心脏的淋巴系

1.在63具胎儿及小儿尸体上,用心外膜下淋巴管注射的方法,观察了心外膜下毛细淋巴管的配布情况,以及心外膜下淋巴管的走行及所注入的淋巴结。2.在心室外膜下可分出深浅两层毛细淋巴管网;但在胎儿及新生儿则仅有一层。心室外膜下毛细淋巴管注入心室外膜下淋巴管;后者最后合成左、右淋巴干。心房的外膜下仅有一层毛细淋巴管网,注入心房外膜下淋巴管;后者注入冠状沟内的淋巴管,而合入左、右淋巴干,一部分可直入左支气管肺淋巴结或气管前淋巴结。 3.左淋巴干多是经过肺动脉后淋巴结(14例),而入左支气管肺淋巴结、气管权淋巴结及气管右侧的气管旁淋巴结;一部分直入左支气管肺淋巴结(9例)、气管权淋巴结(4例)及右支气管肺淋巴结((1例)。 4.右淋巴干多是注入主动脉弓淋巴结(15例),或经主动脉前淋巴结(4例)再入主动脉弓淋巴结;少数例入肺动脉后淋巴结(3例)及右支气管肺淋巴结(1例)。 5.有3例,右淋巴干在肺动脉的后方合入左淋巴干。另外3例,左、右淋巴千在左支气管肺淋巴结、肺动脉后淋巴结或右支气管肺淋巴结相遇。 6.左淋巴干收纳左心大部分及右心室靠前纵沟部分的淋巴,右淋巴干收集右心大部分及左心室靠后纵沟部分的淋巴;但心房及动脉圆锥处的一部分淋巴管,可不入左、右淋巴干,l}接注入局部淋巴结。     

胎儿肺动脉起源异常8例尸体解剖分析

目的 对胎儿先天性肺动脉起源异常进行分析,提高该疾病诊断的精准性。方法 回顾性分析8例胎儿先天性心脏畸形病例,均经尸体解剖证实,检查胎儿心血管畸形及内脏的畸形情况,对肺动脉异位起源进行分析总结。结果 肺动脉异位起源8例,其中男胎4例,女胎4例,孕期为孕21～28周。右肺动脉起自升主动脉1例,同时存在主-肺动脉间隔缺损、主动脉弓离断并室间隔完整,即Berry综合征。双侧肺动脉融合起自左头臂干1例,患者伴肺动脉闭锁等多种心血管畸形及心外脏器畸形。左肺动脉和右肺动脉分别起自不同主动脉或分支3例,均伴随肺动脉闭锁,1例左肺动脉起自左头臂干,右肺动脉起自降主动脉及右侧动脉导管;1例左肺动脉起自右动脉导管,右肺动脉起自右锁骨下动脉;1例左肺动脉起自左动脉导管,右肺动脉起自降主动脉。左肺动脉吊带3例,肺动脉主干发出右肺动脉,右肺动脉发出左肺动脉形成血管环。结论 胎儿肺动脉异位起源是一种少见血管畸形,其走行复杂多样,可伴随心内畸形及心外脏器畸形。     

多层螺旋CT三维成像行国人气道径线分析

目的探讨国人气管和主支气管各种解剖径线的长度与角度.方法用多层螺旋CT三维成像法测定300例成年健康体检者锁骨胸骨端水平气管内径、锁骨胸骨端水平到隆突的气管长度,左、右主支气管和右中间支气管内径与长度,以及左、右主支气管长轴与矢状面的夹角.结果男性气管、左、右主支气管、右中间支气管内径与长度,以及右上肺叶支气管开口内径均大于女性,而女性左、右主支气管长轴与矢状面夹角均大于男性(p<0.05或0.01).成年人与老年人上述各解剖径线值差异无显著(p>0.05).左主支气管内径值与右主支气管内径值呈高度相关,且分别与气管内径值和身高呈高度相关(p<0.01).结论通过多层螺旋CT三维成像法测定国人气管、左和右主支气管径线值,获左、右主支气管内径值与患者身高和气管内径测量值的回归方程,可预测其左、右主支气管内径值.     

