Details of Congenital Cardiovascular Disease. Atrial Septal Defect. Pulmonary Stenosis. Tetralogy of Fallot

What are Congenital Cardiovascular Diseases?

Congenital cardiovascular disease is the most common type of congenital malformations. With the development of cardiac diagnostic methods and surgical treatment techniques, the vast majority of congenital cardiovascular diseases can now obtain a clear diagnosis and corrective surgery.


What are Causes of Congenital Cardiovascular Diseases?

Due to the disturbance of the fetal heart during development, some developmental halts or defects, and some of the degenerates are not completely degraded.


1. Environmental factors around the fetus. Viral infections in the uterus during early pregnancy are more common after rubella virus infection, which often cause arterial duct occlusion and pulmonary artery stenosis, followed by Coxsakie virus infection (Coxsakie) which can cause endocardial elastic fibers Disease, in addition to amniotic lesions, mechanical stress around the fetus, maternal nutritional disorders, vitamin deficiency and metabolic diseases, maternal cytotoxic drugs or prolonged radiation exposure may be related to the occurrence of this disease.


2. Genetic factors: 5% of patients with congenital heart disease occur in the same family, the same or similar disease, may be caused by genetic abnormalities or chromosomal aberrations.


3. The incidence of open ductus arteriosus and atrial septal defects in other plateau areas is high, and the occurrence may be related to hypoxia. Some congenital heart diseases are gender-prone.


What is Classification of Congenital Cardiovascular Diseases?

According to hemodynamics combined with pathophysiological changes, they can be classified into three categories:


1. No shunt: No shunt on the left and right sides, no cyanosis, such as pulmonary artery stenosis, aortic stenosis, aortic constriction, primary pulmonary artery dilatation, primary pulmonary hypertension or right heart.


2. Left-to-right shunts have abnormal channels between the left and right heart cavities or the main and pulmonary arteries. The pressure on the left side is higher than that on the right. The left arterial blood enters the right venous blood through the abnormal channels. For example, atrial septal defect, ventricular septal defect, arterial duct is not closed. There is main pulmonary artery septal defect, partial pulmonary vein malformation and drainage. Valsalva sinus aneurysm breaks into the right heart. Generally there is no cyanosis.

If pulmonary hypertension occurs in the late stage and there is a bidirectional or right-to-left shunt, cyanosis appears, also known as advanced cyanosis.


3. Right-to-left shunts: abnormally increased pressure in the right ventricle or pulmonary arteries, and blood flow into the left ventricle or aorta through abnormal channels.

Generally, cyanosis occurs shortly after birth, such as tetralogy of Fallot, triplet of Fallot, tricuspid atresia, permanent arterial stem, large vessel borrowing, Eisenmenger’s syndrome, and so on.


What is the Diagnosis of Congenital Cardiovascular Diseases?

Common typical congenital heart disease, diagnosis can be made by symptoms, signs, electrocardiogram, X-ray and echocardiography, and can estimate the hemodynamic changes, degree and extent of lesions, to determine a treatment plan.

For patients with other malformations and complex congenital heart disease, combined with cardiac catheterization or cardiovascular angiography to understand the extent, type and scope of abnormal lesions, comprehensive analysis, make a clear diagnosis, and formulate a treatment plan.

What is the Prognosis of Congenital Cardiovascular Diseases?

Generally depends on the type and severity of the malformation, the timing of surgery and preoperative cardiac function for those who are suitable for surgical correction, depending on whether there are complications. No shunt or left to right shunt, mild asymptomatic, electrocardiogram and X-ray abnormalities, and moderate and severe can be corrected by surgery.

The prognosis is better if the two-way shunt of severe pulmonary hypertension has occurred. The prognosis is poor of right to left shunt or compound malformations. Those with more severe conditions should strive for early surgery. The lighter can choose the timing of surgery, preferably around 10 years old.


In congenital cardiovascular disease, ventricular septal defect, open ductus arteriosus and tetralogy of Fallot are more likely to be complicated by infective endocarditis. This affects the prognosis and requires attention to prevention and treatment.


What is Atrial Septal Defect?

