flow bundle entered the ventricle through the common atrioventricular valve in diastolic period, and the atrioventricular regurgitation was seen in systolic period.
The main artery originated from the right ventricle in parallel. The aorta was in front of the right. The diameter of the aorta was obviously widened and the pulmonary artery was narrow.
Color Doppler showed that the flow signal of pulmonary artery was bright and colorful, and the flow velocity of pulmonary artery was 137cm / s.
Aortic arch blood flow section
discuss
summary:
Atrioventricular septal defect (AVSD) is also called endocardial cushion defect (ECD) or atrioventricular common channel malformation. It is a complex congenital heart malformation that the embryo of the endocardial cushion does not develop well and it fails to fuse with the primary septum, the conical septum and the muscular ventricular septum, resulting in the atrioventricular septal defect and the atrioventricular valve abnormality. Atrioventricular septal defect can be divided into complete atrioventricular septal defect (CAVSD), partial atrioventricular septal defect (pavc) and transitional atrioventricular septal defect (tavc). CAVSD is the most serious disease, accounting for about 3% of congenital heart malformation.
According to the anatomic connection of anterior chordae tendineae, CAVSD can be further divided into:
Type A is the most common, accounting for about 75%. The anterior flap has tendinous cords attached to the top or both sides of the interventricular septum.
Type B is rare. The anterior mitral chordae tendineae are attached to the papillary muscle of the right ventricular surface of the interventricular septum through the interventricular septum.
There is no bicuspid or tricuspid valve in type C common atrioventricular valve. The tendinous cord is floating, accounting for about 25%
If the common atrioventricular valve is evenly connected with the two ventricles, it is called balanced atrioventricular septal defect. If most of the common atrioventricular valve is connected with one ventricle, it is called unbalanced type. If the right ventricle is obviously enlarged and the left ventricle is underdeveloped, it is called the right ventricular dominant type. If the left ventricle is obviously enlarged and the right ventricle is underdeveloped, it is called the left ventricular dominant type.
Among the congenital heart diseases detected by fetal echocardiography, CAVSD accounted for 14% - 17%.
CAVSD should be wary of macrovascular malformation and other cardiac malformations, Fetal CAVSD is often associated with chromosomal abnormalities, about 50% of which are associated with chromosomal abnormalities, especially trisomy 21 syndrome (about 60%), which is also the main cause of multiple malformations of CAVSD. But not all pregnant women with CAVSD have chromosomal abnormalities.
Diagnostic points
Complete atrioventricular septal defect (CAVSD):
1. CAVSD showed that the normal atrioventricular crisscross structure disappeared in the four chamber section, and the four chambers were connected, only one group of atrioventricular valve opening and closing activity was seen.
2. Room interval length: room interval length. It should be 2:1 in normal condition and 1:1 in complete endocardial cushion defect. In other words, short ventricular septum should be highly vigilant.
3. Different degrees of common atrioventricular regurgitation can be seen in systole by color Doppler flow.
4. Multiple malformations, such as CAVSD with macrovascular abnormality, are more common in fetal period. CAVSD should be wary of macrovascular malformation and other intracardiac malformations. The most common malformations were other cardiac malformations, respiratory system malformations and motor system malformations.
5. Fetal CAVSD is often associated with chromosomal abnormalities, about 50% of which are associated with chromosomal abnormalities, especially trisomy 21 syndrome (about 60%). But not all pregnant women with CAVSD have chromosomal abnormalities.
Differential diagnosis:
CAVSD should be differentiated from VSD, VSD with large vascular malformation and single ventricle
Direct signs of VSD: local echo loss of VSD; 2) indirect signs: left ventricular volume overload, pulmonary artery dilation, etc. in moderate or above VSD, left ventricle and left atrium are often enlarged due to more left to right shunt flow. Long term left ventricular volume overload can lead to pulmonary artery pressure rise and pulmonary artery dilation.
Single ventricle often has complex malformation. The atrioventricular valves on both sides or common atrioventricular valves are connected with the main chamber. More than 50% of the atrioventricular valves on one side and the atrioventricular valves on the other side are connected with the main chamber for diagnosis. The main chamber and residual chamber are mostly arranged in front and back directions. Color Doppler showed that the blood flow from both sides of the atrium was injected into the main cardiac cavity.
The main differences in the types of DORV are the spatial relationship of the great arteries, the location of VSD and the presence or absence of outflow obstruction. The abnormality of the five chamber section of DORV showed that the VSD and the two arteries originated from the anterior chamber (right ventricle). DORV is also the most common combined malformation of CAVSD.
Prognosis: CAVSD combined with many kinds of cardiovascular malformations are more common in fetus, and it is difficult to survive after birth. Therefore, it is very important to detect CAVSD with other macrovascular malformations and make a comprehensive exploration for the prognosis of the fetus.
reference
1. Analysis of the ultrasonic characteristics of fetal endocardial defect with large artery malformation by sun Lanlan and Wu Yafeng
2. Guidelines for fetal echocardiography, translated by Liu Lin, translated by he Yihua, reviewed by Beijing Science and Technology Press, July 2017, Third Edition
3. Autopsy analysis of 35 cases of fetal complete atrioventricular septal defect by Zhong Pingping and Gu Yiqun
4.A Practical Guide to FetalEchocardiography NORMAL AND ABNORMALHEARTS second Edition AIfred AbuhamadRabih Chaoui
5. The relationship between fetal atrioventricular septal defect and chromosomal abnormality
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