A hybrid surgical transcatheter strategy for treating severe para-right atrioventricular valvular regurgitation in a patient with left atrial isomerism

Paravalvular leaks are a recognized complication of valve replacement surgery. We report a 47-year-old man with left atrial isomerism, interrupted left sided inferior caval vein with unilateral left sided superior caval vein, a common atrium, and anomalous pulmonary venous connection to the coronary sinus, who had recurrent severe para-right atrioventricular (AV) regurgitation with gross right heart failure following tricuspid valve (TCV) replacement. He underwent a hybrid surgery-transcatheter treatment strategy in the cardiac catheterization laboratory, which led to significant improvement in hemodynamics and symptoms. This to our knowledge is the first reported case of a minimally invasive approach to para-right sided AV valve regurgitation.     

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.     

Persistence of Left Supracardinal Vein in an Adult Patient with Heart–Hand Syndrome and Cardiac Pacemaker

A patient with a sporadic heart–hand syndrome, which includes thumb hypoplasia, septum primum atrial septal defect, and cleft mitral valve is described. During attempted placement of a pacemaker lead, persistence of left superior and inferior vena cava was found in addition to the right‐sided caval veins. This corresponds to persistence of left‐sided supracardinal vein present during fetal development.     

Unusual drainage of the inferior caval vein in left atrial isomerism

Two of the 26 cases of left atrial isomerism in our clinical material had an unusual drainage of the inferior caval vein. In one, the inferior caval vein crossed from left to right infradiophragmatically and then drained into the right-sided morphologically left atrium. In the second case, there was azygos continuation to both right and left superior caval veins. These variations have major surgical significance.     

Absent right superior caval vein (vena cava) with normal atrial arrangement.

Eight cases of absent right superior caval vein (vena cava) with normal atrial arrangement from the Great Ormond Street database were reviewed. In each case the right subclavian vein and jugular vein drained into a persistent left superior caval vein through a bridging vein. In six cases the left superior caval vein drained into the right atrium by way of the coronary sinus, and in two cases it drained into the left atrium directly because of the complete unroofing of the coronary sinus. The frontal plane P wave axis was displaced leftwards and upwards in four out of seven cases in which an electrocardiogram was available. No case showed arrhythmia on a standard electrocardiogram preoperatively. Echocardiograms were available in four cases and in each case diagnosis of the anomalous connections of the systemic vein was possible. Only one third of the cases showed a crescentic shadow of the persistent left superior caval vein on chest x ray. A chest x ray and electrocardiogram do not provide the means of reliable diagnosis of this condition, though they may suggest the possibility of its existence. Definitive diagnosis requires cross sectional echocardiography or angiocardiography or both.     

Absent right superior caval vein (vena cava) with normal atrial arrangement

Eight cases of absent right superior caval vein (vena cava) with normal atrial arrangement from the Great Ormond Street database were reviewed. In each case the right subclavian vein and jugular vein drained into a persistent left superior caval vein through a bridging vein. In six cases the left superior caval vein drained into the right atrium by way of the coronary sinus, and in two cases it drained into the left atrium directly because of the complete unroofing of the coronary sinus. The frontal plane P wave axis was displaced leftwards and upwards in four out of seven cases in which an electrocardiogram was available. No case showed arrhythmia on a standard electrocardiogram preoperatively. Echocardiograms were available in four cases and in each case diagnosis of the anomalous connections of the systemic vein was possible. Only one third of the cases showed a crescentic shadow of the persistent left superior caval vein on chest x ray. A chest x ray and electrocardiogram do not provide the means of reliable diagnosis of this condition, though they may suggest the possibility of its existence. Definitive diagnosis requires cross sectional echocardiography or angiocardiography or both.     

Persistent left‐sided superior vena cava – a pacing challenge

A left sided superior vena cava (LSVC) occurs in 0.3% of the population. LSVC normally drains into the right atrium through a dilated coronary sinus. We illustrate two cases of dual chamber permanent pacemaker implantation by using (1) left subclavian vein in a 35‐year‐old woman with symptomatic Mobitz type II atrioventricular block; and (2) right subclavian vein in a 64‐year‐old man who was hospitalized with bradycardia, complete heart block, and alternating bundle branch block.

