Prenatal Diagnosis of a Retroesophageal Left Brachiocephalic Vein: Two Case Reports

A retroesophageal left brachiocephalic vein is an extremely rare anomaly and has only been reported in 6 postnatal cases. Two prenatally diagnosed cases are reported. On the 3‐vessel view, the vein appears as an aberrant vessel transversely coursing behind the aorta and trachea, which subsequently drains into the superior vena cava, giving rise to a U‐shaped configuration. On color Doppler sonography, the U sign is bicolored. This anomaly should prompt the sonographer to carefully assess for other congenital heart defects, suggest consideration for genetic testing, and alert the cardiologist because it could affect central line procedures and cardiac interventions after delivery.     

Prenatal Diagnosis of the Fetal Retroesophageal Left Brachiocephalic Vein: Case Series and Review of the Literature

A retroesophageal left brachiocephalic vein (LBCV) is a highly rare anomaly. We retrospectively analyzed 7 cases of a retroesophageal LBCV that were prenatally diagnosed from a database of fetal echocardiogram of 31,356 cases. The 3-vessel view and the long-axis view are the main views for confirming a fetal retroesophageal LBCV. An isolated retroesophageal LBCV is rare, and it is typically associated with congenital heart defects, especially conotruncal defects and a right aortic arch. An isolated fetal retroesophageal LBCV has a good prognosis and does not need surgical treatment.     

A Simple Rule for Prenatal Diagnosis of Total Anomalous Pulmonary Venous Return

The objective of this series was to describe a simple rule for prenatal diagnosis of total anomalous pulmonary venous return (TAPVR). Fourteen fetuses had a prenatal diagnosis of TAPVR by the simple rule, including the following components: (1) the major criterion, which was the absence of a connection between the pulmonary vein and the left atrium; and (2) at least 1 of the following minor criteria: (a) the presence of a vascular confluence behind the atria, (b) abnormal spectral Doppler waveforms in the pulmonary veins, (c) a smooth posterior wall of the left atrium, (d) increased retroatrial space, (e) a dilated coronary sinus (cardiac type), (f) a dilated superior vena cava or brachiocephalic vein, and (g) an additional vessel on the 3‐vessel/3‐vessel and trachea view or a vertical descending vein. All were accurately diagnosed, and none were missed by the diagnosis. In summary, the simple rule described is helpful in increasing the number of accurate prenatal diagnoses of TAPVR.     

Defibrillator Implantation in a Patient with a Persistent Left Superior Vena Cava

The implantation of a transvenous cardioverter defibrillator (PCD 7217B) was performed in a patient with a persistent left superior vena cava. The defibrillation electrodes were positioned in the right ventricle and the superior vena cava via the right subclavian vein. A subcutaneous patch had to be implanted at the left lateral chest wall to achieve sufficient defibrillation thresholds. Three weeks later the system had to be removed because of a generator pocket infection. During the second implantation we placed one electrode in the persistent left superior vena cava perpendicular to the electrode in the right ventricle. Using this configuration transvenous defibrillation was possible without an additional subcutaneous patch.     

Asymptomatic partial anomalous pulmonary venous return masquerading as pulmonary vein occlusion following radiofrequency ablation

Recent advances in the field of cardiac electrophysiology have resulted in the rapid growth of radiofrequency ablation for treatment of arrhythmias. Pulmonary vein stenosis or occlusion is a rare, but well described adverse outcome. Fortunately, the concomitant evolution of multislice computed tomography (CT) scanners has provided an excellent non-invasive method of monitoring for this complication. We recently encountered a case on multislice CT that initially appeared to be a pulmonary vein occlusion related to the procedure, but on further evaluation was found to be an asymptomatic case of partial anomalous pulmonary venous return.     

Totally anomalous pulmonary venous connection and complex congenital heart disease: prenatal echocardiographic diagnosis and prognosis.