This congenital heart disease is the most common, accounting for 20-25% of the total number of congenital heart diseases, more common in women.


What is the Pathological anatomy Atrial Septal Defect?

According to different embryonic development and pathological anatomy, it is divided into three types:


I. The secondary hole is not closed most often. The defect is far from the atrioventricular valve. During embryonic development, the primary atrial septum is overabsorbed or the secondary atrial septal developmental disorder fails, and the two cannot be fused. According to the location of the secondary hole, it is divided into four types:

i. Central type: the most common

ii. Inferior cavity type (low position): the position is low, and there is no obvious boundary with the entrance of inferior vena cava.

iii. Superior cavity type (high): located below the superior vena cava opening; often accompanied by right pulmonary vein malformations, leading to the right atrium or superior vena cava.

iv. Mixed type: high and low defects exist simultaneously.


II. The primary foramen is not closed, accounting for about 5-10%, and the defect is large. Because the primary atrial septum stops growing prematurely, it does not fuse with the endocardial cushion, leaving a crack.

Divided into:

i. Simple type: there is a complete endocardial cushion at the lower edge of the defect, and there are no cracks in the mitral and tricuspid leaves.

ii. Part of the atrioventricular channel: it is the most common type of primary foramen unclosing. At the lower edge of the primary foramen, that is, the upper part of the ventricular septum, where the mitral and tricuspid valves are attached. Mitral valve mitral valve division often occurs, causing mitral regurgitation, causing left ventricular ventricular blood flow and left and right atrium communication. 

iii.  Complete atrioventricular passage: In addition to some atrioventricular passages, there is still a tricuspid septal valve split, which causes the mitral and tricuspid septa to form two common valves before and after, and the upper part of the ventricular septal defect.


iv. The primary and secondary atrial septum of the common atrial septum does not develop, forming a single atrial cavity.


v. The ovary foramen is not closed. In normal people, 20-25% of the primary and secondary atrial septums are not completely fused and cause the ovary foramen to remain closed. Generally does not cause cardiac chamber shunt.


The disease is often associated with other congenital malformations, such as partial drainage of the pulmonary veins, malformation of the left superior vena cava, mitral stenosis (Lutembacher syndrome), pulmonary stenosis (Trilogy of Fallot), ventricular septal defect, and tricuspid valve downward movement Wait.


What is the Pathophysiology of Atrial Septal Defect?

Under normal circumstances, the right atrium and wall of infants are thicker than adults, and their compliance is poor. With age, the pressure in the left atrium is higher than that in the right atrium. Blood flow from the left atrium to the right atrium through the atrial septal defect opening. Therefore, the tricuspid valve mouth increases the right ventricular and pulmonary circulation blood flow. The right ventricular diastolic load increases, and early pulmonary arterial spasm forms dynamic pulmonary hypertension, with the course of the disease.

Pulmonary arteriolar sclerosis, vascular lumen becomes smaller, pulmonary hypertension becomes worse, obstructive pulmonary hypertension is formed, and right ventricle and right atrium hypertrophy and dilatation occur.

In the late stage of pulmonary hypertension, right heart failure occurs, right atrial pressure is higher than left atrium, bidirectional shunt or right to left shunt can occur, and cyanosis appears, commonly known as Eisenmenger’s syndrome. The left to right shunt flow and the size of the defect and the left, Right ventricular pressure difference is directly proportional.


What are the Clinical manifestations of Atrial Septal Defect?

1. Symptoms are related to the size of the defect and whether it is combined with other deformities. If it is simple and the defect is small, it is often asymptomatic. Most patients with large defects have chest tightness, shortness of breath, and fatigue after fatigue due to pulmonary congestion. Infants and young children are prone to respiratory infections. Primary foramen defects or common atrial symptoms appear early and severe and progress rapidly.


2. The physical signs of large defects can affect development, antrum of the heart, the apex pulse to the left is a lifting pulse. The heart boundary expands to the left, and there is a grade 2-3 soft hairy systolic murmur in the intercostal space of the left margin of the sternum, without fine tremors. There is a short diastolic murmur in the tricuspid valve area, and a second hypertone in the pulmonary valve area Fixed division. If pulmonary hypertension is already present, some patients have pulmonary arterial ejection sounds and early diastolic water-spatter murmurs due to the relative insufficiency of the pulmonary valve in the pulmonary valve area (Graham Steell). If it is a primary pore defect, a systolic whistle-like noise can be heard at the apex.


