Congenital pulmonary atresia

When women reach the middle stage of pregnancy, they will undergo some examinations to check whether the fetal organs are fully developed, such as examinations for fetal heart disease. However, such examinations are not 100% accurate. Some children still develop congenital pulmonary atresia after birth. Pulmonary atresia refers to a congenital heart disease. Although it can be treated surgically, it will require several operations in the later stages, but it is ultimately curable.

Pulmonary atresia is a rare congenital heart disease, accounting for about 1% to 3% of congenital heart diseases. Pulmonary atresia with intact ventricular septum refers to atresia of one or more of the main pulmonary artery, pulmonary valve, and left or right bifurcation of the pulmonary artery, often accompanied by varying degrees of hypoplasia of the right ventricle and tricuspid valve. The ventricular septum was intact and the relationship of the great arteries was normal. Accompanied by ventricular septal defect, also known as pseudo-truncus arteriosus, is the most serious type of tetralogy of Fallot. Due to the closure or absence of the pulmonary valve, there is no channel between the right ventricle and the pulmonary artery, and the pulmonary trunk itself may also be closed or poorly developed.

More than 90% of infants develop cyanosis at birth or very shortly after birth, and the condition progressively worsens. The degree of cyanosis depends primarily on blood flow to the lungs via the ductus arteriosus and other systemic-pulmonary arterial communications. If the ductus arteriosus is small and cyanosis is severe, survivors will have clubbing of the fingers and toes. Growth and development disorders, often with palpitations and shortness of breath after activities, but squatting is rare. If the body-lung communication is larger and the cyanosis is lighter, respiratory tract infections are more likely to occur, and heart failure may often occur early. If the ductus arteriosus tends to close, cyanosis will progressively worsen. The arterial oxygen partial pressure of critically ill children can drop to 20 mmHg, and the blood oxygen saturation is only about 40%. Right heart failure is more common in patients with tricuspid regurgitation, with hepatomegaly, edema and gallop rhythm in the apical area. If the baby is born naturally, he or she will generally develop well, but will have cyanosis, dyspnea and metabolic acidosis after birth.

treat

The treatment of congenital pulmonary atresia has always been one of the most concerned issues for many patients with congenital pulmonary atresia. Wang Qiang, Department of Pediatric Cardiac Surgery at Beijing Fuwai Hospital, pointed out that congenital pulmonary atresia is often accompanied by large systemic-pulmonary collaterals and systemic-pulmonary shunts. In order to establish normal blood flow into the lungs, the left and right pulmonary arteries are poorly developed, which will improve after surgery and the collaterals will close. Elective surgery or staged surgery at a later stage can sometimes achieve the goal of radical cure.

The development of the pulmonary artery determines the complexity of surgery for congenital pulmonary atresia. If the pulmonary artery develops well, radical surgery can be performed.

Type 1 and 2 congenital pulmonary atresia are rarely associated with somatic pulmonary collaterals. Systemic pulmonary collaterals are very dangerous, and the patient may not be able to get off the operating table. Patients with congenital pulmonary atresia must undergo angiography to observe whether systemic pulmonary collaterals exist. If so, intervention should be performed first, and then surgery. During the interventional occlusion, the child developed symptoms of hypoxia, and timely surgery was performed to relieve the patient's hypoxia.

The ductus arteriosus of type 3 and 4 congenital pulmonary atresia is basically closed and is mainly maintained by systemic pulmonary collaterals. A systemic-pulmonary shunt surgery is performed first, and then the development of the pulmonary vessels is observed. Outpatient follow-up is performed until the pulmonary vascular development improves and radical surgery is performed at an appropriate time.