After accessing the subclavian vein, the pacing leads were advanced into the LSVC, which was situated to the left of the vertebral column in the mediastinum. The leads followed the course of the LSVC medially before entering into the right atrium. Once inside the right atrium, the ventricular lead made a U‐turn towards the tricuspid valve and into the right ventricle by shaping the stylet, and it was helped by right atrial contraction. An active fixation atrial lead was used in both cases to secure a satisfactory location within the right atrium. A small volume of contrast can be injected into the pacing sheath to visualize the coronary sinus opening into the right atrium, and the right ventricle. Fluoroscopy in oblique views can be helpful in guiding the atrial lead into the anteriorly positioned atrial appendage.

In emergency transvenous ventricular temporary pacing where the subclavian or internal jugular vein is used, it is important to recognize the presence of a LSVC. The lead should first be directed into the right atrium and then looped back into the right ventricle. Excessive force must be avoided to prevent cardiac perforation and tamponade. If this is not successful, access through a femoral vein should be attempted.     

Heart transplantation after Fontan's procedure with bilateral cavopulmonary connections

We present a young female patient with three caval drainages after complete Fontan's procedure and with bilateral bidirectional cavopulmonary connections who underwent orthotopic heart transplantation. Instead of reconstructing the hypoplastic innominate vein the persistent left superior caval vein was reanastomosed via the recipient's coronary sinus into the right atrium.     

A patient with bilateral superior and inferior caval veins

We present a case with a very uncommon form of anomalous connection: a normal systemic venous connection to the right atrium existing in combination with left superior and inferior caval veins draining into the left atrium (associated with a large ostium primum atrial septal defect). To our knowledge the present report concerns the third case with this very rare congenital anomaly diagnosed during life.     

Pacemaker implantation in patients with persistent left superior vena cava

Implantation of a permanent pacemaker system is most commonly performed by puncturing the left subclavian vein and introducing the pacemaker lead(s) through the superior caval vein to the right atrium and/or ventricle. Occasionally, a persistent left superior caval vein is encountered peroperatively, complicating the procedure. This article describes three such patients and provides a review of the literature regarding one of the most common anomalies of the thoracic vessels.     

Long term complete circulatory exclusion of the right side of the heart Hemodynamic observations

Long-term, complete circulatory exclusion of the right side of the heart was achieved in dogs by: (1) performing an end-to-side anastomosis between the left subclavian and left pulmonary artery as a preliminary procedure, (2) transplanting the inferior caval vein into the left atrium, (3) draining the superior vena cava into the pulmonary artery, and (4) ligating the previously established subclavian-pulmonary artery anastomosis. Sixteen out of 76 animals survived the third stage of the procedure; 9 dogs lived for two weeks or longer after the completion of the procedure. Five of the animals survived one year or longer following complete circulatory exclusion of the right heart. Except for some cyanosis of the mucous membranes and exertional dyspnea, the animals appeared to be and behaved like normal dogs. Hemodynamic observations one year after the completion of the experiment were characterized by the following findings: (1) Normal pressures in the left heart and systemic arteries. (2) Moderately increased pressure in the inferior caval area in some animals. (3) More pronounced pressure elevation in the superior caval area in most animals. (4) “Respiratory” characteristics of the pressure curve in the right pulmonary artery. (5) Desaturation of the arterial blood due to the right-to-left shunt. (6) Increase in the circulating blood volume.     

Permanent pacemaker implantation via left superior vena cava without communication with the coronary sinus

The persistence of the left superior vena cava is a rare venous anomaly and usually does not produce hemodynamic disturbances. Left sided cardiac device implantation has increased the awareness of this rare anomaly. In most cases, left superior vena cava connects to the right heart via coronary sinus. We describe a rare case of successful permanent pacemaker implantation via left superior vena cava-accessory hemiazygos-hemiazygos-inferior vena caval communication.     

静脉畸形、迂曲、狭窄时永久起搏导线置入的方法探讨

经静脉造影或观察导丝走形证实 6例患者存在静脉畸形、迂曲、狭窄 ,其中 5例高龄患者置入永久起搏器时 ,其上腔静脉系统迂曲、狭窄 ,无法使用起搏器穿刺套装内的导丝及鞘管将导线送到起搏部位 ,另 1例为永存左上腔静脉合并有右上腔静脉缺如。试用 175cm 0 .0 35长导丝以及 6 8FINPUT鞘替代普通起搏器穿刺套装。结果 :使用175cm 0 .0 35长导丝以及 6 8FINPUT鞘顺利地将起搏导线送入右心房中下部 ,安全地完成置入手术 ,无并发症。结论 :一旦送入导线或导丝困难 ,应积极地进行血管造影 ,不应盲目的推送 ,使用 175cm长导丝增加支撑力 ,结合IN PUT鞘管通过狭窄或纡曲延长的血管段 ,给起搏导线提供一个光滑的通道 ,可顺利的将起搏导线送入心房及心室。     

