Interruption Of Inferior Vena Cava Research Articles

Transesophageal Echocardiographic Assessment of Single Ventricle Physiology with Interrupted Inferior Vena Cava in a Case of Heterotaxy Syndrome Posted for Kawashima Procedure and Hepatic Vein Rerouting

Transesophageal echocardiography (TEE) is essential in delineating the anatomy, physiology, and perioperative management of single ventricle (SV) lesions. SV lesion is commonly associated with heterotaxy syndrome causing abnormal lateralization of organs across the body’s left-right axis. It is manifested as right atrial or left atrial isomerism. Patients with SV physiology require the Glenn procedure for surgical palliation whereas Fontan operation as definitive intervention. However, in a patient with interrupted inferior vena cava (IVC), the Kawashima procedure is used for definitive palliation. We report a 15-year-old female child diagnosed with SV physiology and interrupted IVC in association with heterotaxy syndrome posted for the Kawashima procedure and hepatic vein rerouting and illustrate the importance of perioperative TEE in delineating the anatomy, decision-making, and confirming the success of repair.

Abstract 17847: Typical Right Atrial Flutter Ablation Using Subclavian Venous Access in Patients With Interrupted or Occluded Inferior Vena Cava

Introduction: Ablation for typical atrial flutter (AFL) traditionally involves vascular access from the femoral vein to facilitate advancing catheters through the Inferior Vena Cava (IVC) to the right atrium (RA). In patients with interrupted or occluded IVC, this procedure is challenging to perform. We report a case series of typical right AFL ablation using subclavian access in interrupted or occluded IVC. Cases: A 63-year-old female with typical AFL refractory to medical therapy was referred for radiofrequency ablation (RFA). She had a history of surgically corrected atrial septal defect at age 4 years with known IVC interruption draining into RA via an azygous venous extension. Another case involved a 41-year-old male with quadriplegia and typical AFL who underwent an attempted RFA from femoral access that was aborted due to massive thrombosis of IVC. Decision making: In both cases, two sheaths were placed into the left subclavian venous system using ultrasound guidance. A coronary sinus catheter and irrigated tip ablation catheter were advanced into the coronary sinus and RA respectively. Additionally, in the second case, an intracardiac echocardiography probe was inserted via separate venous access. Using the electroanatomic mapping system, entrainment and activation mapping confirmed a tricuspid isthmus-dependent AFL. RFA was performed using an open irrigated RFA catheter in a linear fashion resulting in the termination of the tachycardia to sinus rhythm. In the first case, ablation extended from the tricuspid annulus to the os of the hepatic vein at the 06:00 position. Post ablation, hemostasis was achieved using a suture-mediated closure device. At follow-up, both patients were asymptomatic without evidence of AFL on extended-duration cardiac monitoring. Conclusion: In patients with interrupted or occluded IVC, the subclavian veins can be accessed for mapping and ablation of typical AFL when traditional femoral venous access is challenging.

Prenatal diagnosis and clinical outcomes of isolated interruption of the inferior vena cava: an analysis of 12 cases

Objectives: This study aimed to comprehensively investigate the clinical manifestations and outcomes of prenatally diagnosed isolated interrupted inferior vena cava (IIVC) with azygos continuation to shed light on the significance of prenatal diagnosis.
 
 Methods: A longitudinal study involved 12 fetuses prenatally diagnosed with IIVC and azygos continuation. Detailed fetal anomaly scans and echocardiography were performed, and pediatric cardiologists confirmed postnatal diagnoses. Genetic testing and extensive follow-ups were also performed.
 
 Results: The study confirmed the high diagnostic accuracy of prenatal identification, with 100% postnatal confirmation. Most patients exhibited favorable outcomes, emphasizing the importance of prenatal diagnosis. Genetic testing revealed normal chromosomal arrangements in all tested cases.
 
 Conclusion: Our study underscores the vital role of prenatal diagnosis in managing isolated IIVC cases and highlights their generally favorable prognosis. This study contributes to understanding this rare vascular anomaly and its implications for clinical practice.

