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Coarctation of the Aorta  主动脉缩窄

Anatomy of the heart, normal

click image to enlarge

What is coarctation of the aorta?

什么是主动脉缩窄?

Coarctation of the aorta is a congenital (present at birth) heart defect involving a narrowing of the aorta. The aorta is the large artery that carries oxygen-rich (red) blood from the left ventricle to the body. It is shaped like a candy cane, with the first section moving up towards the head (ascending aorta), then curving in a C-shape as smaller arteries that are attached to it carry blood to the head and arms (aortic arch). After the curve, the aorta becomes straight again, and moves downward towards the abdomen, carrying blood to the lower part of the body (descending aorta).

主动脉缩窄是一种先天性(出生时就有的)心脏病。主动脉是把富含氧的(红的)血从左心室输送到全身的大动脉。它的形状像一个糖果手杖。第一部分向上朝向头部(升主动脉),然后弯曲成一个C型,供应头部和上肢的小动脉连接与此(主动脉弓),拐弯之后主动脉又变成直的,向下朝向腹部,输送血液到身体的下部(降主动脉)

The narrowed segment called coarctation can occur anywhere in the aorta, but is most likely to happen in the segment just after the aortic arch. This narrowing restricts the amount of oxygen-rich (red) blood that can travel to the lower part of the body. Varying degrees of narrowing can occur.

狭窄的部分称为缩窄,可发生在主动脉的任何地方,但是最容易发生在紧接主动脉弓后的部分。这个狭窄限制了可以输送到身体下部的富含氧的(红的)血量。不同程度的狭窄可能发生。

Anatomy of a heart with a coarctation of the aorta

click image to enlarge

The more severe the narrowing, the more symptomatic a child will be, and the earlier the problem will be noticed. In some cases, coarctation is noted in infancy. In others, however, it may not be noted until school-age or adolescence.

狭窄越是严重,患儿将有更显著症状,并且会较早的发现问题,有些病例,在婴儿期就发现有缩窄。然而,其他一些病例直到学龄或青春期可能还没有发现问题。

Seventy-five percent of children with coarctation of the aorta also have a bicuspid aortic valve - a valve that has two leaflets instead of the usual three.

75%的患有主动脉缩窄的儿童同时患有主动脉瓣二叶畸形-主动脉瓣只有两个瓣叶代替了通常的三个瓣叶。

 

Coarctation of the aorta occurs in about 6 to 8 percent of all children with congenital heart disease. Boys have the defect twice as often as girls do.

主动脉缩窄的发生率为所有先天性心脏病患儿的6-8%。男孩儿是女孩儿的两倍。

What causes coarctation of the aorta?

什么引起了主动脉缩窄?

Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most of the time this heart defect occurs sporadically (by chance), with no clear reason for its development.

有些先天性心脏病患儿可能带有遗传因素,可以由基因缺陷,染色体异常引起,或环境暴露,使得一些家庭比较多的发生心脏问题。绝大多数这种心脏病是偶发的(偶然的),没有明确的原因。

Why is coarctation a concern?

为什么关注缩窄

Coarctation of the aorta causes several problems, including the following:

主动脉缩窄会因起下述的严重问题:

  • The left ventricle has to work harder to try to move blood through the narrowing in the aorta. Eventually, the left ventricle is no longer able to handle the extra workload, and it fails to pump blood to the body efficiently.
  • 左心室不得不努力工作以推动血液通过主动脉中的狭窄段。最终,左心室承担不了额外的工作负荷,不能有效地把血液有效地输送到全身。
  • Blood pressure is higher above the narrowing, and lower below the narrowing. Older children may have headaches from too much pressure in the vessels in the head, or cramps in the legs or abdomen from too little blood flow in that region. Also, the kidneys may not make enough urine since they require a certain amount of blood flow and a certain blood pressure to perform this task.
  • 血压在狭窄近端升高,在狭窄远端降低。较大的患儿,由于头部的血管受到过高的压力可能会头痛,或者由于过少的血液供应下肢或腹部可能会抽筋。还可能会少尿,因为肾脏需要一定的血流和血压来进行它的工作。
  • The walls of the ascending aorta, the aortic arch, or any of the arteries in the head and arms may become weakened by high pressure. Spontaneous tears in any of these arteries can occur, which can cause a stroke or uncontrollable bleeding.
  • 升主动脉、主动脉弓、头部和上肢的任何动脉壁在高压下可能会变得薄弱。这些动脉可能会发生自发性破裂,导致休克或无法控制的出血。
  • There is a higher than average chance of developing an infection in the lining of the heart or aorta known as bacterial endocarditis.
  • 较一般人有更多的发生细菌性心内膜炎的机会。
  • The coronary arteries, which supply oxygen-rich (red) blood to the heart muscle, may narrow in response to elevated pressure. 
  • 供应富含氧的(红的)血到心肌的冠状动脉对升高的压力的反应可能会变狭窄。

