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The association of left isomerism with tetralogy of Fallot (TOF) is rare and reported as scattered case reports. Complex congenital cardiac and noncardiac malformations are known to be associated with left isomerism, but right-sided obstructive lesions are rare. We present our experience with a case of left isomerism with bilateral superior vena cava, interrupted inferior vena cava, and TOF managed by atrial septation and intracardiac repair.

No abstract available Keywords: Atrial fibrillation ablation; Interrupted inferior vena cava; Left atrial isomerism; Sinus node dysfunction; Transseptal access via internal jugular veins.

Triple duodenal obstruction is an extremely rare disorder—apparently only two cases were reported in the English literature. We present a third case complicated with some cardiovascular anomalies (interrupted inferior vena cava, left-sided inferior vena cava, preduodenal portal vein, and ventricular septal defect) and intestinal malrotation. Operative procedure to reconstruct continuity of the duodenal lumen was a combination of longitudinal incision, partial web resection, and transverse closure at the first obstruction site together with diamond-shaped duodeno-duodenostomy between the oral side of the second obstruction and the anal side of the third obstruction.

Background Pulmonary vein isolation (PVI) has entrenched itself as one of the main approaches for the treatment of paroxysmal symptomatic atrial fibrillation (AF). Pulmonary vein isolation prevents focal triggers from pulmonary veins from initiating AF paroxysms. As standard—PVI is performed through the inferior vena cava (IVC) approach, through the femoral vein. However, there are conditions when this approach is not appropriate or is not available. Case summary We report a case of a 53-year-old male who was referred to Pauls Stradins Clinical University Hospital for PVI due to worsening AF. Due to the rare anatomical variant of the venous system, the standard approach to PVI could not be applied. Interrupted cava inferior did not allow for femoral vein and IVC access. We had to figure out a different path—a combination of internal jugular and subclavian veins was used. Transseptal puncture was performed under transoesophageal echocardiography (TOE) control with a puncture needle stiletto. Pulmonary veins were isolated successfully, no complications were observed, and the patient was discharged in sinus rhythm. Discussion In some patients, PVI cannot be done through the standard IVC approach. In such cases, a different venous access must be chosen. Our patient had a rare variant of interrupted IVC and we had to use superior vena cava approach for the procedure. The difficulty of this approach is that procedure instruments are not designed for non-standard venous access; however, a combined use of TOE, general anaesthesia, and contact force-guided ablation has succeeded in isolating patients’ pulmonary veins.

Atrial septal defect combined with interrupted inferior vena cava (IVC), which is a rare condition, makes it impossible to apply totally endoscopic surgery (TES). The most difficulty is how to drain blood from the posthepatic segment of the IVC. In this study, we report a case of a 44-year-old female patient who was diagnosed with secundum atrial septal defect combined with interrupted IVC with azygos-hemiazygos continuation. This patient underwent successful repair by TES with transthoracic cannulation for the posthepatic segment of the IVC.

Percutaneous closure of secundum atrial septal defect (sASD) in children with interrupted inferior vena cava is challenging, especially in case of deficient aortic rim. Trans-jugular access is generally preferred in this scenario. Patients with situs inversus and sASD also carry technical difficulties for transcatheter closure because of the orientation of the atrial septum. We report a successful case of percutaneous closure of a sASD with deficient aortic rim using an occlutech figulla flex II ASD device through the left internal jugular vein in a child with situs inversus, dextrocardia, and interrupted IVC. This case was facilitated by the absence of left-sided hub of the Occlutech device to provide stable opening of the device into the left atrium, whereas the ball-connection of the delivery system allowed an angle of almost 180 degrees between the device and the atrial septum.

An atrial septal defect is a rare anomaly in patients with interrupted inferior vena cava, which renders the percutaneous intervention more complex; and hence, innovative approaches should be sought. Dextrocardia further complicates the procedure, and traditional atrial septal device deployment methods cannot be employed. We report a successful percutaneous secundum atrial septal defect closure by a novel deployment strategy along with balloon dilation of associated severe valvular pulmonary stenosis in a patient with dextrocardia and interrupted inferior vena cava.

