No obvious precordial pulsation on inspection
Introduction
Introduction There is no obvious pre-cardiac pulsation in Ebstein syndrome, which is bulging and quiet in the anterior region. There is no obvious anterior pulsation in the heart. Ebstein syndrome, also known as Ebstein malformation, refers to the tricuspid valve and/or posterior flap occasionally with the anterior flap moving down to the proximal apical right ventricular wall, accounting for 0.5% to 1.0% of congenital heart disease. The disease is a rare disease, first reported in 1866 by Ebstein. This disease is also known as the tricuspid valvular deformity. Occasionally, family history, the mother's early pregnancy in lithium patients whose progeny are susceptible to the disease, in the functional right ventricle, systolic blood pressure can be normal, and diastolic blood pressure is often increased, similar to constrictive pericarditis. Both atrial systolic and diastolic blood pressures were elevated. There may be systolic pressure difference on both sides of the pulmonary valve. There may be diastolic pressure difference on both sides of the tricuspid valve. The former may be too long due to a tricuspid leaflet and partially block the right ventricular outflow tract. The latter is due to tricuspid malformation and tricuspid valve. The mouth is narrow.
Cause
Cause
There are no obvious reasons for the pulsation of the precordial area:
Can be seen in Ebstein syndrome, the cause: the main pathological anatomy of this malformation is tricuspid valvular deformity right ventricular ventricularization and functional right ventricular stenosis. The position of the right atrioventricular ring is normal (often enlarged). The anterior lobes of the tricuspid valve are normally attached to the annulus fibrosus, while the attachment points of the temporal and posterior lobes are obviously moved down and down the endocardium of the right ventricular wall. The extent of the movement and the manner of attachment vary from person to person, even if the attachment point of the valvular valve is adjacent to the annulus fibrosus, but because the leaflets are too long, they can often adhere to the right ventricular wall at different distal ends of the annulus.
In addition, these leaflets can also be attached to the ventricular septum by the deformed chordae and the right ventricular apex. The posterior mitral tricuspid flap is often underdeveloped or completely absent as described above can cause tricuspid regurgitation. In a small number of patients, the tricuspid valve merges into a aponeurosis in the ventricular lumen, with a hole in the middle or lateral margin, and the right atrial blood flow must be injected into the ventricle through this pore, thus impeding the evacuation of the atria. As the tricuspid valve moves down, the right atrium captures part of the right ventricle. The ventricular wall of this captured area becomes thinner and the room is enlarged, and the right atrium cavity is obviously enlarged. The right ventricle of the room belongs to the right atrium in function, but retains the characteristics of the right ventricular muscle in terms of electrical activity. The larger the right ventricle part of the room, the smaller the functional right ventricular cavity. The right ventricle of the room can not be inserted into the right ventricle. On the contrary, it is like a ventricular wall tumor. When the ventricle contracts, it expands contradictively, thus disturbing the right ventricular ejection. Ebstein syndrome often has heart chamber traffic (as seen in 80% of cases). This heart chamber traffic can be an open orbital defect or a septal defect.
A small number of cases may be associated with other congenital malformations, such as aortic coarctation, ventricular septal defect pulmonary stenosis or atresia, patent ductus arteriosus or corrected large vessel translocation. In the latter case, the anatomical right ventricle, which is functionally the left ventricle of the systemic circulation, can clinically have mitral regurgitation and is therefore referred to as the left Ebstein deformity. The pathophysiological changes of this malformation depend on the presence or absence of functional right ventricular volume and the extent of tricuspid regurgitation in pulmonary stenosis. If the functional right ventricular cavity of the pulmonary stenosis is significantly reduced and the tricuspid regurgitation is severe, the blood volume of the right ventricle will be reduced. It will be clinically characterized by early onset symptoms and poor prognosis. On the contrary, the hemodynamic changes are light, and the clinical manifestations are mild symptoms and better prognosis. As mentioned above, this kind of malformation often has heart room traffic. If the tricuspid valve is very light and there is atrial septal defect, then left to right shunt can occur at the atrial level; or because the tricuspid lesion is light, and the foramen ovale is closed, there will be no shunt. In the third case, the tricuspid malformation is severe and the right atrial pressure is elevated, resulting in a right to left shunt at the atrial level.
