Polyrhythmic heart sound
Introduction
Introduction "Multi-sound" heart sound is the main clinical manifestation of Ebstein syndrome. Ebstein syndrome, also known as Ebstein malformation, refers to the tricuspid valve and/or posterior valve occasionally attached to the proximal apex with the anterior flap. On the right ventricular wall, it accounts 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. 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, and the second component of the split first heart sound is often in the form of a click sound, which is called the "sail sign". The tricuspid valve area may have mild systolic murmur and short diastolic mid-term murmur, in addition to cyanosis, clubulite (toe) jugular vein systolic positive pulsation. Siber believes that the most characteristic signs of this malformation are two groups: 1 hairpin with a quiet precardiac region; 2 a first heart sound, a split second heart sound, an enhanced third heart sound, or a fourth heart sound. Quartet.
Cause
Cause
The cause of the disease is unclear. Occasionally, family history reports that the mother of the mother who is taking lithium in the early pregnancy is prone to the disease. The main pathological anatomical features of this malformation are tricuspid valvular deformity, right ventricular atrialization and functional right ventricular stenosis. In addition, a small number of cases can be combined with other congenital malformations, such as aortic coarctation, ventricular septal defect, pulmonary stenosis or atresia, patent ductus arteriosus or corrected large blood 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.
Examine
an examination
Related inspection
Electrocardiogram cardiac vascular ultrasound
The following points have a reference value when diagnosing this disease:
1. The symptoms of bruising in the neonatal period are obvious, and later alleviate or disappear completely. After the elderly, cyanosis reappears.
2. Cyanosis symptoms combined with tachyarrhythmia should first consider the 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, heart function I ~ II heart light ~ moderate increase, intracardiac shunt left to right mainly right between the right atrium and functional right ventricle, no angiography The prognosis is good without the need for surgery or just closing the intracardiac defect.
2 stenosis type: cyanosis, heart function, grade II or higher, mild to moderate heart enlargement, pulmonary circulation, blood flow decreased, enlarged right atrium and functional right ventricle, pressure difference, intracardiac shunt, right-to-left angiography Double ball sign 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 right atrium There are great double-ball signs that require surgery.
Diagnosis
Differential diagnosis
The diagnosis should be differentiated from the following symptoms:
1. Coming horse heart sound: normal adult heart beat has two heart sounds, called first heart sound and second heart sound, galloping horse is the additional heart sound after the second heart sound, and the original first and second The rhythm of the heart sounds is similar to the sound of the horseshoes touching the ground when the horse runs, so it is called the galloping horse. According to the different time of the occurrence of galloping, it can be divided into early diastolic galloping, diastolic late galloping and overlapping galloping, which is the most common early diastolic galloping, which appears after the second heart sound 0.12-0.18 Within seconds; late diastolic gallopism occurs later, before the start of systole, which is 0.1 second before the first heart sound, so it is also called systolic galloping; when there is simultaneous diastolic early gallop and diastolic late horse At the time of the law, plus the first heart sound and the second heart sound, it sounds like four sounds that occur in parallel, also known as "four temperament", "the locomotive runs the horse". It is generally believed that early diastolic galloping is caused by excessive ventricular diastolic load, decreased myocardial tension and decreased compliance, so that when the ventricle is dilated, blood filling causes wall vibration. Therefore, the appearance of galloping horse is an important sign of severe myocardial damage.
2. Heart sound is low and blunt: low heart sound refers to low heart rate. Low heart bluntness is not necessarily morbid. When the chest wall is thick, obesity, etc., the auscultation heart sound can be low and blunt. In some cases, it can also occur, such as pericardial effusion, heart failure, shock, etc.
3. The first heart sound hyperthyroidism: the left ventricular filling is reduced in the mitral stenosis, the mitral valve position is lower in the late diastolic, and second, the systolic period is shortened due to the decrease of the left ventricular blood volume, and the left ventricular pressure is rapid. Ascending, the low-order mitral valve suddenly becomes tense and closes, thus producing a high-key and crisp first heart sound, which sounds like a slap, commonly referred to as the slap-first heart sound. In complete atrioventricular block, the atrial and ventricular beats are not related, forming a phenomenon of compartment separation. When the atrium and ventricle are simultaneously contracted, the first heart sound is extremely loud, usually called "cannon sound". ".
