医学分享-临床心脏病学50例：病例2-房间隔缺损（ASD）

导语 本文介绍房间隔缺损相关知识，同时原文中有大量的医学单词，可以通过不断地积累和学习，记住这些单词的正确拼写和发音，防止一开口就出错。

Congenital Heart Diseases

第一节 先天性心脏病

Case 2 Atrial septal defect 病例2   房间隔缺损

Case Presentation 病例介绍 A 36-year old woman was referred to a physician by a gynecologist, for preoperative assessment prior to elective hysterectomy. The patient had multiple uterine fibroids on ultrasonography and complained of excessive bleeding during menstruation. For the past 6 months, she had been complaining of exertional dyspnea and fatigue, which were attributed to anemia as a result of blood loss. She denied complaints of chest pain, palpitations or dizziness. 一名36岁的女性由妇科医生转诊至医生，进行择期子宫切除术前的术前评估。患者超声检查发现多发性子宫肌瘤，并抱怨（主诉）月经期间出血过多。对于过去6个月，她一直抱怨劳力性呼吸困难和疲劳，这被归因于失血导致的贫血。她否认主诉胸痛、心悸或头晕。

There was no history of cyanotic spells, joint pains or recurrent respiratory infections during her childhood. The patient was married, had 2 sons aged 11 and 9 years and she had never been hospitalized for any major illness or surgical procedure. 在她的童年时期，没有紫绀发作、关节疼痛或反复呼吸道感染的病史。患者已婚，有2个11岁和9岁的儿子，从未因任何重大疾病或手术住院。

重点单词【要注意音标和单词的分割】 gynecology  英 [ˌgaɪnɪˈk ɒ lədʒi]   美 [ˌgaɪn ə ˈk ɑ lədʒi]     n.妇科学 gynecologist  美 [ˌgaɪn ɪ ˈk ɒ lədʒist]  n. 妇产科医生 ( 注意， gy 的发音 , 是 gai ，而不是 dʒai) hysterectomy   英 [ˌhɪstəˈ rektəmi ]   美 [ˌhɪstəˈ rektəmi ]   n.子宫切除(术) uterine fibroids  英 [ˈjuːtər aɪ n ˈfaɪbrɔɪdz]  美 [ˈjuːtər aɪ n ˈfaɪbrɔɪdz]   子宫纤维瘤;子宫肌瘤 ultrasonography   英 [ˌʌltrəsəˈnɒɡrəfɪ]   n.超声检查术；超声检查法  (注意本单词发音的分割， ultraso + nography，重音在后 ) menstruation  英 [ˌmenstrʊˈeɪʃən]  n.月经；行经 exertional  英 [ɪgˈz ɜː ʃən(ə)l] 美 [ɪgˈz ɜ rʃən(ə)l]  adj.劳累性的  dyspnea  英 [dɪspˈnɪə] 美 [ˈdɪspˌniə]  n.呼吸困难;   palpitations  英 [ˌpælpɪˈteɪʃnz]   美 [ˌpælpɪˈteɪʃnz]  n.心悸(复数) cyanotic  英 [ˌsaɪəˈnɒtɪk]  adj.发绀的；青紫的    

On examination there was mild anemia but no  cyanosis , icterus or sign of congestive heart failure. The pulse was 90 beats/min. regular, with a BP of 136/80 mm Hg in the right arm. The apex beat was normal in location with a sustained left parasternal heave on palpation . The S1 was normal with a loud P2; no S3 or S4 sound was heard. The S2 components namely A2 and P2 were widely spaced and the time gap between them did not increase further during inspiration. A short systolic murmur was heard over the upper left  sternal border . The murmur was not preceded by an ejection click or accompanied by a  palpable thrill and did not radiate to the neck. The lung fields were clear on  auscultation . 检查时有轻度贫血，但无紫绀、黄疸或充血性心力衰竭迹象。脉搏为90次/分。右臂血压为136/80 mm Hg。心尖搏动位置正常，触诊时左侧胸骨旁持续隆起。S1正常，P2响亮；没有听到S3或S4声音。S2的组分，即A2和P2间隔很宽，在吸气期间，它们之间的时间间隔没有进一步增加。左胸骨上缘听到短暂的收缩期杂音。杂音之前，没有弹射咔嗒声，也没有伴有明显的震颤，也没有辐射到颈部。听诊肺野清晰。

