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Computed tomography angiography (CTA) was performed under general anaesthesia on a 7-month-old toy poodle that was referred with the chief complaints of salivation and neurological symptoms. The CTA revealed a rare form of posthepatic portosystemic shunt (PSS) via the suspected persistent left umbilical vein communicating with the internal thoracic vein in addition to an azygos continuation of the caudal vena cava (CVC). The patient underwent surgery for partial ligation of PSS on Day 4 after the initial examination. On Day 71, after the initial examination, a second surgery was performed for complete ligation. Approximately 10 years have passed since the patient's second surgery, and he is still healthy, and generally in good condition. Although the morphology of the shunt in this case was unusual and was accompanied by an azygos continuation of the CVC, a favourable course of treatment was obtained by ligating the shunt vessel. This case report suggests that CTA can reveal the complex morphological characteristics like our case. Surgical treatment in this case resulted in favourable progress, similar to that in dogs with commonly observed extrahepatic PSS., (© 2024 The Author(s). Veterinary Medicine and Science published by John Wiley & Sons Ltd.)

Intrahepatic interruption of the inferior vena cava (IVC) with continued hemizygous is a very rare abnormality and sometimes it may be accompanied by other cardiovascular abnormalities. Continuation of the hemizygous vein draining into the right atrium through the left superior vena cava (LSVC) is much rarer. In this paper, we have presented a patient who had simultaneous IVC interrupted with persistent LSVC and suffered from Atrioventricular nodal reentrant tachycardia (AVNRT). Finally, radiofrequencies (RF) catheter ablation for AVNRT was successfully performed through a left subclavian vein access., (© 2024. The Author(s).)

Patients with functionally single ventricle and interrupted inferior vena cava may develop progressive cyanosis soon after the Kawashima operation. Therefore, early redirection of the hepatic venous return to the pulmonary circulation is recommended. To avoid performing an early redo sternotomy, we propose to prepare these patients for the interventional Fontan-Kreutzer at the time of the Kawashima operation using a technical modification of the approach reported by Prabhu and coworkers in 2017. The technique described here uses an expanded polytetrafluoroethylene conduit interposed between the hepatic veins and the right pulmonary artery. This graft is everted and divided into two portions with a pericardial patch. The lower one is widely opened and anastomosed side-to-side to the atrium. A few months after the operation, percutaneous Fontan-Kreutzer completion can easily be performed using covered stents to open the patch and at the same time close the opening between the conduit and the atrium.

A 68-year-old man was referred to our hospital because of mediastinal tumor on chest computed tomography (CT). Contrast-enhanced CT showed azygos continuation of the inferior vena cava (IVC). The retro-hepatic IVC was absent superior to the renal veins. The IVC continued into the dilated azygos vein, which joined the superior vena cava. The hepatic vein drained directly into the right atrium. The mediastinal tumor was close to the dilated azygos vein. Video-assisted thoracoscopic resection of the mediastinal tumor was performed, using four ports and CO 2 insufflation. Histological examination of the resected specimen revealed a pericardial cyst without malignancy. After a favorable postoperative course, the patient was discharged 4 days after surgery. It is important to recognize this anomaly before thoracic surgery, because transection of the azygos vein can be fatal. Video-assisted thoracoscopic resection of mediastinal tumor close to the azygos vein using CO 2 insufflation avoids injury to the azygos vein.

