胎儿主动脉弓离断的产前超声诊断

目的探讨胎儿主动脉弓离断的超声诊断特点。方法回顾性分析产前超声诊断为胎儿主动脉弓离断的35胎胎儿资料,同时纳入180胎正常胎儿。二维超声判断胎儿内脏及心脏位置,获取四腔心切面、左右心室流出道切面、三血管切面及三血管-气管切面等横断面,并扫查腔静脉长轴切面、主动脉弓切面及动脉导管弓切面等矢状切面。测量主动脉弓离断胎儿左右心室内径比值及主动脉与肺动脉内径比值,并与相应孕周的正常胎儿进行比较。结果产前二维超声诊断A型、B型及C型主动脉弓离断分别为16、18及1胎,均伴室间隔缺损,共27胎经尸体检查或产后检查证实产前诊断,3胎诊断错误,余5例新生儿失访。四腔心切面及三血管切面发现主动脉弓离断胎儿左右心室内径比值和主动脉与肺动脉内径比值显著小于相应孕周正常胎儿,差异有统计学意义(P均0.01)。矢状切面对主动脉追踪连续扫查可确定主动脉的分支及走行,可明确诊断及分型。A型、B型及C型离断升主动脉走行及分支分别呈现典型的"W型"、"Y型"及"I型"。矢状面扫查及横断面扫查对胎儿主动脉弓离断的诊断率分别为90.00%(27/30)及56.67%(17/30),差异有统计学意义(P0.01)。结论产前二维超声可诊断胎儿主动脉弓离断,横断面扫查可发现某些线索,矢状面连续扫查对诊断胎儿主动脉弓离断具有重要意义。     

超声诊断胎儿先天性血管环1例

<正>孕妇26岁,孕1产0,乙肝表面抗原阳性,婚前接触苯、甲苯3年,无先天性心脏病家族史。孕34周产前超声:胎儿双顶径8.74cm,头围30.51cm,股骨长约6.67cm,肝、胃、肾脏、膀胱及四肢正常;胸骨旁四腔心切面示心房正位,心室右襻,大动脉起源正常,左右心室基本对称,降主动脉位于脊柱前方;升主动脉长轴切面示主动脉根部发出左侧颈总动脉,沿气管左侧绕行(图1A);主动脉弓切面示主动脉弓部仅见右侧颈总动脉、右侧锁骨下动脉(图1B);三血管-气管切面示肺动脉和动脉导管位于气管左侧,主动脉弓位于气管右侧,左锁骨下动脉发自降     

先天性血管环产前超声诊断价值和意义

目的探讨产前超声诊断及鉴别诊断先天性血管环中完全性血管环的临床价值和意义。方法将经超声产前检查结合产后影像学检查或者引产后病理确诊的完全性血管环胎儿12例为研究对象,分析其产前超声的主要表现。结果本组中1例双主动脉弓构成完全性血管环,9例右主动脉弓、左位动脉导管、左锁骨下动脉构成完全性血管环,2例右位主动脉弓、左位动脉导管、迷走左无名动脉构成完全性血管环。结论临床产前诊断先天性完全性血管环最重要的切面为三血管气管,产前超声检查能较准确的诊断以及鉴别诊断常见的先天性完全性血管环,临床应用价值肯定。     

超声心动图诊断胎儿冠状静脉窦扩张

目的探讨胎儿冠状静脉窦(CS)扩张的超声诊断思路。方法回顾性分析145胎正常冠状静脉窦胎儿(正常组)和72胎CS扩张胎儿(CS组)的资料,于非标准四腔心切面下显示CS长轴,测量CS的矢状切面面积。采用二维超声结合彩色多普勒超声对所有胎儿分别进行胎儿四腔心切面、左右心室流出道切面、三血管切面、大动脉短轴切面、主动脉弓切面、动脉导管弓切面及腔静脉长轴切面扫查,观察CS的特点。结果胎儿CS矢状切面面积与孕周呈正相关(正常组:r=0.954,P0.05;CS组:r=0.904,P0.05),同孕周正常组胎儿CS矢状切面面积小于CS组(P均0.01)。产前超声诊断72胎CS扩张的胎儿中,52胎为永存左上腔静脉,15胎为完全型肺静脉异位引流,5胎右心压力负荷增大。结论胎儿CS矢状切面的面积与孕周呈正相关;胎儿心脏超声检查发现CS扩张时应同时考虑其他心内畸形;临床应通过多切面、多角度扫查,分析和诊断引起CS扩张的病因。     

右位心的超声心动图诊断

本文报告了11例右位心患者的切面超声心动图和声学造影检查结果。根据右房位置将右位心分为孤立型、反转型和未定型三种类型,提出了右位心超声心动图的诊断疗法和注意事项。     