What are the symptoms of coarctation of the aorta?

主动脉缩窄有什么症状?

Symptoms noted in early infancy are caused by moderate to severe aortic narrowing. The following are the most common symptoms of coarctation of the aorta. However, each child may experience symptoms differently. Symptoms may include:

  • irritability
  • pale skin
  • sweating
  • heavy and/or rapid breathing
  • poor feeding
  • poor weight gain

中至重度的狭窄在婴儿早期即会引起症状,以下是最常见的主动脉缩窄的症状,每一个患儿可能有不同的症状,包括:

易激惹

皮肤苍白

多汗

沉重的和/或快速的呼吸

难喂养

体重增长缓慢

Mild narrowing may not cause symptoms at all. Often, a school-aged child or adolescent is simply noted to have high blood pressure or a heart murmur on a physical examination. Some may complain of headaches or cramps in the lower sections of the body.

较轻的狭窄可能没有症状。常是,学龄儿童或青少年仅在查体时发现高血压或心脏杂音,有的可能会诉说头痛或身体下部抽筋。

The symptoms of coarctation of the aorta may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

主动脉缩窄的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 coarctation of the aorta diagnosed?

怎样作出主动脉缩窄的诊断?

Your child's physician 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 obstruction from the right ventricle to the pulmonary artery. Symptoms your child exhibits will also help with the diagnosis.

在查体时你的孩子的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通过狭窄的部分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 your child's 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:

  • chest x-ray- diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
  • 胸部X线检查:用一种看不见的电磁能量束投射在胶片上产生内部组织,骨骼,和脏器的影像的诊断性的检查。
  • electrocardiogram (ECG or EKG)- a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.
    心电图(ECG或EKG):一种记录心脏电活动的测试,可以显示不正常的心律(arrhythmias或dysrhythmias),还可以发现心肌损害。
  • echocardiogram (echo)- a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
    超声心动图(echo):一种通过把超声波记录在电子传感器上再转换成心脏和心脏瓣膜的活动影像来评价心脏的结构和功能的方法。
  • magnetic resonance imaging (MRI)- a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
  • 磁共振成像:一种使用把大磁铁,射频,和计算机结合在一起的设备产生出体内器官和结构的详细影像的诊断方法。

Treatment for coarctation of the aorta: 

主动脉缩窄的治疗:

Specific treatment for coarctation of the aorta will be determined by your child's physician based on:

主动脉缩窄的特效治疗将由你孩子的医生基于以下几点作出决定:

  • your child's age, overall health, and medical history
  • extent of the disease
  • your child's tolerance for specific medications, procedures, or therapies
  • expectations for the course of the defect
  • your opinion or preference
  • 你孩子的年龄,全面的健康情况,和病史。
  • 病的程度。
  • 你的孩子对于特殊的药物,手术,或其他治疗的耐受性。
  • 疾病过程的预后。
  • 你的意见或选择。

Coarctation of the aorta is treated with repair of the narrowed vessel. Several options are currently available.

主动脉缩窄是用修复狭窄血管的方法来治疗的。目前有几种方法可以选择。

Your child's coarctation of the aorta may be repaired surgically in an operating room or by a cardiac catheterization procedure. The surgical repair is performed under general anesthesia. The narrowed area is either surgically removed, or made larger with the help of surrounding structures or a patch.

你孩子的主动脉缩窄可以在手术室里用外科修复的办法或者心导管介入的方法进行治疗。外科修复是在全身麻醉下进行。狭窄的部分或是手术切除,或是利用周围的结构或补片加宽。

The cardiac catheterization procedure may also be an option for treatment. During the procedure, the child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist will pass an inflated balloon through the narrowed section of the aorta to stretch the area open. A small device, called a stent, may also be placed in the narrowed area after the balloon dilation to keep the aorta open.

