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To analytically depict the associated malformations of polysplenia syndrome (PS) in adults via computed tomography (CT). The incidence of malformations associated with PS in twelve adult patients was retrospectively analyzed via CT imaging. The number of splenic nodules ranged from three to twelve; the splenic nodules were located in the left upper quadrant in nine patients and in the right upper quadrant in three patients. A short pancreas was present in all twelve patients. Midgut malrotation was present in eight patients. Situs inversus totalis was present in two patients. Nine patients presented the absence of hepatic segmental inferior vena cava (IVC), with the hepatic vein directly converging into the right atrium and the continuation of the azygos vein. The preduodenal portal vein was present in six patients. Left lung heterotaxy was found in nine patients. The inferior vena cava was bilateral in one patient. Aberrant right subclavian arteries, bilateral common carotid arteries sharing trunks, abnormal renal vein branching and routing, and abdominal portal vein branching were also found in individual patients. PS is a complex malformation syndrome involving multiple systems. The most common malformation is short pancreas, and other malformations, such as left lung heterogeneity, hepatic segmental IVC agenesis with continuation of the azygos vein, midgut malrotation, preduodenal portal vein, and left atrial heterotaxy, have relatively high prevalence rates.

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.

The inferior vena cava, the principal retroperitoneal venous trunk, carries deoxygenated blood from the lower extremities, pelvis, and abdominal viscera to the right atrium. Complex inferior vena cava malformations can radiologically mimic pulmonary space-occupying lesions, substantially complicating clinical diagnosis and therapeutic planning. We present the case of a patient in whom contrast-enhanced chest computed tomography incidentally revealed a right-sided intrathoracic mass, initially suspected to be a primary or metastatic pulmonary malignancy. Subsequent dynamic contrast-enhanced computed tomography demonstrated heterogeneous, avid enhancement during the venous phase, raising the possibility of a vascular anomaly. Dedicated magnetic resonance imaging ultimately delineated the mass as a congenital inferior vena cava malformation. This case underscores that intricate inferior vena cava anomalies may masquerade as intrapulmonary masses; misguided surgical intervention could precipitate severe hemorrhagic or thromboembolic sequelae. Multimodality imaging—integrating high-resolution computed tomography and magnetic resonance imaging—is therefore indispensable for accurate differential diagnosis.

Dilation of the coronary sinus is often a result of excessive volume overload from congenital anomalies of systemic venous return to the heart. These abnormalities are often discovered incidentally later in life when a patient requires cardiac imaging, cardiac catheterization, or thoracic surgery. The most common abnormality is a persistent left superior vena cava. Inferior vena cava malformation is less common, yet several different anomalies can arise. The presence of persistent left superior vena cava or inferior vena cava anomalies requires further evaluation to rule out congenital heart disease in infants. Knowledge of technically challenging systemic venous anatomy is beneficial prior to procedures necessitating central venous access such as a central line, cardiac catheterization, and intracardiac device implantation. We present an unusual case of persistent LSVC and IVC both draining directly into a severely dilated coronary sinus that was diagnosed by fetal echocardiogram and later confirmed postnatally by transthoracic echocardiogram and computed tomography angiography. To our knowledge this is the second reported case of IVC drainage into the CS and the first case that reports this as a prenatal diagnosis.

We describe a 12-year-old boy with agenesis of the intrahepatic segment of the inferior vena cava (a rare congenital anomaly) in whom an electrified coronary guidewire with electrocautery was used percutaneously to perforate and open the atretic segment, followed by stent implantation. This technique may be a safe and feasible option for paediatric patients, offering a therapeutic alternative for complex cardiac anatomies.

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

The objective of this work is to perform a virtual planning of surgical repairs in patients with congenital heart diseases-to test the predictive capability of a closed-loop multi-scale model. As a first step, we reproduced the pre-operative state of a specific patient with a univentricular circulation and a bidirectional cavopulmonary anastomosis (BCPA), starting from the patient's clinical data. Namely, by adopting a closed-loop multi-scale approach, the boundary conditions at the inlet and outlet sections of the three-dimensional model were automatically calculated by a lumped parameter network. Successively, we simulated three alternative surgical designs of the total cavopulmonary connection (TCPC). In particular, a T-junction of the venae cavae to the pulmonary arteries (T-TCPC), a design with an offset between the venae cavae (O-TCPC) and a Y-graft design (Y-TCPC) were compared. A multi-scale closed-loop model consisting of a lumped parameter network representing the whole circulation and a patient-specific three-dimensional finite volume model of the BCPA with detailed pulmonary anatomy was built. The three TCPC alternatives were investigated in terms of energetics and haemodynamics. Effects of exercise were also investigated. Results showed that the pre-operative caval flows should not be used as boundary conditions in post-operative simulations owing to changes in the flow waveforms post-operatively. The multi-scale approach is a possible solution to overcome this incongruence. Power losses of the Y-TCPC were lower than all other TCPC models both at rest and under exercise conditions and it distributed the inferior vena cava flow evenly to both lungs. Further work is needed to correlate results from these simulations with clinical outcomes.

Ventricular premature contractions (VPC) originating from right ventricular outflow tract is the most common type of ventricular arrhythmias in clinic settings, which can be effectively cured by catheter ablation. Interruption of the inferior vena cava (IVC) is a rare vascular anomaly resulting from aberrant development during embryogenesis. Herein, we reported a very rare case of VPC originating from right ventricular outflow tract (RVOT) with interruption of IVC, which was successfully ablated by transfemoral vein approach. We reported a very rare case of ventricular premature contractions originating from right ventricular outflow tract with interruption of inferior vena cava, which was successfully ablated by transfemoral‐azygos vein approach.

Background The variety of shocks in neonates, if not recognized and treated immediately, is a major cause for fatality. The use of echocardiography may improve assessment and treatment, but its reference values across gestational age (GA) and birth weight (BW) are lacking. To address the information gap, this study aimed at correlating GA and BW of newborns with nonhemodynamic abnormalities, and at evaluating the usefulness of such reference values in neonates with early onset septic (EOS) -shock. Methods A total of 200 normal newborns were enrolled as controls and subdivided into groups based on GA, BW, days of age, and patent ductus arteriosus (PDA). Echocardiography was used to document inferior vena cava diameter (IVC), inferior vena cava collapsibility index (IVC-CI), and inferior vena cava to abdominal aorta ratio (IVC/AO). In addition, 18 neonates with EOS shock were recruited and evaluated using echocardiography. Results Among the control newborns, IVC and AO were significantly increased with GA and BW ( P  < 0.05) but IVC-CI and IVC/AO did not correlate with GA, BW, day of age, and PDA. Compared to the control group, the EOS-shock group had significantly decreased IVC and IVC/AO, and increased IVC-CI ( P  < 0.05). The cut-off values for indicating EOS-shock were > 34.15% for IVC-CI, < 47.58% for IVCmin/AO, and < 66.11% for IVCmax/AO. Conclusions The IVC-CI, IVCmin/AO, and IVCmax/AO indices are applicable to all neonates. Although the number of neonates with EOS-shock in our study is small, the cut-off values showed usefulness for diagnosis. Further research is needed to determine the application of the indices in a larger population and among other populations, especially for clinical application in treatment of shock among neonates.