OBJECTIVE: The purpose of this study was to determine the accuracy of prenatal cardiac diagnosis, prognosis, and outcome of totally anomalous pulmonary venous connection (TAPVC) and to determine echocardiographic clues in the prenatal diagnosis of isolated TAPVC or TAPVC in association with other complex congenital heart disease (CHD). METHODS: We reviewed our 13-year experience of prenatal diagnosis of TAPVC. Thirteen fetuses were identified with the diagnoses of TAPVC. We systematically analyzed the individual pulmonary veins by color and pulsed Doppler imaging, the presence of a pulmonary venous confluence, the pulsed and color Doppler evaluation of the vertical vein, and sites of connections. Prenatal diagnosis was confirmed by postnatal echocardiography, cardiac catheterization, surgery, or autopsy. RESULTS: The mean gestational age at diagnosis of TAPVC was 26.3 weeks (range, 20-33 weeks). There were 8 fetuses with TAPVC and right isomerism, 3 fetuses with other associated CHD, and 2 with isolated TAPVC. There were 7 fetuses with supracardiac TAPVC, 4 with infracardiac TAPVC, and 2 with mixed TAPVC. Pulmonary vein color and pulsed Doppler data were available in 10 of 13 fetuses. The pulmonary venous confluence was visualized in all fetuses except 1. The vertical vein was visualized in all fetuses. Five fetuses had suspected signs of obstruction. The diagnosis was confirmed postnatally or at autopsy in 12 cases. Eight patients underwent surgery; 6 died, and 2 were alive. Two patients had compassionate care and died; 3 pregnancies were terminated. CONCLUSIONS: It is possible to diagnose accurately complex CHD, including the pulmonary venous connections. When diagnosed prenatally, TAPVC carries a poor prognosis.     

孤立性小腿深静脉血栓的超声诊断

目的　探讨孤立性小腿深静脉血栓的声像图特点及超声诊断方法。方法　应用彩色多普勒超声检查小腿肌肉内深静脉 (比目鱼肌静脉、腓肠肌静脉 )、胫后静脉、腓静脉、胫前静脉 ,以及静脉以上近端深静脉是否有血栓形成。结果　检出的 13例孤立性小腿深静脉血栓中 ,77%为小腿肌肉内深静脉受累 ,少数为腓静脉及胫后静脉受累 ,未发现胫前静脉单独受累者 ;比目鱼肌内的单支静脉血栓为最常见的类型 ,且半数合并近端深浅静脉返流。结论　超声检查能够有效地检出孤立性小腿深静脉血栓病变 ,小腿深静脉特别是肌肉内深静脉应包括在下肢深静脉检查常规之中。     

Isolated Fetal Intra‐Abdominal Umbilical Vein Varix

This series describes a single center's experience in follow‐up and management of fetuses with an isolated fetal intra‐abdominal umbilical vein varix. All cases with a fetal intra‐abdominal umbilical vein varix that were diagnosed or referred to our medical center over 15 years were followed and managed. The definition of a fetal intra‐abdominal umbilical vein varix used was a segment dilated to 9 mm or greater or at least 50% wider than the diameter of the adjacent umbilical vein. Over the 15‐year period, our center had approximately 65,000 births with 28 cases of isolated fetal intra‐abdominal umbilical vein varices: a prevalence rate of 1 case per 2300 births. Three of the 28 cases (10.7%) had intrauterine growth restriction. Five of 30 fetuses (17%) showed turbulent flow in the varix. We had no cases of intrauterine fetal death, and 27 of the 28 neonates had good outcomes. In contrary to earlier reports, we found that when a fetal intra‐abdominal umbilical vein varix is isolated, a good fetal outcome is expected. On the basis of our experience, we have changed our policy and do not recommend inducing preterm labor. Nevertheless, close fetal surveillance until delivery is warranted.     

3个月龄患儿完全性肺静脉异位引流的围术期护理

目的探讨3个月龄患儿完全性肺静脉异位引流围术期的护理,提高术前术后的监护水平及护理问题的解决方法。方法本院完成了27例3个月以内的完全性肺静脉异位引流患儿在低温体外循环下矫治术,术后通过维护循环功能的稳定,预防低心排和心包填塞;采取综合措施预防肺动脉高压;积极防治呼吸机相关性肺炎以及术后体温维持、营养支持等治疗护理措施。结果本组5例患儿延迟关胸,术后21～56 h在床边顺利关胸,其中术后并发肺出血1例,经应用东莨菪碱治疗、增加呼吸机PEEP＋5～＋8 H2O, 加强呼吸道管理后好转并治愈;另有4例患儿发生肺部感染,经抗感染后痊愈;无1例出现严重心律失常（可能与手术方式有关）; 27例患儿除1例术后21 h因低心排抢救无效死亡,其余26例经精心治疗和护理,均痊愈出院。结论3个月以内完全性肺静脉异位引流患儿病情严重,术后护理难度大,通过精心护理可明显提高低龄患儿手术成功率和治愈率,降低术后并发症。     