Orthostatic proteinuria due to inferior vena cava interruption without nutcracker phenomenon in an old obese female: a case report and literature review

BackgroundNutcracker syndrome (NCS) caused by left renal vein (LRV) entrapment, is one of the most common causes of orthostatic proteinuria. In stereotype, orthostatic proteinuria is often accompanied by left renal vein obstruction and is found in young and underweight individuals. Here, we report a rare case with orthostatic proteinuria in an old obese female caused by a rare type of congenital inferior vena cava (IVC) interruption.Case presentationA 65-year-old obese woman, who suffered from fluctuated proteinuria, had been misdiagnosed as chronic glomerulitis for 30 years. Instead of having any sign of NCS, she had a unique type of IVC interruption. Most venous blood from infrarenal IVC and right kidney drained into her LRV, and then through the expanded communicating vessel, drained into the left ascending lumbar vein which extended as hemiazygos vein. To the best of our knowledge, this is one of the first cases reported of orthostatic proteinuria attributed to the subsequent hemodynamic irregularity caused by IVC interruption without nutcracker phenomenon.ConclusionAdult-onset orthostatic proteinuria is relatively rare, hard to be recognized and could be misdiagnosed as chronic glomerulonephritis. The case provided a novel differential diagnostic condition for those who suffered from fluctuated proteinuria of unknown causes.

Congenital venous anomalies associated with retrocaval ureter: evaluation using computed tomography.

Retrocaval ureter is a rare congenital anomaly resulting from anomalous development of inferior vena cava (IVC) and not from anomalous of the ureter. The anomaly always occurs on the right side due to regression of right supracardinal vein and persistence of right posterior cardinal vein. Retrocaval ureter tends to be associated with various vena cava anomalies because of the embryogenesis. We aimed to identify the prevalence of associated congenital venous anomalies (CVA) resulting from cardinal vein development in adults with retrocaval ureter using computed tomography (CT) images. The study included 22 adults with retrocaval ureter. We evaluated CT findings and determined the incidence of associated CVA using thin slice data sets from CT scanner with 64 or more detectors. We compared the prevalence of CVA in the retrocaval ureter group (mean age: 57 ± 19 years) and in the control group of 6189 adults with normal ureter (mean age: 66 ± 14 years). In the retrocaval ureter group, 4 (18.2%) adults had CVA including double IVC, right double IVC, preisthmic IVC with horseshoe kidney, and preaortic iliac confluence. One of 2 adults with preaortic iliac confluence had right double right IVC. In the control group, 49 (0.79%) adults had CVA including 37 double IVC, 11 left IVC, and 1 IVC interruption azygos continuation. Fifteen horseshow kidneys were found. The prevalence of associated CVA in the retrocaval ureter group was higher than that in the control group (p < 0.001). Retrocaval ureter is frequently associated with CVA. Various CVA with retrocaval ureter could happen because of abnormal development of not only the right posterior or supra cardinal vein but also other cardinal veins.

PO-04-086 IMPLANTATION OF A LEADLESS PACEMAKER FOR HYBRID DUAL CHAMBER PACING IN A PATIENT WITH A FAILING RIGHT VENTRICULAR LEAD AND HIGH SURGICAL RISK: A CASE REPORT

Mechanical atrial sensing in Micra AV (Medtronic) leadless pacemakers facilitates atrioventricular (AV) synchrony in patients with AV block despite single chamber pacing. Little data is available on the efficacy of Micra AV atrial tracking in patients with concomitant sinus node dysfunction requiring atrial pacing. We present a case that demonstrates the efficacy and utility of Micra AV pacing in the context of transvenous atrial pacing. N/A A 49-year-old woman with complete heart block, sinus node dysfunction, right-sided dual-chamber pacemaker, and subsequent right atrial (RA) lead dysfunction treated with RA lead capping and new implantation, presented to our institution for pacemaker management. Device interrogation revealed rising RV lead impedances and pacing thresholds, for which lead extraction and re-implantation was recommended. Pre-operative imaging revealed partial flow obstruction in the right subclavian vein, an interrupted inferior vena cava, and a persistent left superior vena cava (SVC) with no right SVC connection. As urgent intra-operative cardiopulmonary bypass in the event of vascular rupture would prove difficult, the patient was deemed not a candidate for lead extraction. In an effort to prevent SVC syndrome, the decision was made to implant a Micra AV leadless pacemaker and program the old RV lead to off. The patient’s anatomy precluded a conventional femoral venous approach to Micra implantation, so implantation was achieved via the right internal jugular vein. The procedure was successful without intraprocedural complication. To allow for atrial pacing and to promote AV synchrony, the dual chamber pacemaker was programmed to AAIR 70/100 bpm with the leadless pacemaker programmed to VDD 50/105 bpm. Quarterly device interrogations in the 12 months following Micra implantation demonstrated 77-92% RA pacing and 99% RV pacing with atrial tracking from the Micra device at 76-83% with optimized atrial sensing. This case exemplifies the clinical scenario in which hybrid dual chamber pacing may be indicated. The case also highlights the atrial tracking capability of the Micra AV leadless pacemaker in the setting of an AAIR transvenous pacemaker, ultimately demonstrating the feasibility and potential application of hybrid dual chamber pacing in patients in need of RV lead revision but with prohibitive surgical risk or limited venous access.

