Azygos continuation of interrupted inferior vena cava in association with sick sinus syndrome

Various diagnostic and therapeutic procedures of the right side of the heart and the systemic venous system have increased the need for ready access to the inferior vena cava (IVC) through the transfemoral route. Anatomical variations or obstruction of the IVC can make these procedures difficult. The case of 47 year old woman with an interrupted infrahepatic IVC with azygos continuation accompanied by sick sinus syndrome and a structurally normal heart is reported. Negotiating a temporary pacing lead from the IVC to the right atrium was difficult. Ultimately, the lead took the course from the IVC to azygos vein to superior vena cava to right atrium to right ventricular apex. Permanent VVI pacing through the right subclavian route was uneventful, as the superior vena cava and its tributaries had a normal course. An awareness of the existence of these anomalies before pacing can lead to the use of an alternative route for pacing, which may avoid undue delay of an otherwise urgently needed procedure.     

Ablation of atrial flutter by the femoral approach in the absence of inferior vena cava.

This report describes a case of isthmus-dependent atrial flutter ablation by the femoral approach in a 77-year-old patient with a previously unknown absence of the inferior vena cava (IVC). Multi-row detector CT angiography indicated the absence of the perihepatic IVC, whereas the venous blood is drained into the superior vena cava (SVC) via the vena azygos. An ablation catheter could be advanced through the right femoral vein reaching the right heart via vena azygos and SVC. Despite looping of the catheter, ablation and termination of atrial flutter were performed successfully. This is the first report of an inferior-to-superior approach for ablation of atrial flutter in the absence of the perihepatic IVC.     

Atrial tachycardia originating from the hepatic segment of inferior vena cava in interruption of inferior vena cava with azygos continuation

Interruption of inferior vena cava (IVC) with azygos continuation is a rare venous anomaly, and arrhythmogenic IVC is also rarely reported. Arrhythmogenicity of the hepatic segment of IVC in interruption of IVC has never been reported. We describe the case of a 37-year-old female with interrupted left IVC with azygos continuation to right superior vena cava and atrial tachycardia originating from the hepatic segment of IVC.     

Partial anomalous pulmonary venous return showing anomalous venous return to the azygos vein.

Partial anomalous pulmonary venous return (PAPVR) is a congenital heart disease with a reported incidence of autopsied case. The location of the anomalous pulmonary venous return is usually the right atrium, superior vena cava (SVC), and sometimes the brachiocephalic vein, inferior vena cava (IVC) or coronary venous sinus. Recently we experienced a rare case of PAPVR showing anomalous right total pulmonary venous return to the azygos vein. Furthermore, downward translocation of the right upper lobe bronchus was evident. This rare case is reported along with a review of the related literature.     

Effort-induced macroscopic hematuria revealing segmental and congenital absence of inferior vena cava

Absence of inferior vena cava (IVC) is an uncommon congenital abnormality with few clinical repercussions. We report the case of a 39 year old man with chronic pelvic pain, in whom a macroscopic hematuria episode occurring during exercise led to the discovery of an echographic pelvic venous stasis syndrome. Abdominal and pelvic computed tomography scanning then magnetic resonance imaging of inferior vena cava revealed absence of the postrenal segment of IVC with azygos continuation and considerable collateral venous derivations, leading to pelvic cavernoma.     

The lateral chest film as an unreliable indicator of azygos continuation of the inferior vena cava.

Recognition of azygos continuation of the inferior vena cava (AC-IVC) is necessary to prevent unnecessary surgery, to modify the approach to right heart catheterization and to avoid potentially fatal azygos ligation at the time of thoracic surgery. Absence of the inferior vena cava (IVC) shadow on the lateral chest film has been claimed to be an important feature of AC-IVC. Our findings, however, indicate that AC-IVC is frequently associated with a normal appearing IVC shadow on lateral chest film study. When the findings on frontal chest film examination suggest AC-IVC, a caval shadow on the lateral view does not exclude the diagnosis. There persists a need for further radiographic evaluation and, if necessary, angiographic confirmation. The lateral chest film does not provide reliable evidence for or against the diagnosis of azygos continuation of the inferior vena cava.     

Cross-sectional echocardiographic diagnosis of systemic venous return.

