Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies

We report a case of a healthy, asymptomatic 6-year-old boy in whom an anomalous right pulmonary vein was noted to drain into both the inferior vena cava and left atrium in association with findings consistent with scimitar syndrome. The anomalous pulmonary vein took a very circuitous route through the lungs before draining into the left atrium, a condition previously termed "meandering pulmonary vein." To aid in the diagnosis, cardiovascular magnetic resonance imaging and magnetic resonance angiography were used to delineate this complex course and the connection of the anomalous pulmonary vein. To our knowledge, this is the 1st reported case of a meandering pulmonary vein with dual drainage to the inferior vena cava and left atrium in association with other anomalies.     

Asymptomatic Congenital Atresia of the Left Inferior Pulmonary Vein: A Case Report

We present an unusual fortuitously discovered case of atresia of the left inferior pulmonary vein with drainage via anastomosis to the left upper pulmonary vein and the left atrium. Atresia of the pulmonary veins is a very rare anomaly that results from defective incorporation of the pulmonary venous system into the left atrium. The initial diagnosis, drawn from plain radiographs, was confirmed by cardiac catheterization and pulmonary angiography. The appearance of clinical signs, prognosis, and the need for treatment depend on how well substitute drainage is performed.     

Diagnosis, management, and follow-up of systemic venous drainage via a single left superior caval vein into the left atrium

A 15-month-old boy presented with asymptomatic hypoxaemia due to right-to-left venous shunting via a left superior caval vein emptying into the left atrium, in absence of right superior caval vein. The diagnosis, suspected by contrast echocardiography, was confirmed by computed tomography and angiography. The child underwent surgical correction of the systemic anomalous return by tunnelling the left superior caval vein towards the right atrium. An asymptomatic narrowing inside the intra-atrial baffle developed 6 months later.     

Extension of bronchogenic carcinoma through pulmonary vein into the left atrium detected by echocardiography

This is the case of a 46-year-old female recently diagnosed with a squamous cell bronchogenic carcinoma that spread through the pulmonary veins into the left atrium. This mass was initially seen on surface echocardiography as emanating from the pulmonary vein and subsequently confirmed to be arising from the right superior pulmonary vein by transesophageal echocardiography.     

The angiographic diagnosis of the connection of the persistent left vena cava superior to the left atrium by the absence of the roof of the coronary sinus in a child with a Holmes' heart

We describe a patient with angiographic findings of a left superior persistent vena cava draining in the left atrium with absence of the coronary sinus' roof and coexisting with Holmes' heart. The diagnosis can be made by selective angiography in the left atrium, pulmonary vein or at the left superior vena cava. We want to emphasize the importance of knowing this sort of anomaly before surgical treatment.     

房性心动过速的射频消融治疗

探讨房性心动过速 (简称房速 )的电生理机制、标测方法及射频消融结果。 2 3例房速 :右房房速 15例 ;左房房速 8例 ,其中左上肺静脉口房速 3例、右上肺静脉口房速 2例、左下肺静脉口下方房速 1例、右下肺静脉口下方房速 2例。结果 :15例右房房速成功 13例 ,成功率为 87% ;左房房速 8例全部成功 ,成功率为 10 0 % ,总成功率为91.3%。成功消融靶点的A波较体表心电图P′波提前 44± 6ms。随访 2～ 36个月 ,无复发。结论 :射频消融治疗房速 (包括左房非经典部位房速 )是一种安全有效的方法     

Two‐ and four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation in the diagnosis of fetal isolated partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare malformation. We describe a case of PAPVC, in which the left pulmonary veins coursed to the left innominate vein through a vertical vein and finally drained into the right superior vena cava; the right pulmonary veins were connected to the left atrium. Tracing the origin and destination of abnormal vessels presented at the three‐vessel and trachea view is useful for the diagnosis. Four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation facilitates the identification of the drainage of fetal pulmonary veins, which should be considered as a complementary modality in obstetric ultrasonic examination when cardiac abnormalities are suspected.     