Two-to-one Conduction Block Between Left Atrium and Right Lower Pulmonary Vein Preceding Complete Vein Isolation During Radiofrequency Current Ablation

A 56-year-old patient with paroxysmal atrial fibrillation who developed a transient 2:1 block between the left atrium and right inferior pulmonary vein during a single application of radiofrequency current was described. The production of transient and complete atriovenous block by a single application of radiofrequency current demonstrates that a single connection between the pulmonary veins muscle and the left atrium may exist. (PACE 2004; 27[Pt. I]:829–830)     

彩色多普勒超声心动图诊断新生儿心下型肺静脉异位引流

目的 探讨彩色多普勒超声心动图在心下型肺静脉异位引流诊断中的价值。方法 患者为３例出生６天、７天及２５天的新生儿。采用Ｈｐ Ｓｏｎｏｓ５５００型彩色多普勒超声诊断仪,探头频率１２ＭＨｚ,经胸行二维超声心动图及彩色多普勒血流图检测。结果 心下型肺静脉异位引流的超声心图特征为左房明显小,房水平右向左分流,其后方见肺总静脉、与左房无交通,追踪其瞳向右下穿过膈肌、于肝右叶后方入门静脉或肝静脉。剑下主动脉短     

产前超声测量正常胎儿左头臂静脉

目的 应用产前超声观察胎儿左头臂静脉,建立正常胎儿左头臂静脉管径的正常参考值范围。方法 选取384胎23~39周正常胎儿,应用二维超声显示胎儿左头臂静脉,对能够清晰显示者测量管径,并分析其与孕周的关系,对二维超声显示不明确者应用CDFI进一步进行检测。结果 二维超声对胎儿左头臂静脉的显示率为88.54%（340/384）,联合CDFI对胎儿左头臂静脉的显示率为91.93%（353/384）。正常胎儿左头臂静脉管径与孕周呈直线相关（r²=0.95、P<0.001）。以孕周为自变量（X）、左头臂静脉管径为因变量（Y）,直线回归方程为Y=-1.063+0.152X。结论 产前超声能够显示并测量胎儿左头臂静脉,有助于评价胎儿心血管发育。     

产前超声诊断胎儿食管后左头臂静脉

目的 观察产前超声诊断胎儿食管后左头臂静脉（RELBCV）的价值。方法 回顾性分析10胎产前超声诊断RELBCV胎儿，观察其超声表现、伴发畸形、染色体异常及妊娠结局等；结合产后随访结果分析产前超声诊断RELBCV的效能。结果 产前超声检出单纯RELBCV 5胎，RELBCV伴心内畸形3胎，伴心外畸形2胎。9胎RELBCV超声三血管气管（3VT）切面图像见左头臂静脉（LBCV）走行于食管和气管后方，与扩张的奇静脉汇合后汇入上腔静脉而形成"U"形血管环；1胎RELBCV见LBCV分为2支，前支较纤细，走行于主动脉弓上方，后支走行于主动脉弓平面食管后方，均汇入上腔静脉。6胎接受羊膜腔穿刺染色体核型及基因组疾病检测，1胎致病性染色体微结构异常，1胎染色体缺失，4胎均未见明显异常。4胎接受无创DNA检测，结果均提示非整倍体异常风险较低。7胎正常出生后经超声心动图证实RELBCV。结论 胎儿RELBVC产前超声表现为LBCV走行于食管或气管后方，与奇静脉汇合后流入上腔静脉，形成"U"形血管环；产前超声检出胎儿RELBCV时，应全面筛查是否伴有其他系统异常，必要时行产前基因组疾病检测。     

Prenatal Diagnosis of Isolated Right Pulmonary Agenesis Using Sonography Alone

Pulmonary agenesis is a rare congenital anomaly, estimated to complicate around 1 per 15,000 pregnancies, in which there is complete absence or severe hypoplasia of one or both lungs, frequently associated with other abnormalities. A prospective prenatal diagnosis is a challenge, and a substantial proportion of cases are diagnosed by fetal magnetic resonance imaging, postnatal computed tomography, or postmortem. Thus, there are only a few reported cases of prenatal diagnosis in the literature. We report the prenatal diagnosis of isolated right lung agenesis diagnosed with sonography alone at a relatively early gestational age. We also present a systematic review of the literature for this condition to accompany this case study.