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Atrioventricular Canal (AV Canal or AVC)房室通道(AV通道或AVC)What is atrioventricular canal?什么是房室通道?Atrioventricular canal is a congenital (present at birth) heart defect. As the fetus is growing, something occurs to affect heart development during the first 8 weeks of pregnancy, and certain areas of the heart do not form properly. AV canal is a heart problem that involves several abnormalities of structures inside the heart, including the following:房室通道是一种先天性(出生时就存在的)心脏病。在胎儿发育时,在妊娠的头8周内发生了什么事,干扰了心脏的发展,使心脏的某个区域没有正常的形成。AV通道是一种心脏病,它包括心内结构的几个异常,陈述如下:
Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped out to the body through the aorta.正常情况下,低氧血(蓝)从身体回到右心房,进入右心室,然后泵入肺,在这里它得到氧气。氧合血(红)从肺里返回到左心房,再进入左心室,然后泵出去通过主动脉到达全身。 An atrial septal defect allows oxygen-rich (red) blood to pass from the left atrium, through the opening in the septum, and then mix with oxygen-poor (blue) blood in the right atrium.房间隔缺损让来自左心房的氧合血(红)通过房间隔上的开口和右心房里的低氧血(蓝)混合。 A ventricular septal defect allows oxygen-rich (red) blood to pass from the left ventricle, through the opening in the septum, and then mix with oxygen-poor (blue) blood in the right ventricle.室间隔缺损让来自左心室的氧合血(红)通过室间隔上的开口和右心室里的低氧血(蓝)混合。
Abnormalities of the mitral or tricuspid valves allow blood that should be moving forward from the ventricle into either the pulmonary artery or the aorta to instead flow backward into the atria.二尖瓣或三尖瓣的异常让血流向后反流入心房,而这本来是应该从心室向前运动进入肺动脉和主动脉的。 Atrioventricular canal defects occur in about 5 percent of all congenital heart disease cases and are more common in infants with Down syndrome.房室通道大约占所有先天性心脏病病例的5%,在唐-综合征(先天性愚形)的婴儿较常见。 What causes atrioventricular canal?什么引起房室通道?The heart is forming during the first 8 weeks of fetal development. It begins as a hollow tube, then partitions within the tube develop that eventually become the septa (or walls) dividing the right side of the heart from the left. Atrial and ventricular septal defects occur when the partitioning process does not occur completely, leaving openings in the atrial and ventricular septum. The valves that separate the upper and lower heart chambers are being formed in the latter portion of this eight-week period, and, for some reason, they do not develop properly, either.心脏形成于胚胎发育的头8周内。开始它象一个中空管,然后在管内中隔开始发育,最终成为间隔,把其分为左右两个部分。当这个分割过程不完全时就会在房和室间隔上留下缺口,产生房和室间隔缺损。在这8周的后期,瓣膜开始形成,它把心腔的上部和下部分开,由于某种原因,它们也没有发育完全。 There may be genetic influences on the development of atrioventricular canal. Consider the following statistics:参考以下的统计学结果,遗传因素可能影响房室管的发育。
Other chromosome abnormalities (in addition to Down syndrome) are linked to the development of atrioventricular canal. Maternal age can have an effect on the prevalence of AV canal, which may be related to the increased probability of a woman giving birth to a child with Down syndrome as she gets older.其他的染色体异常(除唐-综合症之外)与房室通道的发生有联系。母亲的年龄对房室通道的流行有影响,可能与高龄母亲生的孩子患唐-综合症的可能性增加有关。 Why is atrioventricular canal a concern?为什么房室通道受到关注?If not treated, this heart defect can cause lung disease. When blood passes through both the ASD and VSD from the left side of the heart to the right side, then a larger volume of blood than normal must be handled by the right side of the heart. This extra blood then passes through the pulmonary artery into the lungs, causing higher pressure than normal in the blood vessels in the lungs.如果不治疗,这种心脏病可以引起肺的病变。当血流从心脏的左侧通过房间隔缺损和室间隔缺损到右侧时,比正常多的大量的血必然加重了右心的负担,造成在肺血管里比正常高的压力。 The larger the volume of blood that goes to the lungs, the higher the pressure. The lungs are able to cope with this extra pressure for a while, depending on exactly how high the pressure is. After a while, however, the blood vessels in the lungs become damaged by the extra pressure.越大量的血涌入肺会造成越高的压力,最初肺还可以应付这额外的压力,然而,时间长了,这额外的压力对肺内血管造成损伤。 