No pulmonary artery closure sensation on palpation

Introduction Palpation without pulmonary occlusion is a clinical manifestation of Ebstein syndrome. Ebstein syndrome, also known as Ebstein malformation, refers to the tricuspid valve and/or posterior flap occasionally attached to the right ventricular wall of the proximal apex with the anterior flap, 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

The cause of Ebstein syndrome:

The main pathological anatomical features of this malformation are right ventricular atrialization 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.

an examination

The incidence of Ebstein syndrome can be early or late, the symptoms can be light and heavy, and the signs can be varied. 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.

The following points have a reference value when diagnosing this disease:

1. The symptoms of bruising in the neonatal period are obviously reduced or completely disappeared, and the cyanosis reappears after the elderly.

2. Cyanosis symptoms combined with tachyarrhythmia should first consider this disease

3. Right-to-left shunt congenital heart disease, less pulmonary blood and no right ventricular hypertrophy.

4. The heart is enlarged, but the pulsation in the anterior region is very weak. There is a "multi-temporal" heart sound during auscultation.

5. Pulmonary blood is less and the heart is enlarged. The aorta and pulmonary artery are small, and the heart is like a balloon.

6.P-port high, but no right ventricular hypertrophy.

7. The right heart lead shows a complete right bundle branch block and a small multi-phase wide QRS wave.

8. There are blue-purple with B-type pre-excitation syndrome, and the pulmonary blood is less.

9. There are QR wave and T wave inversion on the V1~4 lead.

10. Echocardiography shows the tricuspid attachment point moving down

Ebstein syndrome can be classified into 3 types based on clinical manifestations and hemodynamic changes during diagnosis:

1 light type: no or mild cyanosis, cardiac function I ~ II grade heart light ~ moderate increase, intracardiac shunt mainly left to right, no difference between right atrium and functional right ventricle angiography without ball The levy does not require surgery or only closes the intracardiac defect, and the prognosis is good.

2 stenosis type: cyanosis is obvious, heart function is above grade II, heart is mild to moderately increased, pulmonary circulation blood flow is reduced, there is pressure difference between enlarged right atrium and functional right ventricle, intracardiac shunt is right to left, cardiovascular The angiography sees the double ball sign and requires surgery.

3 insufficiency type: no or mild cyanosis, heart function level II or above, heart severity increased, no pressure difference between right atrium and functional right ventricle, intracardiac shunt can be left to right or right to left, angiography visible The right atrium is extremely large, with a double ball sign and requires surgery.

Other ancillary examinations: the diagnosis of Ebstein syndrome, clinical symptoms and signs may give important tips, but to make an accurate diagnosis depends on the following auxiliary examinations, especially selective angiography.

1. Electrocardiogram: The amplitude of the P wave is increased and/or broadened, and sometimes the visible traces are most clearly defined by II, III, aVF and V1. Some people think that the degree of P wave changes is related to the prognosis. People with normal P waves are often asymptomatic; those with significant abnormalities are not only often symptomatic but also easily die rapidly in a short period of time. The PR interval often prolongs complete or incomplete right bundle branch block almost in every patient. Limb leads and right chest leads often have low voltages. Occasionally, right ventricular hypertrophy, but no left ventricular hypertrophy. The QRS complex in the V1~4 lead is Qr-type with T-wave inversion, which is a specific electrocardiogram change of this malformation. About 5% to 25% of patients with this malformation have pre-excitation syndrome (type B). Among those with congenital heart disease and pre-excitation syndrome, 30% were Ebstein malformations. Therefore, clinically, when congenital heart disease with pre-excitation syndrome, the possibility of Ebstein malformation should be suspected. This type of malformation can occur in a variety of arrhythmias, with paroxysmal supraventricular tachycardia being common, even in the absence of pre-excitation syndrome, such as atrial arrhythmias, atrial flutter, or atrial fibrillation.

2. X-ray examination: mild deformity, heart enlargement is not obvious, pulmonary blood is normal. In patients with moderate or severe deformities, the heart expands to the sides, mainly for the enlargement of the right atrium. Under the fluoroscopy, the heart beat is not obvious, and it is not commensurate with the enlarged heart, similar to the X-ray signs of pericardial effusion or pulmonary stenosis with heart failure. Because the right atrium enlarges and the right ventricular outflow tract shifts to the left, the heart shadow can be square or funnel shaped, and a few lesions can be spherical. Pulmonary blood is reduced, and the aortic node is normal or small.

3. Echocardiography: The most typical manifestation is an increase in the amplitude of the anterior tricuspid valvular motion with a closure delay (at least 0.04 s after the mitral valve is closed). In addition, if the probe is placed in a large heart chamber (the right ventricle of the room) where the right ventricle is generally displayed, it can be seen that the EF slope of the tricuspid valve (the early closing movement of diastole) is slowed down.