Anomalous unilateral single pulmonary vein mimicking partial anomalous pulmonary venous return

We report a case in which the clinical findings of a partial anomalous pulmonary venous return were present but with termination of the anomalous pulmonary vein into the left atrium. There was hypoplasia of the right lung, dextroposition of the heart, and a loop-like vascular density in the right lung field. Cardiac catheterization revealed no shunt, and on pulmonary angiograms, a large common right pulmonary vein draining the entire right lung and entering the left atrium by way of a single orifice. Possible embryologic explanations include atresia of the right inferior pulmonary vein and persistence of the ipsilateral superior pulmonary vein.     

Identification of left atrial origin of ectopic tachycardia during right atrial mapping: analysis of double potentials at the posteromedial right atrium

INTRODUCTION: The high posteromedial right atrium is adjacent to the left atrium near the right superior pulmonary vein. We hypothesized that analysis of electrograms at this site could distinguish left from right atrial tachycardia. METHODS AND RESULTS: Atrial mapping was performed in 16 patients with left atrial origin ectopic tachycardia (11 patients with right superior pulmonary vein origin and 5 patients with other left atrial tachycardias). During left atrial tachycardia, earliest right atrial activation was recorded at the high posterior right atrium in 14 of 16 patients. At all of these 14 early sites, double potentials were recorded during tachycardia. The first potential was a far-field signal from left atrium as indicated by the following: (1) during sinus beats, the timing of the two potentials reversed such that the left atrial one was late; (2) ablation at the right atrial site did not decrease the amplitude of the first potential, but did decrease the amplitude of the second potential; and (3) the timing of activation at the adjacent left atrium agreed with that of the first potential. In the 11 right superior pulmonary vein tachycardias, the first potential was markedly earlier than the p wave onset, but in left atrial tachycardias with other origins it was later. In a control group of six patients with pacing to simulate right atrial tachycardia, double potentials were recorded in the posterior right atrium, but the timing of components did not reverse during sinus rhythm. CONCLUSION: For some left atrial ectopic tachycardias, particularly those originating from the right superior pulmonary vein, recognition of left versus right atrial origin can be accomplished during right atrial mapping by analysis of double potentials in the posteromedial right atrium.     

Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD. None of them had elevated pulmonary pressure. The lesion, suggested by clinical findings, was diagnosed either at angiography, which in four cases demonstrated an abnormal pulmonary venous return, or at colour-coded doppler echocardiogram, or at surgery. In every case, surgical correction consisted of closure of the often low-sited ADS by a patch which diverted the inferior vena cava into the right atrium and the abnormal venous return towards the left atrium. The short--and long-term results of surgery were excellent. The authors review the literature concerning this unusual association of ASD with an abnormal drainage of the inferior vena cava into the left atrium.     

Pulmonary vein leiomyosarcoma extending into the left atrium

In this paper, the authors report the case of a 28-year-old man with pulmonary vein leiomyosarcoma presenting subacute respiratory distress. Thoracic computed tomography and transoesophagal ultrasonographic examination of the heart suggested the diagnosis of a heart tumour revealed by the obstruction of the mitral valve and pulmonary oedema. Emergency cardiac surgery revealed the mass to be a leiomyosarcoma, probably extending from the right inferior pulmonary vein and extending into the left atrium. The clinical evolution was complicated because of a sudden local relapse. The patient underwent a second cardiac intervention involving lower right lobectomy followed by adjuvant chemotherapy with an ifosfamide-adriamycin combination. This treatment failed to control the disease and a third cardiac intervention was necessary with second-line gemcitabine-paclitaxel adjuvant chemotherapy. Further recurrences were observed with metastases first in the scalp and then in the spine and adrenal glands leading to the death of the patient 2 years after the diagnosis.     