Venous Thromboembolism in a Patient with Interrupted Inferior Vena Cava and Compressed Azygos Continuation: A Case Report and Review of Literature.

We report an unusual case of a 40-year-old male patient who experienced painful swelling of the left lower limb that persisted for 1week. Imaging modalities not only confirmed the diagnosis of acute iliofemoral venous thrombosis and pulmonary embolism (PE), but also an incidental finding of interrupted inferior vena cava (IVC). This congenital anomaly is uncommon but rarely associated with venous thromboembolism (VTE). The azygos continuation was compressed by the descending aorta against the 11th thoracic vertebrae, which was identified as the cause of VTE. He was treated successfully with anticoagulation and compression therapy. The patient was discharged with lifelong oral Rivaroxaban and remained asymptomatic. In the literature, only 9 cases of interrupted IVC-associated PE were identified but none was due to significant venous compression.

Percentages and Gender Distribution of Anatomic Variations of Inferior Vena Cava, Renal Veins, and Posterior Lumbar Tributaries of the Left Renal Vein Left Renal Vein Drainage Variations

Objective: The aim of this study was to investigate percentages and gender distribution of the variations of the inferior vena cava (IVC), renal veins, and posterior lumbar tributaries of the left renal vein (LRV).&#x0D; Materials and Methods: For this cross sectional observational study, the computed tomography (CT) images of 1949 patients were evaluated retrospectively.&#x0D; Results: In the present study, percentages of double IVC, left IVC, and IVC interruption with azygos continuation were 0.5%, 0.2%, and 0.1%, respectively; circumaortic left renal vein (CLRV) was 6% and retroaortic left renal vein (RLRV) was 4.2%. Multiple renal vein variations were 24.1% on the right, but none on the left. Posterior lumbar tributaries of the renal veins were 0.15% on the right and 48% on the left. There was no difference between genders in terms of LRV and IVC variations. While the incidence of multiple right renal veins was statistically significantly higher in male patients compared to female patients (p = 0.045), the opposite was true for the posterior lumbar tributaries of the LRV (p = 0.035).&#x0D; Conclusion: The venous system has a wide variety of variations, and the renal venous circulation is supported by number variations on the right in men and collaterals on the left in women.

Computed tomography and magnetic resonance imaging of congenital thoracic systemic venous anomalies

We present the imaging findings of thoracic systemic venous anomalies diagnosed by computed tomography and magnetic resonance imaging. Persistent left superior vena cava is the commonest anomaly of the thoracic systemic veins encountered either incidentally as an isolated finding or associated with congenital heart disease. Inferior vena cava (IVC) interruption with azygos continuation is the second most common anomaly, which may also be isolated or be associated with left isomerism syndrome. The article will also discuss other rarer systemic venous anomalies including retroaortic brachiocephalic vein and IVC drainage into the left atrium. Finally, the impact of pre-procedure reporting of thoracic systemic venous anomalies on the choice of intervention and patient outcome will be addressed.

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

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. 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). KONAR-MF™ device can be used safely and effectively for transcatheter closure of PDA with unusual anatomy or in unusual circumstances.

RARE CASE OF MASSIVE DVT AFTER MRNA COVID-19 VACCINATION IN A PATIENT WITH CONGENITAL IVC ANOMALY

RARE CASE OF MASSIVE DVT AFTER MRNA COVID-19 VACCINATION IN A PATIENT WITH CONGENITAL IVC ANOMALY

Feasibility of Endovascular Recanalization in Post-Thrombotic Syndrome of Intentionally Interrupted Inferior Vena Cava.