多层螺旋CT三维成像行国人气道径线分析

目的探讨国人气管和主支气管各种解剖径线的长度与角度.方法用多层螺旋CT三维成像法测定300例成年健康体检者锁骨胸骨端水平气管内径、锁骨胸骨端水平到隆突的气管长度,左、右主支气管和右中间支气管内径与长度,以及左、右主支气管长轴与矢状面的夹角.结果男性气管、左、右主支气管、右中间支气管内径与长度,以及右上肺叶支气管开口内径均大于女性,而女性左、右主支气管长轴与矢状面夹角均大于男性(p<0.05或0.01).成年人与老年人上述各解剖径线值差异无显著(p>0.05).左主支气管内径值与右主支气管内径值呈高度相关,且分别与气管内径值和身高呈高度相关(p<0.01).结论通过多层螺旋CT三维成像法测定国人气管、左和右主支气管径线值,获左、右主支气管内径值与患者身高和气管内径测量值的回归方程,可预测其左、右主支气管内径值.     

汉族成人心脏右房室瓣连合区解剖类型和临床意义

目的为临床心脏右房室瓣病变的诊治提供解剖学资料.方法对171例中国汉族成人心脏行右房室瓣主瓣连合区和主副瓣连合区的形态学观测. 结果主瓣连合区分为单主瓣和联合主瓣间连合区及单主瓣和单主瓣间连合区.主副瓣连合区分为单主瓣和副瓣间连合区及联合主瓣和副瓣间连合区.结论主瓣连合区和主副瓣连合区都有明确位置,它们可以密闭心室与心房的交通,使血液不能逆流入心房.在外科手术时,必须要注意它们的解剖分型.     

胎儿超声心动图在诊断先天性心脏病中的临床意义

目的探讨超声心动图在产前筛查胎儿先天性心脏病的作用。方法以胎儿四腔心切面为基础,左心长轴切面,左、右室流出道及心底短轴、主动脉弓和三血管为主要切面。对1021例22w~28w胎儿心脏进行筛查,发现严重心脏畸形者予以引产,其它异常者追踪至分娩,进行新生儿超声心动图检查,明确先天性心脏病类型。结果发现胎儿各种先天性心脏畸形40例（伴胎儿多发畸形5例）。其中：室间隔缺损10例,心内膜垫缺损4例,法洛氏四联症5例,单腔心1例,左、右心发育不良各1例,右室双出口2例,三尖瓣下移畸形1例,永存动脉干1例,完全性大动脉转位2例,矫正型大动脉转位2例,心脏肿瘤3例,右位心1例,完全性肺静脉畸形引流1例,主动脉狭窄2例,动脉导管提前收缩2例,动脉导管走形扭曲1例。结论胎儿超声心动图是产前筛查胎儿先天性心脏病的有效方法。     

28例胎儿心脏畸形彩色多普勒超声检查回顾性分析

目的探讨彩色多普勒超声在胎儿心脏畸形筛查中的作用和检查方法。方法回顾性分析笔者所在医院2012年10月-2015年12月间发现的28例心脏畸形胎儿的临床资料,观察各种心脏畸形在不同切面的超声表现。结果单心房、单心室5例,左心室发育不良2例,室间隔缺损3例,右室双出口5例,法洛四联症5例,完全性大动脉转位2例,单纯肺动脉狭窄3例,三尖瓣下移畸形1例,右位主动脉弓2例。结论四腔心,左右流出道,三血管切面对胎儿心脏畸形的宫内诊断有较高的敏感性和特异性,是诊断胎儿先天性心脏病的首选和常用的方法。     

胎儿超声四腔心切面法联合腹部横切面法诊断胎儿右位心的临床价值

目的探讨四腔心切面法联合腹部横切面法诊断胎儿心脏右位心的临床价值。方法回顾2004-2013年9年244 619例孕妇中超声发现的24例胎儿心脏右位心。结果 24例胎儿心脏右位心,单纯性右位心15例、伴心脏多发畸形5例,伴室间隔缺损2例,伴心外畸形2例。其中单纯性右位心15例中,2例首次产前超声没有发现,在例行产前超声复查中纠正并发现单纯性右位心。结论四腔心切面法联合腹部横切面法是诊断胎儿心脏右位心的最佳手段,而胎儿体位、孕周大小、羊水多少、操作经验都将影响超声判断胎儿心脏右位心的准确性,因此有必要对胎儿进行定期复查,以减少或预防胎儿心脏右位心的漏误诊。     