心导管介入治疗可以是另一个选择。使患儿镇静,一条小的、细的、柔软的导管在腹股沟处插入血管并且引导进入心脏。一旦导管进入心脏,心脏专家就把一个球囊导管通过主动脉的狭窄段,膨胀球囊使此处加宽扩大。一个小的装置,称为支架,在球囊扩张之后可能被置入在此处以保持主动脉宽敞。

Some infants will be very sick, requiring care in the intensive care unit (ICU) prior to the procedure, and could possibly even need emergency repair of the coarctation. Others, who are exhibiting few symptoms, will have the repair scheduled on a less urgent basis.

有的婴儿可能非常虚弱,治疗前需要在监护室(ICU)护理,而且甚至可能需要解除缩窄的紧急治疗,其他表现出少数症状的患儿,在不那么紧急的情况下,将会预先安排修复的日程。

After surgery, infants will return to the intensive care unit (ICU) to be closely monitored during recovery.

手术后,婴幼儿将返回监护室(ICU)在恢复期得到密切的监护。

While your child is in the ICU, special equipment will be used to help him/her recover, and may include the following:

当你的小孩在监护室时,以下的特殊的设备将可能用来帮助他/她康复。

  • ventilator - a machine that helps your child breathe while he/she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he/she is too sleepy to breathe effectively on his/her own. Many children remain on the ventilator for a while after surgery so they can rest.
  • 呼吸机:一个当你孩子在麻醉下手术时帮助他/她呼吸的机器。一个细小的塑料管插入气管并与呼吸机连接,当你的孩子沉睡不能自己有效地呼吸时,它给你的小孩通气。手术后许多患儿继续使用呼吸机一段时间,如此他们可以得到休息。
  • intravenous (IV) catheters - small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medicines that help your child recover from the operation.
  • 静脉内(IV)导管:一条细小的塑料管经过皮肤插入血管,输注你的孩子手术后康复所需要的静脉液体和重要的药物。
  • arterial line - a specialized IV placed in the wrist, or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU.
  • 动脉管:一个专门的动脉内小插管,安置在腕部或身体其他可以触到脉搏的地方,当你的小孩在手术期间或在ICU里时用来连续测量血压的。
  • nasogastric (NG) tube - a small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.
  • 鼻胃(NG)管:一个细小的,柔软的管子,用来持续排空胃内在手术期间可能聚集的胃酸和胃内气体。
  • urinary catheter - a small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys to remove excess fluid from the body.
  • 导尿管:一个细小的,柔软的管子从膀胱引流尿液并且精确的测量机体产生了多少尿液,这对于判断心功能如何有帮助。在手术后,心脏比以前会有一点虚弱,并且,因此,机体可能开始保持液体,引起水肿和虚胖。可能会给与利尿药以帮助肾脏从身体里排出过多的液体。
  • chest tube - a drainage tube will be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.
    胸管:一根胸腔引流管保持切口缝合以后胸腔里没有集血。出血可能发生在术后数小时或甚至几天。
  • heart monitor - a machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values.
  • 心脏监视器:一个机器持续的显示你孩子的心律的图形,并且监视心率,动脉血压,和其它的数值。

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, and these will be explained to you. The staff will give you 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。

Long-term outlook after coarctation of the aorta surgical repair:

主动脉缩窄外科修复以后的长期前景:

Most children who have had a coarctation of the aorta surgical repair will live healthy lives. Activity levels, appetite, and growth should eventually return to normal.

绝大多数经过主动脉缩窄的外科修复的儿童将会过上健康的生活。活跃程度,食欲,和生长将会恢复正常。

As the child grows, the aorta may once again become narrow on occasion. If this happens, a balloon procedure or operation may be necessary to repair the coarctation.

随着孩子的成长,有时主动脉可能会再次变狭窄。如果发生了,球囊导管介入或手术对于修复缩窄可能是必要的。

Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis after discharge from the hospital.

你孩子的心脏专家可能会劝告你在出院以后使用抗生素以防治细菌性心内膜炎。

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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