肺动脉闭锁

概述

肺动脉闭锁伴室间隔缺损（VSD）是一种出生时即存在的心脏问题。也就是说，这是一种先天性心脏缺陷。

这类肺动脉闭锁患者心肺之间的瓣膜没有完全形成。这个瓣膜称为肺动脉瓣。血液无法从心脏右下腔室（即右心室）流至肺部。肺动脉闭锁伴室间隔缺损（VSD）患者心脏的两个泵血腔室之间还存在一个孔洞。

VSD 使血液能够流入并流出心脏右下腔室。部分血液还可能流经一个名为动脉导管的自然开口。动脉导管通常在出生后不久闭合。但药物可以使其保持开放。

肺动脉闭锁患儿的肺动脉及其分支可能非常小甚至不存在。如果这些血管缺失，体内的大动脉（主动脉）上会形成其他动脉。由这些动脉帮助向肺部供血。这类动脉称为粗大体肺侧支动脉（MAPCA）。

肺动脉闭锁伴室间隔缺损是一种危及生命的状况。肺动脉闭锁患儿无法获取足够的氧气。需要接受药物和一项或多项医疗程序或手术来改善血流并修复心脏。

Types

症状

Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include:

• Blue or gray skin. This change may be harder or easier to see depending on skin color.
• Fast breathing or shortness of breath.
• Tiredness.
• Poor feeding.

When to see a doctor

Pulmonary atresia with ventricular septal defect, also called PA-VSD, is typically found during pregnancy or soon after birth. If your baby has symptoms of this condition after birth, call a healthcare professional right away.

病因

The cause of pulmonary atresia with ventricular septal defect, also called PA-VSD, is not clear. Most congenital heart conditions happen during the first six weeks of pregnancy. The major blood vessels that run to and from the heart also begin to grow at this time. This is when a congenital heart defect such as pulmonary atresia may occur.

In PA-VSD, the pulmonary valve isn't fully formed. There also is a hole in the heart called a ventricular septal defect. The hole lets blood flow into and out of the right lower heart chamber. Some blood also may flow through a natural opening called the ductus arteriosus. The ductus arteriosus usually closes soon after birth. But medicines can keep it open.

In babies with pulmonary atresia, the lung arteries can be very small. Or they may be missing. If the blood vessels are missing, other vessels form on the body's main artery, called the aorta. These new vessels help send blood to the lungs. They are called major aortopulmonary collateral arteries, also called MAPCAs.

风险因素

It's not clear what increases the risk of pulmonary atresia with ventricular septal defect. Possible risk factors for congenital heart conditions in general may include:

• 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.
• Some medicines. Some medicines taken during pregnancy may increase the risk of congenital heart conditions. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk with your healthcare team about the medicines you take.
• Genetics. Changes in some genes may affect how a baby's heart forms. For example, people with Down syndrome are often born with heart conditions.
• Diabetes. Having type 1 or type 2 diabetes during pregnancy may change how a baby's heart forms. Diabetes that develops during pregnancy is called gestational diabetes. It typically doesn't increase a baby's risk of congenital heart conditions.
• Rubella, also called German measles. Having rubella during pregnancy can change how a baby's heart forms. A blood test can be done before pregnancy to see if you're immune to rubella. If you're not, a vaccine is available.

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