keyword
https://read.qxmd.com/read/38098271/absence-of-ductus-venosus-a-comparison-of-2-distinctive-fetal-autopsy-cases-and-embryologic-perspectives
#21
JOURNAL ARTICLE
Elaine S Chan, Ian Suchet, Weiming Yu, David Somerset, Nancy Soliman, Verena Kuret, Rati Chadha
In fetal circulation, oxygenated blood from the placenta flows through the umbilical vein into the ductus venosus (DV), then enters the inferior vena cava, and subsequently reaches the right atrium of the heart. The DV serves as a shunt, allowing this oxygen-rich blood to bypass the liver. The absence of the DV (ADV), also known as agenesis of the DV, is a rare congenital anomaly. Without a DV, blood from the umbilical vein must follow alternative routes to the heart. In ADV cases, blood from the umbilical vein must follow 1 of 2 primary drainage patterns: either an extrahepatic shunt or an intrahepatic shunt...
December 14, 2023: Pediatric and Developmental Pathology
https://read.qxmd.com/read/38073688/type-ii-abernethy-malformation-with-cystic-fibrosis-in-a-12-year-old-girl-a-case-report
#22
Li-Jie Zhang, Xing-Yu Liu, Teng-Fei Chen, Zhong-Ya Xu, Han-Jun Yin
BACKGROUND: Abernethy malformation, also known as congenital extrahepatic portosystemic shunt, is an uncommon malformation resulting from aberrant development of the portal venous system. Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the CFTR gene. It mainly affects the exocrine glands of the respiratory, digestive and reproductive systems. It is considered extremely rare in the Asian population. We present a clinical case involving a pediatric patient of Asian descent who was diagnosed with Abernethy malformation and CF...
November 16, 2023: World Journal of Clinical Cases
https://read.qxmd.com/read/38059116/a-congenital-portosystemic-shunt-in-a-child-with-heterotaxy-situs-inversus-polysplenia-and-interrupted-inferior-vena-cava-with-azygous-continuation
#23
Victoria Carvajal, Saigopala Reddy, Vani Gopalareddy, Adrienne Bean, Gonzalo Wallis
Congenital portosystemic shunts are rare vascular malformations in which portal venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. We report a case of a 4-year-old girl with heterotaxy syndrome, polysplenia, and situs inversus presenting with persistent hypoxemia who was found to have pulmonary arteriovenous malformations (PAVMs) and hypoxemia secondary to a congenital portosystemic shunt. Management of this patient's PAVMs involved endovascular occlusion of the portosystemic shunt with subsequent resolution of hypoxemia...
December 2023: ACG Case Reports Journal
https://read.qxmd.com/read/38046676/ultrasonographic-measurements-of-the-inferior-vena-cava-diameter-in-newborns-is-it-a-useful-tool-for-choosing-an-umbilical-venous-catheter
#24
JOURNAL ARTICLE
Francesca Galdo, Antonella Trappan, Francesca Cossovel, Carmen Rodriguez-Perez, Luca Ronfani, Paolo Montaldo, Cristina Bibalo, Laura Travan, Francesco Maria Risso
OBJECTIVES: The primary outcomes of this study were to evaluate the diameters of the inferior vena cava (IVC) in a cohort of newborns and the correlation between newborn weight and IVC diameter. The secondary outcome was to evaluate the concordance between the measurements performed by the two investigators. METHODS: Two blind examiners performed an ultrasonographic (US) evaluation of the IVC diameter in neonates with a weight ranging from 2 to 4 kg. The exclusion criteria included hemodynamic instability, known vascular malformations, and major congenital malformations...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/37937258/an-unusual-cause-of-cyanosis-after-intra-extra-cardiac-fontan-procedure-anastomotic-leakage-between-conduit-and-inferior-vena-cava
#25
Jiajun Xu, Weize Xu, Jin Yu, Shanshan Shi, Qiang Shu, Zhuo Shi
No abstract text is available yet for this article.