As pressure builds up in the lungs, the flow of blood from the left side of the heart to the right side and on to the lungs will diminish. This helps preserve the function of the lungs, but causes yet another problem. Blood flow within the heart goes from areas where the pressure is high to areas where the pressure is low. If the septal defects are not repaired, and lung disease begins to occur, pressure in the right side of the heart will eventually exceed pressure in the left. In this instance, it will be easier for oxygen-poor (blue) blood to flow from the right side of the heart, through the ASD and VSD, into the left ventricle, and on to the body. When this happens, the body does not receive enough oxygen in the bloodstream to meet its needs, and children may become cyanotic, or exhibit a blue coloring in their skin, lips, and nailbeds.随着肺内压力的升高,左向右到肺的血流会减少。这对保护肺的功能有帮助,但也带来了另外的问题。心内的血液总是从高压的区域流向低压的区域。如果没有修补间隔的缺损,肺的病变开始发生,右侧的压力最终将会超过左侧的压力。这时,右心的低氧血(蓝)就容易通过房缺和室缺分流到左心,然后到全身。当这发生时,身体得不到血液中足够的氧来满足自身的需要,患儿就会变得紫绀,皮肤、口唇、和甲床呈现蓝色。 Because blood is pumped at high pressure through the septal openings, the lining of the right and left ventricles will become irritated and inflamed. Bacteria in the bloodstream can easily infect this injured area, causing a serious illness known as bacterial endocarditis.由于血液是在高压下泵出通过间隔的缺损,左右心室的内膜就会变得敏感和充血。血流中的细菌就容易感染这受伤的区域,引起细菌性心内膜炎这样严重的病。 What are the symptoms of an atrioventricular canal defect?什么是房室通道的症状?The size of the septal openings will affect the type of symptoms noted, the severity of symptoms, and the age at which they first occur. The larger the openings, the greater the amount of blood that passes through from the left side of the heart to the right and overloads the right heart and the lungs.间隔缺损的大小将影响到症状的类型,严重程度,和最初发生的年龄。缺损越大,越大量的血从左向右分流并且使右心和肺超负荷。 Symptoms occur in infancy. The following are the most common symptoms of AVC.症状发生在婴儿期。以下是AVC最常见的症状。 However, each child may experience symptoms differently. Symptoms may include:每一个患儿可能有不同的症状,包括:
As the pressure in the lungs rises, blood within the heart will eventually "shunt" through the septal openings from right heart to the left. This allows oxygen-poor (blue) blood to reach the body, and cyanosis will be noted. Cyanosis gives a blue color to the lips, nailbeds, and skin. The symptoms of AVC may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.随着肺内压力的增高,最终,心内的分流成为右向左分流,使得低氧(蓝)血到达全身,并且出现紫绀。自感时的口唇、甲床、和皮肤发蓝。AVC的这些症状可能与其他的疾病或心脏问题相似。要请你的孩子的医生明确诊断。 How is AV canal diagnosed?房室通道是怎样诊断的?Your child's pediatrician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the opening from the left side of the heart to the right. Symptoms your child exhibits will also help with the diagnosis.在查体时你的孩子的儿科医生可能听到心脏的杂音,而让你的孩子去看一位儿童心脏专家以明确诊断。心脏的杂音是由于血流从左心向右通过缺损口时产生的湍流而引起,你的孩子出现的症状亦有助于作出诊断。 A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. However, other tests are needed to help with the diagnosis, and may include the following:一位儿童心脏病专家专门研究先天性心脏病的诊断和医疗方法,和随后心脏问题在儿童期可能的发展。心脏专家将进行体检,听诊你的孩子的心和肺,作一些有助于诊断的观测。在胸部杂音听得最清楚的位置,以及杂音的响度和性质(粗糙的,吹风样的,等等)对于你的孩子可能患有什么样的心脏问题,将给心脏专家一个初步的意向。然而,有助于诊断的其他的一些检查也是需要的,可能包括以下的检查:
Treatment for atrioventricular canal:房室通道的治疗:Specific treatment for atrioventricular canal will be determined by your child's physician based on:的特殊治疗将由你孩子的医生根据以下几点作出决定:
AV canal is treated by surgical repair of the defects. However, medical support (i.e., medications) may be necessary until the operation is performed. Treatment may include:房室通道是通过外科手术修补缺损来治疗。然而,直到手术时药物治疗可能是必要的。治疗可能包括:
The operative methods used to repair atrioventricular canal have improved greatly in the past decade, and the operation has a high likelihood of success. Most children undergo surgery by the age of 6 months. Children with Down syndrome may develop lung problems earlier than other children, and may need to have surgical repair at an earlier age.修补房室通道的手术方法在过去的十年里有很大的改进,使手术的成功有高的可能性。多数孩子在6个月时经受手术。患唐-综合征的孩子可能比其他孩子早的发生肺的问题,和可能需要在较小的年龄接受手术修补。 The operation is performed under general anesthesia.手术要在全身麻醉下进行。 The atrial and ventricular septal defects are often closed with a pericardial patch made from the membrane that covers the outside surface of the heart, or a patch made from synthetic material. The valve repair technique will differ according to the abnormality and the surgeon's preferences.房和室间隔缺损经常用心包片修补,或者是合成材料做的补片。根据瓣膜的畸形和外科医生的偏爱瓣膜的修复技术将有所不同。 Postoperative care for your child:你孩子的术后护理Children will spend time in the intensive care unit (ICU) after an AV canal repair. During the first several hours after surgery, your child will be very drowsy from the anesthesia that was used during the operation, and from medications given to relax him/her and to help with pain. To help your child rest, these medications might be continued overnight or longer. As time goes by, your child will become more alert.在房室通道修补术后患儿将在监护室(ICU)待些时间,在术后最初几个小时里由于手术中用的麻醉药,肌松药和镇痛药的作用,你的孩子将在昏睡中。为了帮助你孩子休息,可能继续应用这些药物过夜或更长的时间。随着时间的推移,你孩子将逐渐的清醒。 While your child is in the ICU, special equipment will be used to help him/her recover, and may include the following:当你的孩子在ICU里时,某些特殊的设备将用来帮助他/她康复,包括以下的:
Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.当你的小孩在ICU里时可能需要这里没有提到的设备提供支持。医院的职员将向你说明所有的这些必需的设备。 Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.使用几种不同的药物治疗,将使你的孩子尽可能的保持舒适;有的止痛,有的解除焦虑。职员将会告诉你怎样最好的使你的孩子安静和舒适。 After discharged from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while at home, and these will be explained to you. The staff will give you written instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.从ICU转出之后,在回家之前你的孩子要在医院的另一个单元里恢复几天。在你的孩子出院之前,你将要学习在家里怎样照顾你的孩子。你的孩子可能需要服药一段时间,并且这些将向你解释。在你的孩子出院之前,职员将给你关于药物治疗,活动限制,和随访约定的指导。 Caring for your child at home following AV canal repair:房室通道手术后在家里护理你的孩子Most infants and older children feel fairly comfortable when they go home. Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child's physician will discuss pain control before your child is discharged from the hospital.大多数婴儿和大一些的孩子在回家时感觉相当不错。止痛药比如醋氨酚或布洛芬,可以用来保持你孩子舒服。在出院前你孩子的医生会和你讨论控制疼痛的方法。 Often, infants who fed poorly prior to surgery have more energy after the recuperation period, and begin to eat better and gain weight faster. However, high-calorie formulas may be needed for several weeks or months after surgery to help your child catch up growth-wise. Tube feedings may also be helpful until your child is able to feed better.经常的,有些术前很难喂的婴儿在恢复期后有了较大的活力,开始吃得好一些和较快的增加体重。然而,在手术后高卡路里的配方可能还需要给数周或数月以帮助你孩子的生长发育赶上正常。胃管饲养可能还会有帮助,直到你的孩子吃得好为止。 After surgery, older children usually have a fair tolerance for activity. Your child may become tired easily, and sleep more right after surgery, but, within a few weeks, your child may be fully recovered.手术后,大一些的孩子通常活动能力有改善。你的孩子可能容易疲劳,睡得好一些,但是,在数周内,你孩子可能完全痊愈了。 You may receive additional instructions from your child's physicians and the hospital staff.你可能从你孩子的医生和医院的职员那里得到进一步的指导。 Long-term outlook after AV canal surgical repair:房室通道手术后的长期效果Many children who have had an AV canal defect repair will live healthy lives. Activity levels, appetite, and growth will eventually return to normal in most children. Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis for a specific time period after discharge from the hospital.许多孩子在接受房室通道手术后将过上健康的生活,活动能力,食欲,和生长发育最终恢复正常。在出院后的一段时间内你孩子的心脏专家将给于抗生素预防细菌性心内膜炎。 Some children will still have some degree of mitral or tricuspid valve abnormality after AV canal repair surgery. This may require another operation in the future to repair the leaky valve(s).有些患儿在房室通道手术后,仍然有一定程度的二尖瓣或三尖瓣异常,这可能需要将来再次手术修复漏的瓣膜。 Children with Down syndrome will benefit from special programs that enhance their mental and physical development. Your child's physician can assist you in locating such programs in your community.伴有唐-综合征的患儿将从一种特殊的促进他们的智力和体力发展的教育项目获益。你孩子的医生能够帮助你找到离你们最近的这种项目。 Consult your child's physician regarding the specific outlook for your child.教请你孩子的医生关于你孩子的前景。
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