Background: Patients with heterotaxy, single ventricle and interrupted inferior vena cava are at risk of developing significant pulmonary arteriovenous malformations and cyanosis, and inequitable distribution of hepatic factor has been implicated in their development. We describe our experience with a technique for hepatic vein incorporation that reliably provides resolution of cyanosis and presumably equitable hepatic factor distribution., Methods: A retrospective review of a single-surgeon experience was conducted for patients who underwent this modified Fontan operation utilizing an extracardiac conduit from the hepatic veins to the dominant superior cavopulmonary connection. Preoperative characteristics and imaging, operative details, and postoperative course and imaging were abstracted., Results: Median age at operation was 5 years (2-10 years) and median weight was 19.6 kg (11.8-23 kg). Sixty percent (3/5) of patients had Fontan completion without cardiopulmonary bypass, and follow-up was complete at a median of 14 months (range 1-20 months). Systemic saturations increased significantly from 81% ± 1.9% preoperatively to 95% ± 3.5% postoperatively, P = .0008. Median length of stay was 10 days (range: 7-14 days). No deaths occurred. One patient required reoperation for bleeding and one was readmitted for pleural effusion. Postoperative imaging suggested distribution of hepatic factor to all lung segments with improved pulmonary arteriovenous malformation burden., Conclusions: Hepatic vein incorporation for patients with heterotaxy and interrupted inferior vena cava should optimally provide equitable pulmonary distribution of hepatic factor with resolution of cyanosis. The described technique is performed through a conventional approach, is facile, and improves cyanosis in these complex patients.

This report describes a case of isthmus-dependent atrial flutter ablation by the femoral approach in a 54-year-old woman with a previously unknown absence of the inferior vena cava (IVC) and dual chamber pacemaker. Despite looping of the catheters, ablation and termination of atrial flutter were performed successfully without function alteration of the pacemaker leads.  This is the first report of an inferior-to-superior approach for ablation of atrial flutter in the absence of the perihepatic IVC with the presence of chronic indwelling leads in the area targeted for radiofrequency.

The azygos system of veins varies greatly in its mode of origin, but the variation in which the azygos vein is a continuation of the inferior vena cava (IVC) is rare. During an oesophagectomy, the azygos vein typically is transected as a requirement of the surgery. In this case, the enlarged azygos and its arch were a continuation of the IVC. During our procedure, we first established a bypass between the right femoral vein and the jugular vein in case of injury to the azygos vein, and we then performed a McKeown oesophagectomy without transecting the azygos vein. Our experience suggests that an oesophagectomy in cases with an azygos vein continuation of the IVC is feasible. An adequate medical examination and careful reading of the imaging is crucial for the safety of these surgical procedures. An appropriate surgical approach should be selected according to the location of the tumour, the size of the tumour and its anatomical features. The establishment of a veno-venous bypass and protection of the azygos arch in patients whose azygos vein is a continuation of IVC is necessary.

Aims: Inferior vena cava (IVC) interruption is a rare anatomic variant where the azygos vein (AV) drains the blood from the IVC to the upper part of the right atrium via the superior vena cava. Here, we report balloon cryoablation of the pulmonary veins (PVs) via superior access in a patient with atrial fibrillation., Methods and Results: After the first failed ablation attempt due to IVC interruption, balloon cryoablation with a 28-mm Arctic Front Advance cryoballoon (Medtronic CryoCath LP, Quebec, Canada) via superior access was performed; it requires only a single transseptal puncture (TP), and the patient had optimal PV anatomy. Deflectable electrodes were inserted into the right ventricle and coronary sinus from the right femoral vein. The right internal jugular vein was accessed using an SL0 transseptal sheath and BRK needle. The TP was performed under transoesophageal echocardiographic guidance with a Safe Sept wire because the septum was stiff. All PVs were engaged: the left using an ablation catheter before balloon insertion and the inferior following a 'push-up' technique because of a leak above the veins. Cryothermal energy was delivered after checking for occlusion. The procedure lasted 210 min, fluoroscopy time was 78 min, and air-kerma dose was 194 mGy. During the 6-month follow-up, no episodes of atrial fibrillation were detected on several Holter recordings., Conclusions: Successful PV isolation in patients with AV continuation of an interrupted IVC can be safely performed using superior access with balloon cryoablation, after several modifications of standard equipment., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