Unilateral absence of pulmonary artery is a rare developmental anomaly. Infrahepatic inferior vena cava interruption is a well-recognized but uncommon developmental anomaly. Presence of both these anomalies in a single individual is extremely rare. A 58-year-old man with a history of recurrent lower extremity deep vein thrombosis and venous insufficiency presented to our emergency department with bilateral calf pain and swelling. Ultrasound demonstrated extensive deep vein thrombosis throughout bilateral lower extremities. Computed tomography angiography showed smooth tapering of the right pulmonary artery with absent distal most segment. To our knowledge, there is only 1 case report in the literature so far with both the abnormalities present in a single individual.

We present a case of a 31-year-old male with a large atrial septal defect, who was found to have interrupted inferior caval vein with azygous continuation to the superior caval vein, which precluded transcutaneous closure by device. The defect was successfully closed with a 33 mm Occlutech Figula septal occluder using a sub-mammary small thoracotomy incision and per-atrial approach without using cardiopulmonary bypass. The patient was discharged home after 48 hours of procedure.

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.

We herein report successful cryoablation of paroxysmal atrial fibrillation via right jugular vein in a patient with interrupted inferior vena cava. We preferred cryoablation instead of radiofrequency ablation in the treatment of our patient. For stronger support in aiming the Brockenbrough needle toward the septum, we manuallly curved the needle with a 120° angle about 6 cm proximal to the tip. After successful transseptal puncture, we performed balloon dilatation in the septal puncture zone to facilitate passage. Cryoablation has theoretical advantage, particularly in challenging anatomies, in which it eliminates the need for point-by-point ablation around the pulmonary vein.

Echocardiographic subcostal windows are very helpful to evaluate the junction of inferior vena cava (IVC) to right atrium (RA). Absence of intra hepatic segment of IVC, interruption of IVC, should be suspected when IVC-RA junction cannot be seen, especially in association with heterotaxy syndrome.

Background Interrupted inferior vena cava (IVC) is a rare venous anomaly that complicates the treatment of patients who require electrophysiology (EP) procedures. Methods We describe five consecutive cases of patients with interrupted IVC who presented to the EP laboratory requiring interventional procedures including catheter ablation for atrial fibrillation and supraventricular tachycardia and left atrial appendage closure. All cases were successfully completed utilizing a variety of approaches to vascular access including transseptal puncture via transhepatic and internal jugular approaches. Conclusion Procedures in the EP lab can be performed successfully in patients with interrupted IVC.

Pulmonary vein isolation (PVI) with Arctic Front cryoballoon has been an effective treatment for paroxysmal atrial fibrillation.1 The recent 2016 European Society of Cardiology guidelines and Heart Rhythm Society Guideline on the management of atrial fibrillation have established PVI as a cornerstone catheter ablation strategy during the primary index ablation for patients with symptomatic and drug-refractory atrial fibrillation.2 Furthermore, these guidelines recognized that cryoballoon catheter ablation can be an effective alternative to the traditional radiofrequency ablation catheters that have been historically used.2 The performance of PVI is typically achieved via the inferior vena cava (IVC) through the access of the femoral veins; however, congenital anomalies or venous occlusion may inhibit the use of this approach. In these rare circumstances, access to the cardiac chambers may be achieved via the superior vena cava or via retrograde aortic approaches. Unfortunately, these approaches are less favorable owing to reduced catheter control, manipulation, and stability.3, 4, 5 The purpose of this case report is to illustrate the safety and efficacy of performing PVI with the Arctic Front cryoballoon catheter via the transhepatic approach among patients with congenital anomalies. Access to the right atrium via the percutaneous transhepatic approach is an alternative method when traditional means are not feasible. The percutaneous transhepatic approach has been shown to be effective, with a complication rate of

Femoral venous approach is the classic route of percutaneous atrial septal defect (ASD) closure. But in patients with interrupted inferior vena cava (IVC) with azygos continuation the normal connection is lost rendering transcatheter intervention more complicated and innovative approaches should be sought. Transhepatic approach has been used with success but this approach is technically demanding and could lead to major complications. We report successful percutaneous secundum ASD closure in 2 patients with interrupted IVC. Transjugular route was used to close the secundum ASD in a 15-month-old girl. Percutaneous ASD closure through femoral venous route was performed in a 49-year-old woman. We found that both these approaches of percutaneous ASD device occlusion can be performed with success and safety.