双源CT在单心室中的诊断价值EI北大核心CSCD

探讨单心室的双源CT(DSCT)特征及DSCT对单心室患者的诊断价值。回顾分析2009年-2011年期间四川大学华西医院14例单心室患者的DSCT影像表现,并与手术及心导管结果对照。结果发现:(1)按照Anderson分型方法,14例单心室患者中,左心室型7例(50.0%),右心室型3例(21.4%),未定心室型4例(28.6%)。(2)合并畸形:肺动脉狭窄11例(78.6%),双侧上腔静脉6例(42.9%),房间隔缺损5例(35.7%),共同房室瓣5例(35.7%),单心房4例(28.6%),大动脉转位3例(21.4%),内脏反位3例(21.4%),心房异构2例(14.3%),右位心2例(14.3%),右位主动脉弓2(14.3%),心耳并置1例(7.1%),完全性肺静脉异位引流1例(7.1%),无脾综合征1例(7.1%),动脉导管未闭1例(7.1%)。(3)左心室型的附属心腔多位于主心腔的前方,占5例(71.4%);而右心室型的附属心腔均位于主心腔的后方,占3例(100%)。(4)主、肺动脉以共同起于主心室多见,占8例(57.1%);主动脉瓣多位于肺动脉瓣的右侧,占9例(64.3%)。(5)其中10例单心室分型与手术结果对照,符合率达100%。DSCT对单心室诊断分型的准确率高,对主、肺动脉及冠状动脉的病变也可很好地显示。     

先天血管环畸形的解剖变异及外科治疗

目的 总结先天血管环畸形的解剖变异及外科治疗经验.方法 回顾性分析2009年3月-2012年2月收治的21例先天血管环畸形患儿的临床资料.21例中男10例,女11例;年龄4个月～3岁,平均1.1岁.患儿术前均行心脏超声、64排CT检查,其中双主动脉弓8例、右位主动脉弓伴左侧韧带6例、右位主动脉弓伴迷走左锁骨下动脉2例、肺动脉吊带4例、左位主动脉弓伴右侧动脉导管1例,合并法洛四联症4例、室间隔缺损2例、单心房1例、完全性肺静脉异位引流1例、肺静脉狭窄1例.21例均在全麻下行畸形矫治术,其中低温体外循环下手术矫治12例,非体外循环手术9例（7例采用左后外侧切口,2例采用左腋下切口）.结果 所有患儿均顺利完成手术.其中体外循环下手术的患儿,体外循环34～83 min,中位数47 min;主动脉阻断19～50 min,中位数26 min.1例患儿术后出现呼吸功能不全,再次插管呼吸机辅助呼吸.所有患儿均康复出院,于出院后1、3、6、12个月复查,此后每年复查1次.21例患儿平均随访3.6年.随访期间患儿呼吸道症状消失或明显缓解,生长发育正常.术后1个月复查胸部CT气管重建示：气管狭窄较前明显改善.结论 根据先天血管环的解剖变异及是否合并心内畸形选择相应的外科治疗方案,可取得较好的治疗效果.     

Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein

PURPOSE: Four pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins. MATERIALS AND METHODS: From January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies. RESULTS: Clinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired. CONCLUSION: The clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.     