What are the Laboratory and Other Inspections of Atrial Septal Defect?

1. The X-ray diaphragmatic pulmonary vascular shadow increased, the pulmonary artery trunk bulged and the pulsation increased, the right atrium and right ventricle increased, the aortic node contracted, and the primary ventricular defect may have an enlarged left ventricle.


2. The ECG is often combined with incomplete or complete right bundle branch block, right ventricular hypertrophy.


3. Echocardiography: The popliteal pulmonary artery widens, the right atrium and right ventricle enlarge, and the atrial septum is continuously interrupted. Acoustic contrast showed abnormal shunts. Ultrasound Doppler; systolic left-to-right shunt spectrum can be measured on the right side of the atrial septum.


4. Cardiac Catheterization The right heart catheter found that the blood oxygen content in the right atrium was higher than that of the superior vena cava by 1.9% by volume. In 70% of cases, the heart catheter could enter the left atrium from the right atrium through the defect opening. Through the right heart catheter can measure the pressure and calculate the partial flow of various parts.


If there are suspected primary hole defects, pulmonary artery stenosis, pulmonary vein malformation and other abnormalities, cardiovascular angiography can be considered.


What is the Diagnosis and Differential Diagnosis of Atrial Septal Defect?

Typically, diagnosis can be made based on X-rays, electrocardiograms, echocardiograms, and cardiac catheterization, but attention should be paid to the identification of the following diseases.


1. Ventricular septal defect X-ray and ECG are similar to the primary hole defect, but the ventricular septal defect has a rough contractive murmur between the III-IV intercostal space of the left margin of the sternum, accompanied by fine tremors. Cardiac catheterization can also help identify.


2. Mild pulmonary valve stenosis should be distinguished from secondary hole defects. Pulmonary valve stenosis X-ray showed that the pulmonary blood vessels were scarce. The right heart catheter entered the ectopic pulmonary artery at the right edge of the right atrium.


3. Some pulmonary vein malformations are drawn into the right atrium, X-rays, chest radiographs or tomograms have abnormal vein shadows. The right heart catheter shows that the catheter enters the guiding pulmonary artery at the right edge of the right atrium.


4. Primary pulmonary artery dilatation: Pulmonary artery dilatation has a systolic ejection sound in the pulmonary valve area, and there is no abnormal electrocardiogram. X-rays show that the pulmonary artery is dilated, but there is no pulmonary congestion, and there is no shunt in cardiac catheterization.


5. The signs of primary pulmonary hypertension and ECG are similar to atrial septal defect. X-ray showed enlargement of right atrium and right ventricle, expansion of pulmonary artery and pulmonary artery trunk, but the distal pulmonary artery became thinner and smaller, the outside of the pulmonary field was clear, and cardiac catheterization showed elevated pulmonary artery pressure without abnormal shunt.


What is the Treatment of of Atrial Septal Defect?

Where there are abnormalities in the X-ray and the electrocardiogram, the calculated partial flow of the right ventricle catheter has reached more than 40% of the pulmonary circulation blood flow, and those who have clinically obvious symptoms should be operated as soon as possible. Age is preferably 5-10 years. Patients with existing pulmonary hypertension with two-way shunt or left-to-right shunt are not suitable for surgery.



What is Ventricular Septal Defect?

About 20% of the total number of congenital heart disease, can exist alone or coexist with other deformities.


What is the Pathological anatomy of Ventricular Septal Defect?

According to the location of the defect, it can be divided into five types:


i. The supraventricular defect is located in the right ventricular outflow tract, above the superior ventricular sacrum and below the main and pulmonary valves. In a few cases, the main and pulmonary valves are incompletely closed.


ii. The supraventricular defect is located in the ventricular septal membrane. This type is most common, accounting for about 60-70%.


iii. The posterior septal defect is located in the right ventricular inflow tract, behind the tricuspid septum, accounting for about 20%.


iv. The muscle defect is located in the apex of the trabecular muscle defect. During the systole, the interval myocardial contraction makes the defect smaller, so the left-to-right partial flow is small.


v. The common ventricle and ventricular septal membrane and muscle are not developed, or there are multiple defects, which are rare.