Bronchogenic cyst causing atrial fibrillation by impinging the right inferior pulmonary vein

Atrial fibrillation is the most common sustained cardiac arrhythmia and is usually associated with underlying structural heart disease, but may also occur in isolation--the entity of "lone" atrial fibrillation. Recently, attention has been directed to the pulmonary veins as a source of the arrhythmia through identification of rapidly firing ectopic foci within the covering myocardial sleeves. We describe a 38-year-old man who presented with treatment-resistant atrial fibrillation and a posterior mediastinal mass. Electrophysiological studies demonstrated abnormal foci of electrical activity at the entrance of the right inferior pulmonary vein into the left atrium. Surgical exploration revealed a bronchogenic cyst that distorted and stretched the right inferior pulmonary vein as it traversed the posterior mediastinum towards the left atrium. Restoration of sinus rhythm without recurrence of atrial fibrillation characterized the clinical course after surgical resection of the mass. This case demonstrates that a retro-cardiac bronchogenic cyst can cause atrial fibrillation by impinging on a pulmonary vein.     

64排螺旋CT心脏扫描在显示非心房颤动患者肺静脉和左心房结构中的价值

目的探讨64排螺旋CT心电门控血管造影在显示非心房颤动患者肺静脉和左心房结构中的价值。方法回顾性分析2007年8～12月160例行64排螺旋CT心电门控心脏血管造影患者的肺静脉和左心房解剖结构,利用工作站后处理,通过最大密度投影(MIP)、多平面重组(MPR)及容积再现(VR)图像了解肺静脉数目、开口大小,左心房囊样结构,并评价其临床应用价值。结果160例患者共发现肺静脉642根。3支肺静脉者8例(5.00%),其中左侧共干者4例,右侧共干者4例;5支肺静脉者10例(6.25%),其中左侧3支者6例,右侧3支者4例;4支肺静脉者142例(88.75%)。左上肺静脉最大径为(24.30±4.54)mm、最小径为(17.76±4.24)mm、开口面积为(352.92±124.11)mm~2;左下肺静脉最大径为(19.10±4.45)mm、最小径为(12.27±3.52)mm、开口面积为(201.96±93.65)mm~2;右上肺静脉最大径为(22.99±5.04)mm、最小径为(16.19±4.87)mm、开口面积为(316.53±144.20)mm~2;右下肺静脉最大径为(18.63±4.60)mm、最小径为(14.46±3.48)mm、开口面积为(219.20±95.85)mm~2。有左心房囊样结构者25例(15.63%),其中男性21例,女性4例,年龄38～74岁,平均57岁。25例患者共发现囊样结构29个(有4例存在2个囊样结构),19个(65.51%)位于左心房右顶壁,6个为Ⅰ型,13个为Ⅱ型;6个(20.69%)位于左顶壁,3个为Ⅰ型,3个为Ⅱ型;4个(13.80%)位于左心房左下壁,3个为Ⅰ型,1个为Ⅱ型;未发现位于右下壁者。结论64排螺旋CT心电门控心脏扫描能够很好地显示肺静脉的数目、开口大小,左心房囊样结构,为研究肺静脉和左心房提供了一个很好的工具。     

Atrial natriuretic polypeptide is removed by the lungs and released into the left atrium as well as the right atrium in humans

To examine the sites of release and removal of plasma atrial natriuretic polypeptide plasma levels in the femoral vein, right atrium, pulmonary artery, pulmonary capillary bed, left atrium and aortic root were measured in 11 control subjects and 22 patients with mitral stenosis. Mean plasma natriuretic polypeptide levels in the femoral vein, right atrium, pulmonary artery, pulmonary capillary bed, left atrium and aortic root were, respectively, 64 +/- 29, 124 +/- 72, 103 +/- 44, 83 +/- 30, 106 +/- 46 and 101 +/- 35 pg/ml in the control subjects and 321 +/- 170, 500 +/- 234, 458 +/- 266, 356 +/- 209, 434 +/- 222 and 432 +/- 217 pg/ml in the patients with mitral stenosis. In both the control subjects and the patients with mitral stenosis, there was a significant increase between the femoral vein and the right atrium and between the pulmonary capillary bed and the left atrium and a significant decrease between the pulmonary artery and the pulmonary capillary bed. Blood samples were also taken simultaneously from the pulmonary vein and the pulmonary capillary bed, as well as from the pulmonary artery and the left atrium, in 25 patients (11 control subjects, 5 patients with mitral stenosis and 9 patients with atrial septal defect). There was no difference in plasma atrial natriuretic polypeptide levels between the pulmonary capillary bed and the pulmonary vein in these 25 patients. It is concluded that atrial natriuretic polypeptide 1) is released into the left as well as the right atrium, and 2) is removed by the lungs.     