重点单词【要注意音标和单词的分割】 anemia 英 [əˈniːmɪə]   美 [əˈnimiə]   n. 贫血;贫血症 cyanosis 英 [ˌsaɪəˈnəʊsɪs]   美 [ˌsaɪəˈnoʊsɪs]   n. 发绀，发绀病；苍白病；黄萎病 icterus 英 [ˈɪktərəs] n.（植物因过冷或湿度过大而发生的）黄化病; congestive 美 [kənˈdʒɛstɪv]  adj. (疾病)充血性的 apex 英 [ˈeɪpeks]   美 [ˈeɪpeks]   n. 顶;顶点;最高点 (注意发音不是ai) sternal 英 [ˈstɜːnəl] adj.胸骨的; para-sternal 胸骨旁的        sternal boarder 胸骨板 heave 英 [hiːv]   美 [hiːv]   v. 隆起;(用力)举起，拖，拉，抛;(强烈而有节奏地)起伏;(常指吃力地)缓慢发出(声音);恶心  n.举;起伏;拖;拉;抛 palpation 英 [pælˈpeɪʃn]   美 [pælˈpeɪʃn]   n.触诊，扪诊      （On palpation, 经触诊） systolic 英 [sɪˈstɒlɪk]  adj. 心缩期的 murmur 英 [ˈmɜːmə(r)]   美 [ˈmɜːrmər]   n. 咕哝;低语;喃喃声;嘟囔;接连从远处传来的低沉的声音;（胸部的）杂音 v. 低语;喃喃细语;连续发出低沉的声音;(私下)发怨言，发牢骚 precede 英 [prɪˈsiːd]   美 [prɪˈsiːd]   v. 先于;在…之前发生(或出现);走在…前面 palpable 英 [ˈpælpəbl]  adj.可触摸的；有形的;明显的；易察觉的；可感知的;可触知的; thrill 英 [θrɪl]   美 [θr ɪ l]  n. 激动;兴奋;震颤感;兴奋感; vt. 使非常兴奋;使非常激动 auscultation 英 [ˌɔːskəlˈteɪʃn]   美 [ˌɔːskəlˈteɪʃn]  n.听诊  (注意 auscul+tation，重音在后）    （On auscultation，经听诊）    

Clinical Discussion 临床讨论 From the history and physical examination, this young woman had effort intolerance with an ejection murmur in the pulmonary area. Typical causes of such a murmur are innocent hemic murmur (Still’s murmur), pulmonary valve stenosis, pulmonary hypertension and atrial septal defect. 

从病史和体格检查来看，这名年轻女性存在用力不耐受，肺部出现射血杂音。这种杂音的典型原因是无害的（单纯的）贫血性杂音（Still 杂音）、肺动脉瓣膜狭窄、肺动脉高压和房间隔缺损。

The murmur of pulmonary stenosis may be preceded by an ejection click and accompanied by a palpable thrill. The P2 component of S2 is muffled and the splitting between A2 and P2 is wide, but widens further during inspiration.  肺动脉狭窄的杂音之前可能有射血咔嗒声，并伴有明显的震颤。S2的P2成分被抑制（掩盖），A2和P2之间的分裂很宽，但在吸气期间进一步扩大。 An innocent hemic murmur is not associated with a loud P2 or wide splitting of S2. Pulmonary hypertension of any etiology can produce a systolic murmur with loud P2 but wide fixed splitting of S2 is only a feature of atrial septal defect.  单纯的（无害的）缺血性杂音与响亮的P2或S2的宽分裂无关。任何病因的肺动脉高压均可产生收缩期响亮的P2杂音，但是，S2裂宽固定，仅是房间隔缺损的一个特征。 ECG of the patient showed sinus rhythm with incomplete right bundle branch block and a rightward QRS axis. X-ray chest showed enlarged right-sided chambers with dilated main pulmonary artery, prominent hila and pulmonary plethora. 

患者心电图显示窦性心律，呈现不完全右束支传导阻滞，QRS轴向右偏移。X线胸片显示右心室扩大，主肺动脉扩张，肺门突出，肺（血量）过多。

On ECHO, the right atrium and right ventricle were dilated and a signal drop-out was noticed in the interatrial septum. On colour Doppler, an abnormal flow map was observed extending across the area of echo drop-out, from the left atrium to the right atrium (Fig. 2.1). There were no abnormalities of the cardiac valves and the estimated pulmonary artery pressure was normal. Therefore, the definite diagnosis in this case is atrial septal defect (ASD). 在ECHO上，右心房和右心室扩张，并注意到房间隔信号下降。在彩色多普勒上，观察到异常血流图跨越回声下降区域，从从左心房延伸至右心房（Fig. 2.1）。心脏瓣膜无异常，估计肺动脉压力正常。因此，本例明确诊断为房间隔缺损（ASD）。

