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Congenital portosystemic shunts (CPSSs) are rare vascular anomalies characterized by abnormal connections between the portal/splanchnic veins and the systemic veins. CPSSs often occur as an isolated congenital anomaly, but they can also coexist with congenital heart disease (CHD). Owing to their myriad consequences on multiple organ systems, familiarity with CPSS is of tremendous importance to the care of patients with CHD. The rationale and timing for interventions to embolize CPSS in this scenario are discussed. Specific shunt embolization techniques are beyond the scope of this article. [ABSTRACT FROM AUTHOR]

Pulmonary vein isolation is a primary therapeutical option for controlling atrial fibrillation, particularly in cases complicated with heart failure or previously ineffective pharmacological antiarrhythmic treatment. Different techniques share the punction of the femoral vein followed by the insertion of endocardial catheters through the inferior vena cava. When an interrupted vena cava inferior is present, another approach is needed to reach the target area for ablation. We present a case and the circumstances of a 48-year-old female, when transjugular pulmonary vein isolation was performed due to persistent atrial fibrillation.

Introduction: Catheter ablation of atrial fibrillation (AF) has emerged as the most effective therapy. However, rare anatomical abnormalities such as situs inversus totalis, dextrocardia, or interrupted inferior vena cava can make ablation challenging. Methods and Results: We report a case of a 55‐year‐old woman with situs inversus totalis, dextrocardia, surgical atrial septal defect repair, left‐sided dual chamber pacemaker in place, and symptomatic recurrent persistent AF who underwent successful pulmonary vein and posterior wall isolation by the superior access from the left internal jugular vein. Conclusions: It is a feasible and safe approach with support of transesophageal echocardiography and multiple emerging technologies. [ABSTRACT FROM AUTHOR]

Key Clinical Message: This case report describes a successful procedure involving pulmonary vein isolation (PVI) and left atrial appendage (LAA) closure with a watchman device in a 78‐year‐old male with atrial fibrillation and an interrupted inferior vena cava. Due to the vascular anomaly, a transhepatic approach was used, which proved successful. [ABSTRACT FROM AUTHOR]

Key Clinical Message This case report describes a successful procedure involving pulmonary vein isolation (PVI) and left atrial appendage (LAA) closure with a watchman device in a 78‐year‐old male with atrial fibrillation and an interrupted inferior vena cava. Due to the vascular anomaly, a transhepatic approach was used, which proved successful.

Background: Transcatheter patent ductus arteriosus (PDA) closure with unusual morphology associated with multiple constrictions or unusual circumstances such as interruption of inferior vena cava (IVC) is still challenging. This study evaluates the use of KONAR-MF™ (Lifetech Scientific Co Ltd., Shenzhen) for transcatheter closure of PDA in such situations. Materials and Methods and Results: Between January 2021 and October 2021, 24 patients from three different tertiary care centers underwent PDA device closure using the KONAR-MF™ device and are included in the study. Patient demographics, echocardiographic assessment, procedural details including the approach, device details, complications, and follow-up data were recorded. The median age and weight of the cohort were 2 years (0.5-41) and 10 kg (5-98), respectively. The indications for using KONAR-MF™ were (a) unusual morphology of PDA in 14, long tubular PDA in 8, and long tubular with multiple constrictions in 6 patients and (b) unusual circumstances such as interrupted IVC in 6 and inability to cross PDA antegradely in 4 due to dilated main pulmonary artery. The procedure was successful in all the patients and no procedure-related complications were noted in this cohort. Median follow-up of 9 months (range: 5-18) showed no residual shunt and only one patient had flow acceleration across the left pulmonary artery (peak Doppler gradient 16 mmHg). Conclusions: KONAR-MF™ device can be used safely and effectively for transcatheter closure of PDA with unusual anatomy or in unusual circumstances. [ABSTRACT FROM AUTHOR]