Ventricular septal defects, with defects between 0.1 and 3 cm, are larger in the membrane and smaller in the muscle, which is also called Roger’s disease. If the defect is less than 0.5cm, the partial flow is small, and there are usually no clinical symptoms. The right ventricle enlarges mainly in the small defect, and the left ventricle enlarges significantly in the large defect.


What is Pathophysiology of Ventricular Septal Defect?

Left to right shunts are generated at the ventricular level, and the amount of shunt depends on the size of the defect. In the case of a large defect, pulmonary circulation blood flow increases significantly. After flowing into the left atrium and ventricle, it flows into the right ventricle through the defect opening at the ventricular level and enters the pulmonary circulation. Therefore, the left and right ventricles increase, the left and right ventricles increase, and the pulmonary circulation blood flow increases As a result of increased pulmonary arterial pressure, the right ventricular systolic load also increases, and eventually enters the obstructive pulmonary hypertension phase, which can appear bidirectional or right-to-left shunt.


What are the Clinical manifestations of Ventricular Septal Defect?

1. The symptoms of small defects, can be asymptomatic. In large defects, the symptoms appear early and obvious, which affects development. Heart palpitations, asthma, fatigue, and susceptibility to lung infections. Heart failure can occur in severe cases. When there is obvious pulmonary hypertension, cyanosis may occur, and the disease is susceptible to infective endocarditis.


2. Signs: The apical pulsation increases and shifts to the lower left, and the heart boundary expands to the lower left. The typical signs are a grade 4-5 rough systolic murmur in the left margin of the sternum III-IV intercostal space, which is transmitted to the anterior region of the heart, with fine tremors in the systole. . If the partial flow is large, there may be functional diastolic murmurs in the apex. Pulmonary valve secondary hyperphony and division. Severe pulmonary hypertension, the diastolic murmur of the relative pulmonary valve insufficiency in the pulmonary valve area, and the systolic murmur of the original septal defect can be weakened or disappeared.


What are the Laboratory and other inspections of Ventricular Septal Defect?

1. The heart shadow of those with small X-ray defects is unchanged. When the defect is moderately large, the heart shadow increases to varying degrees, mainly in the right ventricle. In large defects, the left and right ventricles are enlarged, the pulmonary artery bulges, and the pulmonary vascular shadow is enhanced. In severe pulmonary hypertension, the lateral band of the pulmonary field is clear.


2. The ECG small defects were abnormal. If the defect is moderate or larger, the right ventricle or left and right ventricular hypertrophy is shown.


3. Echocardiography: The left atrium, left and right ventricle diameters increase, and the interventricular septum echoes are interrupted continuously. Doppler ultrasound: The maximum turbulence can be measured by tracking the defect from the right ventricle to the hole and the left ventricle.


4. Cardiac Catheterization: The level of blood oxygen in the right ventricle is higher than 0.9% of the volume in the right atrium. Occasionally, the catheter can reach the left ventricle through the defect. Depending on the amount of shunt, the pulmonary or right ventricular pressure increases to varying degrees.


What is the Diagnosis of Ventricular Septal Defect?

According to typical signs, X-rays, electrocardiograms, echocardiograms, and cardiac catheterizations can confirm the diagnosis, but it should be noted that when the disease is complicated by arterial catheters, the murmur of the latter is often masked by loud murmurs of ventricular septal defects. When it is easy to miss diagnosis, or when the ventricular septum is a subvalvular defect, the left to right shunted blood flows directly into the pulmonary artery, causing the pulmonary artery blood oxygen content to be higher than that of the right ventricle, which is easily misdiagnosed as an open duct. Therefore, if necessary, a clear diagnosis of ascending aorta can be made.


What is the Differential Diagnosis of Ventricular Septal Defect?