重点单词【要注意音标和单词的分割】 muffled 英 [ˈmʌfld]   美 [ˈmʌfld]   adj. 沉闷的;压抑的;模糊不清的 v.压抑(声音);使(声音)降低;使听不清;裹住，覆盖，蒙住(以保暖)    muffle的过去分词和过去式

stenosis 英 [stɪˈnəʊsɪs]   美 [stɪˈnoʊsɪs; stəˈnoʊsɪs]   狭窄;狭窄症

etiology 英 [ˌiːtiˈɒlədʒi]   美 [ˌiːtiˈɑːlədʒi]  n. 病因学，病原学，致病源

hila  [ˈhilə]    n.种脐；（淀粉的）脐点；门  (hilum的复数） hilum 英 [ˈhaɪləm]  n.种脐;（淀粉粒的）脐点;门; plethora 英 [ˈpleθərə]   美 [ˈpleθərə]   n. 过多;过量;过剩    

Figure 2.1: Color flow map extending from left atrium to right atrium(彩色血流图，从左心房延伸到右心房) In ASD， breach in the continuity of the interatrial septum creates a left-to-right shunt between the atria (Fig. 2.2). The septal defect occurs due to complexity of its embryological development. Most (75%) ASDs occur in the mid-portion of the septum, in the region of the foramen ovale and are termed as ostium secundum ASD. Some ASDs occur lower down the inter-atrial septum and are termed as ostium primum ASD (Fig. 2.3).  在ASD中，房间隔连续性的破裂造成左向右的心房间分流（图2.2）。间隔缺损的发生，是由于胚胎发育的复杂性导致。大多数（75%）ASD发生在隔膜的中部，即卵圆孔区域，因此称为继发孔型房间隔缺损（卵圆孔ASD）。一些ASD发生在房间隔下方，由此被命名为原发孔型房间隔缺损（Fig. 2.3）。 Ostium primum ASDs are associated with cleft leaflets, regurgitation of the atrioventricular valves and are also known as endocardial cushion defect. An uncommon variety of ASD in the upper portion is sinus venosus defect, which is accompanied by anomalous pulmonary venous connections (Table 2.1). Inferior vena caval defects are very rare. An ASD maybe associated with trisomy 21 (Down’s syndrome) or abnormalities of the hand(Holt Oram syndrome).

原发孔型ASD与小叶裂开、房室瓣反流相关，也叫心内膜垫缺损。上段部分一种不常见的ASD是静脉窦缺损，伴有异常的肺静脉连接（Table 2.1）。下腔静脉缺损非常罕见。ASD可能与21三体（唐氏综合征）或手部异常（霍尔特-奥拉姆综合征）有关。

重点单词 【要注意音标和单词的分割】

breach 英 [briːtʃ]   美 [briːtʃ]   vt. 违反;违背;在…上打开缺口 n. 破坏;(对法规等的)违背，违犯;辜负;(关系的)中断，终止;突破口

embryological  英 [ˌ em b rɪə ˈlɒdʒɪkl]  adj.胚胎学的;胚胎的; foramen 英 [fɒˈreɪmɛn]   美 [fəˈreɪmən]  n.（骨头中的）孔，小孔，胚孔ovale 英[əʊ'veɪl]   美[oʊ'veɪl] 卵圆形 （注：重音在后） foramen ovale（胚）卵圆孔，第二中隔孔 ostium   英 [ˈɒstɪəm ] 卵圆孔（口） secundum   英 [ sɪ'kʌndəm] 第二孔道 primum 英[p'rɪməm] 美[p'rɪməm] adj. 原始的 ostium primum 原始孔     ostium secundum 第二孔/继发孔 cleft 英[kleft] 美[kleft]n. 裂缝; 裂口; cleave的过去式和过去分词; .v. 劈开，剁开，割开( cleave的过去式和过去分词 )

regurgitation 英[rɪˌɡɜ:dʒɪ'teɪʃn]美[rɪˌɡɜdʒɪ'teɪʃn]n.回流; 反刍; 翻胃; 胃反

vena 英['viːnə] 美[ 'vinə]n.<拉>静脉

caval 英[k æ 'vəl] 美[k æ 'vəl] 腔静脉的 venosus[vi' nəu sis] 静脉的 (注：重音在后） anomalous英 [əˈnɒmələs]   美 [əˈnɑːmələs]   adj.反常的；异常的   【派生词】anomalously adv. trisomy 英['traɪs əʊ mɪ] 美['traɪˌs oʊ mi] n. 三（染色）体性，三（染色）体细胞    

Table 2.1 : 房间隔缺损的类型(Types of atrial septal defect)

