什么是房屋管道缺陷？
房室的管道缺陷或AV管是几个紧密相关的心脏问题的组合，导致心脏中心存在很大的缺陷。该疾病也是先天性的，也称为心室间隔缺陷或心内膜垫缺陷，这意味着它出生时存在，并且每10,000名新生儿中有两人发生。它通常与唐氏综合症.
When the heart is properly divided, blood from the lungs does not mix with blood from the body; however, with an AV canal, blood moves freely among the four heart chambers. If left untreated, AV canal can cause many problems involving the heart and lungs.
Like many congenital heart conditions, AV canal defect isn’t actually a single defect but rather a group of closely associated defects in various combinations and with varying degrees of severity:
- atrial septal defect
- ventricular septal defect
- abnormalities of the AV valves (mitral and tricuspid) that separate the upper heart chambers (atria) from the lower chambers (ventricles), often resulting in one large “common” valve rather than two separate valves
In addition, there are other associated heart defects such as专利导管动脉桥,transposition of the great arteries, unbalanced AV canal defects, and aortic arch hypoplasia.
房室管缺陷的症状是什么？
患有房屋管道缺陷（AV运河）的儿童通常会在生命的头几周到几个月内出现症状。这些症状可能包括：
- 喂食时不感兴趣或累人
- 体重增加不佳
- 疲劳
- sweating
- 苍白的肤色
- cool skin
- rapid breathing
- heavy breathing
- 快速心率
- 拥挤的呼吸
- blue color
如果您的孩子有这些症状中的任何一种，您的儿科医生可能会将您转介给儿科心脏病专家进行测试，诊断和治疗的确定。
导致房室管缺陷的原因是什么?
When the heart is forming during the first eight weeks of fetal development, it begins as a hollow tube. Over time, partitions that form within the tube eventually become the walls dividing the right side of the heart from the left.
当分区过程不完全发生时，会发生心房和心室间隔缺陷，并在心房和心室壁中留下开口。分离上层和下心腔的阀在这个八周结束时形成，并且通常无法正常发育。通常，有一个大型公共阀位于心脏的上和下腔之间，而不是两个单独的AV阀（三尖瓣和二尖瓣），使血液在瓣膜上方和下方的腔室之间自由流动 - 混合氧气 - 混合氧气- 富裕和贫血的血液。
Do genetics play a role in atrioventricular canal defect?
Genetics may play a role in the development of AV canal defect. Many children have associated genetic syndromes. About 15 to 20 percent of children with唐氏综合症也有AV运河缺陷。
How we care for atrioventricular canal defect
At Boston Children’s Hospital ourCardiac Surgery team治疗世界上一些最复杂的小儿心脏病，总体成功率超过98％，这是全国最高的大儿科心脏中心。特别是，在过去的二十年中，用于修复AV运河缺陷的方法已大大改善，并且该操作很有可能成功。
Our areas of innovation for atrioventricular canal defect
波士顿儿欧宝彩票平台童医院本森家庭心脏中心处理最复杂的AV运河,经常include children operated on at other centers. We are committed to constantly refining and improving our techniques and procedures.
Children with unbalanced AV canal, traditionally treated as single ventricle defect, are now being considered for biventricular repair. This is achieved using innovative techniques, developed in Boston Children’sComplex Biventricular Repair Program并在实验室中用3-D打印的心脏进行完善，以确保维修在一生中持久耐用。
心室管道缺陷|Diagnosis & Treatments
How is atrioventricular canal defect diagnosed?
If your newborn has any symptoms that suggest acongenital heart defect，您的儿科医生会将您转介给儿科心脏病专家。心脏病专家将进行体格检查，并听取心杂音. The location in the chest where the murmur is heard, as well as the sound and character of the murmur itself, will give the cardiologist an initial idea of what kind of heart problem your baby may have.
What tests are used to diagnosis atrioventricular canal defect?
Some of the following medical tests will be used to diagnose AV canal and its associated defects:
- 心电图(EKG or ECG) is usually the first test used to diagnose AV canal.
- 超声心动图is also commonly used for diagnosis.
- Cardiac magnetic resonance imaging（心脏MRI）有时在特殊情况下用于诊断AV管，也可以用于帮助计划手术。
- Cardiac catheterizationis only used in rare cases to diagnose AV canal.
如何治疗心室管道缺陷？
Specific treatments for atrioventricular canal defect (AV canal) depend on the extent of the disease — which can range from a single defect to a full combination of defects (complete). AV canal is almost always treated by surgical repair of the defects. Medications may be helpful and improve symptoms until the operation is performed.
Most children with complete AV canal undergo surgery by the age of 3 to 6 months. Children with partial and transitional AV canal undergo surgery later — 1 to 2 years old. Children with唐氏综合症可能比其他儿童更早出现症状，并且可能需要在更早的时候进行手术。
Treatments may include:
- 喂食时可能会疲倦并且可能无法饮食的婴儿的医疗管理，可以增加体重（来自更浓缩的母乳或配方的营养支持会使婴儿更多的卡路里或其他形式的营养助剂利尿剂，例如Lasix，例如Lasix，Lasix，Lasix，帮助肾脏从肺和身体中去除多余的液体。）
- ACE（血管紧张素转换酶）抑制剂，例如卡托普利或依那普利，可以帮助心脏的血液向前泵入体内地高辛并增强心肌，使其能够更有效地泵送
- 修复涉及以下组件的AV运河的手术：首先，创建两个独立的功能AV阀，一个针对心脏的每一侧；其次，要关闭各种间隔缺陷（心室和心房），并解决其他任何其他缺陷
- 3-D echocardiography to help optimize surgical treatment of the defect to help prolong durability of the valve repair
Care after AV canal surgery
After your baby's operation and hospital stay (usually five to seven days), he or she will need to be followed by a pediatric cardiologist, who will offer recommendations for post-operative follow-up care, including:
- wound care while your baby is healing
- 一个营养计划，以鼓励体重增加
- an oral hygiene program to prevent infection
- 适当的运动方案，以建立体重并实现健身
As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.
What is the long-term outlook for atrioventricular canal defect
Many children who've had an AV-canal repair will live healthy lives. Activity levels, appetite, and growth typically return to normal in most children. Some children will still have some degree of mitral- or tricuspid-valve abnormality or leakage after surgery, which may require another operation in the future. Children with AV canal will need lifelong monitoring (some will need medication), since they will always be at some risk for心律不齐，感染，心脏衰竭, or中风.
Your cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years, and even adulthood. Most people who’ve had congenital heart disease repair will have an ongoing relationship with their cardiologist. We will prevent and treat complications and will advise on daily life issues, such as activity levels, nutrition, and any precautions related to becoming pregnant.