The importance of being isomeric

In the normal individual, the parietal components of the body are mirror‐imaged and appropriately described as isomeric. The thoraco‐abdominal organs, in contrast, are lateralized. However, in “visceral heterotaxy,” the thoraco‐abdominal organs also show some degree of isomerism, best seen in the arrangement of the bronchial tree. Whether isomerism can be found within the heart remains controversial. One of two recent publications in this journal emphasized the crucial features of bronchial isomerism; the other, in contrast, confused the situation of isomerism within the heart. In this review, we show how the topic of cardiac isomerism is clarified by concentrating on the anatomical features of the cardiac components and determining how best they can be described. Appropriate manipulation of developing mice produces unequivocal evidence of isomerism of the atrial appendages, but with no evidence of ventricular isomerism. In hearts from patients with so‐called “heterotaxy,” only the atrial appendages, distinguished on the basis of the pectinate muscles lining their walls, are uniformly isomeric, permitting the syndrome to be differentiated into the subsets of left as opposed to right atrial appendage isomerism. Thus, controversies are defused by simply describing the isomerism of the atrial appendages rather than “atrial isomerism,” recognizing the frequency of abnormal venoatrial connections in these settings. Any suggestion of ambiguity is removed by the equally simple expedient of describing all the variable cardiac features, describing the arrangements of the thoracic and abdominal organs separately should there be discordances. Clin. Anat. 28:477–486, 2015. © 2015 Wiley Periodicals, Inc.     

Left cardiac isomerism in the Sonic hedgehog null mouse

Sonic hedgehog (Shh) is a secreted morphogen necessary for the production of sidedness in the developing embryo. In this study, we describe the morphology of the atrial chambers and atrioventricular junctions of the Shh null mouse heart. We demonstrate that the essential phenotypic feature is isomerism of the left atrial appendages, in combination with an atrioventricular septal defect and a common atrioventricular junction. These malformations are known to be frequent in humans with left isomerism. To confirm the presence of left isomerism, we show that Pitx2c, a recognized determinant of morphological leftness, is expressed in the Shh null mutants on both the right and left sides of the inflow region, and on both sides of the solitary arterial trunk exiting from the heart. It has been established that derivatives of the second heart field expressing Isl1 are asymmetrically distributed in the developing normal heart. We now show that this population is reduced in the hearts from the Shh null mutants, likely contributing to the defects. To distinguish the consequences of reduced contributions from the second heart field from those of left–right patterning disturbance, we disrupted the movement of second heart field cells into the heart by expressing dominant‐negative Rho kinase in the population of cells expressing Isl1. This resulted in absence of the vestibular spine, and presence of atrioventricular septal defects closely resembling those seen in the hearts from the Shh null mutants. The primary atrial septum, however, was well formed, and there was no evidence of isomerism of the atrial appendages, suggesting that these features do not relate to disruption of the contributions made by the second heart field. We demonstrate, therefore, that the Shh null mouse is a model of isomerism of the left atrial appendages, and show that the recognized associated malformations found at the venous pole of the heart in the setting of left isomerism are likely to arise from the loss of the effects of Shh in the establishment of laterality, combined with a reduced contribution made by cells derived from the second heart field.     

Characterization of atrial morphology and sinus node morphology in heterotaxy syndrome: an autopsy-based study of 41 cases (1950-2008)

BackgroundHeterotaxy syndrome affects the sidedness of heart, lungs, and abdominal viscera, and is associated with complex congenital heart disease. Cardiac sidedness is defined by the position of the morphological right atrium and may be normal, mirror-image, or isomeric. The sinus node has been reported to be present bilaterally in right isomerism and absent bilaterally in left isomerism, although exceptions may occur.ObjectiveOur aim was to evaluate bilaterally the presence or absence of sinus node tissue in autopsy-derived hearts from patients with heterotaxy and to correlate the findings with the sidedness of the two atria.MethodsAutopsy and clinical records were reviewed from 41 cases with heterotaxy. From the cardiac specimens, tissue was collected bilaterally from expected sinus node sites. Sinus node tissue was categorized microscopically as normal, hypoplastic, indeterminate, or absent.ResultsIn hearts thought to show right atrial isomerism, sinus node tissue was detected bilaterally in 54%, was absent on one side in 43%, and was absent on both sides in 3%. For cases with apparent left atrial isomerism, a single sinus node was present in the left-sided atrium in 75% of cases and was absent bilaterally in 25%.ConclusionsBilateral sinus nodes were observed in only 54% of cases with right isomerism, and bilateral absence of sinus nodes was documented in only 25% of cases with left isomerism. Thus, our findings indicate that the sinus node is not a morphologically right-sided structure, and its presence therefore is not consistently related to the sidedness of the atria.     