2023: World journal of pediatric surgery
https://read.qxmd.com/read/37908335/volume-overload-impedes-the-maturation-of-sarcomeres-and-t-tubules-in-the-right-atria-a-potential-cause-of-atrial-arrhythmia-following-delayed-atrial-septal-defect-closure
#26
JOURNAL ARTICLE
Zhuoya Dong, Dian Chen, Sixie Zheng, Zheng Wang, Debao Li, Yingying Xiao, Sijuan Sun, Lincai Ye, Lisheng Qiu, Yuqing Hu, Haifa Hong
Introduction: Adult patients with atrial septal defects (ASD), the most common form of adult congenital heart disease, often die of arrhythmias, and the immaturity of cardiomyocytes contributes significantly to arrhythmias. ASD typically induces a left-to-right shunt, which then leads to the right atrium (RA) volume overload (VO). Whether or not VO contributes to RA cardiomyocyte immaturity and thereby causes arrhythmias in adult patients with ASD remains unclear. Methods: Here, we developed the first neonatal RA VO mouse model by creating a fistula between the inferior vena cava and abdominal aorta on postnatal day 7...
2023: Frontiers in Physiology
https://read.qxmd.com/read/37904806/automated-segmentation-of-3d-cine-cardiovascular-magnetic-resonance-imaging
#27
JOURNAL ARTICLE
Soroosh Tayebi Arasteh, Jennifer Romanowicz, Danielle F Pace, Polina Golland, Andrew J Powell, Andreas K Maier, Daniel Truhn, Tom Brosch, Juergen Weese, Mahshad Lotfinia, Rob J van der Geest, Mehdi H Moghari
INTRODUCTION: As the life expectancy of children with congenital heart disease (CHD) is rapidly increasing and the adult population with CHD is growing, there is an unmet need to improve clinical workflow and efficiency of analysis. Cardiovascular magnetic resonance (CMR) is a noninvasive imaging modality for monitoring patients with CHD. CMR exam is based on multiple breath-hold 2-dimensional (2D) cine acquisitions that should be precisely prescribed and is expert and institution dependent...
2023: Frontiers in Cardiovascular Medicine
https://read.qxmd.com/read/37855807/transcatheter-closure-of-superior%C3%A2-sinus%C3%A2-venosus-defects
#28
REVIEW
Alban-Elouen Baruteau, Sébastien Hascoet, Sophie Malekzadeh-Milani, Clément Batteux, Clément Karsenty, Vlad Ciobotaru, Jean-Benoit Thambo, Alain Fraisse, Younes Boudjemline, Zakaria Jalal
Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited...
November 13, 2023: JACC. Cardiovascular Interventions
https://read.qxmd.com/read/37850759/a-remarkable-case-report-of-an-interrupted-inferior-vena-cava-with-hemiazygos-and-transhepatic-continuation
#29
JOURNAL ARTICLE
Federica Masino, Gianmichele Muscatella, Manuela Montatore, Rossella Gifuni, Giuseppe Guglielmi
Inferior vena cava (IVC) interruption with azygos/hemiazygos continuation is an extremely uncommon congenital vascular anomaly, which may present with multiple variants. As a result, it is challenging to find in the literature the same anatomical variant. We report a unique case of an interrupted IVC with hemiazygos and transhepatic continuation in an 83-year-old female patient. The case was evaluated by performing Computed Tomography (CT) as imaging modality, with a multiphase protocol, able to detect accurately this complex vascular anomaly...
October 17, 2023: Acta Bio-medica: Atenei Parmensis
https://read.qxmd.com/read/37811155/scimitar-syndrome-with-large-atrial-septal-defect-and-a-rare-partial-anomalous-venous-drainage-in-an-adult-a-case-report
#30
Thisara Samarawickrama, Namal Wijesinghe, Subash Karunarathna, Anjali Biyanwila
BACKGROUND: Scimitar syndrome is a very rare congenital cardio-pulmonary disease with anomalous right pulmonary vein draining either partially or completely into the inferior vena cava. It is called Scimitar syndrome due to the classical appearance in the chest X-ray, which resembles the curved blade of Turkish sword 'Scimitar'. It commonly associates with atrial septal defect (ASD), hypoplasia of the right lung, dextroposition of the heart, and pulmonary hypertension (PHT). CASE SUMMARY: A 67-year-old lady, diagnosed with atrial fibrillation and moderate PHT 3 years ago, presented with worsening bilateral ankle oedema and New York Heart Association class III shortness of breath...
