congenital IVC

Inferior vena cava (IVC) anomalies are uncommon congenital causes of deep vein thrombosis (DVT). KILT syndrome (kidney and IVC abnormalities with leg thrombosis) has only been described as case reports in the literature. Therefore, the characteristics, evaluation, and management of patients with KILT syndrome have not yet been standardized. This study aimed to systematically review and analyze the clinical and radiographic data and treatment of previously reported cases of KILT syndrome. In this systematic review, we performed a literature search of the PubMed, Scopus, and Web of Science databases in December 2023, with no restrictions on the publication date...

BACKGROUND: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis. HFD is highly dependent on the local hemodynamics...

Univentricular heart anomalies represent a group of severe congenital heart defects necessitating early surgical intervention in infancy. The Fontan procedure, the final stage of single-ventricle palliation, establishes a serial connection between systemic and pulmonary circulation by channeling venous return to the lungs. The absence of the subpulmonary ventricle in this peculiar circulation progressively eventuates in failure, primarily due to chronic elevation in inferior vena cava (IVC) pressure. This study experimentally validates the effectiveness of an intracorporeally-powered venous ejector pump (VEP) in reducing IVC pressure in Fontan patients...

Inferior vena cava (IVC) atresia is a rare congenital anomaly. Standardized treatment is not well defined due to its uncommon presentation, with this pathology associated with an increased risk of unprovoked lower extremity deep vein thrombosis (DVT). We present a case of a 32-year-old man who was admitted for bilateral lower extremity edema and pain and was found to have bilateral extensive iliofemoral and femoropopliteal DVT, absence of IVC filling, and extensive tortuous collateralization arising from the pelvic veins to the azygos vein...

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Scimitar syndrome, a rare congenital cardiac anomaly, involves abnormal pulmonary vein drainage into systemic veins, leading to distinct imaging features resembling a curved-blade sword. This case report presents a unique instance of scimitar syndrome in Pakistan, emphasizing its clinical importance and the challenges of management. A 26-year-old female with a history of recurrent pulmonary infections and respiratory symptoms since childhood was diagnosed with scimitar syndrome. Radiological assessments, including chest X-rays, computed tomography pulmonary angiograms (CTPA), and transthoracic echocardiography, confirmed the presence of a curved vessel originating from the right hemidiaphragm and connecting with the inferior vena cava (IVC)...

Patent foramen ovale (PFO) is the most common interatrial septal abnormality. The indications for PFO device closure are still being evaluated, with the most common reason being to prevent cerebrovascular accidents (CVA) caused by paradoxical embolism of deep vein thrombosis (DVT) in the lower extremities. This procedure is usually performed through percutaneous intervention using femoral vein access. Here, we present a case of PFO closure using a transhepatic approach, as femoral vein access was not feasible due to an interrupted inferior vena cava (IVC)...

Intestinal malrotation and duplication of the inferior vena cava are rarely diagnosed in adult patients; however, incidence is likely underestimated as they are usually asymptomatic. These congenital malformations have been previously reported in the same patient twice but never with colonic obstruction or ischaemia. A 25-year-old female presented with nausea, vomiting, obstipation, and abdominal pain, and on computed tomography of the abdomen and pelvis was diagnosed with a caecal volvulus and pneumatosis coli associated with intestinal malrotation requiring emergency right hemicolectomy...

INTRODUCTION AND IMPORTANCE: Raghib syndrome is a rare congenital complication consisting of the termination of the left superior vena cava (LSVC) in the left atrium, an unroofed coronary sinus, and an atrial septal defect most often found in the posterior-inferior angle of the atrial septum. Both a right-to-left and a left-to-right intracardiac shunt exist. In most circumstances, they do not show any symptoms. PRESENTATION OF CASE: The patient presented with a persistent left superior vena cava draining into the left atrium, an unroofed coronary sinus, and a secondary atrial septal defect (ASD)...

BACKGROUND: Situs inversus is a rare congenital anatomical variant that involves a group of anomalies regarding the arrangement of intrathoracic and intraabdominal organs. Being able to find in the abdominal region the liver, gallbladder, inferior vena cava, and head of the pancreas and ascending colon on the left side of the abdomen, while on the right side there is the spleen, the stomach, the body of the pancreas, the ligament of Treitz, descending colon among others. In this same way, the thoracic organs, lungs and heart, are changed in their position in a mirror translocation...