A founder truncating variant in GDF1 causes autosomal‐recessive right isomerism and associated congenital heart defects in multiplex Arab kindreds

The genetic basis of congenital heart malformations associated with disruption of left–right (L–R) asymmetry is broad and heterogenous, with variants in over 25 genes implicated thus far. Of these, deleterious variants in the Growth/Differentiation Factor 1 (GDF1) gene have been shown to cause heterotaxy with varied complex heart malformations of left–right patterning, in 23 individuals reported to date, either in monoallelic or biallelic state. We report three unrelated individuals exhibiting right isomerism with congenital heart defects, each originating from a consanguineous kindred of Arab‐Muslim descent. Using whole exome sequencing, a shared novel homozygous truncating c.608G > A (p.W203*) variant in the GDF1 gene was revealed as the molecular basis of their disease. Subsequently, targeted sequencing of this variant showed full segregation with the disease in these families, with a total of over 15 reportedly affected individuals, enabling genetic counseling, prenatal diagnosis, and planning of future pregnancies. Our findings further confirm the association of biallelic GDF1 variants, heterotaxy and congenital heart defects of left–right patterning, and expand the previously described phenotypic spectrum and mutational profile. Moreover, we suggest targeted screening for the p.W203* variant in relevant clinical circumstances.     

Left atrial isomerism: clinicopathologic findings in a 14-year-old boy

Visceral heterotaxy syndromes have been associated with complex congenital heart disease and several attempts have been made to classify these lesions. One area of controversy that remains is the existence of atrial isomerism. In this case report we provide definite anatomic evidence of the existence of left atrial isomerism, wherein both the atria have smooth walls, finger-like appendages, and absence of the pectinate muscles and sinus node.     

胎儿动脉导管缺如的尸体解剖分析

目的对胎儿先天性动脉导管缺如进行尸体解剖分析,以提高该疾病诊断的精准性。方法回顾2011年1月至2019年9月北京安贞医院经超声心动图畸形筛查发现胎儿先天性心脏畸形引产而进行尸体解剖病例400例,检查胎儿心血管畸形及内脏的畸形情况,对其动脉导管缺如进行分析总结。结果400例胎儿先天性心脏病中,动脉导管缺如24例,检出率6%,男女各12例,左位主动脉弓19例,右位主动脉弓5例,21例具有肺动脉主干,3例无肺动脉主干及分支。具体情况如下:(1)肺动脉狭窄伴动脉导管缺如15例,伴发主要心血管畸形:单心室6例,房间隔缺损6例,单心房4例,右心房异构4例,右心室双出口4例,静肺脉异位引流4例,法洛四联症3例,永存左上腔静脉3例;(2)肺动脉闭锁伴动脉导管缺如7例,3例无肺动脉主干,4例肺动脉闭锁伴有狭窄的肺动脉主干,均可见体-肺动脉侧支;(3)法洛四联症伴肺动脉瓣缺如综合征及动脉导管缺如1例,肺动脉扩张;(4)主-肺动脉间隔缺损伴动脉导管缺如1例,肺动脉正常。动脉导管缺如伴发主要心外畸形:脾缺如7例,肺异常7例,内脏反位7例。结论胎儿动脉导管缺如常合并复杂先天性心血管畸形;动脉导管缺如合并肺动脉闭锁常伴体-肺动脉侧支;内脏畸形与伴随的先天性心血管畸形有关。     

Atresia of left coronary ostium and left main coronary artery

An uncommon coronary anomaly incidentally found at autopsy of a 76-year-old woman is reported. There was no history of cardiovascular symptoms or clinical signs of ischemic heart disease. The autopsy disclosed that the patient had no left coronary ostium and that the left main coronary artery was atretic. However, the rest of the left coronary system, including the left anterior descending coronary artery and the left circumflex coronary artery, were intact in their normal positions. Arterial flow to the left coronary system was supplied by a dilated collateral vessel that originated from the right main coronary artery and coursed between the right ventricular infundibulum and the aorta. Our case suggests that atresia of the left coronary ostium and the left main coronary artery, often associated with death at an early age, may also be compatible with symptom-free longevity. Moreover, attention is focused on the nomenclature of single coronary anomalies.