先天性心脏病合并右位主动脉弓的诊断与治疗

目的 探讨先天性心脏病合并右位主动脉弓的诊断与治疗。 方法 回顾性分析2012年1月1日至2013年4月1日北京儿童医院小儿心脏中心27例先天性心脏病合并右位主动脉弓行外科手术治疗患儿的临床资料,其中男20例,女7例;年龄（10.96±12.08） 个月,体重（7.70±3.13） kg。法洛四联症14例,室间隔缺损9例,动脉导管未闭1例;肺动脉吊带1例;单纯双主动脉弓2例;均合并右位主动脉弓。其中13例合并血管环畸形,包括迷走左锁骨下动脉畸形和双主动脉弓畸形。所有患儿均接受手术治疗,在矫治心内畸形的同时,进行左锁骨下动脉移植,矫治迷走左锁骨下动脉畸形,双主动脉弓主要是切断一侧非优势弓。 结果 围术期死亡3例,其中1例术后无法停体外循环死亡,1例未合并血管环的患儿术后7 d死亡,1例合并双主动脉弓患儿术后并发急性呼吸窘迫综合征（ARDS） 死亡;1例放弃治疗。随访23例,随访时间3～17个月,心脏超声心动图提示心内畸形矫治满意,恢复良好,双侧上肢动脉波动良好。 结论 对先天性心脏病合并右位主动脉弓患者行外科矫治时应慎重,术前尽量完善相关检查,选择增强CT、磁共振成像检查,明确诊断,制定手术计划。一期同期矫治血管环畸形及其它先天性心脏病,手术效果满意,近期效果良好。     

超声诊断胎儿支气管囊肿1例

<正>孕妇26岁,孕1产0,既往体健,本次怀孕无药物接触史,妊娠初期有感冒史。孕32周胎儿超声检查:胎儿双顶径7.81cm,股骨长5.32cm,羊水指数12.03cm,肝、胃、肾脏、膀胱及四肢正常;超声心动图于胎儿右心室流出道切面动脉导管与左肺动脉间探及2.22cm×1.73cm囊性包块,壁薄、边界清晰、内部透声良好(图1A),CDFI未见明显血流信号(图1B);气管-左右主支气管冠状切面扫查示囊性包块位于气管隆突下,主动脉弓切面扫查示囊性包块位于主动脉弓下(图1C)。超声诊     

镜面右位心合并复合畸形一例

患者女,11岁。3岁时出现活动后心悸、气促、轻度紫绀。查体心尖搏动位于右锁骨中线第5肋间外侧7.5 cm,胸骨右缘外侧第3、4肋间可见2.0 cm×3.0 cm搏动区,心尖部及胸骨右缘可触及震颤,叩诊心界向右侧扩大,心尖部及胸骨右缘第2肋间可闻及4/6级收缩期喷射性杂音,向颈背部传导,P2消失。超声心动图提示:镜面右位心、右心室双出口、双向上腔静脉、肺动脉口重度狭窄、室间隔缺损(V SD)、房间隔缺损(A SD);16层CT血管成像(CTA)检查示:镜面右位心、双上腔静脉、V SD、A SD、右心室双出口、右心室流出道狭窄和肺动脉发育良好;胸部X线片示:右位…     

产前超声诊断胎儿动脉导管提前闭合1例

正孕妇37岁,单胎妊娠,孕37周,常规产检超声发现胎儿右心增大;既往孕1产0,无特殊病史,否认近期用药史,近5天日均进食葡萄约500g。胎儿超声:四腔心切面(图1A)见心胸比0.46,右心增大,右心室壁运动良好,有极少量心包积液;三尖瓣口见瓣叶活动,血流暗淡,流速46cm/s,关闭时大量反流,反流束约占右心房面积50%,反流速度2.9m/s;卵圆孔增大,直径8.4mm,并见右向左分流;三血管切面见动脉导管呈沙漏样,主动脉端纤细,直径仅1.2mm,CDFI未见血流信号通过(图1B),频谱未测及;肺动脉增宽,与主动脉比值约1.5,见窄带样低速高阻型频谱,流速29cm/s;静脉导管a波反向;超声心动图心衰评分8分。     

Castor支架治疗右位心、迷走右锁骨下动脉合并Stanford B型主动脉夹层1例

右位心、迷走右锁骨下动脉合并Stanford B型主动脉夹层罕见。我们采用Castor分支型支架对1例81岁女性患者实施主动脉腔内隔绝获得成功。与常规B型夹层的主动脉腔内隔绝术比较, 术前应通过主动脉CTA、超声等明确右位心、主动脉及其分支、尤其迷走右锁骨下动脉解剖、夹层的破口位置及其比邻;通过术前影像学以及术中造影寻找出充分展现主动脉弓部形态及其分支的投射角, 并由熟练掌握心血管介入技术的医师完成是手术成功的关键。     