October 2023: European Heart Journal. Case Reports
https://read.qxmd.com/read/37797526/retrocaval-ureter-in-a-child-presenting-at-a-regional-referral-hospital-in-south-western-uganda-a-case-report
#31
Oscar Atwine, Denis Mucunguzi, Johnson Ebine, Racheal Ainomugisha, Joshua Muhumuza, Marvin Mutakooha Mwesigwa
INTRODUCTION AND IMPORTANCE: Retrocaval ureter is a rare developmental abnormality of the inferior vane cava. Commonly presents in the 3rd and 4th decades of life hence a rare presentation in the pediatric population. This condition presents a surgical challenge both in terms of diagnosis and management due to costly investigations and few urology specialists in resource limited settings. We present a case of retrocaval ureter surgically managed in a resource limited setting with excellent outcome...
October 3, 2023: International Journal of Surgery Case Reports
https://read.qxmd.com/read/37774925/prognostic-role-of-inferior-vena-collapsibility-index-in-congenital-heart-disease-a-validation-study
#32
JOURNAL ARTICLE
Alexander C Egbe, Omar Abozied, William R Miranda, Ahmed Younis, Luke Burchill, Sriharsha Kandlakunta, Marwan H Ahmed, Snigdha Karnakoti, Heidi M Connolly
BACKGROUND: A recent study showed that inferior vena cava collapsibility index (IVCCI) <60% had better prognostic performance as compared to the American Society of Echocardiogram (ASE) criteria for estimating right atrial pressure (RAP). However, this study was based on a selected cohort of adults with congenital heart disease (CHD) that underwent right heart catheterization and limiting the generalizability of the results. The purpose of this study was, therefore, to validate the prognostic performance of IVCCI in a more representative sample of adults with CHD, which would in turn, improve generalizability of the results...
January 1, 2024: International Journal of Cardiology
https://read.qxmd.com/read/37771518/experience-in-retrocaval-ureter-at-saint-paul-s-hospital-millennium-medical-college-a-case-series
#33
Adugna Getachew Mideksa, Tolesa Yadeta Huluka, Masresha Solomon Dino, Mensur Mohammed Ahmed
Retrocaval ureter is a rare congenital vascular anomaly described as the passage of the ureter behind the inferior vena cava (IVC) and then turning around the IVC to attain the final lateral position. The condition is usually associated with obstruction in the ipsilateral kidney, causing different degrees of hydronephrosis and complications associated with urinary stasis, such as stone formation. Imaging has a crucial role in the diagnosis and management of retrocaval ureter. CT urography may be the procedure of choice to confirm the diagnosis and avoid retrograde ureteropyelography...
2023: Research and Reports in Urology
https://read.qxmd.com/read/37743384/the-inadequate-oxygen-delivery-index-and-its-correlation-with-venous-saturation-in-the-pediatric-cardiac-intensive-care-unit
#34
JOURNAL ARTICLE
Rohit S Loomba, Enrique G Villarreal, Saul Flores, Juan S Farias, Alex Constas
Continuous monitoring software, T3, has an integrated index called the inadequate oxygen delivery index 50% (IDO2-50) which displays a probability that the mixed venous saturation is below a user-selected threshold of 30-50%. The primary aim of this study was to determine the correlation of the IDO2-50 with a measured venous saturation. The secondary aim of this study was to characterize the hemodynamic factors that contributed to the IDO2-50. This single-center, retrospective study aimed to characterize the correlation between IDO2-50 and inferior vena cava (IVC) saturation...
September 24, 2023: Pediatric Cardiology
https://read.qxmd.com/read/37725857/congenital-scoliosis-associated-with-total-situs-inversus-a-rare-case-report
#35
Indah Mirasari, Karya Triko Biakto, Ira Nong, Leonard Christianto Singjie
INTRODUCTION AND IMPORTANCE: Situs inversus totalis is a rare congenital abnormality characterized by a mirror-image transposition of both the abdominal and the thoracic organs. Vertebral anomalies causing congenital scoliosis are classified on the basis of failures of formation, segmentation, or both. The hallmark of surgical treatment is early intervention before the development of large curvatures. The surgical treatment of a congenital deformity mandates the use of neurological monitoring to minimize the risk of perioperative neurological deficit...