1. The atrial septal defect of the primary hole is not closed type, see atrial septal defect section.


2. Pulmonary artery stenosis Patients with pulmonary artery stenosis have normal or reduced second tone in the pulmonary artery region. X-rays show that the pulmonary vessels are scarce. There is no shunt in the right heart catheterization. There is a systolic pressure step between the right ventricle and the pulmonary artery.


3. Obstructive hypertrophic cardiomyopathy. Obstructive cardiomyopathy murmur is jet, loud changes with body position and breathing, X-ray shows that the pulmonary artery trunk is not protruding, and the pulmonary vascular shadow is not much.


4. The supraventricular supraventricular septal defect is located under the aortic valve, which causes the aortic valve to be incomplete due to the loss of support of the aortic valve. It should be noted that the aortic sinus aneurysm has broken into the right heart and the aortic pulmonary artery defect. Identification. Examination of the right ventricle showed that the right ventricle was shunted horizontally, ascending aorta angiography showed that the left ventricle had contrast agent reflux, and the right ventricle was advanced in advance.


What is the Treatment of Ventricular Septal Defect?

1. Medical treatment Prevents and treats infective endocarditis, pulmonary infection and heart failure.


2. Surgical treatment of defect repair under direct vision, small defect, normal X-ray and ECG without surgery, if with or without pulmonary hypertension, left to right shunt is the main, the best surgical effect, 4-10 The age is appropriate. If the symptoms appear early or have heart failure, surgery can also be performed in infants and children. Significant pulmonary hypertension, bidirectional or right-to-left shunt is the main, surgery is not suitable.


What is Pulmonary artery stenosis (Pulmonary stenosis)?

It refers to the narrowing of the outlet of the pulmonary artery, resulting in obstruction of right ventricular bleeding, including stenosis of the pulmonary valve, stenosis of the right ventricle funnel and stenosis of the pulmonary valve, the main trunk and branches of the pulmonary artery.


What is Pathological Anatomy of Pulmonary stenosis?

1. Valvular pulmonary artery stenosis is most common. Three valve leaves are fused into a circular cone shape, with a small hole with a diameter of 2-4 mm in the center. In some cases, there are only two leaves. The pulmonary artery trunk is narrowed and dilated.


2. The right ventricular funnel stenosis: The entire funnel is thickened to form long and narrow channels. It can also be a muscular diaphragm type with a ring-shaped stenosis, which causes the third ventricle. If there is also a valve type stenosis, it is called mixed stenosis.


3. Pulmonary arterial stenosis can involve part or all of the common pulmonary artery trunk, and can also extend to the left and right branches, often with stenosis before and after expansion.


What is the Pathophysiology of Pulmonary stenosis?

Pulmonary artery outlet stenosis, obstruction of right ventricular bleeding, increased right ventricular systolic load, increased right ventricular pressure, normal or reduced pulmonary arterial pressure, systolic pressure gradient before and after stenosis, long-term can cause right ventricular hypertrophy, and right heart failure .


What are the Clinical Manifestations of Pulmonary stenosis?

1. Symptoms: People with mild stenosis are generally asymptomatic, and those with moderate to moderate stenosis may have asthma after exertion, fatigue, palpitations and fainting. Late heart failure may occur.


If it is accompanied by atrial septal defect or ovary foramen, right-to-left shunt, also known as trilogy of Fallot, with cyanotic pestle-like fingers (toes).


2. The signs and severe stenosis, poor development. The anterior region of the heart bulges, and the heart dullness zone expands significantly. There is a grade 4-5 rough systolic murmur in the second intercostal space of the left margin of the sternum. Conducts to the left axilla, under the clavicle and left shoulder. The narrow murmur in the funnel is the loudest, and the part is low. The noise is related to the degree of stenosis, accompanied by fine tremor in systole. Pulmonary valve second sound weakened or disappeared. In patients with mild to moderate stenosis, systolic jetting sounds can be heard in the pulmonary valve area.


What are the Laboratory and Other Inspections for Pulmonary stenosis?