We describe here a thoracic esophageal cancer with an unusual type of duplicated inferior vena cava. A 58-year-old man was referred to our hospital because a tumor in his lower esophagus had been identified by endoscopy and radiology. Computed tomography scans showed an unusual type of duplicated inferior vena cava characterized by both common iliac veins flowing back into the left-sided inferior vena cava, which drained into the azygos vein, whereas the right-sided one had no drainage. Esophagectomy was performed 3 weeks later after preoperative chemotherapy. Because the patient could have developed thrombosis of the left-sided inferior vena cava and severe hypotension caused by decreased venous return to the heart if the azygos vein had been severed, the azygos vein was preserved. Thus, when performing surgery for thoracic esophageal cancer, the surgeon should check for a duplicated inferior vena cava and preserve the azygos vein if necessary.

A 58-year-old woman with symptomatic multiple monomorphic premature ventricular beats of a right ventricular outflow tract origin was referred for ablation. An inferior vena cava interruption with azygos continuation was discovered during catheter placement. This case describes positioning of the noncontact mapping array and successful radiofrequency ablation in this challenging anatomy., (©2011, The Authors. Journal compilation ©2011 Wiley Periodicals, Inc.)

We describe a child dependent on parenteral nutrition who developed loss of all conventional central venous catheter insertion sites coupled with very unusual collateralization due to extensive thrombosis of both the superior and inferior vena cava, including the azygos vein. We successfully achieved trans-hepatic anterior access to the infra-diaphragmatic vena cava. Since the success rate for this technique is low due to catheter dislodgement, the procedure was surgically assisted for increased safety and efficacy., (Copyright © 2012 Elsevier Inc. All rights reserved.)

A 17-year-old girl visited our hospital for an operation to correct a partial anomalous pulmonary venous return. All the right-side pulmonary veins returned to the azygos vein, which had no connection to the cardiac chamber through the great venous system, such as the superior or inferior vena cava. The azygos vein lacked a normal azygos arch and was connected by multiple connections only to the paravertebral plexus. For the surgical correction, a pericardiostomy was created in the right posterior pericardial wall and azygos vein which was divided at the diaphragm level and was anastomosed to the left atriotomy area through the pericardiostomy site., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Pulmonary arteriovenous malformations caused by abnormal hepatic flow distribution can develop in patients with a single ventricle with an interrupted inferior vena cava. However, preoperatively determining the hepatic baffle design that optimizes hepatic flow distribution is far from trivial. The current study combines virtual surgery and numeric simulations to identify potential surgical strategies for patients with an interrupted inferior vena cava., Methods: Five patients with an interrupted inferior vena cava and severe pulmonary arteriovenous malformations were enrolled. Their in vivo anatomies were reconstructed from magnetic resonance imaging (n = 4) and computed tomography (n = 1), and alternate virtual surgery options (intracardiac/extracardiac, Y-grafts, hepato-to-azygous shunts, and azygous-to-hepatic shunts) were generated for each. Hepatic flow distribution was assessed for all options using a fully validated computational flow solver., Results: For patients with a single superior vena cava (n = 3), intracardiac/extracardiac connections proved dangerous, because even a small left or right offset led to a highly preferential hepatic flow distribution to the associated lung. The best results were obtained with either a Y-graft spanning the Kawashima to split the flow or hepato-to-azygous shunts to promote mixing. For patients with bilateral superior vena cavae (n = 2), results depended on the balance between the left and right superior inflows. When those were equal, connecting the hepatic baffle between the superior vena cavae performed well, but other options should be pursued otherwise., Conclusions: This study demonstrates how virtual surgery environments can benefit the clinical community, especially for patients with a single ventricle with an interrupted inferior vena cava. Furthermore, the sensitivity of the optimal baffle design to the superior inflows underscores the need to characterize both preoperative anatomy and flows to identify the best option., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Azygos continuation of inferior venacava (IVC) is well known in association with interrupted IVC. We report azygos connection of uninterrupted IVC in a young child with complex univentricular heart. This peculiar anatomy was made suitable for univentricular repair by combining percutaneous device closure of prehepatic right limb of IVC and bidirectional Glenn shunt. The azygos vein acted as a conduit for IVC flow to superior venacava as in Kawashima's operation. This case highlights a rare IVC morphology. Also this innovative strategy allowed simple solution to a challenging problem and is first time described in the literature.