September 16, 2023: International Journal of Surgery Case Reports
https://read.qxmd.com/read/37677007/a-neonatal-heterotopic-rat-heart-transplantation-model-for-the-study-of-endothelial-to-mesenchymal-transition
#36
JOURNAL ARTICLE
Gregor Gierlinger, Lavinia Rech, Sitaram M Emani, Pedro J Del Nido, Ingeborg Friehs
Endocardial fibroelastosis (EFE), defined by subendocardial tissue accumulation, has major impacts on the development of the left ventricle (LV) and precludes patients with congenital critical aortic stenosis and hypoplastic left heart syndrome (HLHS) from curative anatomical biventricular surgical repair. Surgical resection is currently the only available therapeutic option, but EFE often recurs, sometimes with an even more infiltrative growth pattern into the adjacent myocardium. To better understand the underlying mechanisms of EFE and to explore therapeutic strategies, an animal model suitable for preclinical testing was developed...
July 21, 2023: Journal of Visualized Experiments: JoVE
https://read.qxmd.com/read/37654671/atrial-transseptal-catheterisation-challenging-scenarios-and-techniques-to-overcome-them
#37
REVIEW
Maiwand Mirwais, Pasquale Santangeli
Atrial transseptal catheterisation is a fundamental skill of any interventional electrophysiologist. In this review, various scenarios that pose unique challenges to atrial transseptal catheterisation are discussed. These scenarios include post-surgical or congenital malformations of the interatrial septum, presence of interatrial septal closure devices, absent or obstructed inferior vena cava and complex congenital heart disease after palliative surgery. Transseptal catheterisation in all of the above situations is feasible and can be performed safely with the aid of dedicated tools and specific techniques...
2023: Arrhythmia & Electrophysiology Review
https://read.qxmd.com/read/37649933/extensive-deep-vein-thrombosis-with-intraabdominal-extension-triggered-by-oral-contraceptive-use-in-a-young-patient-with-interrupted-inferior-vena-cava-with-azygos-continuation-a-case-report
#38
Mosa R Abu Sabha, Zinah A Bairmani, Khadeejeh M Alfroukh, Osayd Nassar, Mutaz Albakri
Congenital malformations of the inferior vena cava (IVC) are rare and often asymptomatic, typically discovered incidentally during imaging. However, these anomalies can result in circulatory stasis, impede venous return, and serve as predisposing factors for thrombus formation. Here, we present a unique case of a 28-year-old female patient who was found to have interrupted IVC with azygos continuation, an exceedingly rare IVC anomaly, during a work-up of extensive bilateral deep vein thrombosis (DVT) with an intraabdominal extension which was triggered by recent combined oral contraceptive pills (OCP) use...
July 2023: Curēus
https://read.qxmd.com/read/37589961/congenital-anomalous-drainage-of-the-inferior-vena-cava-and-hepatic-veins-into-the-left-atrium-associated-with-long-qt-syndrome-multimodality-imaging-pitfalls-and-caveats
#39
Elaheh Malakan Rad, Hassan Radmehr, Ashkan Taghizadeh, Hamidreza Pouraliakbar
Isolated congenital anomalous drainage of the inferior vena cava (IVC) and partial hepatic veins (HV) into the left atrium (LA) via an interatrial communication (IAC), associated with the normal connection of the IVC to the right atrium is exceedingly rare. Therefore, there is a dearth of knowledge regarding the management of these cases. To date, there has been no report of abnormal IVC drainage caused by abnormal IVC-IAC alignment. Much more frequently, patients have an abnormal connection or abnormal drainage mediated by a persistent Eustachian valve that allows blood to pass from the inferior vena cava to the left atrium...
August 17, 2023: Echocardiography
https://read.qxmd.com/read/37586754/pulmonary-homograft-failure-presents-as-abdominal-ascites
#40
JOURNAL ARTICLE
Kimberly Renae Ding, Jamil Aboulhosn, Priya Pillutla
Ascites is a condition typically attributed to liver disease but can be cardiac in aetiology as well. We report of an atypical case of pulmonary homograft failure status-post the Ross-Yacoub procedure as an underlying cause of anasarca. A man in his mid-30s with a history of congenital heart disease presented with abdominal distension and exercise intolerance. His prior history was relevant for congenital bicuspid aortic valve with critical aortic stenosis. Abdomen and pelvis CT revealed a grossly dilated inferior vena cava while transthoracic echocardiogram showed severe homograft pulmonary stenosis and regurgitation...
August 16, 2023: BMJ Case Reports
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