Key Clinical Message Isolated persistent left superior vena cava (SVC) in the absence of right SVC is a rare congenital variant of thoracic venous drainage with the left subclavian and jugular veins that drain into the right atrium through the coronary sinus. Inferior vena cava interruption with azygos continuation is another congenital anomaly resulting in venous drainage of the lower extremities via a typically dilated azygos vein. Although both variants are generally asymptomatic and incidentally detected, these can have clinical implications in specific circumstances and in particular during device implantation. We report a case of pacemaker implantation in which both anatomical variants were present.

An 11-month-old girl with an unbalanced atrioventricular septal defect, coarctation of the aorta and an interrupted inferior vena cava with hemiazygos continuation post-surgical status of coarctation repair and pulmonary artery banding underwent a Kawashima operation. Severe hypoxia occurred immediately after she was weaned from cardiopulmonary bypass, which was successfully resolved by the ligation of the hemiazygos vein. Postoperative enhanced computed tomography detected significant venovenous malformation from the hemiazygos vein to the left renal vein.

We present a male newborn referred to pediatric cardiology due to a heart murmur, whose echocardiographic evaluation showed an intrahepatic inferior vena cava interruption with azygos continuation and two small mesoseptal ventricular septal defects. Although mainly incidental, the early diagnosis of interrupted inferior vena cava can be crucial to avoid complications of invasive vascular procedures.

Interruption of the inferior vena cava with azygos continuation is a rare congenital anomaly. This anomaly becomes clinically important during cardiac interventions.

Plastic bronchitis is a rare life-threatening complication of the Fontan operation. Transcatheter Fontan fenestration can ameliorate symptoms by decompressing elevated venous pressures. Transcatheter creation of a fenestration can be technically challenging in cases with complex venous anatomy. We report a case of a 5-year-old boy with heterotaxy, dextrocardia with unbalanced atrioventricular canal (AVC), atrial and visceral situs inversus, left-sided superior vena cava (SVC), and left-sided interrupted inferior vena cava (IVC) with azygos continuation. With few modifications to the equipment, a successful Fontan fenestration with stent implantation was performed via transjugular approach. At 2-year follow-up, his symptoms of plastic bronchitis improved significantly.

A 76-year-old woman with paroxysmal atrial fibrillation (CHA2DS2-VASc score 4) and recurrent nose and gastrointestinal bleeds was referred for a Watchman left atrial appendage closure device (Boston Scientific, Natick, Massachusetts) implantation. Transthoracic echocardiography showed a mildly

Femoral venous route is routinely used for percutaneous closure of atrial septal defects (ASDs). However, a situation may arise where transfemoral approach is not feasible. We describe a successful transjugular closure of a moderate-sized ASD in a 49-year-old symptomatic man with interrupted inferior vena cava, using a novel deployment technique, which helped in overcoming difficulties such as maintaining stable sheath position and minimizing risk of air embolism.

Abnormalities of the systemic and pulmonary veins can occur in isolation or in conjunction with other congenital heart defects. When venous anomalies present in isolation it can be particularly difficult to detect them during routine fetal anomaly scans as these are relatively small extra-cardiac structures. However, advances in ultrasound technology with higher resolution on 2D and colour Doppler ultrasound have facilitated and improved prenatal detection. The systemic venous variations and abnormalities, which will be discussed in this chapter include persistent left superior vena cava, interrupted inferior vena cava and agenesis of the ductus venosus. Anomalies of the pulmonary veins including total or partial anomalous pulmonary venous connections may be difficult to diagnose in isolation. Disorders of laterality which can be associated with systemic and/or pulmonary venous abnormalities as well as intracardiac defects will also be addressed.

A 15year old girl who underwent surgical correction of ventricular septal defect and patent ductus arteriosus ligation in childhood presented with atrial tachycardia of crista terminalis origin and counterclockwise atrial flutter. She also had associated interruption of inferior vena cava which continued as azygous vein and left superior vena cava which drained via coronary sinus into the right atrium. She underwent radiofrequency ablation of both the tachycardias via internal jugular vein and azygous vein approach using 3D electroanatomical mapping system.