犬肺静脉肌袖电生理随龄性改变

目的对比研究老龄和成年犬肺静脉肌袖电生理特性.方法采用经导管房间隔穿刺技术,应用程序刺激和短阵快速起搏分别对正常自然老龄和成年实验犬肺静脉、上腔静脉、左房、右房、以及冠状静脉窦的有效不应期、激动传导及心房颤动诱发阈值进行标测.结果老龄犬的左、右心房,上腔静脉,冠状静脉窦和肺静脉的有效不应期较成年犬均有不同程度的缩短,其中左心房,上腔静脉和肺静脉有效不应期的随龄性变化有统计学差异(P＜0.01).老龄犬的肺静脉不应期较其左房的不应期明显缩短,而成年犬肺静脉与左房的不应期则无显著性差异.老龄犬的肺静脉和上腔静脉内激动传导呈显著递减性传导.在多数老龄犬的肺静脉、左心房和上腔静脉,快速短阵起搏及程序刺激能诱发持续时间＞30s的房颤.结论老龄犬肺静脉肌袖有易于房颤发生的电生理变化,提示心脏老龄化可能是房颤发生的潜在基质.     

Coronary ischemia induced by radiofrequency ablation in the left atrium

INTRODUCTION: We report three cases of transient, reversible coronary ischemia occurring after radiofrequency ablation in the left atrium. METHODS AND RESULTS: A 56-year-old man with a left atrial tachycardia that was mapped to the septum and roof of the atrium using a noncontact mapping developed 5-mm ST elevation in the anterolateral leads. Coronary angiography showed an occluded diagonal that was opened using intracoronary nitrate, which led to resolution of the ST changes. A 57-year-old man undergoing right upper pulmonary vein ablation developed 6-mm ST elevation in leads V1-V4, II, III, and aVF. Coronary angiography showed normal coronaries with slow flow into the left anterior descending artery, which resolved with nitrates. A 50-year-old man undergoing left lower pulmonary vein ablation developed 3-mm reversible inferior ST elevation. All patients were adequately anticoagulated after transseptal access to the left atrium. CONCLUSION: Ablation in the left atrium, at the roof, septum, and left inferior wall, can cause transient coronary ischemia, possibly due to spasm, which can be reversed with intracoronary nitrates. This phenomenon has not been previously described.     

Impending paradoxical embolism visualized by echocardiography combined with pulmonary embolism: a case report

A 43-year-old man was admitted to our hospital complaining of dyspnea on exertion and dizziness. Transthoracic echocardiography revealed a mobile mass (3.5 x 1.0 cm) attached to the left atrial septum and transesophageal echocardiography showed the mass in the right atrium protruding through the patent foramen ovale into the left atrium. A mobile snake-like thrombus was apparent in the right atrium and right ventricle. The diagnosis was pulmonary embolism with impending paradoxical emboli.     