A 39-year-old female patient was referred for ablation of recurrent episodes of atrioventricular nodal reentrant tachycardia. A combination of an anomalous inferior vena cava with azygos continuation and a persistent left superior vena cava was discovered. A nonfluoroscopic navigation system was very useful for catheter ablation of the tachycardia in this unusual case of anomalous venous system of the heart.

Catheter ablation of the slow pathway for atrioventricular nodal re-entrant tachycardia (AVNRT) is not always possible due to congenital or acquired obstruction of the inferior vena cava (IVC). Although a superior access has been proposed as an alternative approach, a poor manoeuvrability and a lower stability of the ablation catheter may be potential problems. We report a case of slow pathway ablation for AVNRT in a patient with an azygos continuation using a hypoplastic but uninterrupted IVC.

Background: Portal hypertension is a common disease and its major surgical therapeutic approaches include devascularization and shunting. This study was undertaken to investigate the effects of combined splenocaval or mesocaval C shunt and portoazygous devascularization (combined procedures) on portal hypertension., Methods: The clinical data of 150 patients with portal hypertension who had undergone combined procedures at the First Affiliated Hospital of Zhengzhou University from May 1990 to May 2003 were analyzed retrospectively., Results: The mean free portal pressure (FPP) was 25.6+/-1.83 mmHg, 18.0+/-2.07 mmHg and 18.4+/-2.19 mmHg before operation, after splenectomy plus splenocaval or mesocaval C shunt, and combined procedures, respectively. There was no operative death in all patients. The 1-7 year follow-up of 100 patients showed rebleeding in 3 patients, encephalopathy in 4, thrombosis of artificial vascular graft in 3, and dying from liver failure in 2., Conclusions: The combined procedures can not only decrease portal pressure but also preserve hepatic blood flow to some extent. It may be one of the best choices for treating portal hypertension in China.

We report an unusual case of a patient with diminished cardiac output caused by systemic venous occlusions of unclear etiology with a history of idiopathic fibrosing mediastinitis and retroperitoneal fibrosis. On thoracic, abdominal, and pelvic venograms, the patient was previously known to have superior vena caval and infrarenal inferior vena caval occlusions. Subsequent studies revealed retrograde flow through the azygous and hemiazygous veins and numerous caval-portal anastomoses providing venous return from the upper and lower extremities. After an extensive hypercoagulable state work-up, no abnormalities were found. To relieve the symptoms, the patient underwent an elective right thoracotomy with azygous vein to right atrial bypass graft. The patient has had complete amelioration of the disabling symptoms and participates in many activities that he could not tolerate previously. Because this condition and procedure are unreported, we present a novel surgical treatment option to palliate symptoms caused by concurrent superior and inferior caval obstructions by improving venous return to the heart.

Ultrasonography, angiography, magnetic resonance imaging, and exploratory laparotomy of a 2-year-old wheaten terrier with lethargy, exercise intolerance, and ascites revealed segmental aplasia of the caudal vena cava with azygos continuation, complicated by thrombus formation. Surgeries were performed on the blind-ended vessel to remove thrombi, enhancing shunting of blood through the azygos vein.

Massive systemic-to-hepatic venous collaterals developed in a 8-year-old boy with left isomerism and azygos connection of the inferior vena cava who had undergone total cavopulmonary shunt leaving the hepatic vein connected to the common atrium 5 years previously. A hepatic vein-to-azygos vein connection via an autologous pericardial roll successfully ameliorated cyanosis and physical activity. Hepatic vein-to-azygos vein connection is technically easy and is favorable in providing balanced distribution of hepatic venous blood to both lungs. We recommend this technique as an effective alternative to hepatic vein-to-pulmonary artery connection in patients developing collateral vessels after total cavopulmonary shunt.