BACKGROUND: Right atrial pressure plays a critical role as a hemodynamic parameter in diagnosing pulmonary hypertension and other cardiac diseases, as well as guiding the treatment and prognosis of various cardiac disorders. If there is no obstruction between the inferior or superior vena cava (SVC) as central veins and the right atrium, the pressures in these veins could be considered equal to the right atrial pressure. This study aimed to examine the correlation between echocardiographic methods for estimating right atrial pressure and invasive measurements of central venous pressure (CVPi ) in infants and children with congenital heart disease during the 48 h after cardiac surgery and to establish regression equations for echocardiographic estimation of central venous pressure (CVPe )...

BACKGROUND: . Atrial fibrillation is the most common sustained cardiac arrhythmia in adults and it is associated with a high burden of mortality and morbidity worldwide. Catheter ablation is increasingly used to improve symptoms and prognosis in selected patients. Lower limb venous access with subsequent transseptal approach to the left atrium is the standard procedure for atrial fibrillation catheter ablation. CASE PRESENTATION: . We report an unusual case of complex venous anomaly with a left-sided inferior vena cava with hemiazygos continuation to a persistent left superior vena cava draining in an enlarged coronary sinus in a patient with persistent atrial fibrillation scheduled for transcatheter ablation...

The azygos venous system is a crucial conduit of the posterior thorax and potentially vital collateral pathway. However, it is often overlooked clinically and radiologically. This pictorial essay reviews the normal azygos venous anatomy and CT findings of congenital variations and structural changes associated with acquired pathologies.

The widespread use of computed tomography (CT) for diagnosing and screening abdominal conditions often reveals rare, asymptomatic anomalies. There is a wide range of documented congenital variations in the anatomy of the inferior vena cava (IVC) and hepatic veins. In this report, we detail an exceptionally unusual variant of the IVC that follows a frontward and intraliver course, terminating at the anterior section of the right atrium. To gain a deeper insight into this anomaly, we employed 3D reconstruction techniques using the software Slicer and Blender...

The greater thoracic vessels are central to a well-functioning circulatory system and are often targeted in congenital heart surgeries, yet the structure and function of these vessels have not been well studied. Here we use consistent methods to quantify and compare microstructural features and biaxial biomechanical properties of the following six greater thoracic vessels in wild-type mice: ascending thoracic aorta, descending thoracic aorta, right subclavian artery, right pulmonary artery, thoracic inferior vena cava, and superior vena cava...

The inferior vena cava (IVC) and superior vena cava are the main conduits of the systemic venous circulation into the right atrium. Developmental or procedural interruptions of vena cava might predispose to stasis and deep vein thrombosis (DVT) distal to the anomaly and may impact the subsequent rate of pulmonary embolism (PE). This study aimed to review the various etiologies of developmental or procedural vena cava interruption and their impact on venous thromboembolism. A systematic search was performed in PubMed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines per each clinical question...

Scimitar syndrome is a rare congenital anomaly characterized by partial or total anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We report a case of a 67-year-old female who presented with cough and dyspnea and was diagnosed with scimitar syndrome and pulmonary arterial hypertension based on comprehensive imaging and hemodynamic evaluation. This case highlights the importance of considering scimitar syndrome as a cause of pulmonary hypertension even in adult patients.

BACKGROUND: Double inferior vena cava (DIVC) is a rare vascular malformation. With advances in radiological techniques and diagnosis, more and more types of DIVC were identified and diagnosed. Recognition of the variety of DIVC seen on imaging is essential for subsequent venous interventions. CASE PRESENTATION: A 77-year-old man presented with low back pain with left lower limb pain for 1 month. Scattered petechiae above the skin surface on the left lower leg, especially on the extensor surface, with flaking and mild tingling of the skin, were noted 3 weeks ago...

INTRODUCTION: Down syndrome is due to trisomy 21 and is characterized by intellectual disability, dysmorphic facial features, congenital malformations, and gastrointestinal abnormalities. There is an increased appreciation of congenital portosystemic shunts in Down syndrome patients. Congenital portosystemic shunts have been associated with many defects in body systems, including cardiac, metabolic, and neurological. CASE PRESENTATION: Herein, we describe a portosystemic shunt in a Down syndrome patient that resulted in hyperammonemia with altered mental status and choreiform movements...

Congenital anomalies of inferior vena cava are increasingly being recognized with the technical advancements and increased utilization of cross-sectional imaging techniques. Duplication of inferior vena cava classically involves duplication of the infrarenal segment, where both inferior vena cava ascend on either side of the abdominal aorta until they form a confluence at the level of the renal veins. It has been extensively described in literature with few reports of more complex variation in the form of duplicated infrarenal inferior vena cava with azygos or hemiazygos continuation...