1. No abnormality was found in X-ray cases. In patients with moderate to severe stenosis, the pulmonary vascular shadow is scarce, the lung field is clear, with the right ventricle and the right atrium enlarged. Valvular stenosis has a protruding pulmonary artery trunk, funnel stenosis and mixed stenosis with a pulmonary artery segment depression.


2. ECG ECG changes are related to right ventricular pressure. People with moderate to severe stenosis have incomplete right bundle branch block, right ventricular hypertrophy and strain, and some cases have right atrial hypertrophy.


3. Echocardiography: Right ventricle and right atrium are enlarged. Can understand the nature, location and extent of pulmonary stenosis. Doppler ultrasound can detect the systolic turbulence spectrum in the pulmonary artery.


4. Cardiac Catheterization: The right ventricle pressure increases, and there is a systolic pressure step between the right ventricle and the pulmonary artery. Under normal circumstances, the pressure step should be less than 1.33kpa (10mmHg). The pressure step of mild stenosis increases but is less than 5.33kpa (40mmHg), the pressure step of moderate stenosis is 5.33 ~ 13.3kpa (40-100mmHg), and the pressure step of severe stenosis exceeds 13.3kpa (100mmHg). The pressure curve is continuously recorded from the pulmonary artery to the right ventricle to determine the type of stenosis.


5. Cardiovascular angiography revealed that the emptying time of the right ventricle and pulmonary artery was prolonged, which could show the shape, scope and degree of narrowing of the right ventricle, pulmonary valve, pulmonary artery and its branches, and helped determine the surgical plan.


What is the Diagnosis and Differential Diagnosis?

Diagnosis can usually be made based on physical signs, X-rays, and electrocardiograms. Cardiac catheterization and right heart angiography can further show the pathological anatomy of the right ventricle, pulmonary valve, and pulmonary artery. Pay attention to identify the following diseases.


1. Atrial septal defect: See the atrial septal defect segment.


2. Ventricular septal defect see ventricular septal defect.


3. The primary pulmonary artery dilatation is similar to mild pulmonary valve stenosis, but the primary pulmonary artery dilatation and systolic murmur is soft without tremor, the second pulmonary valve sound is normal, and there is no pressure step between the right ventricle and the pulmonary artery during cardiac catheterization. Diversion.


What is the Treatment of Pulmonary stenosis?

1. Medical treatment Prevent and treat pulmonary infection, heart failure or infective endocarditis.


The valvular pulmonary artery stenosis can be performed by percutaneous puncture balloon dilation angioplasty. Because the trauma is small, no thoracotomy is needed and it is easy for patients to accept.


2. Surgical treatment: feasible valvulotomy or hypertrophic muscle bundle resection. If the symptoms are obvious, severe stenosis or right heart failure should be performed as soon as possible.

The surgical indications are as follows:

i. The symptoms worsen progressively.

ii. The pressure difference between right ventricle and pulmonary artery is more than 5.33kpa (40mmHg).

iii. The right ventricular systolic pressure is> 8.00 kpa (60 mmHg), and the average right ventricular pressure is> 3.33 kpa (25 mm Hg).

iv. X-ray and ECG showed left ventricular hypertrophy.




 What is Tetralogy of Fallot?

The most common form of cyanotic congenital heart disease (70-75%), is a complex congenital malformation, including pulmonary artery stenosis, ventricular septal defect, aortic straddling the defective ventricular septum or right and right ventricular hypertrophy kind of deformity. The incidence is similar in men and women.


What is Pathological Anatomy of Tetralogy of Fallot?

The basic pathological changes of this disease are ventricular septal defect and pulmonary artery stenosis, right ventricular hypertrophy and aortic stride are the consequences of the first two deformities.


Pulmonary artery stenosis is more common in the funnel stenosis.

The third ventricle is formed due to poor development of the right ventricular outflow tract, thickening of the endocardium, and diffuse or localized thickening of the funnel.

Ventricular septal defects are mostly located below the origin of the ascending aorta, are similar in size to the aortic valve openings, and are supraventricular.

The aortic straddle is a relative malformation, which can gradually increase as the aorta develops. Right ventricular hypertrophy is a compensatory result of pulmonary artery stenosis, and the wall thickens, which can approach and exceed the left ventricle.