In the first two cases, there is often no cyanosis in the clinic. In the third case, there are a few patients with cyanosis. Even if there is no obvious right-to-left shunt, the difference in arteriovenous oxygen is increased due to low cardiac output, and there may be mild cyanosis in the clinic. In addition, in the functional right ventricle, systolic blood pressure is normal, and diastolic blood pressure is often increased, similar to constrictive pericarditis. Both atrial systolic and diastolic blood pressures were elevated. There may be systolic pressure difference on both sides of the pulmonary valve. There may be diastolic pressure difference on both sides of the tricuspid valve. The former may be too long due to a tricuspid leaflet and partially block the right ventricular outflow tract. The latter is due to tricuspid malformation and tricuspid valve. The mouth is narrow.
Examine
an examination
Related inspection
Chest CT examination of dynamic electrocardiogram (Holter monitoring) Doppler echocardiography chest MRI
There is no obvious diagnosis of pulsation in the anterior region:
It can be seen in Ebstein syndrome, the symptoms can be early or late, and the symptoms can be light and heavy. In severe cases, there may be obvious cyanosis and congestive heart failure after birth; those with mild deformity may not have obvious symptoms until adulthood. The most prominent symptoms of this malformation are cyanosis and congestive heart failure.
The main signs of Ebstein syndrome include: bulging and quiet pre-cardiac region (no obvious pre-cardiac pulsation, palpation without pulmonary artery closure); the first heart sound and the second heart sound are clearly split, may be enhanced In the third heart sound, the fourth heart sound can also appear; the second component of the split first heart sound is often in the form of a click sound; the so-called "sail sign"; the tricuspid valve region can exhibit soft systolic murmurs and Short diastolic mid-term murmur.
In addition, there is a sacral systolic positive pulsation of the jugular finger (toe). Siber believes that the most characteristic signs of this malformation are two groups: 1 hairpin with a quiet precordial region; 2 a first heart sound, a split second heart sound, an enhanced third heart sound, or a fourth heart sound. A quartet composed. Complications such as heart failure, arrhythmia, cerebral embolism and brain abscess can be combined.
Diagnosis
Differential diagnosis
There is no obvious differential diagnosis of pulsation in the anterior region of the heart:
1, the elderly pheochromocytoma crisis: chromaffin cells, mainly distributed in the adrenal medulla, sympathetic ganglion and other chromophores, responsible for the synthesis, storage and release of norepinephrine and adrenaline. Tumors derived from mature chromaffin cells are called pheochromocytoma, and clinical manifestations are characterized by a series of syndromes caused by excessive amounts of catecholamines (norepinephrine and adrenaline), with hypertension being the most prominent symptom. Because the tumor releases a large amount of catecholamine into the blood, resulting in severe clinical syndromes, such as hypertensive crisis, hypotension shock and severe arrhythmia, it is called pheochromocytoma crisis.
2. Congenital heart disease: Congenital cardiovascular disease is the most common type of congenital malformation. With the progress of cardiac diagnosis methods and surgical treatment techniques, most of the congenital cardiovascular diseases can obtain clear diagnosis and surgical correction treatment, and the prognosis is significantly improved.
3, the premature contraction of the atrioventricular junction: premature contraction of the atrioventricular junction is also called premature beats (JPBs), referred to as premature ventricular premature beats or crossover premature beats. It refers to an excitement that occurs in advance in the atrioventricular junction area before the sinus excitement has not yet been issued.
4. Primary cardiac lymphoma: Primary cardiac lymphoma is a rare lymphoma. The World Selected Works reported only 76 cases in 1949-1996 for more than 40 years. Some autopsy data showed that the percentage of disseminated lymphoma involving the heart in autopsy materials was as high as 20%.
