Congenital mitral valve deformity
Introduction
Introduction to congenital mitral valve malformation Congenital mitral valve malformation can lead to mitral stenosis and/or regurgitation. Fisher reported 2 cases of congenital mitral valve malformation in 1902, and cases reported after the 1960s are increasing. Congenital mitral valve malformation often with atrioventricular tube malformation, aortic dislocation, single ventricle, atrial septal defect, ventricular septal defect, left ventricular outflow tract or aortic outlet stenosis, aortic coarctation, tetralogy of Fallot, etc. Congenital cardiovascular disease exists in combination. Separate congenital mitral valve malformation is rare, accounting for only 0.6% of autopsy materials for congenital cardiovascular disease, and 0.21 to 0.42% of clinical cases. The mitral valve consists of an annulus, leaflet, chordae, papillary muscle and left atrial wall. Any one or several dysplasia of the constitutive structure can produce congenital mitral valve malformation. Dysplasia, resulting in multiple stenosis in the left atrioventricular blood flow channel. basic knowledge The proportion of illness: 0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: atrioventricular tube malformation, single ventricle, ventricular septal defect, tetralogy of Fallot
Cause
Congenital mitral valve malformation
Congenital mitral stenosis is a rare congenital disease, the cause of which is still unclear. At present, it is mainly thought that abnormalities in the process of embryonic development lead to hypoplasia. Considering the etiology and pathology, the disease can be divided into the following. Several types:
(1) If the dysplastic mitral valve is attached to a single group of left ventricular posterior papillary muscles by a short chordae, and the ultrasound appears to be mitral echo enhancement, activity is limited, and the left ventricular single group can be displayed. Papillary muscles, short chordae attachment and mitral open restriction, parasternal short axis, apical view and M-mode ultrasound also show mitral openness limitation, then called parachute mitral valve malformation
(2) Double-hole mitral valve malformation
Ultrasound is characterized by left atrioventricular valve doubling, shown as two separate flaps, and the parasternal short axis view is optimal. The apical view also shows two separate flaps and their respective subvalvular support structures. And nipple dysplasia, two-hole mitral valve malformation combined with varying degrees of mitral regurgitation.
(3) Congenital mitral valve malformation of asymmetrical papillary muscles
The ultrasound of congenital mitral stenosis in the two groups of papillary muscles showed enhanced mitral echo and limited openness. The anterior lateral papillary muscle was the dominant papillary muscle and the posterior middle papillary muscle was smaller.
(4) Congenital mitral valve malformation of the rotation of the papillary muscles
Ultrasound showed that the left ventricular papillary muscles of the patient showed a significant rotation, which became internal anterior and posterior, and the direction of the mitral valve also rotated.
(5) Congenital mitral valve malformation of three groups of papillary muscles
There was a normal offset relationship between the left atrioventricular valve and the right atrioventricular valve, but ultrasound showed three groups of left ventricular papillary muscles, three joint sites and three valve leaflets.
Prevention
Congenital mitral valve malformation prevention
There are no effective preventive measures, timely surgical treatment, attention to some potential complication and active treatment, is the only effective measure to prevent more serious harm, in the surgical effect, mitral stenosis cases, valve repair and plastic surgery The operative mortality rate was 25 to 4%, and the operative mortality of patients with mitral regurgitation was only 3-1%. The operative mortality of valve replacement in mitral stenosis was 2 to 6%. The mitral regurgitation was incomplete. For 3%, although there were more than half of the left atrium in the left atrium, there was residual diastolic pressure difference between the left ventricle or systolic murmur in the apical area, but the heart function recovered to grade I in nearly 9% of cases.
Complication
Congenital mitral valve malformation complications Complications, atrioventricular tube malformation, single ventricular ventricular septal defect, tetralogy of Fallot
The disease is often associated with atrioventricular tube malformation, aortic dislocation, single ventricle, atrial septal defect, ventricular septal defect, left ventricular outflow tract or aortic outlet stenosis, aortic coarctation, tetralogy of Fallot etiology and other congenital heart vessels Symptoms exist in combination, severe cases can be complicated by pulmonary hypertension. For children undergoing surgery, there may be some postoperative complications, including low cardiac output syndrome, respiratory dysfunction and severe arrhythmia.
