three hearts
Introduction
Introduction to Sanfangxin Sanfangxin is a congenital cardiovascular malformation that is divided into two parts by the fibrosis of the left or right atrium due to embryonic developmental disorders. Is a rare congenital heart disease, accounting for about 0.1% of congenital heart disease, usually refers to the three-room heart refers to the left atrium is divided into the atrium and the true left atrium (connected with the left atrial appendage, and through the mitral valve And left ventricular traffic). The right three-room heart is less common, accounting for only about 8% of the three-room heart case. There are many types of three-room heart classification. According to the position of the auxiliary room, it can be divided into three types: a, b and c: a type, the auxiliary room is located above the true left atrium; b type, the auxiliary room is located behind the true left atrium; c Type, the auxiliary room rides across the room interval. From the hemodynamics, according to the traffic conditions of the atrium and the true left atrium, and the left and right atrial traffic, it is roughly divided into two types of mitral stenosis and atrial septal defect. basic knowledge Sickness ratio: 0.0002%-0.0005% Susceptible people: no specific population Mode of infection: non-infectious Complications: arrhythmia pulmonary hypertension
Cause
Three-hearted heart disease
Congenital factors (90%)
The cause of this disease is not very clear. It is generally believed that the common venous trunk in the embryonic period failed to fuse with the left atrium, and the enlargement of the common pulmonary veins constitutes part of the left atrium, and the original left atrium is not integrated into the sub-atrium, and the original Abnormal development of the ventricle, abnormal diaphragm in the left atrium, the left atrium is divided into the auxiliary room and the real left atrium.
Pathogenesis
Hemodynamic changes depend on the size and concomitant malformation of the diaphragmatic orifice in the atria. The hemodynamics of the left three-chamber heart is similar to the mitral stenosis. The left septum is only a few millimeters in diameter, which can cause pulmonary venous return stasis, pulmonary stagnation, pulmonary edema and pulmonary hypertension, and partial pulmonary vein abnormal reflux or A septal defect between the right atrium and the secondary atrium creates a left-to-right shunt. If the atrial septal defect is close to the intrinsic atrial lumen, it is a right-to-left shunt.
Prevention
Three-room heart prevention
There is no effective preventive measure for this disease, but the surgical effect is good. The pulmonary arterial pressure may drop to normal after surgery. For infants with congestive heart failure, the mortality rate is high. Therefore, early detection, early diagnosis and early treatment are still treated. A key to this disease.
Complication
Three-compartment heart complications Complications, arrhythmia, pulmonary hypertension
The diseases that are easily complicated by the disease are as follows:
1, arrhythmia
Because most of the three chambers are combined with other intracardiac malformations, the operation is difficult, and there are many intracardiac incisions. It is easy to damage the conduction beam and cause atrioventricular block. The suture site pulls the conduction beam and causes local tissue trauma. The edema also causes postoperative operation. Conduction block, electrolyte imbalance, myocardial ischemia, hypoxia and reperfusion injury caused by surgery, it is easy to cause postoperative malignant arrhythmia, severe cardiac arrest, timely detection and early treatment is the key, postoperative Arterial blood was taken within 30 min after ICU for blood gas analysis, timely treatment of water, electrolyte imbalance, acid-base imbalance, oxygen supply imbalance, until every indicator was normal, blood gas analysis was performed every 2 to 4 hours to avoid various causes. Arrhythmia induced by hypoxia.
2, pulmonary hypertension crisis
Pulmonary hypertension is a severe syndrome caused by a sharp increase in pulmonary artery pressure in the short term. It is often accompanied by a significant decrease in cardiac output and oxygen saturation. Pulmonary hypertension can occur in both preoperative and intraoperative conditions. Increased pressure, increased heart rate, decreased oxygen saturation, irritability of patients, active treatment at this time, the outcome of pulmonary crisis is sometimes irreversible, often occurs after 18 to 48 h after surgery, can be advanced or Delayed, the key to treatment is prevention, avoiding the cause of pulmonary hypertension crisis, adequate sedation early in the postoperative period to prevent agitation, micro-pumping sedative morphine, Domecan, propofol according to doctor's advice; appropriate extension of assisted breathing time, Fully oxygen supply, keep the airway unobstructed, clear the secretions of the respiratory tract in time, and gently suck the sputum. Before and after sucking, give high concentration of oxygen to the respiratory sac and expand the lungs to prevent agitation, pain, increased oxygen and pulmonary vasoconstriction, lung Increased vascular resistance, thereby inducing pulmonary hypertension, while closely observing changes in pulmonary arterial pressure, continuously monitoring the lungs through intraoperative pulmonary or mandibular pressure tubes Pulse pressure, pulmonary artery pressure when the circulation close to or greater than 1/2, using the prescribed vasodilators such as sodium nitroprusside, prostaglandin E1, and control the pressure in the pulmonary artery 1/3 or less circulation, preventing pulmonary hypertension crisis occurred.