Saccular aortic aneurysm that resembled a mediastinal neoplasm

INTRODUCTIONSaccular aortic arch aneurysms in unusual sites may be misdiagnosed as a neoplasm. We present the case of a rare saccular aortic arch aneurysm between trachea and esophagus that resembled a mediastinal neoplasm in the preoperative findings.PRESENTATION OF CASEA 63-year-old male with an abnormal mediastinal shadow on chest X-ray was referred to the hospital. An axial plain computed tomogram of the chest revealed mediastinal soft tissue next to the right side of the aortic arch resembling a neoplasm originating from the gap between the trachea and the esophagus. The coronal view constructed by enhanced 64-row multi detector computed tomography revealed the soft tissue was an aneurysm arising from the inner side of the aortic arch. An aortic arch replacement was performed via a median sternotomy.DISCUSSIONA thoracic aortic aneurysm sometimes behaves like a mediastinal neoplasm. The multiple cross-sectional image from multidetector computed tomography was useful for the correct diagnosis of such an aneurysm.CONCLUSIONThe possibility of an aneurysm should be considered whenever a mass in contact with the aortic wall is identified.     

Repair of aortic coarctation and aneurysm of ductus arteriosus in the presence of right-sided arch and descending aorta

Association of right aortic arch and coarctation of the aorta is rare. A patient with aortic coarctation was presented to our clinic. Detailed radiological work-up revealed aortic coarctation, aneurysm of ductus arteriosus, mirror image brachiocephalic vessels, right-sided arch, and right-sided descending aorta. The patient underwent successful operative repair through a right thoracotomy. She is normotensive and doing well two years postoperatively.     

Saccular aneurysm in the right-sided aortic arch: A successfully corrected case

We report the surgical treatment of a rare case of true aortic aneurysm in a right-sided aortic arch. A 49-year-old female patient with obstructive respiratory problems demonstrated a true aneurysm with a diameter of 58 mm located at the right-sided aortic arch between the right carotid artery and right subclavian artery. Surgery was successfully performed by replacing the arch including the aneurysm with a prosthetic graft. The positions of true aneurysms in the right-sided aortic arch can be divided into two subtypes: first, the transverse arch between the right carotid artery and right subclavian artery, and second, the base of the subclavian artery, the Kommerell's diverticlum. The region is informative for consideration of the surgical approach toward aneurysms of this entity. (J Vasc Surg 1997;25:941-4.)     

General anesthesia in a case of right-sided aortic arch with Kommerell's diverticulum diagnosed on preoperative examination

Kommerell's diverticulum is a rare anomaly of the aortic arch. A 59-year-old man was scheduled for open reduction and internal fixation of his right proximal tibial fracture under general anesthesia. We diagnosed right-sided aortic arch by the chest X-ray and thoracic computed tomography. His trachea and esophagus were compressed by the aortic arch. He had complained of no dyspnea or dysphagia. Respiratory difficulty might be caused by muscle relaxants, intermittent positive pressure ventilation, change of intrathoracic pressure, postural change and overloaded infusion during general anesthesia in a case of right-sided aortic arch. We performed lumbar epidural anesthesia and inserted an i-gel after general anesthesia induction preserving spontaneous respiration in preparation for controlled ventilation or tracheal intubation via an i-gel. We could accomplish the operation uneventfully and he was discharged on POD 53. A supraglottic airway such as an i-gel was a useful device in the present case of right-sided aortic arch with Kommerell's diverticulum.     

Right-sided double aortic arch in an adult

A 27-year-old woman complained of a severe stridor caused by a right-sided double aortic arch with a right-sided descending thoracic aorta. A smaller left-sided aortic arch had an atretic segment located between the left common carotid artery and an aortic diverticulum from which the left subclavian artery originated. Through a left fourth thoracotomy, the atretic segment, which caused a compression of the trachea and esophagus, was ligated and divided. The ligamentum arteriosum could not be identified on that side. After the operation she was completely relieved of her symptoms.     

