成人先天性心脏病

部分型肺静脉回流异常

部分型肺静脉回流异常是一种出生时即存在的罕见心脏问题。也就是说，它是一种先天性的心脏缺陷。

它还有其他名称：

• PAPVR。
• 部分肺静脉连接异常。
• PAPVC。

在这种状况中，肺部的一些血管附着在心脏上错误的位置。这些血管称为肺静脉。

在正常心脏中，富含氧气的血液从肺部流向左上心腔（左心房），然后流经全身。

在 PAPVR 中，来自肺部的血液流向右上心腔（右心房），从而会有多余的血液流到心脏右侧。这可能导致右心腔肿胀。

部分 PAPVR 患者的上心腔之间还有一个孔，称为房间隔缺损。这个小孔使血液流入上心腔之间，从而可能导致其他心脏问题。先天患有特纳综合征的婴儿有更高风险罹患 PAPVR。

症状

症状取决于是否有其他心脏问题。PAPVR 的常见症状是呼吸困难。

病因

The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. Most congenital heart defects happen as the unborn baby's heart is forming before birth. An unborn baby also is called a fetus.

Changes in the genes, some medicines or health conditions, and environmental factors may play a role. Lifestyle choices, such as smoking during pregnancy, also may increase the risk of congenital heart defects in the baby.

风险因素

What increases the risk of PAPVR is not well known. Possible risk factors for congenital heart defects may include:

• Diabetes. Having type 1 or type 2 diabetes during pregnancy may change how the unborn baby's heart forms. Diabetes that develops during pregnancy is called gestational diabetes. It generally doesn't increase a baby's risk of congenital heart defects.
• Genetics. Changes in some genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. A child born with Turner syndrome also has an increased risk of PAPVR.
• Rubella, also called German measles. Having rubella during pregnancy can cause changes in an unborn baby's heart. 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.
• Some medicines. Some medicines taken during pregnancy may increase the risk of some congenital heart conditions. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk with a healthcare professional about the medicines you take.
• Smoking. If you smoke, quit. Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions.
• Alcohol use. Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby.

诊断

如果 PAPVR 伴随其他心脏问题出现，则可在出生后不久诊断出。如果病情较轻，可能要到成年才能诊断出。

医务人员会进行体格检查，并用听诊器听诊心脏。可能会听到嗖嗖声（心脏杂音）。

部分型肺静脉回流异常使用超声心动图来诊断。这项检查利用声波生成心脏跳动的图像，可显示肺静脉和心室的大小，还可测量血流速度，帮助诊断心脏穿孔。

如果需要更多信息，可能会进行如心电图（ECG 或 EKG）、胸部 X 线检查或 CT 扫描等其他检查。

治疗

如果存在以下情况，可能需要手术修复心脏：

• 心脏中有大量富氧血和贫氧血混合。
• 疾病导致多处肺部感染。

如果您没有症状，可能不需要手术。如果您因另一种心脏疾病需要手术，外科医生可能会在施行手术期间一并修复 PAPVR。

PAPVR 修复手术期间，心外科医生会：

• 将肺静脉重新连接到左上心腔。
• 闭合心脏穿孔。

部分型肺静脉回流异常患者终生都需要定期体检，以确定是否存在并发症。最好是看在先天性心脏病方面接受过专业训练的医疗服务提供者。此类医务人员称为先天性心脏病科医生。

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