Atrial noninvasive activation mapping of paced rhythm data

INTRODUCTION: Atrial arrhythmias have emerged as a topic of great interest for clinical electrophysiologists. Noninvasive imaging of electrical function in humans may be useful for computer-aided diagnosis and treatment of cardiac arrhythmias, which can be accomplished by the fusion of data from ECG mapping and magnetic resonance imaging (MRI). METHODS AND RESULTS: In this study, a bidomain-theory-based surface heart model activation time (AT) imaging approach was applied to paced rhythm data from four patients. Pacing sites were the right superior pulmonary vein, left inferior pulmonary vein, left superior pulmonary vein, coronary sinus, posterior wall of right atrium, and high right atrium. For coronary sinus pacing, the AT pattern of the right atrium was compared with a CARTO map. The root mean square error between CARTO geometry (85 nodal points) and the surface model of the right atrium was 8.6 mm. The correlation coefficient of the noninvasively obtained AT map of the right atrium and the CARTO map was 0.76. All pulmonary vein pacing sites were identified. The reconstructed pacing site of right posterior atrial pacing correlates with the invasively determined pacing catheter position with a localization distance of 4 mm. CONCLUSION: The individual anatomic model of the atria of each patient enables accurate noninvasive AT imaging within the atria, resulting in a localization error for the pacing sites within 10 mm. Our findings may have implications for imaging of atrial activity in patients with focal arrhythmias or focal triggers.     

An unusual opacity of the right base

The scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable malformation of the right lung characterized by an abnormal right sided pulmonary venous drainage in the inferior vena cava, malformation of the right lung, abnormal arterial supply and sometimes cardiac malformations. We present a case in which this diagnosis was suspected on an abnormal routine chest radiograph in a 38-year-old asymptomatic woman. Most patients are asymptomatic; symptomatic patients have a marked left-to-right shunt or a severe congenital heart disease. They usually suffer from shortness of breath, asthenia or repeated chest infections. Usually, the posteroanterior chest radiograph can confirm the diagnostic. It shows the abnormal vein draining into the inferior vena cava as a curved vascular shadow with a scimitar like appearance. However, in some cases, when the scimitar vein is masked by the overlying cardiac shadow, computed tomography, angiography and magnetic resonance imaging can be helpful by showing the abnormal vein and its insertion into the inferior vena cava. Scimitar syndrome seldom necessitates surgical intervention. However, repeated lung infections can sometimes require lobectomy or pneumonectomy, left-to-right shunt vascular surgery to redirect the scimitar vein into the left atrium.     

Quantitative study of nerves of the human left atrium.

OBJECTIVES: To quantify and study the distribution of innervation of the left atrium and the pulmonary veins in humans. BACKGROUND: Damage to cardiac nerves has been hypothesized as the explanation for successful radiofrequency ablation of atrial fibrillation. METHODS: From January 2003 to September 2003, histologic and quantitative studies of innervation of the left atrium and the pulmonary veins was performed in 43 consecutive necropsied adult hearts (30 men and 3 women; mean age 45.5 +/- 12.4 years). The left atrium was sectioned in 1-cm slices from left to right, with the plane of section perpendicular to the long axis of the heart. Sections of the pulmonary veins at their ostia and sections 1 cm away of this structure also were obtained. Nerve fiber density was counted manually for each case and expressed as the mean number per slice. RESULTS: Numerous epicardial nerve fibers and ganglia having distinct patterns of distribution in the left atrium were found. Nerve density was significantly higher at the ostia of the four pulmonary veins than in their distal part (7.1 +/- 2.1 vs 5.2 +/- 1.3 for left upper pulmonary vein; 6.3 +/- 1.5 vs 5.2 +/- 1.7 for right upper pulmonary vein; 7.4 +/- 2 vs 5.9 +/- 2 for left lower pulmonary vein; 6.7 +/- 1.8 vs 3.9 +/- 1.3 for right lower pulmonary vein). The left superior vein was significantly more innervated than the right inferior vein (12.3 +/- 3 vs 10.6 +/- 1.4). Gradients of innervation were found from right to left (9.8 +/- 4.6 vs 18.5 +/- 6.6, P < .05) and from the front to the rear of the atrium (17.2 +/- 6.4 vs 20.7 +/- 6.5, P < .05). The same heterogeneous distribution was observed at the myocardial level but with thinner nerve fibers, making quantification difficult. Only very thin nerve fibers were present in the endocardium. CONCLUSIONS: The human left atrium exhibits several gradients of innervation at discrete sites. These findings may have clinical implications for radiofrequency ablation of atrial fibrillation.