Surgical plication of inferior vena cava was commonly used until the 1980s for prevention of pulmonary embolism, associated with high incidence of post-thrombotic syndrome (PTS). This study aims to assess the feasibility and safety of endovascular iliocaval recanalization after intentionally surgically interrupted inferior vena cava by external plicating clip. Endovascular iliocaval recanalizations in relation to previous vena cava clip plication were extracted from the retrospective French multicentre database and further analysed. All procedure data were retrospectively reviewed, including technical aspects, technical success and outcomes (clinical response and ultrasound stent patency). From 2016 to 2018, 8 patients from 4 different centres underwent endovascular iliocaval recanalization for PTS in relation to previous caval interruption by clip. Recanalization of iliocaval occlusion through the clip and stent reconstruction were successfully performed for all of them without complications. After angioplasty, plicating clip was opened in U shape (n = 3) or ovalized without significant residual stenosis (n = 5). Patency of the inferior vena cava was maintained for all patients with a mean follow-up of almost two years after stenting. All patients clinically improved. In this small cases series, endovascular recanalization and stenting of surgical vena cava clip plication seem technically feasible without morbidity. Restoration of blood flow through the iliocaval occlusion, using non-invasive endovascular technique, even as late treatment may be durable, with improvement of patient's venous symptoms. 4, Short Communication.

An adult with left isomerism having an interrupted inferior vena cava and persistent left superior vena cava associated with partial atrioventricular canal defect and complete heart block — a case report

BackgroundPersistent left superior vena cava is a well-recognized thoracic venous abnormality, most of which do not present with any symptoms. Its opening into the left atrium is uncommonly encountered and, if present, is noted to be associated with various other complex intracardiac malformations, which further complicates its management. Herein, we highlight a case of an adult with persistent left superior vena cava with a spectrum of other cardiac anomalies rarely encountered with it, which was effectively managed by tailored surgical intervention.Case presentationA 26-year-old female on meticulous evaluation was found to have left isomerism with an interrupted inferior vena cava and hemiazygos continuing as a persistent left superior vena cava. It was also associated with common atrium, partial atrioventricular canal defect, and complete heart block. This complex array of anomalies was managed surgically. Common atrium was closed with atrial neo-septation using autologous pericardial patch tailored in a way, such that the persistent left superior vena cava drained into right atrium with left atrial appendage being on right side of neo-septum. Cleft in the left atrioventricular valve was also repaired. Despite preoperative complete heart block, the patient had an uneventful postoperative recovery and was in atrial fibrillation with controlled ventricular rate, thus precluding the need for permanent pacemaker implantation.ConclusionsA rare spectrum of adult congenital cyanotic heart disease was successfully managed with thorough preoperative evaluation and judicious intraoperative management. Surgical management should be tailored based on specific anatomy for reduction of morbidity and optimum results.

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

AimsThe 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.MethodsWe 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.ResultsThe 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.ConclusionThe 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.

Spectrum of thoracic systemic venous abnormalities using multidetector computed tomography

BackgroundAnomalies of the thoracic systemic venous return vary widely and range from those with completely normal physiology to severe right to left shunting thus requiring surgical correction. The aim of the study is to enhance the awareness of multidetector computed tomography (MDCT) role in evaluation of systemic venous abnormalities, and be familiar with the imaging characteristics of even the extremely rare abnormalities.ResultsAmong 270 examined patients, 15.19% had systemic venous abnormalities. Inferior vena cava (IVC) congenital anomalies accounted for (24.4%) of the detected abnormalities (prevalence: 3.7% among the studied population) where IVC interruption with azygos continuation was the most common detected IVC abnormality accounting for 17.7% of the detected abnormalities (prevalence: 2.6%), while IVC thrombosis accounted for 21.9% (prevalence: 3.3%). Persistent left sided superior vena cava (SVC) accounted for 14.6% of the detected abnormalities (prevalence: 2.2%), while SVC syndrome represented 19.5% (prevalence: 2.9%) and SVC aneurysm represented 2.4% (prevalence: 0.37%). Retroaortic brachiocephalic vein (BCV) and BCV thrombosis accounted for 7.3% each (prevalence: 1.1). Finally, persistent levo-atrial cardinal vein represented 2.4% of the detected abnormalities (prevalence: 0.37%).ConclusionsMDCT is a non-invasive modality that can provide detailed information about the systemic thoracic veins before surgical or interventional procedures, especially in patients with congenital anomalies.

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

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. 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. 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. 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.

Bilateral chylothorax: A late complication of Kawashima procedure despite normal pulmonary pressures.

