Total Anomalous Pulmonary Venous Connection (TAPVC)
Total de conexión venosa pulmonar anómala
What is it?
A defect in the veins leading from the lungs to the heart.
在TAPVC中，血液不能从肺部到心脏再到身体的正常途径. Instead, 肺部的静脉以不正常的位置连接到心脏，这个问题意味着含氧血液进入或泄漏到错误的腔室.
Information for Parents of children with TAPVC
What causes it?
In most children, the cause isn't known. Some children can have other heart defects along with TAPVC.
How does it affect the heart?
In the right atrium, 来自肺静脉的富氧(红色)血液与来自身体的低氧(蓝色)血液混合. 部分混合物通过房间隔(房间隔缺损)进入左心房. 它从那里进入左心室，然后进入主动脉，最后进入身体. The rest of the blood flows through the right ventricle, into the pulmonary artery and on to the lungs. 通过主动脉进入身体的血液没有正常的氧气量, which causes the child to look blue.
How does TAPVC affect my child?
Symptoms may develop soon after birth. In other children, symptoms may be delayed. 这部分取决于肺静脉流向右心房时是否被阻塞. 严重的肺静脉阻塞往往会使婴儿呼吸困难，看起来更蓝(含氧量更低).
What can be done about the defect?
This defect must be surgically repaired in early infancy. At the time of open-heart surgery, 肺静脉重新连接到左心房，房间隔缺损被关闭.
What activities can my child do?
有修复TAPVC的儿童可以建议限制他们的体力活动以他们自己的耐力. 一些竞技运动如果有残留的肺静脉阻塞，可能会有更大的风险, or if the child has heart rhythm problems. 您孩子的儿科心脏病专家将帮助您确定适当的活动量.
What will my child need in the future?
如果在婴儿期早期进行手术修复，远期前景非常好. However, 你的孩子需要定期接受儿科心脏病专家的随访, once your child reaches adulthood, 终身定期随访一位在先天性心脏缺陷方面接受过特殊训练的心脏病专家. 需要跟进以确保任何遗留的问题, 例如肺静脉阻塞或心律失常, are treated. 有些孩子可能需要药物，心导管插入术甚至更多的手术.
What about preventing endocarditis?
患有TAPVC的儿童患心内膜炎的风险增加. 询问你的儿科心脏病专家，在进行某些牙科手术之前是否需要服用抗生素，以帮助预防心内膜炎. See the section on Endocarditis for more information.
Congenital Heart Defect ID sheet
Adults with TAPVC
What causes it?
In most cases, the cause isn't known. Some patients can have other heart defects along with TAPVC.
How does it affect the heart?
In the right atrium, 来自肺静脉的富氧(红色)血液与来自身体的低氧(蓝色)血液混合. 部分混合物通过房间隔(房间隔缺损)进入左心房. 它从那里进入左心室，然后进入主动脉，最后进入身体. The rest of the blood flows through the right ventricle, into the pulmonary artery and on to the lungs. 通过主动脉进入身体的血液没有正常的氧气量, which causes the child to look blue.
How does TAPVC affect me?
Symptoms may develop soon after birth. In other patients, symptoms may be delayed. 这部分取决于肺静脉流向右心房时是否被阻塞. 严重的肺静脉阻塞往往会使婴儿呼吸困难，看起来更蓝(含氧量更低). It's rare for an adult to have uncorrected TAPVC. 存活到成年的患者通常只有轻微的紫绀，没有堵塞的迹象. 症状包括呼吸短促和不能运动.
If my TAPVC was fixed in childhood, what can I expect?
这种缺陷几乎总是在婴儿期或儿童期通过手术修复. At the time of open-heart surgery, 肺静脉重新连接到左心房，房间隔缺损被关闭. 儿童期手术后预后良好，晚期并发症少见. 极少数情况下，会发生肺动脉重连部位的肺静脉阻塞. Occasionally abnormal heart rhythms can also occur.
What if the defect is still present? Should it be repaired in adulthood?
It's rare when the TAPVC hasn't been repaired in childhood, but most adults in this category are able to have surgery.
Ongoing Care
What will I need in the future?
有TAPVC病史的患者应定期由具有成人先天性心脏病专业知识的心脏病专家进行检查，以寻找不常见的问题. The long-term outlook is excellent, 通常不需要药物，也不需要更多的手术或导尿管. 如果你正在接受任何类型的非心脏手术或侵入性手术，你也应该咨询一位在照顾患有先天性心脏病的成年人方面有专业知识的心脏病专家.
Medical Follow-up
如果需要的话，你的心脏病专家可以用无创检查来监测你. 这些包括心电图、动态心电图、运动压力测试和超声心动图. 它们将有助于显示是否需要进行更多的手术，比如心导管插入术. 只有在心律不正常的情况下才需要用药.
Activity Restrictions
大多数TAPVC修复的成年人将不需要限制他们的体力活动，以自己的耐力. 一些竞技运动如果有残留的肺静脉阻塞，可能会有更大的风险, or if the patient has heart rhythm problems. 你的心脏病专家会帮你确定合适的运动量.
Endocarditis Prevention
Not needed beyond six months after repair.
Pregnancy
由于TAPVC是一种罕见的缺陷，因此关于修复后患者妊娠的信息很少. Unless there is obstruction, or abnormal heart rhythms are poorly controlled, 如果孕前检查没有发现任何问题，大多数做过修复手术的女性应该能忍受怀孕.
Will you need more surgery?
一旦TAPVC被修复，就不太可能需要更多的手术. Rarely, 成人患者可能有梗阻，可能需要根据严重程度进行矫正.
