Squatting phenomenon

Introduction The sputum phenomenon is a special forced position, which is more common in children with congenital tetralogy of Fallot. When the child walks or plays, the special posture of the legs flexing and squatting for a while is followed, and then the normal walking is resumed, and the sputum appears repeatedly. The tetralogy of Fallot is named after the first description of Fallot, also known as the quadriplegia. This disease includes ventricular septal defect, pulmonary stenosis, aortic riding and right ventricular hypertrophy. The first two malformations are basic lesions. The rate is about 11% to 13% of congenital heart disease, and the ratio of male to female is similar.

Cause

Pathogenesis:

Due to stenosis of the pulmonary artery, blood enters the pulmonary circulation, causing compensatory hypertrophy of the right ventricle, increased right ventricular pressure, right ventricular systolic pressure is similar to left ventricular pressure, blood flow through the ventricular septal biphasic shunt, right ventricular blood is large Partially entering the aorta, if the pulmonary valve is atresia, all blood in the right ventricle enters the aorta, and the blood supply to the lung depends on the arterial catheter. Since the aorta is above the left and right ventricles, the left and right ventricles are transported throughout the body. Causes hairpins. Due to pulmonary stenosis, the blood flow exchanged by the pulmonary circulation is reduced, and the cyanosis is more serious. However, because the arterial catheter is not closed, the blood flow of the pulmonary circulation is increased, and the cyanosis may be inconspicuous or light, but with the closure of the arterial catheter and the narrowing of the funnel. Gradually increasing, cyanosis is becoming more and more obvious, and red blood cells and hemoglobin are compensatory.

an examination

Related inspection

Cardiovascular angiography M-mode echocardiography (ME)

Clinical symptoms

1. Most cases have cyanosis several months after birth. After severe birth, they develop convulsions. After exercise, they are short of breath. Children often feel weak and have poor endurance. They may have hypoxia during intense activities, crying or just waking up in the morning. Attack: Sudden breathing difficulties, increased cyanosis, severe cases can cause convulsions, fainting, like sputum during activities is also one of the characteristics of this disease, sputum can increase systemic resistance, reduce right heart blood to the aorta shunt, thereby increasing pulmonary circulation Improve hypoxia; phlegm can reduce the amount of blood return to the lower body, reduce right ventricular diversion, increase circulating oxygen and improve cerebral hypoxia. In a few cases, there may be symptoms such as epistaxis, hemoptysis, embolism or brain abscess. .

2. Signs: Children with growth and development, with cyanosis and clubbing, cardiac auscultation in the left sternal border of the second and third ribs with II-III systolic jet murmur, the loudness of the murmur is related to the degree of pulmonary stenosis, stenosis The more severe the murmur, the weaker the second sound of the pulmonary artery, splitting or disappearing.

diagnosis

According to the medical history, the child has purpura, shortness of breath after exercise, difficulty in breathing, increased tingling, like sputum, poor growth and development, clubbing, and II-III systolic jet murmur between the second and third ribs of the sternum. Electrocardiogram and X-ray examination of right ventricular hypertrophy, aortic widening, shoe heart, echocardiography, cardiac catheterization, cardiovascular angiography and other performance can be diagnosed.

Differential diagnosis

(A), pulmonary stenosis with atrial septal defect with right to left shunt (Fallow's triad). The disease has a late appearance of purpura. The systolic murmur of the second intercostal space on the left sternal border is louder, occupying a longer time, and the second heart sound in the pulmonary valve area is reduced and split. On the X-ray film, the increase of the heart shadow was more significant, and the total dry arc of the pulmonary artery was obviously convex. The performance of right ventricular strain in the electrocardiogram is more obvious. Right heart catheterization, selective indicator dilution curve measurement, or selective cardiovascular angiography revealed that the pulmonary stenosis is a valve type, and the right to left shunt level is at the atrial site, which can establish a diagnosis.

(B), Eisenmenger syndrome. When severe pulmonary hypertension occurs in patients with ventricular septal defect, atrial septal defect, aortic-pulmonary septal defect, or patent ductus arteriosus, the left-to-right shunt is converted to a right-to-left shunt to form Eisenmenger syndrome. The syndrome has a late appearance of purpura; the pulmonary valve area has a contraction jet sound and a systolic hairy murmur, the second heart sound is hyperthyroidism and can be split, and there may be a diastolic murmur of the hair sample; the X-ray examination shows that the total dry arc of the pulmonary artery is clearly protruding, the lung The vascular shadow of the portal is large and the blood vessels in the lung field are small; the right heart catheter examination shows that the pulmonary artery is significantly high pressure, etc., can be identified.

(C), Ebstein deformity and tricuspid atresia. When Ebstein is deformed, the tricuspid valve leaflets and posterior valve leaflets move down to the ventricle, the right atrium enlarges, and the right ventricle is relatively small, often accompanied by atrial septal defect resulting in a right-to-left shunt. 4 heart sounds can often be heard in the anterior region; X-rays show an increase in heart shadow, often spherical, right atrium can be very large; ECG shows right atrial hypertrophy and right bundle branch block; selective right atrial angiography shows increased right Atrial and malformed tricuspid valves can establish a diagnosis. When the tricuspid atresia is completely blocked, the blood in the right atrium enters the left atrium through the patent foramen ovale or atrial septal defect, passes through the mitral valve into the left ventricle, and then passes through the ventricular septal defect or the patent ductus arteriosus to the pulmonary circulation. . X-ray examination showed that the right ventricle was not obvious and the lung field was clear. The electrocardiogram has left ventricular hypertrophy. Selective right atrial angiography establishes a diagnosis.

(D), large blood vessels are misplaced. When the complete vascular dislocation is located, the pulmonary artery originates from the left ventricle, and the aortic origin comes from the right ventricle, often accompanied by atrial or ventricular septal defect or patent ductus arteriosus. The heart is often significantly enlarged, and the X-ray shows pulmonary congestion. Selective right ventriculography can establish a diagnosis. The aortic and pulmonary arteries of patients with right ventricular double outlet in incomplete large vessel dislocation are from the right ventricle, often with ventricular septal defect. X-ray films show significant increase in heart shadow, pulmonary congestion, and selective right ventriculography can be established. diagnosis. If there is a pulmonary stenosis at the same time, the differential diagnosis will be very difficult.

(5) The arterial trunk is permanent. When the arterial stem persists, there is only one set of semi-monthly flaps, which straddle the two ventricles. The pulmonary artery and the brachial artery are all emitted from the arterial trunk, often accompanied by ventricular septal defect. In patients with tetralogy of Fallot, such as pulmonary artery disease, when the pulmonary artery and pulmonary valve atresia are formed, its performance is similar to that of arterial stenosis. Pay attention to the identification of the two. In this regard, selective right ventricular angiography is helpful.

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