Persistent left-sided superior vena cava--a pacing challenge

A left sided superior vena cava (LSVC) occurs in 0.3% of the population. LSVC normally drains into the right atrium through a dilated coronary sinus. We illustrate two cases of dual chamber permanent pacemaker implantation by using (1) left subclavian vein in a 35-year-old woman with symptomatic Mobitz type II atrioventricular block; and (2) right subclavian vein in a 64-year-old man who was hospitalized with bradycardia, complete heart block, and alternating bundle branch block. After accessing the subclavian vein, the pacing leads were advanced into the LSVC, which was situated to the left of the vertebral column in the mediastinum. The leads followed the course of the LSVC medially before entering into the right atrium. Once inside the right atrium, the ventricular lead made a U-turn towards the tricuspid valve and into the right ventricle by shaping the stylet, and it was helped by right atrial contraction. An active fixation atrial lead was used in both cases to secure a satisfactory location within the right atrium. A small volume of contrast can be injected into the pacing sheath to visualize the coronary sinus opening into the right atrium, and the right ventricle. Fluoroscopy in oblique views can be helpful in guiding the atrial lead into the anteriorly positioned atrial appendage. In emergency transvenous ventricular temporary pacing where the subclavian or internal jugular vein is used, it is important to recognize the presence of a LSVC. The lead should first be directed into the right atrium and then looped back into the right ventricle. Excessive force must be avoided to prevent cardiac perforation and tamponade. If this is not successful, access through a femoral vein should be attempted.     

Transesophageal Echocardiographic Findings in Complete Unilateral Anomalous Pulmonary Venous Connection of Right Lung to Right Atrium

Transesophageal echocardiographic findings in a patient with anomalous drainage of both right- sided pulmonary veins into the right atrium are described. The atrial septum was intact and the left- sided veins connected normally with the left atrium.
transesophageal echocardiography, partial anomalous pulmonary venous connection of the right pulmonary veins to the right atrium     

Ablation of right-sided accessory pathways with atrial insertion far from the tricuspid annulus using an electroanatomical mapping system

Ablating Right‐Sided Accessory Pathways With Atrial Insertions Far From Tricuspid Annulus . Background: It is difficult to ablate a right‐sided accessory pathway (AP) with atrial insertion far from the tricuspid annulus (TA). We report our initial experience of ablating this rare AP by a 3‐dimensional electroanatomical mapping system (CARTO). Methods: From January of 2006 to April of 2008, 18 patients with right‐sided APs who failed previous outside ablations were enrolled in this study. Retrograde AP conduction was mapped during pacing at the right ventricular apex by activation‐mapping the right atrium (RA) using a 3‐dimensional electroanatomical mapping system. AP atrial insertion was defined as the earliest retrograde atrial activations and successful ablation of the APs at this site. Results: Among the 18 patients who had failed previous ablation, 10 patients (7 patients with right manifest APs and 3 patients with right conceal APs) had atrial insertions far from the TA. Of the 10 patients, the atrial insertions were found at the base of the RA appendage in 3 patients, at the high lateral RA in 5 patients, at the low lateral RA in other 2 patients. Ablation at the atrial insertions successfully abolished the AP conduction. The mean distance between the atrial insertion sites and the TA was 20.2 ± 2.7 mm. No patients reported recovered AP conduction or recurrent tachycardias after 6‐month follow‐up. Conclusions: The right‐sided APs may have atrial insertion far from the TA. These uncommon variation of APs can be reliably identified and ablated using CARTO system. (J Cardiovasc Electrophysiol, Vol. 22, pp. 499‐505 May 2011)     

Peripheral and intracardiac levels of atrial natriuretic factor during cardiothoracic surgery.

Immunoreactive atrial natriuretic factor (ANF) levels were measured in blood taken from superior vena cava, right atrium, pulmonary artery, pulmonary vein, left atrium, and radial arteries in patients undergoing cardiac surgery. Significant (p less than 0.05) differences were seen between sites. Levels of atrial natriuretic factor were seen to rise from superior vena cava (27.5 pmol/L) to right atrium (54.3 pmol/L) and then fall in pulmonary artery (38.5 pmol/L). A further significant fall was seen in pulmonary vein (32.7 pmol/L) samples. There was no significant change in levels in left atrium (32.1 pmol/L) or radial artery (30 pmol/L). The fall between radial artery levels and superior vena caval levels was also significant. The rise from superior vena cava to right atrium is to be expected as this represents the major site of addition of atrial natriuretic factor to the circulation. We propose that the fall in levels from right atrium to pulmonary artery and from pulmonary artery to pulmonary vein suggests uptake and therefore possible local hormonal action on both right ventricle and pulmonary vasculature. The fall from radial artery to vena cava would be in keeping with atrial natriuretic factor's known systemic uptake and action.     

