完全性房室管缺损(CAVC)
这是什么??
许多术语被用来描述这个复杂的缺陷. 它们包括房室(AV)管, 完整房室管, 完整房室总管, 房室间隔缺损和心内膜垫缺损.
A large hole in the center of the heart affecting all four chambers where they would normally be divided. 当一颗心被正确地分割, 来自肺部的富氧血液不会与来自身体的贫氧血液混合. A CAVC allows blood to mix and the chambers and valves to not properly route the blood to each station of circulation.
房室管缺损是心脏中心的一个大洞. It's located where the wall (septum) between the upper chambers (atria) joins the wall between the lower chambers (ventricles). 室间隔缺损累及上下腔. The tricuspid and mitral valves that normally separate the heart's upper and lower chambers aren't formed as individual valves. Instead a single large valve forms that crosses the defect in the wall between the two sides of the heart.
为患有CAVC儿童的家长提供更多信息
原因是什么??
在大多数儿童中，病因不明. 这是一种非常常见的有染色体问题的儿童心脏缺陷, 21三体(唐氏综合症). 有些儿童除了房室管外还会有其他心脏缺陷.
它是如何影响心脏的?
正常情况下, 心脏的左侧只向身体泵血, 而心脏的右侧只将血液输送到肺部. 儿童耳道缺损, blood can travel across the holes from the left heart chambers to the right heart chambers and out into the lung arteries. 被泵入肺部动脉的额外血液使心脏和肺部更加努力地工作 肺部可能会充血.
耳道缺损对孩子有何影响?
患有房室管缺陷的儿童可能比正常人呼吸更快、更困难. 婴儿可能在喂养和正常生长方面有困难. 症状可能在出生后几周才出现. High pressure may occur in the blood vessels in the lungs because more blood than normal is being pumped there. 久而久之，这会对肺血管造成永久性损伤.
In some infants, the common valve between the upper and lower chambers doesn't close properly. 这使得血液从心脏的下腔向后渗漏到上腔. 这种泄漏，称为反流或功能不全，也会使心脏工作更加困难.
对于这个缺陷可以做些什么?
耳道可以固定. 需要开胸手术来修复这个缺陷. 与其他类型的室间隔缺损不同，房室管缺损不能自行关闭. 药物可以暂时用于缓解症状, 但它们不能治愈这种缺陷，也不能防止对肺部动脉造成永久性损伤.
有严重症状或肺部高血压的婴儿, 手术通常必须在婴儿时期进行. 在手术过程中，外科医生用一个或两个贴片将大洞闭合. Later the patch will become a permanent part of the heart as the heart's lining grows over it. The surgeon also divides the single valve between the heart's upper and lower chambers and makes two separate valves. 这些阀门将尽可能接近普通阀门.
如果婴儿病得很重, 或者有缺陷，可能太复杂，无法在婴儿期修复, 可能需要临时手术来缓解症状和肺部的高压. This procedure (pulmonary artery banding) narrows the pulmonary artery to reduce the blood flow to the lungs. 当孩子长大了, 通过开胸手术切除腱束并修复房室管缺损.
我的孩子会做什么活动?
如果手术关闭了房室管缺损, your child may not need any special precautions regarding physical activities and may be able to participate in normal activities without increased risk. 积极锻炼身体对心血管系统有益, 但有些孩子可能需要限制他们的活动. 与你孩子的儿科心脏病专家讨论这个问题.
我的孩子将来需要什么?
手术后，您的孩子必须由儿科心脏病专家定期检查. 有时需要更多的药物或手术治疗.
手术修复耳道通常能使血液循环恢复正常. 然而，重建后的瓣膜可能无法正常工作. 阀门结构可能泄漏或变窄. 但, 对很多孩子来说, 长期前景是好的, 通常不需要药物或额外的手术.
如何预防心内膜炎呢?
有房室管缺损的儿童在修复前后都可能有心内膜炎的危险. Ask about your child's risk of 心内膜炎 and about your child's need to take antibiotics before certain dental procedures. 请参阅 心内膜炎 了解更多信息.
成人CAVC的更多信息
Some patients with AV canal defects have a small or no opening between the bottom chambers of the heart. They can present in adulthood with findings similar to patients with atrial septal defects; this can be referred to as an "ostium primum" atrial septal defect. 更不常见的是，只有下腔之间的孔存在.
原因是什么??
在大多数情况下，病因尚不清楚. 这是一种非常常见的有染色体问题的儿童心脏缺陷, 21三体(唐氏综合症). 有些病人除了房室管外还有其他心脏缺陷.
它是如何影响心脏的?
正常情况下, 心脏的左侧只向身体泵血, 而心脏的右侧只将血液输送到肺部. 儿童耳道缺损, blood can travel across the holes from the left heart chambers to the right heart chambers and out into the lung arteries. 被泵入肺部动脉的额外血液使心脏和肺部更加努力地工作, 肺部可能会充血. 有时异常单阀有漏气(反流). 这可能会增加心力衰竭的症状.