Thoracoscopic patch repair of a right-sided congenital diaphragmatic hernia in a neonate

Purpose  Minimally invasive techniques continue to expand in pediatric surgery; however, there has been some debate over the appropriate operative technique for the management of congenital diaphragmatic hernias in neonates [1–3]. We present a video of a thoracoscopic patch repair of a right-sided Bochdalek congenital diaphragmatic hernia (CDH) in a 3-day-old male. Methods  Our patient was noted to have a right-sided CDH on chest X-ray following respiratory distress at the time of birth. The patient’s remaining neonatal workup also confirmed hypoplastic transverse aortic arch with coarctation, ventricular septal defect (VSD), and patent ductus arteriosus, which were initially diagnosed by prenatal ultrasound. After monitoring the patient for hemodynamic stability and discussion with the family and involved pediatric cardiothoracic surgeons, the decision was made to proceed with a thoracoscopic repair of the CDH. Results  The large right-sided CDH was noted to involve herniated small bowel, colon, and liver. The diaphragmatic defect was successfully repaired thoracoscopically using a 5 × 5 cm polytetrafluoroethylene (PTFE) patch. The patient was extubated on the second postoperative day and ultimately underwent aortic arch augmentation, VSD closure, and patent ductus arteriosus ligation and division at 1 month of age. There has been no evidence of CDH recurrence in follow-up. Conclusions  As demonstrated by our video, large right-sided congenital diaphragmatic hernias requiring patch repair can be successfully repaired thoracoscopically with appropriate surgeon comfort and experience. This minimally invasive approach may also be used in neonates with associated cardiac defects with appropriate cardiothoracic surgical consultation and support. To our knowledge this is the first reported case of a thoracoscopic repair of a Bochdalek (posterolateral) hernia with a prosthetic patch in a neonate with significant congenital cardiac anomalies. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Surgical treatment of vascular ring including right cervical aortic arch

Cervical aortic arch is a developmental entity consisting of persistence of the right or left third branchial arch and regression of the fourth branchial arches. In most cases, the aorta is redundant and crosses behind the esophagus to the opposite side. In the presence of an aberrant subclavian artery contralateral to the side of the aortic arch and a ligamentum arteriosum, a vascular ring is formed around the trachea and esophagus. Two young patients with right-sided cervical aortic arch, aberrant left subclavian artery, and ligamentum arteriosum presented with dysphagia and exertional dyspnea. In one patient, through a left thoracotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were dissected thoroughly above and below the level of the ring. In addition, the aberrant left subclavian artery was divided at its origin from a large diverticulum and implanted into the left common carotid artery; the aortic diverticulum was resected. In the other patient, who had associated 22q11 chromosomal deletion, in addition to left-sided compression of the trachea and esophagus, there was additional marked compression of the right anterolateral trachea by the redundant ascending aorta. Through a median sternotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were widely mobilized; an additional aortopexy of the ascending aorta to the right of the sternum resulted in the absence of tracheal compression. The cases of the two reported patients illustrate the clinical variability of vascular ring, including a right cervical aortic arch and the consequently versatile surgical approach that is needed to successfully address this combination of vascular anomalies.     

Right-sided patent ductus arteriosus with a right aortic arch and right descending aorta]

We experienced an extremely rare anomaly, i.e. right-sided persistent ductus arteriosus with a right aortic arch and right descending aorta. Reviewing the literature, we found only two cases clinically reported in Japan as far as we know. The diagnosis was established by angiography and MRI. We treated the patient successfully through right thoracotomy.     

Airway management in an infant with double aortic arch

A 2-month old male was admitted due to repeated cyanotic attacks. He had suffered from stridor and retractive breathing since birth. Double aortic arch was diagnosed and the vascular ring formed by the double aortic arch was compressing the trachea. Multirow detector computed tomography showed that he had a right-dominant double aortic arch with left ductus arteriosus and an aberrant left subclavian artery, and that the narrowest part of the trachea, where the diameter was 2.0 mm, was located 9.0 mm above the carina. Airway management in patients with extreme narrowing of the trachea is challenging for anesthesiologists. He was scheduled for ligation and division of the left aortic arch and ductus arteriosus. In the operating theater, anesthesia was slowly induced with sevoflurane (0–4%) in oxygen. After mask ventilation was confirmed to be adequate, a 4.0 mm internal diameter endotracheal tube (ETT) was inserted and advanced smoothly beyond the tracheal stenosis. The tip of the ETT was placed just above the carina using a fiber optic bronchoscope (fiberscope) that was passed through the ETT. Since mechanical ventilation was adequate, vecuronium was administered. Surgery was conducted in the right lateral position and using a left thoracotomy approach. Anesthesia was maintained with sevoflurane (2–3%). After positioning, right one-lung ventilation was performed unexpectedly. However, anesthetic management was achieved without difficult ventilation during surgery. The tip of the ETT was pulled past the stenotic part before transfer to the intensive care unit (ICU). A patent trachea during spontaneous breathing under CPAP (5 and 2 cmH₂O) was confirmed with a bronchofiberscope in the ICU. After weaning from mechanical ventilation, he had the persistence of mild stridor despite improvement of respiratory symptoms.