To determine the sensitivity and specificity of cross-sectional echocardiography in diagnosing anomalous systemic venous return we used the technique in 800 consecutive children with congenital heart disease and whom the diagnosis was ultimately confirmed by angiography. Cross-sectional echocardiography was performed without prior knowledge of the diagnosis in all but 11 patients, who were recalled because of a known abnormality of atrial situs. The sensitivity of cross-sectional echocardiographic detection of various structures was as follows: right superior vena cava 792/792 (100%); left superior vena cava 46/48 (96%); bilateral superior vena cava 38/40 (95%); bridging innominate vein with bilateral superior vena cava 13/18 (72%); connection of superior caval segment to heart (coronary sinus or either atrium) (100%); absence of suprarenal inferior vena cava 23/23 (100%); azygos continuation of the inferior vena cava 31/33 (91%); downstream connection of azygos continuation, once seen, 21/21 (100%); partial anomalous hepatic venous connection (one hepatic vein not connected to the inferior vena cava) 1/1 (100%); total anomalous hepatic venous connection (invariably associated with left isomerism) 23/23 (100%). The specificity of each above diagnoses was 100% except in one infant with exomphalos in whom absence of the suprarenal inferior vena cava was incorrectly diagnosed. Thus cross-sectional echocardiography is an extremely specific and highly sensitive method of recognizing anomalous systemic venous return. It is therefore of great value of planning both cardiac catheterisation and cannulation for open heart surgery.     

Cross-sectional echocardiographic diagnosis of systemic venous return

To determine the sensitivity and specificity of cross-sectional echocardiography in diagnosing anomalous systemic venous return we used the technique in 800 consecutive children with congenital heart disease and whom the diagnosis was ultimately confirmed by angiography. Cross-sectional echocardiography was performed without prior knowledge of the diagnosis in all but 11 patients, who were recalled because of a known abnormality of atrial situs. The sensitivity of cross-sectional echocardiographic detection of various structures was as follows: right superior vena cava 792/792 (100%); left superior vena cava 46/48 (96%); bilateral superior vena cava 38/40 (95%); bridging innominate vein with bilateral superior vena cava 13/18 (72%); connection of superior caval segment to heart (coronary sinus or either atrium) (100%); absence of suprarenal inferior vena cava 23/23 (100%); azygos continuation of the inferior vena cava 31/33 (91%); downstream connection of azygos continuation, once seen, 21/21 (100%); partial anomalous hepatic venous connection (one hepatic vein not connected to the inferior vena cava) 1/1 (100%); total anomalous hepatic venous connection (invariably associated with left isomerism) 23/23 (100%). The specificity of each above diagnoses was 100% except in one infant with exomphalos in whom absence of the suprarenal inferior vena cava was incorrectly diagnosed. Thus cross-sectional echocardiography is an extremely specific and highly sensitive method of recognizing anomalous systemic venous return. It is therefore of great value of planning both cardiac catheterisation and cannulation for open heart surgery.     

Successful blood sampling through azygos continuation with interrupted inferior vena cava. A case report and review of the literature

Interrupted inferior vena cava (IVC) with azygos continuation is a rare congenital anomaly, and is frequently associated with other cardiovascular malformations and situs anomalies, such as left isomerism. These patients usually develop deep vein thrombosis (DVT), and asymptomatic patients above 60 years of age are very rare. Here we report a case of interrupted IVC which we diagnosed in a 72-year-old woman. She was admitted to our hospital suffering from heart failure and supraventricular tachycardia. Echocardiography detected secundum atrial septal defect (ASD). An abnormal paravertebral pleural line on the chest X-rays indicated the existence of venous anomaly. Anatomical images obtained by Multidetector Computed Tomography (MDCT) helped us to successfully perform right heart catheterization procedures through azygos continuation including blood sampling from pulmonary veins. Even in elderly patients, a careful examination of chest X-rays can indicate undiagnosed venous anomalies; thus, it is critically important before planning surgical or interventional procedures.     

Cross-Sectional echocardiographic diagnosis of azygos continuation of the inferior vena cava

Azygos continuation of the inferior vena cava has importance for both the invasive diagnosis of congenital heart disease by catheterization and for surgical treatment. Cross-sectional echocardiography was used to examine 1,000 patients (1 day to 16 years, mean 3.3 years) who also had angiographic or surgical confirmation. Twenty-eight patients (3%) had azygos continuation (left 13, right 14, bilateral 1) and, in 26 patients, the hepatic portion of the inferior vena cava was absent. Azygos continuation was prospectively detected in all and was directly visualized in subcostal scans as a venous structure posterior to the aorta coursing behind the heart and not entering the inferior aspect of either atrium in 26/28 (93%). Azygos connection to the ipsilateral superior vena cava or atrium was correctly predicted in all. The inferior vena cava was visualized in all patients without azygos continuation, except one neonate with omphalocele. We conclude that cross-sectional echocardiography can accurately detect azygos continuation of the inferior vena cava and predict its side and connection.     

