Congenital absence or defect of the pericardium

Introduction to congenital pericardial defects or defects Congenital palpe absorptive or defect (congenitalpericardialabsenceordefect) is a rare form of heart disease, more male than female, 3:1, reported patients aged 11 to 79 years. The disease was first confirmed by RealdoColumbus in 1559, but the first reliable case was reported by Baille in 1793. basic knowledge The proportion of sickness: 0.00001% Susceptible people: no specific population Mode of infection: non-infectious Complications: syncope, patent ductus arteriosus, septal defect, children with tetralogy of Fallot

Congenital pericardial absence or defect cause

(1) Causes of the disease

Originated from congenital pericardial dysplasia.

(two) pathogenesis

Most authors believe that the occurrence of this disease is related to the left Cuvier tube. During normal embryonic development, the left Cuvier tube gradually shrinks and forms part of the left upper intercostal vein. If it shrinks prematurely, it will make the pleural pericardial wrinkle blood supply poor. Pericardial dysplasia, resulting in defects of varying sizes.

Congenital pericardial deficiency or defect prevention

1. Strengthen publicity and education, promote prenatal and postnatal care; strengthen pregnancy care, guide reasonable pregnancy, and avoid the occurrence of fetal congenital malformations.

2. Reasonable arrangement of exercise: The amount of exercise should be controlled, and the sudden movement of the force should be forbidden to prevent a part of the heart from being incarcerated and endangering life.

Congenital pericardial absence or defect complications Complications, syncope, atrial fibrillation, atrial septal defect, tetralogy of Fallot

One third of the cases can be combined with other congenital malformations, such as patent ductus arteriosus, atrial septal defect, congenital mitral stenosis, tetralogy of Fallot and so on.

Congenital pericardium deficiency or defect symptoms Common symptoms Chest pain syncope dyspnea systolic murmur vascular malformation patent ductus arteriosus atrial septal defect dizziness

1. Both men and women can be ill, more men than women (3:1), reported patients aged 11 to 79 years.

2. Most patients with pericardial defects are usually asymptomatic, and a few may have certain non-specific symptoms. The most common symptoms are chest discomfort, occasionally dyspnea, dizziness and syncope. Part of the left pericardial defect may occur at the defect. Some of the heart is incarcerated and life-threatening. The right part of the pericardium defect is rare, and may be accompanied by right chest pain during inhalation, due to right or right ventricular fistula, or pulmonary fistula into the pericardial cavity.

3. The left sternal border can smell I-III systolic murmur. This murmur may be related to the turbulence caused by the variable heart. When the left pericardium is completely deficient, the apical beat can be shifted to the left.

Examination of congenital pericardial defects or defects

1. Electrocardiogram: the right axis of the electric axis, incomplete right bundle branch block, the QRS wave group in the precordial area is transposed clockwise.

2. Echocardiography: Confirmed that the left ventricular contour has localized bulging and pericardial echo fading.

3. CT and MRI: The right pericardium is clearly visible, while the left pericardium is absent, and abnormal lung tissue is wedged between the aorta and the pulmonary artery.

4. Chest X-ray: Inject air into the left pleural cavity about 500ml, take the left lateral position for a while and then take a picture. It is obvious that the gas enters the pericardial cavity, which is the most important method for diagnosing this disease. The heart shape on the chest X-ray Depending on whether or not the heart tissue is removed from the defect, if there is a sputum, there is a prominent "mass" shadow on the heart of the 3rd to 7th anterior intercostals (mostly the second bow of the left heart). The fluoroscopy shows that the beat is strong.

Congenital pericardial absence or defect diagnosis

The disease has no characteristic symptoms and signs. The diagnosis is based on echocardiography to confirm the left ventricular contour with localized bulging and pericardial echo fading. Radionuclide wedge can confirm the diagnosis. In 1995, Connolly et al summarized the echocardiography of 10 patients. The picture is characterized by abnormal ultrasound window (10/10), high heart variability (9/10), abnormal ventricular septal motion (8/10) and cardiac swing motion (7/10). Cardiac catheterization is only available. In combination with other congenital cardiovascular malformations requiring surgical treatment, if left atrial opacity occurs during pulmonary angiography, or the left atrial appendage outlines beyond the left edge of the heart, a left or left atrial deafness is indicated.

Chest pain should be differentiated from angina pectoris. The chest radiograph shows the upper left margin of the left heart. It needs to be differentiated from pulmonary artery dilatation, left atrial appendage aneurysm, mitral valve disease, atrial septal defect and left hilar lymph node enlargement.

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