肺动脉闭锁

诊断

肺动脉闭锁伴室间隔缺损（VSD）常常在出生时或出生后不久诊断出来。可能用于诊断肺动脉闭锁伴 VSD 的检查包括：

• 脉搏血氧测定法。使用连在手部或足部的小传感器测量血液中的氧含量。脉搏血氧测定法简单、无痛。
• 胸部 X 线检查。胸部 X 线检查可显示心脏和肺的形状及大小。
• 超声心动图。该检查利用声波生成心脏跳动的图像。分娩前在母亲腹部进行的超声心动图被称为胎儿超声心动图。可用于诊断肺动脉闭锁。
• 心电图（ECG 或 EKG）。这项快速、简单的检查可显示心脏的跳动情况。医生会将带有传感器的黏性贴片（电极）贴在胸部，有时也会贴在臂部和腿部。传感器通过导线连接到一台计算机，用以打印或显示结果。ECG 可帮助诊断心律不齐。
• 心脏导管插入术。医生将一根软管（即导管）插入血管，通常是腹股沟或手腕处的血管。然后引导至心脏。造影剂通过导管流至心脏动脉。使用造影剂有助于在图像上更清楚地显示动脉。
• 对心脏的 CT 扫描也称心脏 CT 扫描。该检查使用一系列 X 线来生成心脏和血管的图片。这项检查可显示心脏和肺的形状。心脏 CT 扫描可帮助诊断粗大体肺侧支动脉（MAPCA）。了解 MAPCA 对于规划治疗非常重要。

Tests

Tests that may be used to diagnose pulmonary atresia with ventricular septal defect include:

• Pulse oximetry. For this simple test, a small sensor clips onto a hand or foot. It checks the amount of oxygen in the blood.
• Chest X-ray. A chest X-ray shows the shape and size of the heart and lungs.
• Echocardiogram. This is an ultrasound of the heart. Sound waves make images of the beating heart. An echocardiogram of a baby's heart during pregnancy is called a fetal echocardiogram. It can diagnose pulmonary atresia.
• Electrocardiogram, also called an ECG or EKG. This quick test shows how the heart is beating. Patches with sensors, called electrodes, stick to the chest and sometimes to the arms or legs. Wires connect the patches to a computer, which prints or displays results. An ECG can find irregular heartbeats.
• Cardiac catheterization. A doctor places a flexible tube called a catheter in a blood vessel, usually in the groin or wrist. It's guided to the heart. Dye flows through the catheter to heart arteries. The dye helps the arteries show up more clearly on images.
• Heart CT scan, also called a cardiac CT scan. This test uses several X-rays to make pictures of the heart and blood vessels. It shows the shape of the heart and lungs. A cardiac CT can help diagnose major aortopulmonary collateral arteries, also called MAPCAs. Knowing about the MAPCAs is important for planning treatment.

治疗

患有肺动脉闭锁伴室间隔缺损（VSD）的婴儿需要立即接受治疗。治疗方法可能包括药物和一项或多项手术或医疗程序。

手术或其他医疗程序

患有肺动脉闭锁伴 VSD 的婴儿需要接受一项或多项手术或医疗程序来改善血流并帮助心脏更好地工作。治疗取决于肺动脉的结构以及是否有粗大体肺侧支动脉（MAPCA）。

治疗肺动脉闭锁伴室间隔缺损的手术或其他医疗程序可能包括：

• 肺动脉分支导管术。这种治疗方法是为了观察婴儿的心脏。医生有时会用这种方法来重建肺动脉的分支血管。导管末端的球囊可用于拓宽任何狭窄部位。可能在一条称为动脉导管的血管中置入一根硬管（支架）。这条血管位于人体的主动脉与肺动脉之间。支架能使其保持开放，使血液流向肺部。
• 体-肺动脉分流术。婴儿可能需要在出生后的最初几天内接受这项手术，以增加流向肺部的血流量。手术使用一根小的合成管建立供血液流动的连接，称为分流术。布莱洛克-陶西格分流术（BT 分流术）就是一个例子。
• 新生儿完全修复。如果婴儿心脏中的肺动脉发育良好且没有 MAPCA，外科医生可能在婴儿出生后 4 周内进行一次性完全修复。在新生儿完全修复术中，医生会闭合心脏上的孔洞，并在心脏右下腔室与肺动脉之间置入一根带有人工瓣膜的管道。
• 一期完全修复。这种治疗方法又名单源化手术，把所有 MAPCA 连在一起，构成一条新的肺动脉。然后，外科医生将心脏上的孔洞闭合。使用管状移植物（带或不带瓣膜）在心脏右下腔室与肺动脉之间建立一条通路。这种治疗通常在孩子 4 到 6 个月大时进行。
• 分期单源化。如果 MAPCA 很小或有许多变窄区域，则可以分期进行连接它们的手术。这能让动脉在完全修复之前持续生长。通过从主动脉到新建肺动脉之间的小分流能让血液流向肺部。几个月后进行心脏成像检查，确定婴儿是否已具备接受完全修复的条件。

患有肺动脉闭锁伴室间隔缺损（VSD）的婴儿需要由接受过先天性心脏状况治疗培训的医生定期进行检查。

Surgeries or other procedures

A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs.

Surgeries or other procedures may include:

• Catheter procedure. Thin, flexible tubes called catheters are used to look at the baby's heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs.
• Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt.
• Complete repair. This treatment may be done when as a baby is young or after a shunt or stent is placed. During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery.
• Unifocalization. Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it's the first step toward an eventual complete repair.

Babies with pulmonary atresia with ventricular septal defect need regular checkups by a doctor trained in heart conditions present at birth. This type of doctor is called a congenital cardiologist.

临床试验

探索 Mayo Clinic 的研究 测试新的治疗、干预与检查方法，旨在预防、检测、治疗或控制这种疾病。

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