Intermediate term hemodynamic effects of single inferior vena cava valve implant for the treatment of severe tricuspid regurgitation

Severe tricuspid regurgitation (TR) remains a vastly undertreated disease, with sustained elevation of right atrial (RA) pressure directly resulting in chronic end‐organ damage. Recently, bi‐caval valve implantation has been shown to improve symptoms in patients with symptomatic TR who are at high risk for surgery. We present the first report of intermediate term hemodynamic effects of single inferior vena cava (IVC) valve implantation (CAVI) for treatment of severe TR. We performed CAVI on a 66‐year‐old female with severe TR, who suffered from repeat hospitalizations for treatment refractory NYHA class III–IV heart failure symptoms and had prohibitive operative risk. Pre‐implantation right heart catheterization (RHC) revealed a mean RA pressure of 12 mm Hg, an IVC mean pressure of 13 mm Hg, with V‐waves to 16 and 18 mm Hg in the RA and IVC respectively, and a cardiac output (CO) of 3.5 liters per minute (LPM). Postprocedure, mean IVC and RA pressures decreased to 11 and 10 mm Hg, respectively, with CO increasing to 5.1 LPM. At one month, symptoms improved to NYHA class II. At 9 months, mean RA pressure was 5 mm Hg with V‐waves to 7 mm Hg and an improvement in CO to 6.3 LPM. CAVI appears to result in similar decreases in RA pressure at intermediate follow‐up as compared to bi‐caval valve implantation. The favorable hemodynamic effects were likely mediated by redirection of regurgitant blood flow away from the IVC thereby resulting in sustained reduction in right‐sided pressures as well as an increase in CO. © 2017 Wiley Periodicals, Inc.     

A radiofrequency transseptal needle: Initial animal studies

Objectives: The objective of this study was to demonstrate the safety and efficacy of a new transseptal needle design with a radiofrequency (RF) tip combined with the ease of use of the needle configuration. Background: RF transseptal puncture to enter the left heart, with a RF wire‐catheter system is a successful technique in patients with complex access using a standard transseptal needle. Methods: The RF transseptal needle (NRG? Transseptal Needle; Baylis Medical, Montreal, Canada) was designed for RF percutaneous transseptal access to the left heart. Eight pigs underwent transvenous cardiac catheterization with baseline intracardiac electrograms and right atrial pressure waveforms. Transseptal RF puncture was performed followed by left atrial pressure waveform monitoring. Results: An intracardiac electrogram was recorded in each animal while dragging the needle tip from the superior caval vein across the atrial septum and into the inferior caval vein. Contrast staining of the atrial septum was accomplished in all animals, with subsequent RF septal puncture. After 0.1 sec, impedance increased from 300 to 1,200 Ohms with the creation of a vapor layer, and voltage increased steadily to a threshold of 230 volts over the first 0.4 sec. This dielectric breakdown results in tissue vaporization and tissue perforation. The needle's location within the left atrium was confirmed by the pressure waveform and contrast injection. Repeated RF punctures with the NRG? was compatible with various manufactures transseptal sheaths. Conclusion: RF puncture of the interatrial septum using the NRG? Transseptal Needle facilitates an alternative effective technique to enter the left atrium. © 2010 Wiley‐Liss, Inc.     

Misdiagnosis of aorta-right atrial tunnel

First reported by Coto in 1980, aorta-right atrial tunnel (ARAT) is a rare congenital vascular connection between the aortic root and RA. The case report presents a 38-year-old male patient with ARAT. Echocardiography showed a tunnel-like structure which appeared to be a connection between the left coronary sinus and the left atrium although the tunnel was connected to the right atrium. The misdiagnosis may be explained that the images were overlaid and abnormal color flow signal was not detected in RA because of the failure to detect color flow spectrum at the outlet of superior vena cava (SVC). We have discussed the diagnostic experience of the rare congenital cardiac anomaly in echocardiography: (1) carefully detect the origin and termination of abnormal tunnel structure; (2) the outlet of SVC into RA should be detected for possible artery flow spectrum and color flow signal into RA; (3) transesophageal echocardiography should be performed for identifying the diagnosis of ARAT if it is necessary.