4. Right heart catheterization: In the past, Ebstein syndrome was considered to be very dangerous for cardiac catheterization, and it is prone to severe arrhythmia and even life-threatening. Therefore, if you do not consider cardiac surgery, it is best not to do this examination. It is now considered that if the clinical diagnosis is unknown, it should be carried out despite the danger. Under the condition of experienced personnel and rescue equipment, the danger is not great. A group of internationally studied 505 patients with this malformation, 363 patients underwent catheterization and angiography, and 100 patients had arrhythmia, including 13 deaths. The catheter is often coiled in the enlarged right atrium during right heart catheterization. Manipulating the catheter often pushes the tip of the catheter into the left atrium (via the heart chamber) but is difficult to access the right ventricle. The pressure in the right atrium is higher, and the right atrial pressure curve shows a wave and v wave increase. The right ventricle has a high diastolic pressure and the systolic blood pressure is normal or slightly higher.

Pulmonary arterial pressure is normal or low When the catheter is removed from the pulmonary artery to the right ventricle, or from the right ventricle to the right atrium, and the pressure curve is continuously recorded, there may be a systolic pressure difference on both sides of the pulmonary valve, and diastolic pressure on both sides of the tricuspid valve. difference. In most patients, there is a right-to-left shunt at the atrial level, and even a horizontal to left shunt can be found at this level. It should be specifically mentioned here that if the pressure curve and the intracardiac electrocardiogram can be recorded simultaneously, it is often found that there is a transition zone between the right atrium and the functional right ventricle. The pressure recorded in the area is the same as the right atrium and the intracardiac electrocardiogram is the same as the right ventricle. This transition zone is the right ventricle of the room. This finding often contributes to the diagnosis of Ebstein syndrome.

5. Selective right heart system angiography: The main basis for the diagnosis of malformation angiography is: tricuspid malformation, downshift and right ventricularization are generally seen in the lower edge of the right atrium of the developed two incisions, one in the midline of the spine Nearby, it is an intrinsic tricuspid annulus; the second is located on the left side of the spine as a reflection of the tricuspid valve moving downward. Between the two incisions is the right ventricle of the room. In addition, selective right atrial angiography can be found, the right atrium is obviously dilated, the contrast agent passes slowly; the left atrium, the left ventricle, and the aorta are developed early (when the room is in traffic) . With selective right ventricular angiography, tricuspid regurgitation can also be found, and the right ventricular outflow tract is narrowed or dilated.

Differential diagnosis

Complications such as heart failure, arrhythmia, cerebral embolism and brain abscess can be combined.

Heart failure, also known as "myocardial failure," refers to the heart's inability to fight the blood supply that is commensurate with the venous return and body tissue metabolism. Often, the ability of the heart muscle to contract is weakened by various diseases, so that the blood output of the heart is reduced, which is insufficient to meet the needs of the body, and thus a series of symptoms and signs are generated.

Arrhythmia (cardiacarrhythmia) refers to any abnormality in the origin of heart rhythm, heart rate and rhythm, and impulse conduction. The meaning of the words "heart rhythm disorder" or "arrhythmia" is more important than the abnormality of rhythm. Arrhythmia includes both rhythm and frequency anomaly, more exact and appropriate.

Cerebral embolism refers to the abnormality of solid, liquid, gaseous objects (called emboli) along the blood circulation into the cerebral arterial system, causing occlusion of the arterial lumen, leading to necrosis of the brain tissue in the blood supply area of the artery, clinically showing hemiplegia, Sudden numbness, unclear speech and other sudden symptoms of local neurological deficits. The disease accounts for 15-20% of cerebrovascular diseases. The most common embolus is derived from the heart, about 14-48% of patients with rheumatic heart disease develop cerebral embolism; myocardial infarction, endocarditis, atrial fibrillation, heart surgery is easy to induce this disease; non-cardiac emboli Found in the neck atherosclerotic plaque, traumatic fracture or pneumothorax, diving or high-altitude flight decompression, pregnant women production.

A brain abscess is a purulent inflammation caused by a pathogen such as a bacterium, a fungus, or a parasite invading the brain parenchyma, which in turn forms an abscess. The abscess is formed in the brain parenchyma, which is clinically characterized by increased intracranial pressure, localized signs, and infectious symptoms. Brain abscesses caused by the latter two pathogens are rare. It can occur at any age and is more common in children and young adults. The incidence rate is about 2% of inpatients in neurosurgery, and the ratio of male to female is about 2.5:1.

The material in this site is intended to be of general informational use and is not intended to constitute medical advice, probable diagnosis, or recommended treatments.