About 20-25% of this disease has a right aortic arch, and about 15% is accompanied by an open ovoid or atrial septal defect, which is called Fallot pentagram. Its clinical manifestations are similar to those of Fallot’s quadruple disease.

This disease can still coexist with arterial ducts, bilateral superior vena cava, pulmonary vein malformation and drainage, and right heart defects.


What is Pathophysiology of  Tetralogy of Fallot?

The severity of the condition is mainly determined by the degree of pulmonary artery stenosis and the size of the ventricular septal defect.


Pulmonary artery stenosis causes increased right ventricular systolic load and right ventricular hypertrophy. When the right ventricular systolic pressure exceeds the left ventricle, the right ventricular venous blood flows into the left ventricle and the riding aorta through the ventricular septal defect, reducing the arterial oxygen saturation.

The occurrence of cyanosis and secondary erythrocytes, the heavier the pulmonary artery stenosis and the greater the ventricular septal defect, the greater the right-to-left shunt, and the heavier the cyanosis. Increased right ventricular pressure can cause right atrial hypertrophy and reduced pulmonary arterial pressure.


What are Clinical manifestations of Tetralogy of Fallot?

1. Symptoms: Most of them have cyanosis within 6 months after birth, and severe cases appear shortly after birth. In the milder group, cyanosis gradually develops due to the narrowing of the pulmonary valve orifice and the closure of the arterial duct at about 1 year old. Asthma, asthma, fatigue, and squatting position after exercise, which can increase systemic circulation resistance and reduce right-to-left shunt and return to heart blood. Symptoms may be slightly relieved.

In severe cases of cyanosis, seizure-induced fainting seizures, disturbance of consciousness, or even death may occur due to severe hypoxia, which may be caused by a temporary increase in endogenous catecholamine levels and muscle spasm in the right ventricular funnel. , Pulmonary artery blood flow is further reduced, resulting in a sudden increase in right-to-left shunt.


A few cases may have epistaxis, hemoptysis, embolism and cerebral hemorrhage.


Signs: Aster cyanosis and clubbing fingers (toes) are common signs of the disease. Poor development, anterocardial area bulge. Most cases have 2-3 stage systolic murmurs in the III-IV intercostal space of the left margin of the sternum. The murmur is caused by narrowing of the pulmonary artery orifice.

The loudest part of the noise is related to the type of pulmonary artery orifice stenosis.

The loudness of the murmur is inversely proportional to the degree of stenosis.

The heavier the stenosis, the more the right ventricular blood flow shunts to the aorta of the riding span, and the less the blood flow into the pulmonary artery. Pulmonary valve second sound weakened or disappeared.


What are the Laboratory and other inspections of Tetralogy of Fallot?

1. The X-ray heart shadow is normal or slightly larger, the apical circle is bluntly upturned, the lung field is clear, the pulmonary vascular shadow is scarce, and the pulmonary artery is dry and faint. If the pulmonary artery stenosis is valvular, the pulmonary artery bulges, the aortic arch widens, the right ventricle enlarges, and sometimes the right atrium also increases, with 20% of the right aortic arch.


2. The ECG: right ventricular hypertrophy and strain, some people also have right atrial hypertrophy. The electric axis is skewed to the right.


3. Echocardiography: The anterior wall of the aorta and the ventricular septum are continuously interrupted. The ventricular septum is located between the anterior and posterior walls of the aorta. The aorta is widened, the right ventricle is enlarged, the right ventricular anterior wall is thickened, and the outflow tract is narrow.


4. Cardiac catheter examinations may have the following characteristics:

i. The catheter can enter the aorta from the right ventricle through the ventricular septal defect.

ii. There is a systolic pressure step between the right ventricle and the pulmonary artery. Analyzing the continuous pressure curve can determine the location, type and degree of stenosis.

iii. The right ventricular blood oxygen content is higher than that of the right atrium, indicating that there is left-to-right shunt at the ventricular level.

iv. Decreased arterial blood oxygen, indicating a right-to-left shunt.

v. If the aorta, left ventricle, and right ventricular systolic blood pressure are similar, it means that the ventricular septal defect is large and the right span of the aorta is obvious.

vi. Both the red blood cell count and the hemoglobin concentration increased.