Symptom
Symptoms of congenital mitral valve malformation Common symptoms: Qi tremor, congestion, purpura, systolic murmur, sitting, breathing, first heart sound, hyperthyroidism, pulsating heart failure, mitral regurgitation
symptom
The clinical manifestations of congenital mitral valve malformation are similar to acquired mitral valve disease, but the symptoms appear early, and there is no history of rheumatic fever, about 30% of patients within 1 month after birth, 75% after birth Symptoms are present within 1 year. Common symptoms are shortness of breath, sitting breathing, pulmonary edema and recurrent pulmonary infection. Patients with severe disease have congestive heart failure and cyanosis due to complicated pulmonary hypertension.
Physical examination: poor physical growth and development, easy to get tired, typical signs of mitral stenosis are apical diastolic drum-like murmur, and may be accompanied by tremor, first heart sound hyperthyroidism and open slap sound, if leaflet activity is affected The above-mentioned signs are not obvious, and the second pulmonary heart disease in the pulmonary valve area is hyperthyroidism. The pulmonary infection can be heard in the lungs. The apical area of the mitral regurgitation can be seen in the apical area, and the powerful pulsating pulsation is observed. In the apical area, the systolic murmur can be heard to the left iliac crest, and the third heart sound can often be heard. The second heart sound in the pulmonary valve area of the pulmonary hypertension is splitting.
Examine
Examination of congenital mitral valve malformation
Chest X-ray examination: the heart shadow is enlarged, the left atrium enlargement is abnormal, the pulmonary artery is conical, the lung stagnation causes lung vascular shadow thickening, and the severe condition may present signs of pulmonary edema, electrocardiogram examination: typical signs are P wave widening There is a notch, the left chest lead shows an enlarged two-phase P wave indicating left atrial hypertrophy, the mitral stenosis case shows right ventricular hypertrophy, the right axis of the electric axis, and the mitral regurgitation shows left ventricular hypertrophy and strain. Or left and right ventricular hypertrophy, atrial fibrillation is rare, cardiac catheterization and selective left heart angiography: cardiac catheterization and selective left heart angiography can show the location of the lesion, morphology and severity, to identify pulmonary circulation pressure and pulmonary vascular resistance And can identify other congenital cardiovascular diseases.
Echocardiography: Cut-to-face echocardiography can directly show the shape, activity and alignment of the leaflets, determine the size of the valve, the shape of the upper ring and the subvalvular structure, and the ultrasonic pulse Doppler examination can still determine Whether the cusp has reflux and its extent, echocardiography can also examine other coronary vascular lesions.
Diagnosis
Diagnosis and diagnosis of congenital mitral valve malformation
The disease needs to be differentiated from other children with mitral valve disease, such as rheumatic mitral stenosis in children.
Rheumatic mitral stenosis in children, mild symptoms may not occur, children with moderate or higher stenosis may be fatigued, palpitations, shortness of breath, difficulty breathing after activities, accompanied by mild cyanosis of the mouth, flushing of cheeks, so-called "mitral valve" "Skin", severe cases may occur hemoptysis or bloody foam sputum, sitting breathing and paroxysmal nocturnal dyspnea, the child's signs are full of anterior region, apex can be heard low-pitched rumbling diastolic murmur, the range of noise is limited, Often accompanied by diastolic tremor, the first heart sound of the apex is hyperthyroidism, the second sound of the pulmonary artery is hyperthyroidism and splitting, the left sternal border 3 to 4 intercostal or the upper apex of the apex can be heard loud mitral open slap sound (mitral valve open Petal sound), active rheumatoid carditis and enlargement of the left atrium, patients with increased pressure often have atrial arrhythmia, such as atrial premature beats, atrial tachycardia and atrial fibrillation, especially the latter suggest more rheumatoid activities, The most important point that can be distinguished from this disease is that the congenital mitral stenosis presents symptoms early and has no history of rheumatic fever. About 3% of patients are within 1 month after birth, and 75% are within 1 year after birth. Presenting symptoms
