儿童先天性心脏缺陷

完全型肺静脉回流异常（TAPVR）

完全型肺静脉回流异常（TAPVR）是一种出生时即存在的罕见心脏问题。也就是说，它属于先天性心脏缺陷。

它还有其他名称：

• 完全型肺静脉连接异常
• TAPVC

在这种心脏疾病中，肺部血管（称为肺静脉）连接至心脏中错误的部位。

正常心脏中的富氧血液从肺部流向左上心腔（即左心房），然后流经全身。

罹患 TAPVR 时，静脉连接会发生改变。血液流经右上心腔，即右心房。这种血流变化导致贫氧血与富氧血混合。结果，流经全身的血液没有足够的氧气。

TAPVR 的具体类型取决于静脉连接的位置。大多数先天性 TAPVR 患儿没有先天性心脏病家族史。

症状

患有 TAPVR 的婴儿可能出现呼吸困难。低氧水平会导致婴儿皮肤发灰或发蓝。这称为发绀。

医务人员可能会在婴儿刚出生时就注意到 TAPVR 的症状。但有些孩子要等到后来才会出现症状。

病因

Most congenital heart defects are caused by changes that happen early as the unborn baby's heart is developing before birth. An unborn baby is also called a fetus. The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known.

Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role.

风险因素

Possible risk factors for congenital heart defects, including total anomalous pulmonary venous return (TAPVR), may include:

• Rubella, also called German measles. Having rubella during pregnancy can change how the baby's heart develops. A blood test can be done before pregnancy to see if you're immune to rubella. If you're not, you can get a vaccine.
• Diabetes. Having type 1 or type 2 diabetes during pregnancy may change how the baby's heart grows during pregnancy. Diabetes that develops during pregnancy is called gestational diabetes. Gestational diabetes generally doesn't increase the risk of congenital heart disease.
• Genetics. Although TAPVR doesn't usually run in families, changes in genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions.
• Smoking. If you smoke, quit. Smoking during pregnancy or exposure to secondhand smoke increases the risk of congenital heart defects in the baby.
• Alcohol use. Drinking alcohol during pregnancy has been linked to heart conditions in the baby.
• Some medicines. Some medicines taken during pregnancy may increase the risk of congenital heart defects. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk to your healthcare team about the medicines you take.

诊断

孩子的医务人员会进行体格检查，并用听诊器听诊孩子的心脏。可能会听到嗖嗖声（心脏杂音）。

超声心动图可用于诊断完全型肺静脉回流异常。这项检查使用声波来生成心脏运动的图像。超声心动图可以显示肺静脉、任何心脏穿孔和心脏腔室的尺寸。该检查还可显示血液如何流经心脏及心脏瓣膜。

如需更多信息，可能会进行如心电图、胸部 X 线检查或 CT 扫描等其他检查。

治疗

TAPVR 外科手术通常需要在孩子还在婴儿时期时进行。手术时间取决于是否有阻塞。为修复心脏，外科医生会把肺静脉与左上心腔连接，并闭合上心腔之间的孔洞。

完全型肺静脉回流异常患者终生都需要定期进行健康检查，以检查是否存在感染、阻塞或心律问题。接受过先天性心脏病培训的医生会对患者进行护理。这类医务人员称为先天性心脏科医生。

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