房室管缺损对患者有何影响?
High pressure may occur in the blood vessels in the lungs that can lead to permanent damage with 肺动脉高压 that persists into adulthood.
许多以前没有做过修复的成年人都做过 肺动脉高压 (参见艾森曼格综合症). 这种并发症比ASD或室间隔缺损患者更常见. 即使是没有艾森曼格综合症的成年人, 症状包括呼吸急促, 不耐运动和心悸是常见的. 关于身体检查, 由于血液流经缺陷和瓣膜泄漏引起的杂音是常见的.
如果我的房室管缺陷在童年时被关闭，做了什么，我能期待什么?
The surgery to fix these defects involves patching the ASD and the VSD and repairing the heart valve. 修理阀门, the surgeon divides the single valve between the heart's upper and lower chambers and makes two separate valves. 这些阀门尽可能接近普通阀门. It's possible that a temporary operation to relieve symptoms and high pressure in the lungs may have been performed before the definitive operation. This procedure (pulmonary artery banding) narrows the pulmonary artery to reduce the blood flow to the lungs. 当孩子长大了, an operation was done to remove the band and fix the AV canal defect with open-heart surgery. 与其他类型的室间隔缺损不同，房室管缺损不会自行关闭.
手术修复耳道通常能使血液循环恢复正常. For many patients, 长期前景是好的, and no medicines or additional surgery are needed. 因为这是一种更复杂的先天性心脏缺陷, 晚期问题在成人中比在ASD或室间隔关闭后更常见. 随着孩子的成长, 修复可能部分损坏，导致补丁泄漏, 瓣膜渗漏，使流向身体的血液通道变窄. 这些问题可能会增加心脏的负荷并引起症状.
如果仍然存在房室管缺损怎么办? 成年后应该修复吗?
成年后决定是否修复耳道缺损是很复杂的. 这取决于肺的压力和心脏的泵血功能. 然而, 当压力不太高，泵的功能良好, 这些缺陷是可以修复的，成年患者也有可能得到改善. A heart catheterization is almost always required to know whether the defect should be closed. 这些缺陷在导管室是无法关闭或修复的, 然而, 因为它们的位置和修复心脏瓣膜的需要.
你可能遇到的问题
Problems in patients with repaired AV canals depend on whether there are patch leaks and whether there's a lot of valve regurgitation. Shortness of breath, inability to exercise and swelling in the legs are all signs of heart failure. Abnormal heart rhythms may cause palpitations (skipped or rapid heartbeats) and, rarely, fainting. Some patients may need a pacemaker after the repair if the electrical system has been damaged.
未修复的房室管患者常呈蓝色(见 Eisenmenger综合征). 因为心脏左侧的瓣膜漏了, 他们比其他艾森曼格综合症患者更容易患心力衰竭, 因为asd和vsd.
持续的关心
我将来需要什么?
An adult with a repaired or unrepaired AV canal defect must be examined regularly by a cardiologist with experience in adult congenital heart disease. 检查的频率取决于维修问题的严重程度, 存在心律异常和肺动脉高压. 一般来说，你应该每年至少去看一次心脏病专家. You should also consult a cardiologist with expertise in care of adult congenital heart disease if you're undergoing any type of non-heart surgery or invasive procedure or thinking about heart surgery.
医疗
可能需要心力衰竭药物, 尤其对于瓣膜返流的患者, 帮助他们的心脏更好地跳动和/或降低血压. 肺动脉高压患者也可能需要药物治疗. 如果需要的话，你的心脏病专家可以用无创检查来监测你. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms. 当考虑进一步手术时，几乎总是需要心导管插入术.
我能做什么活动?
如果手术关闭了房室管缺损, you may not need any special precautions and may be able to participate in normal activities without increased risk. 然而，心力衰竭和肺动脉高压患者可能需要一些限制.
心内膜炎的预防
Patients with AV canal defects may risk an infection of the heart's inside lining or valves (心内膜炎) before and after surgery. 建议终生预防心内膜炎. 请参阅 心内膜炎 了解更多信息.
怀孕
如果手术后你没有明显的残留问题, 怀孕的风险很低. 如果有瓣膜漏水或心律失常之类的问题, 你患妊娠并发症的风险可能会增加. 如果你有心力衰竭或肺动脉高压，不建议怀孕. Women with unrepaired AV canal defects or who have leftover problems should talk to their cardiologist before deciding to get pregnant. 请参阅 怀孕 和 遗传咨询 了解更多信息. Pregnant women with repaired AV canal defects who are free of significant problems may not require high risk obstetrical care. In contrast those unrepaired AV canal defects, significant valve leaks or 肺动脉高压.
你还需要手术吗?
修复后的阀门功能是一个长期关注的问题. 当一些病人变老时，他们需要用一个机械瓣膜来替换他们的瓣膜. 瓣膜能进一步修复的情况很少见. 其他患者可能需要更多的手术来关闭补丁泄漏.
