全肺静脉异常连接(TAPVC)
这是什么??
从肺到心脏的静脉有缺陷.
In TAPVC, the blood does not take the normal route from the lungs to the heart and out to the body. 而不是, the veins from the lungs attach to the heart in abnormal positions and this problem means that oxygenated blood enters or leaks into the wrong chamber.
给患有TAPVC儿童家长的信息
原因是什么??
在大多数儿童中，病因不明. 有些儿童除了TAPVC还会有其他心脏缺陷.
它是如何影响心脏的?
在右心房, oxygen-rich (red) blood from the pulmonary veins mixes with low-oxygen (bluish) blood from the body. Part of this mixture passes through the atrial septum (atrial septal defect) into the left atrium. From there it goes into the left ventricle, then into the aorta and out to the body. 其余的血液流经右心室, 进入肺动脉，然后进入肺部. The blood passing through the aorta to the body doesn't have a normal amount of oxygen, 是什么导致孩子脸色发青.
TAPVC如何影响我的孩子?
症状可能在出生后不久出现. 在其他儿童中，症状可能延迟出现. This partly depends on whether the lung veins are blocked as they drain toward the right atrium. Severe obstruction of the pulmonary veins tends to make infants breathe harder and look bluer (have lower oxygen levels) than infants with little obstruction.
对于这个缺陷可以做些什么?
这种缺陷必须在婴儿早期通过手术修复. 做心脏直视手术的时候, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed.
我的孩子会做什么活动?
Children with repaired TAPVC may be advised to limit their physical activities to their own endurance. Some competitive sports may have greater risk if there is leftover obstruction in the pulmonary veins, 或者如果孩子有心律问题. Your child's pediatric cardiologist will help determine the proper level of activity.
我的孩子将来需要什么?
When surgical repair is done in early infancy, the long-term outlook is very good. 然而, your child will need regular follow-up with a pediatric cardiologist and, 一旦你的孩子长大成人, lifelong regular follow-up with a cardiologist who's had special training in congenital heart defects. Follow-up is needed to make certain that any remaining problems, such as an obstruction in the pulmonary veins or irregularities in heart rhythm, 对待. Some children may need medicines, heart catheterization or even more surgery.
如何预防心内膜炎呢?
Children with TAPVC are at increased risk for developing endocarditis. Ask your pediatric cardiologist about your child's need to take antibiotics before certain dental procedures to help prevent endocarditis. 请参阅 心内膜炎 了解更多信息.
患有TAPVC的成人
原因是什么??
在大多数情况下，病因尚不清楚. Some patients can have other heart defects along with TAPVC.
它是如何影响心脏的?
在右心房, oxygen-rich (red) blood from the pulmonary veins mixes with low-oxygen (bluish) blood from the body. Part of this mixture passes through the atrial septum (atrial septal defect) into the left atrium. From there it goes into the left ventricle, then into the aorta and out to the body. 其余的血液流经右心室, 进入肺动脉，然后进入肺部. The blood passing through the aorta to the body doesn't have a normal amount of oxygen, 是什么导致孩子脸色发青.
TAPVC对我有什么影响?
症状可能在出生后不久出现. 在其他患者中，症状可能会延迟. This partly depends on whether the lung veins are blocked as they drain toward the right atrium. Severe obstruction of the pulmonary veins tends to make infants breathe harder and look bluer (have lower oxygen levels) than infants with little obstruction. 成人未矫正的TAPVC是罕见的. Patients who survive until adulthood usually have only mild cyanosis and no evidence of blockage. Symptoms include shortness of breath and inability to exercise.
如果我的TAPVC从小就固定了，我还能期待什么呢?
This defect is almost always surgically repaired in early infancy or childhood. 做心脏直视手术的时候, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. The prognosis after surgery during childhood is excellent with uncommon late complications. Rarely, obstruction of the pulmonary veins at the site of reconnection can occur. 偶尔也会出现心律失常.
如果缺陷仍然存在怎么办? 成年后应该修复吗?
TAPVC在儿童期未修复是很少见的, 但这一类的大多数成年人都能做手术.
持续的关心
我将来需要什么?
Patients with a history of TAPVC should be seen periodically by a cardiologist with expertise in adult congenital heart disease to look for uncommon problems. 长期前景非常好, and usually no medicines and no more surgery or catheterization are needed. You should also consult a cardiologist with expertise in caring for adults with congenital heart disease if you're undergoing any type of non-heart surgery or invasive procedure.
医学随访
Your cardiologist can monitor you with noninvasive tests if needed. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms. They'll help show if more procedures, such as a cardiac catheterization, are needed. Medicines may only be required if there are abnormal heart rhythms.
活动的限制
Most adults with repaired TAPVC will not need to limit their physical activities to their own endurance. Some competitive sports may have greater risk if there is leftover obstruction in the pulmonary veins, 或者如果病人有心律问题. Your cardiologist will help determine the proper level of activity for you.
心内膜炎的预防
修复后6个月内无需使用.
怀孕
There is little information about pregnancy in patients with repaired TAPVC since it is such a rare defect. 除非有障碍物, 或者是不正常的心律控制不好, most women who have had repair should tolerate pregnancy provided that a pre-pregnancy evaluation doesn't reveal any problems.
你还需要手术吗?
Once the TAPVC has been repaired, it's unlikely that more surgery will be needed. Rarely, an adult patient may have obstruction that may need to be corrected depending on the severity.