Background : Transcatheter patent ductus arteriosus (PDA) closure with unusual morphology associated with multiple constrictions or unusual circumstances such as interruption of inferior vena cava (IVC) is still challenging. This study evaluates the use of KONAR-MF™ (Lifetech Scientific Co Ltd., Shenzhen) for transcatheter closure of PDA in such situations. Materials and Methods and Results: Between January 2021 and October 2021, 24 patients from three different tertiary care centers underwent PDA device closure using the KONAR-MF™ device and are included in the study. Patient demographics, echocardiographic assessment, procedural details including the approach, device details, complications, and follow-up data were recorded. The median age and weight of the cohort were 2 years (0.5–41) and 10 kg (5–98), respectively. The indications for using KONAR-MF™ were (a) unusual morphology of PDA in 14, long tubular PDA in 8, and long tubular with multiple constrictions in 6 patients and (b) unusual circumstances such as interrupted IVC in 6 and inability to cross PDA antegradely in 4 due to dilated main pulmonary artery. The procedure was successful in all the patients and no procedure-related complications were noted in this cohort. Median follow-up of 9 months (range: 5–18) showed no residual shunt and only one patient had flow acceleration across the left pulmonary artery (peak Doppler gradient 16 mmHg). Conclusions : KONAR-MF™ device can be used safely and effectively for transcatheter closure of PDA with unusual anatomy or in unusual circumstances.

Background/purpose: Inferior vena cava (IVC) interruption is rare and can be detected through prenatal or postnatal imaging. It usually occurs in patients with heterotaxy syndrome with bilateral left-sidedness (left isomerism or polysplenia syndrome), indicating a laterality defect. However, its long-term outcomes remain unclear. Methods: This retrospective study included a patient cohort with evidence of IVC interruption based on imagining data (1980–2019) selected from our institutional database. Results: We included 34 (male/female = 14/20) patients with IVC interruption. Most of the patients had left isomerism of the bronchopulmonary situs (96.4%) and cardiac atrial situs (90.3%). Splenic anomalies, including polysplenia (35.7%), lobulated spleen (39.3%), inversus solitary spleen (10.7%), and asplenia (3.6%), were common. Normal cardiac structure was noted in four (11.8%) patients. Congenital heart disease (CHD) was noted in 30 patients: 7 with simple CHD and 23 with severe CHD. Bradycardia occurred in 47.1% of the patients and was not associated with CHD. Splenic variations were not associated with CHD or bradycardia. The survival rates for the 10-, 20-, and 40-year age groups were 0.880, 0.792, and 0.441, respectively; severe CHD was the only risk factor. Conclusion: IVC interruption can present as an isolated lesion and be associated with CHD. Although bradycardia was common among the patients, CHD severity was the only risk factor for survival. Patients with IVC interruption commonly have left isomerism at the atrial and bronchopulmonary situs, but the spectrum of splenic abnormalities is wide, including polysplenia, lobulated spleen, solitary inversus spleen, and, rarely, asplenia.

Percutaneous transcatheter closure of fossa ovalis atrial septal defect (FOASD) is an established procedure. When femoral venous approach is unfeasible due to interrupted inferior vena cava (IVC) or occluded femoral veins, other interventional methods through transjugular, transhepatic approach, or surgical closure can be performed. We report two cases of transhepatic device closure of FOASD associated with IVC interruption successfully performed without any complications.

Background/purpose: Inferior vena cava (IVC) interruption is rare and can be detected through prenatal or postnatal imaging. It usually occurs in patients with heterotaxy syndrome with bilateral left-sidedness (left isomerism or polysplenia syndrome), indicating a laterality defect. However, its long-term outcomes remain unclear.Methods: This retrospective study included a patient cohort with evidence of IVC interruption based on imagining data (1980-2019) selected from our institutional database.Results: We included 34 (male/female = 14/20) patients with IVC interruption. Most of the patients had left isomerism of the bronchopulmonary situs (96.4%) and cardiac atrial situs (90.3%). Splenic anomalies, including polysplenia (35.7%), lobulated spleen (39.3%), inversus solitary spleen (10.7%), and asplenia (3.6%), were common. Normal cardiac structure was noted in four (11.8%) patients. Congenital heart disease (CHD) was noted in 30 patients: 7 with simple CHD and 23 with severe CHD. Bradycardia occurred in 47.1% of the patients and was not associated with CHD. Splenic variations were not associated with CHD or bradycardia. The survival rates for the 10-, 20-, and 40-year age groups were 0.880, 0.792, and 0.441, respectively; severe CHD was the only risk factor.Conclusion: IVC interruption can present as an isolated lesion and be associated with CHD. Although bradycardia was common among the patients, CHD severity was the only risk factor for survival. Patients with IVC interruption commonly have left isomerism at the atrial and bronchopulmonary situs, but the spectrum of splenic abnormalities is wide, including polysplenia, lobulated spleen, solitary inversus spleen, and, rarely, asplenia. [ABSTRACT FROM AUTHOR]

