Radiofrequency ablation of persistent atrial fibrillation in a patient with situs inversus totalis and interrupted inferior vena cava.

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.

Systematic review of electrophysiology procedures in patients with obstruction of the inferior vena cava.

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.

Transhepatic balloon mitral valvotomy in mitral stenosis with interrupted inferior vena cava.

A 38-year-old female was found to have severe mitral stenosis, severe pulmonary arterial hypertension with moderate tricuspid regurgitation, dilated right atrium, persistent left superior vena cava, and hugely dilated coronary sinus. The scheduled balloon mitral valvotomy via trans-femoral approach was abandoned after the venogram revealed the presence of left-sided inferior vena cava with hemi-azygos continuation draining into coronary sinus via left-sided superior vena cava. Balloon mitral valvotomy was attempted from the right trans-jugular route, but we were unable to puncture the inter-atrial septum due to the hugely dilated coronary sinus and right atrium. A transhepatic approach was used and balloon mitral valvotomy was successfully done with a standard balloon of 24 mm size without any complication. In patients with inferior vena cava anomalies or interruption, a percutaneous transhepatic approach is a feasible alternative for performing balloon mitral valvotomy.

Thoracoscopic AF ablation in situs inversus dextrocardia with interrupted inferior vena cava continuation in azygos vein.

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.

Clinical implications of congenital interruption of inferior vena cava.

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.

Prenatal diagnosis of hepatic interruption of the inferior vena cava with azygos/hemiazygos continuation without structural heart defects: A case series.

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.

Totally endoscopic atrial septal defect repair in a patient with interrupted inferior vena cava with azygos-hemiazygos continuation.

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.

Transcatheter Closure of a Secundum Atrial Septal Defect with Deficient Aortic Rim Through the Left Internal Jugular Vein in a Child with Situs Inversus and Interrupted Inferior Vena Cava: Device's Choice Matters.

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.

How to perform left atrial transseptal access and catheter ablation of atrial fibrillation from a superior approach.

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.

Hybrid closure of a large atrial septal defect using Occlutech Flex II septal occluder in a patient with interrupted inferior caval vein.

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.

Unconventional deployment of atrial septal occluder in a patient with atrial septal defect, dextrocardia, and interrupted inferior vena cava.

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.

Successful cryoablation of atrial fibrillation from jugular approach in patient with interrupted inferior vena cava and azygos continuation.

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.

Retrograde non trans-septal balloon mitral valvotomy in mitral stenosis with interrupted inferior vena cava, left superior vena cava, and hugely dilated coronary sinus.

A 22-year-old woman with severe mitral stenosis was referred to us for further evaluation and management. She was found to have severe mitral stenosis, severe tricuspid regurgitation with dilated right atrium and right ventricle with persistent left superior vena cava and hugely dilated coronary sinus. Valve was suitable for balloon mitral valvotomy. Cardiac catheterization showed interrupted inferior vena cava with azygos continuation to right atrium and large left superior vena cava draining to coronary sinus which was very much dilated. Right trans-jugular approach was tried for balloon mitral valvotomy, but was unsuccessful due to a very large right atrium and coronary sinus. Retrograde non trans-septal approach was used and balloon valvotomy was done successfully using a 24 mm × 40 mm TYSHAK balloon without any major complication. Reduction in the transmitral pressure gradient on cardiac catheterization data and transthoracic echocardiography confirmed successful procedure. Balloon mitral valvotomy can be done successfully in patients with the above unusual cardiac anatomy with no major procedural complications.

Heterotaxy syndrome with malrotation of the gut and interrupted vena cava does not preclude safe procurement of multivisceral graft.

We report the first successful procurement and transplantation of a multivisceral graft from a pediatric donor with polysplenic heterotaxy syndrome, including intestinal malrotation, midline liver with left-sided gallbladder and an interrupted inferior vena cava with azygous continuation. Procurement of the graft presented a surgical challenge in the presence of above anomalies. Modified approach to standard organ procurement and minor technical adaptation enabled successful transplantation. In an era of severe organ shortage of pediatric multivisceral grafts, a valuable organ offer should not lightly be declined for reasons of anatomic imperfections that might be overcome.

Hepatic venous reconstruction using the superficial femoral vein in a right-lobe living donor liver transplant patient with interrupted inferior vena cava.

Anatomical abnormalities in patients with BA often include polysplenia, preduodenal portal vein, interrupted retrohepatic IVC, cardiac abnormalities, and situs inversus. In LDLT patients who had congenital vascular anomalies, additional surgical modifications for the reconstruction of hepatic venous branches are sometimes necessary to prevent venous parenchymal congestion. We report a 12-yr-old female with post-Kasai BA with interrupted retrohepatic IVC who underwent right-lobe LDLT because the left liver graft volume was insufficient. The donor right liver graft had three major hepatic branches, including the RHV, IRHV, and MHV tributary (V8). We performed hepatic venous reconstruction by creating a large, wide triple orifice consisting of the RHV and two SFVs, which were anastomosed to the V8 and IRHV using the donor's SFV as an interposition graft. In conclusion, the reconstruction of venous orifices for right-lobe LDLT patients with the absent retrohepatic IVC is can be carried out using an SFV graft derived from the living donor or the recipient.

Prenatal ultrasonic diagnosis of esophageal atresia and tracheoesophageal fistula combined with interrupted inferior vena cava: a rare case report.

PURPOSE: This is an extremely rare case of complicated fetal esophageal atresia (EA) with tracheoesophageal fistula (TEF) and interrupted inferior vena cava (IVC) diagnosed by prenatal ultrsonography and successfully treated with surgical repair. METHODS: A 35-year-old pregnant woman was referred to our center for prenatal ultrasound, and the fetus was found to have a series of abnormalities, such as an interrupted IVC associated with a dilated azygos vein, an upper neck pouch sign of the thorax, and polyhydramnios. With suspicion of EA with TEF and interrupted IVC, the infant was born at 39 weeks of gestation, and successfully underwent the surgical operation. RESULTS: The baby was doing well after 21 months of follow-up. CONCLUSION: It is beneficial for the prenatal ultrasonic diagnosis of EA with TEF in optimizing labor care, postpartum treatment, and prompting neonatal management.

The Significance of Congenital Portosystemic Shunts in Congenital Heart Disease and the Bizarre Phenomenon of Alternating Portosystemic and Systemic-Portal Shunting.

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.

A case of combined pulmonary vein isolation (PVI) and watchman implant through hepatic vein in a patient with interrupted inferior vena cava (IVC).

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.

Off-label use of KONAR-MF™ occluder for transcatheter closure of patent ductus arteriosus in unusual circumstances.

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.

Complete atrioventricular block in an adult with congenitally corrected transposition of the great arteries with interrupted inferior vena cava.

A 56-year-old man got admitted as he was suffering from dizziness for 3 days. Electrocardiogram (ECG) showed complete atrioventricular (AV) block with ventricular rhythm of 35/min. We found that he had no inferior vena cava (IVC) which drained into right atrium in the middle of temporary pacing lead insertion. Venous drainage into superior vena cava from dilated azygos vein was identified after venogram. Echocardiogram revealed a congenitally corrected transposition of the great arteries (CCTGA). Chest computed tomography (CT) angiogram revealed AV and ventriculoarterial discordance with reversed ventricles and interrupted IVC with azygos continuation. DDD pacemaker was implanted via left axillary vein without any problem.