Ostium secundum ASD（继发型房间隔缺损）

Ostium primum ASD（原发型房间隔缺损）

Sinus venosus defect（静脉窦缺损）

Vena caval defect（腔静脉缺损）

The systolic murmur of ASD is due to increased flow across the pulmonary valve and not due to the shunt. The intensity of murmur does not correlate with the size of the ASD. However, a large ASD is associated with a diastolic flow murmur and a right-sided S3, due to torrential flow across the tricuspid valve.  ASD的收缩期杂音是由于流经肺动脉瓣的流量增加，而非分流导致。杂音的强度与ASD的大小无关。然而，由于三尖瓣的反流，较大的ASD与舒张血流杂音和右侧S3相关。 An accompanying pansystolic murmur due to mitral and/or tricuspid regurgitation is a feature of ostium primum ASD. In ASD, the splitting of S2 is wide and fixed. It is wide because of increased pulmonary ejection time, which delays the P2. Other reasons for wide splitting of S2 are right bundle branch block or pulmonary stenosis (delayed P2) and mitral regurgitation or ventricular septal defect (premature A2). The splitting of S2 is also wide in WPW syndrome Type A, in which there is pre-excitation of the left ventricle. The splitting of S2 is fixed in ASD because the shunt equalizes atrial pressures throughout the respiratory cycle and there is no inspiratory augmentation of right ventricular filling.  由于二尖瓣和/或三尖瓣反流而伴随的全收缩期杂音是原发型ASD的特征。在ASD中，S2的分裂是宽而且固定的。S2增宽，是因为肺射血时间增加，延迟了P2。其他导致S2宽分裂的原因包括右束支传导阻滞或肺动脉狭窄（延迟P2）和二尖瓣反流或室间隔缺损（早发A2）。在A型WPW综合征中，S2分裂也很宽，其中左心室存在预激。S2的分裂在ASD中是固定的，因为分流在整个呼吸循环中平衡了心房压力，并且没有呼吸导致的右心室充盈增强。 On ECHO, since the signal from the interatrial septum is weak, false echo drop-out may be seen even in normal persons.  The subcostal window may be a better option to diagnose an ASD but  transesophageal  echocardiography (TEE) provides excellent visualization particularly in endocardial cushion defects and  sinus venosus ASD . Sometimes, contrast echo is needed to visualize the shunt using agitated saline , which contains air bubbles that cross over the septal defect. 在ECHO（心脏超声）上，由于来自房间隔的信号较弱，即使在正常人中也可以看到假的回声下降。肋下窗可能是诊断ASD的更好选择，但经食管超声心动图（TEE）提供了极好的可视化效果，特别是在心内膜垫缺损和窦静脉ASD中。有时，需要造影剂超声(对比超声）来显示分流，使用的是搅拌盐水(激动盐水），盐水中含有穿过间隔缺损的气泡。 ASD is the commonest congenital heart disease diagnosed in adulthood, with either absent or mild symptoms. It is 7 times more common in females than in males. Complications of ASD in adults are effort intolerance and pulmonary hypertension. Reversal of shunt and right heart failure are rare compared to ventricular septal defect. Atrial tachyarrhythmias including atrial fibrillation are common. Typically, sinus arrhythmia is never observed, because the shunt  negates the effect of inspiration on venous return. Systemic thrombo-embolism can occur due to emboli from peripheral or  pelvic veins, passing across the septal defect ( paradoxical embolism). ASD是成年后诊断的最常见的先天性心脏病，症状或无症状或轻微。女性的发病率是男性的7倍。成人ASD的并发症是用力不耐受和肺动脉高压。与室间隔缺损相比，分流逆转和右心衰竭是罕见的。包括房颤在内的房性快速心律失常是常见的。通常，从未观察到窦性心律失常，因为分流术抵消了吸气对静脉回流的影响。系统性血栓栓塞可发生于外周静脉或盆腔静脉的栓塞，穿过间隔缺损（矛盾栓塞/反常栓塞）。