5. Cardiovascular angiography: Right ventricular angiography shows that the aorta and the left and right ventricles are simultaneously developed, the aorta is widened, and the judging of the pulmonary artery stenosis, degree and type, the branching of the pulmonary artery, the location and size of the ventricular septal defect, and the ascending aorta The degree of riding span is of great value.


6. The blood routine examination red blood cell count, hemoglobin concentration and red blood cell volume were significantly increased.


What is Diagnosis and Differential Diagnosis of Tetralogy of Fallot?

According to clinical symptoms, X-ray, electrocardiogram, echocardiogram, combined with right heart catheterization and angiography can confirm the diagnosis. Attention should be paid to distinguishing from other cyanotic congenital heart disease.


I. Trilogy of Fallot: This disease is pulmonary artery stenosis, atrial septal defect and right ventricular hypertrophy accompanied by right-to-left shunt. However, the cyanosis of the disease appears late and mild, the murmur of the intercostal systole in the left margin of the sternum is louder, the X-ray heart shadow increases significantly, the pulmonary artery trunk is protruding, and the aortic node is small. The right heart catheter and the right heart Radiography also has its own characteristics.


II. Eisenmenger’s syndrome: There is a left-to-right shunt change in the ventricular or aortic levels, with bidirectional shunt or right-to-left shunt caused by increased pulmonary artery pressure and resistance, and a collective name for cases of cyanosis. Because at this time most of the primary signs and symptoms have disappeared. Therefore, attention should be paid to the identification of cyanotic congenital heart disease. However, this disease appears in the late stage of the disease and is lighter. The clubbing fingers are not obvious. The second hypercardia of the pulmonary artery is accompanied by a systolic jet sound or the diastolic murmur of the relative insufficiency of the pulmonary valve. The heart enlarges significantly. X-rays Pulmonary artery trunks are protruding, pulmonary vascular shadows are increased, only the outside of the lungs are scarce, and the characteristics of right heart catheter pulmonary artery pressure and resistance can be identified.


III. Tricuspid valve atresia: The tricuspid valve is closed or absent. The right atrium blood enters the left atrium and left ventricle through the unopened foramen or atrial septal defect, and then passes through the ventricular septal defect or the closed arterial duct to the pulmonary circulation. After birth, she has cyanosis, severe symptoms and manifestations of right heart failure. The electrocardiogram shows an increase in P waves, left ventricular hypertrophy, and X-rays of the right atrium and left ventricle. The right ventricle shrinks. The atrium, ventricle develops in advance and the right ventricle does not develop.


IV. Complete large vessel dislocation, the aorta originates from the right ventricle, the pulmonary artery originates from the left ventricle, often accompanied by atrioventricular septal defect or arterial duct is not closed.

The disease cyanosis appears early. The symptoms are obvious. X-ray shows the heart is enlarged. The lungs Vascular shadows increased, and the cardiac catheter showed that the catheter entered the ascending aorta from the right ventricle. Oximetry has left-to-right shunts, right ventricular angiography shows right ventricular imaging, aorta imaging in advance and pulmonary artery imaging later.


What is the Treatment of Tetralogy of Fallot?

1. Medical treatment: timely control of respiratory infections to prevent infective endocarditis, β-receptor blockers in severe cases to reduce right ventricular outflow tract obstruction and prevent hypoxia.


2. Surgical treatment: Radical mastectomy, including resection of the right ventricular outflow tract hypertrophy, separation of stenotic valves, repair of ventricular septal defects, this operation is more thorough. The age of surgery is preferably 5-8 years. If the symptoms are severe, shunt surgery can also be selected within 3 years of age for anastomosis of the subclavian artery, aorta or superior vena cava with the pulmonary artery to establish body-pulmonary circulation to improve hypoxia and prepare conditions for future radical surgery. Individual patients who cannot undergo radical surgery can also be considered for narrow pulmonary valve opening or funnel incision to reduce right ventricular obstruction, increase pulmonary blood flow, and reduce right-to-left shunts.


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