Introduction: Situs inversus totalis, dextrocardia with interrupted inferior vena cava, and azygos vein continuation concomitant with symptomatic atrial fibrillation requiring ablation. This case was deemed not suitable for percutaneous ablation due to anatomic variations and the lack of case reports in the literature. Methods and Results: We performed bilateral thoracoscopic epicardial ablation and epicardial left atrial appendage exclusion. The direct vision allowed for a complete box lesion set with bipolar radiofrequency device. Patient remained in sinus rhythm at the 12‐months follow‐up. Conclusion: Surgical thoracoscopic epicardial ablation is safe and effective also in congenital defects. Multidisciplinary expertise can offer minimally invasive ablation treatments. [ABSTRACT FROM AUTHOR]

Competing Interests: Disclosures The authors have no conflicts to disclose.

Objectives: To describe fetal spectrum and echocardiographic characteristics of interrupted inferior vena cava (IIVC) with azygos/hemiazygous continuation without other structural heart defects and to evaluate its association with visceral heterotaxy and isomerism, extracardiac and genetic anomalies, and to review neonatal outcome. Methods: This was a retrospective study of 14 fetuses with a confirmed diagnosis of IIVC with normal intracardiac anatomy. The following variables were collected; indication for referral, gestational age at diagnosis; associated isomerism and visceral heterotaxy, heart rhythm, genetic and extracardiac abnormalities, and fetal/neonatal outcome. Results: Among 36 fetuses with IIVC, 14 cases (38.8%) had normal intracardiac anatomy. These IIVC cases correspond to 0.19% (14/7250) of all fetal cardiac examinations, and to 1.5% (14/922) of all cardiac abnormalities. Six patients had visceral abnormalities. Atrial appendage morphology was clearly depicted in three fetuses, both appendages were left. One fetus had bradyarrhythmia revealing atrial ectopic rhythm. Six fetuses did not have any concomitant cardiac or visceral abnormalities, therefore regarded as isolated. All babies were delivered at term with a good prognosis. Conclusion: Our study has shown that almost half of the IIVC cases without intracardiac structural anomalies displayed other findings of isomerism while the other half was isolated benign vascular variant. Therefore, prenatal diagnosis of IIVC should prompt a comprehensive evaluation for cardiac, situs, and visceral anomalies. The outcome is favorable. [ABSTRACT FROM AUTHOR]

Aims: The objective of the study was to conduct a systematic review to describe and compare the different approaches for performing cardiac electrophysiology (EP) procedures in patients with interrupted inferior vena cava (IVC) or equivalent entities causing IVC obstruction. Methods: We conducted a structured search to identify manuscripts reporting EP procedures with interrupted IVC or IVC obstruction of any aetiology published up until August 2020. No restrictions were applied in the search strategy. We also included seven local cases that met inclusion criteria. Results: The analysis included 142 patients (mean age 48.9 years; 48% female) undergoing 143 procedures. Obstruction of the IVC was not known before the index procedure in 54% of patients. Congenital interruption of IVC was the most frequent cause (80%); and, associated congenital heart disease (CHD) was observed in 43% of patients in this setting. The superior approach for ablation was the most frequently used strategy (52%), followed by inferior approach via the azygos or hemiazygos vein (24%), transhepatic approach (14%), and retroaortic approach (10%). Electroanatomical mapping (58%), use of long sheaths (41%), intracardiac echocardiography (19%), transesophageal echocardiography (15%) and remote controlled magnetic navigation (13%) were used as adjuncts to aid performance. Ablation was successful in 135 of 140 procedures in which outcomes were reported. Major complications were only reported in patients undergoing AF ablation, including two patients with pericardial effusion, one of whom required surgical repair, and another patient who died after inadvertent entry into an undiagnosed atrioesophageal fistula from a previous procedure. Conclusion: The superior approach is most frequent approach for performing EP procedures in the setting of obstructed IVC. Transhepatic approach is a feasible alternative, and may provide a "familiar approach" for transseptal access when it is required. Adjunctive use of long sheaths, intravascular echocardiography, electro‐anatomical mapping and remote magnetic navigation may be helpful, especially if there is associated complex CHD. With careful planning, EP procedures can usually be successfully performed with a low risk of complications. [ABSTRACT FROM AUTHOR]