重点单词 【要注意音标和单词的分割】 torrential  英 [t ə ˈrenʃl]   美 [t ə ˈrenʃl]  adj. 倾盆的;倾泻的;如注的 tricuspid  英 [traɪˈkʌspɪd]   美 [traɪˈkʌspəd]  三尖瓣;三尖瓣的;尖瓣;三尖

equalize ['iːkwəlaɪz] v.(使)相等;划一

subcostal  英 [sʌbˈkɒstəl]  adj.肋骨下的；

saline 英 [ˈseɪlaɪn]   美 [ˈseɪliːn]  n. 生理盐水;盐水 adj. 盐的;含盐的;咸的 agitated 英 ['ædʒɪteɪtɪd] 美['ædʒɪtetɪd] adj. 颤抖的，不安的,激动不安的;焦虑的 negates 英 [nɪˈɡeɪts]   美 [nɪˈɡeɪts]   v. 取消;使无效;否定;否认（negate的第三人称单数） pelvic 英 [ˈpelvɪk]   美 [ˈpelvɪk]  adj. 靠近骨盆的;骨盆的 transesophageal[ˌtrænsˈisɒfədʒioʊ] adj. 经食道的   （注意单词的分割，trans+esophageal是一个复合词）

paradoxical 英 [ˌpærəˈdɒksɪkl]   美 [ˌpærəˈdɑːksɪkl]   adj. 矛盾的;似自相矛盾的  （重音在后）

MANAGEMENT ISSUES

管理问题

Most ostium secundum atrial septal defects are amenable to percutaneous device closure. Large ASDs allow large volumes of left-to-right shunt and usually persent with exertional breathlessness and fatigue. They can lead to pulmonary hypertension and right heart failure, although reversal of shunt (right-to-left) is less common than in case of ventricular septal defect. Therefore, ASDs larger than  10 mm in size should ideally be closed before significant pulmonary hypertension develops.

大多数继发孔口房间隔缺损可通过经皮装置（封堵器）闭合。大的ASD存在大量的左向右分流，通常伴有劳力性呼吸困难和疲劳。它们可能导致肺动脉高压和右心衰竭，尽管分流术逆转（从右到左）比室间隔缺损更不常见。因此，尺寸大于10 mm的ASD最好在出现严重肺动脉高压之前关闭。

Smaller ASDs with small volumes of shunt may become progressively more symptomatic as left ventricular compliance declines with age and the degree of shunting increases. Such ASDs that lead to right ventricular dilatation, should be closed during adulthood. Patients with ASD may develop paradoxical emboli which arise in the venous system and cross the septal defect to reach the systemic circulation. ASDs with history of thrombo-embolism should be closed, irrespective of their size (Table 2.2).

随着左心室顺应性因年龄的增长而下降，以及分流的程度增加，分流量较小的ASD可能会逐渐加重症状。此类导致右心室扩张的ASD应在成年期间关闭。ASD患者可能会出现反常的栓塞，栓塞出现在静脉系统，穿过间隔缺损到达全身循环。有血栓栓塞史的ASD应关闭，而无论其大小（Table 2.2）。

Table 2.2 : Indications for surgical closure of ASD (手术关闭ASD的指征）

Large-sized ASD more than 10 mm in size with pulmonary to systemic flow ratio ＞1.5：1.   大于10 mm的大型ASD，肺与全身流量比＞1.5:1。

Medium-sized ASD with dilated right ventricle without significant pulmonary hypertension.   中度ASD，右心室扩张，无明显肺动脉高压。

Small-sized ASD without dilated right ventricle with history of systemic thrombo-embolis.   无右心室扩张的小型ASD，有系统性血栓栓塞史。

Ostium primum atrial septal defects with atrioventricular valvular abnormalities and sinus venosus defects with anomalous pulmonary venous drainage are not amenable to percutaneous device closure because of their complexity. They require a definitive surgical procedure for their correction.

原发型房间隔缺损伴房室瓣异常，以及窦静脉缺损伴异常肺静脉引流，这些情况，由于其复杂性，不适合经皮封堵器封堵。他们需要一个明确的外科手术来矫正。

RECENT ADVANCES 最近的进展 Percutaneous deployment of a closure device for atrial septal defect has been standard practice for several decades. Transesophageal echocardiography (TEE) is widely used to guide the deployment.  经皮放置房间隔缺损封堵器是近几十年来的实践标准。经食管超声心动图（TEE）广泛用于指导(封堵器）的部署。 Recently, intracardiac ultrasound has  been used to accurately assess the anatomy, for better selection and placement  of the device. Vital anatomical information includes size of the defect, the rim  around the defect and proximity to the mitral and tricuspid valves.

最近，心内超声已经用于准确评估解剖结构，以便更好地选择和放置该设备。重要的解剖信息包括缺损的大小、缺损周围的边缘以及二尖瓣和三尖瓣附近。

重点单词【要注意音标和单词的分割】

anatomical 英 [ˌæn ə ˈt ɒ mɪkl]   美 [ˌæn ə ˈt ɑː mɪkl]   adj. 解剖的;解剖学的；身体结构上的 amendable 【 ə ˈmendəbl】  adj. 能改正的；可修改的    

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