What is Pulmonary Atresia?
A rare, severe congenital heart malformation. The basic disease is atresia of pulmonary valve, pulmonary artery trunk or its branches, but atresia of pulmonary valve is more common. According to the presence or absence of ventricular septal defect, it can be divided into two categories: complete pulmonary septal atresia and pulmonary atresia complicated by ventricular septal defect. Children with this disease have cyanosis after birth and mostly die during infancy. Survivors often have intracardiac malformations.
- Chinese name
- Pulmonary atresia
- Foreign name
- Pulmonary atresia
- A rare, severe congenital heart malformation. The basic disease is atresia of pulmonary valve, pulmonary artery trunk or its branches, but atresia of pulmonary valve is more common. According to the presence or absence of ventricular septal defect, it can be divided into two categories: complete pulmonary septal atresia and pulmonary atresia complicated by ventricular septal defect. Children with this disease have cyanosis after birth and mostly die during infancy. Survivors often have intracardiac malformations.
Pulmonary Atresia Pathology and Pathophysiology
- 1. Pulmonary valve atresia, complete ventricular septum, interatrial communication (or ASD, or oval foramen), and PDA are necessary for survival.
- 2. The size of the right ventricle is variable and related to the survival rate. It can be divided into 3 types according to the development of the right ventricle.
- Type I: Also known as three-structure type, the three parts of the right ventricular cavity (inflow tract, muscle trabecular part, and funnel part) all exist, and the right ventricle is close to normal size.
- Type II: Also known as the second structure type, the inflow channel and the funnel are present, but there is no muscle beam.
- Type : Also known as single structure type, it only exists in the inflow section. In the third type, the right ventricle is small and there is almost always a coronary sinus space. The so-called coronary sinus space refers to the abnormal blood vessels in the muscle trabeculae communicating with the coronary arteries. Due to the obstruction of the right ventricular outflow tract, the right ventricle is formed. In ventricular hypertension, the vascular sinus of the trabecular muscle of the right ventricle remains open, especially during systole. A large amount of blood passes through the vascular sinus and the coronary arteries enters the aorta, forming a right-to-left shunt.
- Three types of ventricular septal intact pulmonary artery atresia. A: Normal right ventricle. B: Type I: All three parts of the right ventricle (inflow channel, trabecular muscle, and funnel) are present. C: Type II: Only the inflow channel part and the funnel part are present. D: Type III: Only the entrance to the right ventricle.
- 3. Right ventricular hypertension is often decompressed by dilating sinusoidal blood vessels to the left or right coronary arteries, and tricuspid regurgitation often exists. The size and amount of sinusoidal space is directly proportional to right ventricular pressure. Tricuspid regurgitation is inversely proportional, and surgical mortality is high in cases of proximal coronary obstruction.
- 4. The pathophysiology is similar to tricuspid valve atresia. The right atrium shunts the return blood from the body veins to the left atrium, and the right atrium is enlarged. How much depends on whether the PDA is open or not, and closing it after birth causes death.
Differential diagnosis of pulmonary atresia
- Two-dimensional echocardiography and color Doppler examination are helpful for diagnosis. Cardiac angiography showed that the blind vent of the right ventricle exit had no direct communication with the pulmonary artery and could be diagnosed.
Pulmonary atresia
- X-ray film: The heart rapidly enlarges after birth, and even more complicated with tricuspid valve insufficiency. In both types, the right atrium and left ventricle are enlarged, the left atrium is also slightly enlarged, the ascending arch of the aorta is dilated, and the pulmonary vein texture is significantly reduced. Pulmonary artery segment is depressed or straight in the heart and waist, and the shadow of the heart is more like a boot shape. In the early postnatal period, the heart is progressively enlarged, the aorta is dilated, the pulmonary artery segment is depressed, and the pulmonary vein texture is significantly reduced. The electrocardiogram shows an enlarged atrium. Left axis deviation or left ventricular hypertrophy reflects right ventricular contraction; right ventricular enlargement can see right ventricular hypertrophy. Right ventricular angiography shows that the structure of the right ventricle is superior to venous cardiovascular angiography. At the right ventricular outflow tract the valve level is completely obstructed. The right ventricular cavity shrinks and can show the sinusoid of the myocardium. If the right ventricle is not small or enlarged, tricuspid regurgitation is often seen. From the vein or right atrium, right to left shunts can be seen at the atrial level, the left ventricle and the aorta are early developed, and some blood flows through the arterial catheter into the pulmonary artery.
Pulmonary atresia treatment principles
- This disease can be treated surgically. If the right ventricle and tricuspid valve are well developed, Rastelli surgery is preferred. If the right ventricle and tricuspid valve are not fully developed, modified Fontan surgery or total vena cava-pulmonary arterial connection can be used.