One-stage intracardiac repair surgery for tetralogy of Fallot with absent pulmonary artery

The definition of tetralogy of Fallot with pulmonary valve dysfunction includes pulmonary aneurysm-like enlargement, ventricular septal defect with abnormal paraval, pulmonary valve deficiencies, and pulmonary annulus stenosis. This malformation is about 3% in the quadruple syndrome. In 1947, Cheverz first discovered this deformity and elaborated on its pathological anatomy. In 1973, Litwin et al recommended two procedures. Boston Children's Hospital does not approve of staged surgery, and advocates the first phase of intracardiac repair in the early stage, the treatment effect is improved. In cases of this malformation, traces of the pulmonary valve between the right ventricle and the pulmonary artery are often found, with a ring of jelly-like nodules. A few are completely smooth. The heart enlargement is mainly the enlargement of the right ventricle and the funnel. Pulmonary annulus stenosis and stenosis or stenosis of the funnel and abnormal ventricular septal defect. Most of the severe cases are neonates after birth, and the pulmonary trunk and bilateral pulmonary aneurysms enlarge the compression bronchus. It is worth noting that pulmonary aneurysm-like enlargement can extend to the lung segment and the internal pulmonary artery, hindering bronchial development and alveolar proliferation, thus affecting long-term effects. In addition to the two ventricular systolic pressure and pulmonary blood flow reduction, the pathophysiology of quadruple syndrome with pulmonary valve deficiencies has the following characteristics: 1 indoor shunt. Postpartum cyanosis is due to high pulmonary vascular resistance and pulmonary stenosis sufficient to produce an indoor right to left shunt. However, as the pulmonary vascular resistance decreased 1 week after birth, the right ventricular dysfunction and the reduction of pulmonary regurgitation resulted in a reduction in the right ventricular diastolic load, resulting in an indoor left to right shunt. 2 respiratory insufficiency. The enlarged tumor-like pulmonary artery compresses the bronchus on both sides to produce hypoxia, and the severe one can be fatal due to acidosis. However, in some patients, tracheal bronchial tissue maturation and caliber increase one week after birth, as well as increased right ventricular outflow tract obstruction and pulmonary artery pressure drop and/or pulmonary valve insufficiency reduction are beneficial to the improvement of lung function. 3 right ventricular dysfunction. With ageing to 10 to 15 years of age, due to this deformity, long-term right ventricular outflow tract obstruction and pulmonary valve insufficiency, the right ventricle can not bear stress and capacity overload and produce severe cyanosis and refractory heart failure. In the past, it has been reported that staging surgery is performed on this deformity, but the effect is not good. It is currently believed that early stage of intracardiac repair can achieve the purpose of relieving the expansion of the tumor-like pulmonary artery to the bronchus and correcting the pulmonary regurgitation. Treatment of diseases: ventricular septal defect Indication 1. Severe neonates with quadriplegia and pulmonary valve dysfunction, often with medical treatment is ineffective, emergency treatment of tetralogy of Fallot with pulmonary artery dysfunction in the first stage of intracardiac repair surgery. 2. After the patient has passed the infancy, the patient should be scheduled to undergo surgery at 3 to 5 years of age. Contraindications 1. Poor physical fitness, can not tolerate the operator; 2. Patients with impaired coagulation function; 3. Patients with a history of allergies to anesthetics. Preoperative preparation 1. Severe babies have severe breathing difficulties after birth, prone position can alleviate symptoms. Timely tracheal intubation for mechanically assisted breathing. 2. Actively treat metabolic acidosis and heart failure, as well as sucking and keeping the airway open. 3. After the diagnosis is confirmed by echocardiography, the first-stage intracardiac repair operation with tetralogy of Fallot and pulmonary artery deficiency is performed as soon as possible. 4. The preoperative preparation of mild patients is similar to the simple tetralogy of Fallot. Surgical procedure After the patient enters the operating room, changing from the prone position to the supine position often causes severe cyanosis and life-threatening. At this time, ECG, arterial oxygen saturation and blood pressure should be monitored for tracheal intubation and mechanically assisted breathing in the prone or semi-prone position. After turning over, he was in supine position and was anesthetized. Extracorporeal circulation method and myocardial protection: application of deep hypothermia (16 ~ 18 ° C) to stop the circulation and cold blood cardioplegic coronary perfusion and local heart cooling to protect the myocardium. complication 1. Respiratory insufficiency and inability to detach from the ventilator due to changes in the surrounding pulmonary plexus and the softening of the surrounding bronchus and alveolar reduction, patients with this deformity may deteriorate after the ventilator, arterial oxygen saturation decreased and dioxide As the carbon tension rises, it is necessary to continue the use of mechanically assisted breathing by endotracheal intubation or tracheotomy. If severe pulmonary regurgitation is found, the same aortic valve should be used to connect the right ventricle to the pulmonary artery. Once the ventilator cannot be removed from the ventilator multiple times, intermittent assisted breathing is used to gradually extend the time of spontaneous breathing until the ventilator is disengaged. 2. Pulmonary infection: This is a frequent complication of postoperative patients with this deformity, using antibiotics and nebulization. 3. Pulmonary regurgitation and right heart failure produce mild pulmonary regurgitation after intracardiac repair, pulmonary artery angioplasty, and right ventricle-to-pulmonary valve drainage. The patient can tolerate it. In patients with severe pulmonary regurgitation and right heart failure after right ventricular outflow tract patch, the same type of aortic extracardiac duct should be used or a prosthetic valve placed at the pulmonary valve annulus. 4. Patients with extracardiac obstruction have progressive activity endurance after surgery. Echocardiographic examination confirms that the extracardiac tube is obstructed, and the extracardiac tube should be replaced in time.