3, low cardiac output syndrome
Low cardiac output syndrome is a special type of cardiogenic shock, which is one of the serious complications of cardiopulmonary bypass. After strict control of the amount of fluid in and out, avoiding excessive fluid overload and excessive capacity overload. Invasive blood pressure, central venous pressure, urine volume; appropriate use of vasoactive drugs, should start from a small dose, gradually adjust to the desired effect, avoid hemodynamic fluctuations; timely detection, early treatment of various types of arrhythmia, prevent induction and aggravation Heart plague syndrome.
Symptom
Three-room heart symptoms Common symptoms Shortness of breath, systolic murmur, edema, narrow diaphragm hole
I. Clinical manifestations: The time of symptom onset is related to the size of the diaphragm hole. Severe cases with narrow pores can cause severe pulmonary congestion and shortness of breath shortly after birth, followed by severe pneumonia and congestive heart failure. Cases, symptoms appear later, in early childhood or childhood, large cases of atrial tract are similar to atrial septal defect, clinically asymptomatic, normal life, only a little shortness of breath after the event, most cases can be heard and sprayed at the bottom of the heart Sexual systolic murmurs and diastolic murmurs, sometimes continuous murmurs can be heard. This is due to the high degree of obstruction and the high degree of pressure gradient near the distal end of the tunnel. P2 hyperthyroidism, but also no noise.
Second, the hemodynamic changes depend on the size and concomitant malformation of the diaphragm in the atrium. The hemodynamics of the left three-chamber heart is similar to the mitral stenosis. The left septum is only a few millimeters in diameter, which can cause pulmonary veins. Reflux stasis, pulmonary stagnation, pulmonary edema and pulmonary hypertension, and partial pulmonary vein abnormal reflux or atrial septal defect between the right atrium and the accessory atrium produces a left-to-right shunt. If the atrial septal defect is close to the intrinsic atrial cavity, it is right. Diverted to the left.
Third, the clinical type: In 1964, Ji Zhuyi combined the classification of Loeffler and Niwayama into three types.
Schematic diagram of three-atrial anatomical malformation
There is no communication between the type I accessory room and the true left atrium. The accessory room passes through the foramen ovale or with a complete abnormal return of the pulmonary vein, and the baby dies early.
There are one or several small passages between the type II atrium and the true left atrium, which are further divided into two subtypes from the perspective of clinical surgery:
(1) It is not connected to the right atrium, and the clinical manifestations are similar to the symptoms of mitral stenosis.
(2) Communicating with the right atrium, the clinical manifestations are similar to the symptoms of atrial septal defect or complete pulmonary venous return.
There is a great connection between the type III accessory and the true left house.
Examine
Three-room inspection
In order to correctly diagnose the disease, the following methods can be performed:
(1) X-ray examination: the heart is mild to moderately enlarged, mainly to the right ventricular hypertrophy, with obvious pulmonary hypertension, but the left atrium is not large or only slightly increased, characterized by superior vena cava dilatation, pulmonary interstitial edema Pulmonary artery segment highlights pulmonary motility and venous hypertension.
(2) Electrocardiogram: right axis deviation, right ventricular hypertrophy, P wave increase suggesting right atrial hypertrophy.
(3) Echocardiography: B-mode echocardiography shows an abnormal diaphragm echo in the left atrium and above the mitral valve. Pulsed Doppler ultrasonography can show abnormal diaphragm and visible blood flow through the diaphragm and the interatrial septum. The size of the defect is very helpful for diagnosis.
(4) Cardiac catheterization and angiography: The right heart catheter is characterized by an increase in pulmonary wedge pressure and a low or normal left atrial pressure. About 1/3 of the cases can pass through the atrial septal defect or egg after entering the right atrium. The round hole enters the left atrium, and the left atrial angiography can show that there is an abnormal diaphragm in the left atrium. If the auxiliary room can be displayed, it can be found that there is no contraction in the cardiac cycle, and the shape is kept constant, and the cardiac catheterization is generally not required.
Diagnosis
Three-Diagnostic Diagnosis
The disease needs to be identified with the following diseases:
(1) Hemodynamic changes of congenital mitral stenosis are similar to those of Sanfangxin, so clinical symptoms and signs are difficult to identify. Echocardiographic examination shows left atrial enlargement, no diaphragm or intracardiac shunt, only mitral valve In the stenotic lesions, left atrial angiography showed enlargement of the left atrium, delayed emptying, and no presence of the diaphragm and the third atrium.
(2) Complete pulmonary vein abnormality X-ray chest radiograph was "8" or "snowman". Pulmonary angiography showed abnormal connection of pulmonary veins. Echocardiography showed abnormal pulmonary venous return and atrial septal defect.
(3) Left atrial myxoma When the myxoma partially obstructs the mitral valve, the clinical symptoms are similar to those of the mitral stenosis or the three-chamber heart. Echocardiography shows that the abnormal mass in the left atrium moves with the contraction of the heart.