Agenesis of one or more segments and duplication of the infrarenal segment are, individually, rare abdominal variants of the inferior vena cava (IVC). In this article, the authors report on a case in which these two anomalies occurred concurrently. Details of the embryology are presented insofar as they are necessary for an understanding of this IVC variant. Spiral computed tomography makes it possible to define the vascular morphology and to make a differentiation from lymphadenopathy. Cavography gives an overall picture of the vascular morphology.

Absence of the inferior vena cava (IVC) is an uncommon congenital abnormality. Symptoms of lower extremity venous insufficiency resulting from this anatomic abnormality have been reported only once in the English literature, and no experience with surgical treatment of this condition has been published. We report the case of an otherwise healthy 41-year-old man with an 18-month history of severe venous insufficiency involving the right leg manifested by extensive ulceration that did not respond to aggressive conservative treatment. Duplex findings were not suggestive of venous obstruction or reflux, but venography documented no filling of the common iliac vein or inferior vena cava, and outflow was via collaterals to the azygous and hemiazygous systems. Computed tomography demonstrated complete absence of the inferior vena cava with azygous continuation. A prosthetic bypass from the external iliac to the intrathoracic azygous vein was performed with complete symptomatic relief after a 30-month follow-up period. Venous bypass surgery may play a role in treatment of this rare cause of venous insufficiency.

A case of incomplete endocardial cushion defect associated with mirror-image dextrocardia, IVC defect and azygos connection is reported. Intracardiac defect was corrected under moderate hypothermia with cardiopulmonary bypass. Three venous drainage cannulas were necessary to be indwelled into SVC, hepatic vein and right common iliac vein to maintain adequate venous drainage for extra-corporeal circulation. Thus, anomaly of venous system, which is commonly associated with mirror-image dextrocardia, must be recognized correctly and prepared before intracardiac correction.

An adult case of partial anomalous pulmonary venous drainage of left pulmonary veins with dextrocardia, situs inversus and azygos continuation of inferior vena cava is reported. Surgical repair was accomplished by constructing a baffle to direct the blood flow from left pulmonary veins to the left atrium through the created atrial septal defect. This case is a rare type of PAPVD.

Repeated thrombophlebitis with pulmonary embolism in a 28-year-old female led to the discovery of a complex malformation of the inferior vena cava which required the insertion of a clip. Three years later, recurrence of phlebitis with appearance of bipolar aphthae, arthritis and a meningeal syndrome led to the diagnosis of Behçet disease. Despite various medications for this disorder and heparin or oral anticoagulant therapy, the thromboembolic disease remained active, accompany with new exacerbations of Behçet disease. Diminished plasma fibrinolytic activity was demonstrated, probably resulting from venous endothelial vascularitis, which aggravates the consequences of the preexisting vascular malformation.

Successful surgical correction of transposition of the great arteries in a nine-year-old girl with dextrocardia, primum atrial septal defect, bilateral venae cavae, and azygos continuation of inferior vena cava is reported. The patient was cooled on cardiopulmonary bypass and the operation performed under circulatory arrest at 19 degrees C. nasopharyngeal temperature. The problems of diagnosis and management are discussed.

Twenty eight patients with penetrating injuries to the heart and/or the great vessels are presented. These constituted 9% of all penetrating chest injuries during the period between April 1975 and November 1976. The injuries were confined to the cardio-vascular system in 3 patients only. Twelve patients presented with signs of cardiac tamponade, of whom 2 succumbed intra-operatively due to ventricular fibrillation. Eight patients required respiratory support immediately after operation, of whom one died eventually due to extra-cardiac injuries. The mortality rate in this series was 11%. The remaining 25 survivors have resumed their regular life activities after discharge from the hospital. In none of the cases was the heart-lung machine used for repair.