Congenital Portosystemic Venous Connections and Other Abdominal Venous Abnormalities in Patients with Polysplenia and Functionally Univentricular Heart Disease: A Case Series and Literature Review

Objective. Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemic venous anomalies in patients with polysplenia and inferior vena cava (IVC) interruption who underwent a cavopulmonary anastomosis procedure at our center, and to review the published literature on this topic and the potential clinical importance of such anomalies. Design. Retrospective cohort study and literature review were used. Results. Among 77 patients with heterotaxy, univentricular heart disease, and IVC interruption who underwent a bidirectional Glenn and/or modified Fontan procedure, pulmonary arteriovenous malformations were diagnosed in 33 (43%). Bilateral superior vena cavas were present in 42 patients (55%). Despite inadequate imaging in many patients, a partial PSVC, dual IVCs, and/or renal vein anomalies were detected in 15 patients (19%). A PSVC formed by a tortuous vessel running from the systemic venous system to the extrahepatic portal vein was found in six patients (8%). Abdominal venous anomalies other than PSVC were documented in 13 patients (16%), including nine (12%) with some form of duplicated IVC system, with a large azygous vein continuing to the superior vena cava and a parallel, contralateral IVC of similar or smaller size, and seven with renal vein anomalies. In patients with a partial PSVC or a duplicate IVC that connected to the atrium, the abnormal connection allowed right‐to‐left shunting. Conclusions. PSVC and other abdominal venous anomalies may be clinically important but under‐recognized in patients with IVC interruption and univentricular heart disease. In such patients, preoperative evaluation of the abdominal systemic venous system may be valuable. More data are necessary to determine whether there is a pathophysiologic connection between the polysplenia variant of heterotaxy, PSVC, and cavopulmonary anastomosis‐associated pulmonary arteriovenous malformations.     

Catheter Ablation of Wolff-Parkinson-White Syndrome Associated with Congenital Absence of Inferior Vena Cava

In the present report we describe a patient (a 36-year-old woman with 15 year history of supraventricular tachyarrhythmias) with congenital absence of inferior vena cava (IVC) revealed during radiofrequency (RF) catheter ablation procedure for right postero-septal Wolff-Parkinson-White syndrome (WPW). For the absence of IVC, the ablation procedure was more difficult, because we had to perform the ablation with the catheters (the ablator catheter and the coronary sinus catheter) introduced both through the superior vena cava. The application of RF energy (35 Watt for 60 seconds) at successful site abolished accessory pathway conduction. The following day was performed the venous angiography, showing the absence of the IVC and a venous return via paravertebral venous plexus to the azygous vein and superior vena cava into the right atrium. Computer tomography confirmed the absence of the IVC with azygous continuation. The drainage via the azygous system modified the radiological image on chest roentgenogram of the right mediastinal silhouette. During cardiogenesis fusion of the IVC and organisation of the heart occur between the 33rd to 40th embryonic days. It is therefore possible that some unknown teratogenic mechanism at this critical period might have caused, in the patient, both the developmental arrest of IVC and failure of regression of atrio-ventricular anatomical and electrical continuity in the right postero-septal region.     

A case of diffuse pulmonary arteriovenous fistula.

A 30-year-old Japanese woman was admitted to hospital for dyspnea. She had a history of corrective surgery for a large atrial septal defect and partial anomalous pulmonary venous drainage, which had produced cyanosis in her infancy. However, her cyanosis continued postoperatively. Angiography revealed a double inferior vena cava (IVC), with the left IVC connected with the hemiazygos vein and the right IVC with the left atrium through a very small orifice. Most of the blood from the 2 IVCs flowed into the superior vena cava via the distended azygos and hemiazygos veins. Pulmonary arteriography revealed no abnormal structures. Pulmonary arterial pressure was normal. There was marked pulmonary venous oxygen desaturation. Perfusion lung scintigraphy revealed multiple segmental perfusion defects. These findings suggested the presence of diffuse microscopic pulmonary arteriovenous fistulas bilaterally in the lungs. The patient appears to be the first reported adult case of microscopic and diffuse arteriovenous fistulas. Neither resection of the arteriovenous fistulas nor corrective surgery for the diversion was indicated, and heart-lung transplantation might be the only treatment able to relieve her dyspnea.     

Inferior Vena Cava Anomalies with Portal Vein System Continuation Presenting as Portal Hypertension with a Long-term Follow-up

Inferior vena cava (IVC) anomalies, such as the absence of an intra-hepatic IVC or IVC hypoplasia, are rare. Usually, these anomalies are asymptomatic and cause few clinical issues. We herien report a 53-year-old woman with IVC anomalies who demonstrated both azygos and portal vein system continuation. Over time, this resulted in gradually progressive portal hypertension due to abnormal hemodynamics. The increased inflow from the IVC to the portal vein system for an extended time may contribute to the development of portal hypertension without liver cirrhosis.     