IntroductionChylothorax, a relatively rare congenital heart disease early postoperative complication, is occurring more frequently due to complexity of cardiac surgeries.Presentation of caseWe present a 9-month-old boy who had hypoplastic left heart (HLH) syndrome with interrupted inferior vena cava (IVC) and bilateral superior vena cava (SVC) palliated with left sided modified Blalock-Taussig (MBT) shunt during neonatal period and second stage palliation with left sided bidirectional glen (BDG) procedure and right sided Kawashima procedure develop bilateral chylothorax two weeks after discharge.DiscussionThis is the first reported case in the literature of a patient who developed chylothorax with relatively low Fontan systemic venous pressures after a Kawashima procedure. Clinically important chylothorax may be a marker of poor long-term outcomes, demonstrating an inability to handle overwhelming lymphatic congestion.ConclusionEarly diagnosis of chylothorax in complex cardiac surgeries may permit successful conservative management.

A Rare Case of Successfully Treated Total Anomalous Systemic Venous Connection With Interruption of IVC in an Adolescent

Total anomalous systemic venous connection (TASVC) with interruption of inferior vena cava in the absence of heterotaxy is an extremely rare anomaly. TASVC is diagnosed when both the caval veins and coronary sinus drain into the left atrium. The diagnosis of TASVC is possible only when all the systemic veins (both superior and inferior caval veins) and coronary sinus drain into the left atrium in the setting of normal or mirror-imaged atrial arrangement. The presence of cyanosis despite left-to-right shunt across an atrial septal defect with hypoplastic or small right heart chambers should arouse the suspicion of TASVC. The severity of symptoms and the age of presentation depend highly on mixing at the atrial level across the atrial septal defect. The amount of mixing also decides the growth of the right atrium, right ventricle, main and branch pulmonary arteries. Adequately sized right heart chambers and tricuspid annulus with good mixing at the atrial level facilitate complete correction and event-free survival. Prompt diagnosis is possible with contrast echocardiography performed both from upper and lower limb veins, cath study, and cardiac computed tomography or magnetic resonance imaging. However, it is not uncommon to note that even in the era of advancement in echocardiography, the complete miss diagnosis of this entity as single ventricle physiology and denial to the patient of possible complete surgical correction. We are reporting here a case of successfully treated TASVC with interrupted inferior vena cava in an adolescent.

Clinical implications of congenital interruption of inferior vena cava

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. This retrospective study included a patient cohort with evidence of IVC interruption based on imagining data (1980-2019) selected from our institutional database. 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. 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.

A novel Fontan Y-graft for interrupted inferior vena cava and azygos continuation.

OBJECTIVESTo evaluate the hemodynamicdynamic advantage of a new Fontan surgical template that is intended for complex single-ventricle patients with interrupted inferior vena cava-azygos and hemi-azygos continuation. The new technique has emerged from a comprehensive pre-surgical simulation campaign conducted to facilitate a balanced hepatic flow and somatic Fontan pathway growth after Kawashima procedure.METHODSFor 9 patients, aged 2 to18 years, majority having poor preoperative oxygen saturation, a pre-surgical computational fluid dynamics customization is conducted. Both the traditional Fontan pathways and the proposed novel Y-graft templates are considered. Numerical model was validated against in vivo phase-contrast magnetic resonance imaging data and in vitro experiments.RESULTSThe proposed template is selected and executed for 6 out of the 9 patients based on its predicted superior hemodynamic performance. Pre-surgical simulations performed for this cohort indicated that flow from the hepatic veins (HEP) do not reach to the desired lung. The novel Y-graft template, customized via a right- or left-sided displacement of the total cavopulmonary connection anastomosis location resulted a drastic increase in HEP flow to the desired lung. Orientation of HEP to azygos direct shunt is found to be important as it can alter the flow pattern from 38% in the caudally located direct shunt to 3% in the cranial configuration with significantly reversed flow. The postoperative measurements prove that oxygen saturation increased significantly (P-value = 0.00009) to normal levels in 1 year follow-up.CONCLUSIONSThe new Y-graft template, if customized for the individual patient, is a viable alternative to the traditional surgical pathways. This template addresses the competing hemodynamic design factors of low physiological venous pressure, high postoperative oxygen saturation, low energy loss and balanced hepatic growth factor distribution possibly assuring adequate lung development.Date and number of IRB approval25 October 2019, 280011928-604.01.01.