Percutaneous transcatheter closure of fossa ovalis atrial septal defect (FOASD) is an established procedure. When femoral venous approach is unfeasible due to interrupted inferior vena cava (IVC) or occluded femoral veins, other interventional methods through transjugular, transhepatic approach, or surgical closure can be performed. We report two cases of transhepatic device closure of FOASD associated with IVC interruption successfully performed without any complications. [ABSTRACT FROM AUTHOR]

Interrupted inferior vena cava (IVC) with azygous continuation provides technical challenge in many percutaneous cardiac interventions. They are performed via the femoral venous access route. We describe four such cases in whom, patent ductus arteriosus (PDA) device closure was done in interrupted IVC. All the four cases were done using the femoral route. Two cases were done via retrograde approach through femoral artery access, in which one was closed with a muscular VSD device and the other with a Gianturco coil. The introduction of newer closure devices helps in the retrograde approach. Two cases were done via anterograde approach through the femoral venous route, with a loop through the azygous vein. One child had transient bradycardia due to cardiac stretch which normalized after the device deployment and introducer system removal. Thus, we describe different femoral approaches in interrupted IVC patients for PDA closure, with the difficulties faced and the complications managed.

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

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. [ABSTRACT FROM AUTHOR]

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.

Transcatheter closure of secundum atrial septal defect (ASD II) is considered the treatment of choice when anatomically suitable and clinically indicated. This is routinely done through the inferior vena cava via a femoral venous approach; however, certain anatomical anomalies, such as congenital interruption of inferior vena cava with azygos continuation, render the delivery of the device either difficult or not possible. When this anomaly is also associated with dextrocardia, the technical challenge increases further due to the unusual orientation of the interatrial septum. We describe a case of transcatheter closure of ASD II via left internal jugular vein approach with some modification of the standard technique. We used a combination of veno-arterial rail by parking the super-stiff wire in the descending aorta instead of the usual method of parking it in the pulmonary vein for better support, and over the wire technique during device deployment to maintain the delivery sheath position during device deployment. Changing the vascular access with the modification of the standard technique of closure was the key to success in this patient. < Learning objective: Transcatheter atrial septal defect closure in patients with interrupted inferior vena cava can be successfully performed via trans jugular approach using a combination of veno-arterial rail and over the wire technique as a technical modification to the standard procedure's steps inorder to increase the support while crossing with the delivery sheath and maintain its position during deployment of the device.> [ABSTRACT FROM AUTHOR]

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

Interrupted inferior vena cava (IVC) with azygous continuation provides technical challenge in many percutaneous cardiac interventions. They are performed via the femoral venous access route. We describe four such cases in whom, patent ductus arteriosus (PDA) device closure was done in interrupted IVC. All the four cases were done using the femoral route. Two cases were done via retrograde approach through femoral artery access, in which one was closed with a muscular VSD device and the other with a Gianturco coil. The introduction of newer closure devices helps in the retrograde approach. Two cases were done via anterograde approach through the femoral venous route, with a loop through the azygous vein. One child had transient bradycardia due to cardiac stretch which normalized after the device deployment and introducer system removal. Thus, we describe different fem Thus, we describe different femoral approaches in interrupted IVC patients for PDA closure, with the difficulties faced and the complications managed. [ABSTRACT FROM AUTHOR]

Competing Interests: The authors have no conflicts to disclose. Written patient consent was received for the publication of this article.

We describe a case of an elderly female with complete heart block where temporary pacemaker insertion led to diagnosis of inferior vena cava interruption, leading further to diagnosis of polysplenia syndrome.