Bilateral inferior venae cava combined with the persistent left superior vena cava and hemiazygos continuation of left inferior vena cava with drainage into right atrium: A case report

The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of .3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left-sided venous return with hemiazygos continuation in presence of a right-sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition.     

Relation between caval and pulmonary artery oxygen saturation in children.

The flow relation between that in the superior vena cava and inferior vena cava was studied in order to estimate the oxygen saturation of mixed venous blood in connection with heart catheterisation of children between the ages of 5 and 8 years without cardiac shunts. The investigation includes a group of 19 children examined during halothane anaesthesia and a group of 40 examined under local anaesthesia. The groups were comparable in respect of age and body surface. The results show that the oxygen saturation of mixed venous blood (MVB) is higher under general anaesthesia than during local anaesthesia, and that the oxygen saturation in the former group is highest in the superior vena cava (SVC) and lowest in the inferior vena cava (IVC), while the reverse is the case during examination under local anaesthesia. Based on multiple regression analysis, it is advisable, for children between the ages of 5 and 8 years, to use the formula MVBcalc = -0.85 + 0.81 SVC + 0.18 IVC for the estimation of the oxygen saturation in the mixed venous blood in children under general anaesthesia and the formula MVBcalc = 10.21 + 0.52 SVC + 0.34 IVC for children during local anaesthesia. When estimating mixed venous blood in younger children and during general anaesthesia or both, more emphasis should be laid on the flow through the superior vena cava.     

Absence of the inferior vena cava in a patient with omphalocele: Two-dimensional echocardiographic and cineangiographic findings

Summary We report an unusual form of absence of the inferior vena cava (IVC) in a patient with a repaired omphalocele. Two sets of bilateral paravertebral veins served as the channels of systemic venous return from the lower half of the body. These veins were narrower than typical azygos or hemiazygos continuation in the absence of the IVC, with the result that a catheter from the femoral vein could not reach the right atrium (RA). Other associated venous-side anomalies were present, including a chamber between the hepatic vein (HV) and RA, narrowing and angulation at the junction between the HV and the chamber, and a subaortic innominate vein. All these anomalies were demonstrated by two-dimensional and color Doppler echocardiography. The recognition of these venous anomalies is important for cardiac catheterization or IVC cannulation for cardiopulmonary bypass in patients with omphalocele.     

Severe subvalvular pulmonic stenosis due to a ball‐shaped accessory tissue in an asymptomatic woman with congenitally corrected transposition of great arteries and mesocardia: A rare case report

Congenitally corrected transposition of great arteries (CCTGA) is a rare anomaly described by atrioventricular and ventriculoarterial discordance. On the other hand, mesocardia that is extremely rare includes two relatively well‐defined apexes defined by each ventricle with the major axis of the heart lies in the midline. We describe a rare case of an asymptomatic 20‐year‐old woman with mesocardia, CCTGA, and severe subvalvular pulmonic stenosis due to a ball‐shaped accessory tissue in left ventricular outflow tract.     

Unusual collateral vessel from right subclavian vein to left atrium,a rare complication of superior vena cava obstruction

The most commonly reported collateral systems in the setting of superior vena cava obstruction are azygos venous system, vertebral venous system, external and internal thoracic venous system based on McLntire and Sykes classification. A 49‐year‐old female with renal disease complained dyspnea on exertion. Transesophageal echocardiography showed significant mitral annular calcification, large multi‐lobulated mass at posterior aspect of RA, and complete obstruction of superior vena cava by thrombus formation. Computed tomography angiography showed a collateral vein to the left atrium (LA) roof. This case report is the first one which shows development of collateral vein from right subclavian to LA.     

Percutaneous paraspinal access to the azygos vein to create a neo‐vena cava

We present the case of a child with congenital heart disease repaired in infancy with diffuse central venous occlusions resulting in central venous insufficiency, superior vena cava (SVC) syndrome, and intracranial bleeds. He presented to the catheterization laboratory for multiple transcatheter interventions to recanalize central venous channels which were unsuccessful by conventional methods. Therefore, extravascular venous channels were created to decompress his upper body by creation of a neo‐SVC and his lower body with a neo‐azygos vein to the neo‐SVC. The latter procedure required direct percutaneous access from a paraspinal approach in order to obtain continuity with the azygos vein. At latest follow‐up the patient is clinically well and asymptomatic.