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. [ABSTRACT FROM AUTHOR]

Objective. To share our experience in the prenatal diagnosis of agenesis of ductus venosus (ADV) secondary to interrupted inferior vena cava (IIVC) and how does this affect the outcome. Methodology. A prospective study has been conducted in our center regarding the ADV outcome, since October 2016. In all cases, the main features of abdominal fetal circulation were evaluated in all secondtrimester ultrasound scans, including the presence of a normal drainage of the umbilical vein, via ductus venosus, and the normal location of inferior vena cava (IVC). We present an analysis regarding the outcome of the ADV cases secondary to IIVC and we compare our data with those from the literature. Results. A total of five cases with prenatally diagnosed ADV and IIVC were identified. The gestational age at diagnosis ranged from 18 to 27 weeks. Karyotyping was performed in all cases but one, with an abnormal result in one case. Two cases were part of left isomerism syndrome and had an unfavorable outcome. Two cases did not present other anomalies and had a good outcome, according to the neonatal and pediatric cardiology follow-up. Conclusions. Postnatal outcome in cases with ADV and IIVC mainly depends on the presence of concomitant malformations. In isolated cases, the prognosis is generally good, but counseling is often difficult in such cases, and it is very important to exclude other structural or genetic anomalies. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The standard technique for percutaneous catheter ablation of atrial fibrillation (AF) involves obtaining left atrial access and catheter manipulation from an inferior transfemoral venous access. However, in patients with inferior vena cava interruption, a standard transfemoral venous approach is not possible. In these cases, a percutaneous approach from a superior central vein, such as the internal jugular vein or the axillary/subclavian vein can be considered. In this article, we describe the details of our technique to obtain left atrial catheterization and perform catheter ablation of AF from a superior approach. Our technique involves the use of steerable sheaths, dedicated radiofrequency wires, and intracardiac echocardiography guidance. [ABSTRACT FROM AUTHOR]

Objective: Interrupted inferior vena cava (IVC) is a well-recognized but a rare congenital anomaly due to absence of hepatic portion of the IVC and can therefore pose problems while undertaking cardiac catheterization in these particular patients. We are sharing our experience of cardiac catheterization in patients with interrupted IVC. Study Design: Retrospective descriptive study. Place and Duration of Study: Armed Forces Institute of Cardiology/National institute of heart diseases (AFIC/NIHD) Rawalpindi, from Jan 2017 to Dec 2018. Methodology: This retrospective descriptive study was done at the Paediatric cardiology department of the Armed Forces Institute of Cardiology/National institute of heart diseases (AFIC/NIHD) Rawalpindi and comprised of 34 consecutive cases with interrupted IVC, who underwent cardiac catheterization procedure from January 2017 to December 2018. Results: Total of 34 consecutive patients with interrupted IVC underwent cardiac catheterization procedure (29 diagnostic procedures and 5 interventional procedures) with mean age of 4.3 ± 4.5 years, including 19 males & 15 females. The mean procedural time and fluoroscopy time were 55 & 17 minutes respectively and no major complications were encountered in study population. Conclusion: Cardiac catheterizations in patients with interrupted IVC can be challenging especially in device closure of atrial septal defects and patent ductusarteriosus. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Varicocele represents the main cause of male infertility. Right-sided varicocele is rare and can be due to renal malignancy or a venous abnormality. The most common anomaly of the inferior vena cava (IVC) is interruption of IVC with azygos continuation, which is recognized as an uncommon congenital anomaly. The prevalence of the interruption of IVC is less than 0.3% in the healthy population. We describe the case of a 26-year-old man who had right varicocele because of a right-sided IVC with a retro-aortic left renal vein and azygos continuation. The right and left IVCs received the right and left common iliac veins, respectively, and the left renal vein crossed posteriorly to the aorta and joined the right IVC. The right IVC continued cephalad as the azygos vein within the retrocrural space. Isolated right-sided varicoceles are uncommon, but practitioners should be aware of such a condition. In case of a venous anomaly, clinicians should aware of the association with other important clinical presentations. [ABSTRACT FROM AUTHOR]

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. < Learning objective: Interrupted inferior vena cava with azygos or hemiazygos continuation is a rare congenital anomaly but potentially complicates the usual percutaneous atrial septal defect (ASD) closure and therefore merits an accurate diagnosis before the procedure. Percutaneous ASD device closure can be performed with success and safety through femoral and transjugular approaches in patients with ostium secundum ASD who present with interrupted inferior vena cava.> [ABSTRACT FROM AUTHOR]

Absence of inferior vena cava is an uncommon congenital abnormality. It is usually associated with other structural anomalies, typically left isomerism. We report a case of interrupted inferior vena cava with azygos continuation diagnosed as an isolated finding during routine prenatal ultrasound scan, confirmed by post-natal echocardiography. Detailed ultrasound examination of the fetal anatomy failed to demonstrate other anomalies. The neonatal course of this fetus was uneventful.