Publications by authors named "Yoav Dori"

Background: Health related quality of life (HRQOL) is a patient-reported metric (PRM) that provides a holistic measure of health that is not addressed in traditional outcome measures. The acute responsiveness of HRQOL after pediatric/congenital cardiac catheterization procedures has not, to our knowledge, been studied.

Methods: A single-center prospective cohort study was performed, longitudinally evaluating HRQOL and other PRM in school-age children and adolescents (ages 8-18) undergoing diagnostic and interventional cardiac catheterization procedures prior to their scheduled procedure, and then 1 day, ∼1 month, and ∼3 months after the procedure.

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Purpose: To assess effectiveness of selective lymphatic duct embolization (SLDE) for treatment of thoracic lymphatic flow disorders, performed to maintain patency of the thoracic duct (TD), compared with that of TD embolization (TDE).

Materials And Methods: Records of 39 patients (mean age, 7.6 years [SD ± 7.

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The lymphatic system plays a central role in some of the most devastating complications associated with congenital heart defects. Diseases like protein-losing enteropathy, plastic bronchitis, postoperative chylothorax, and chylous ascites are now proven to be lymphatic in origin. Novel imaging modalities, most notably, noncontrast magnetic resonance lymphangiography and dynamic contrast-enhanced magnetic resonance lymphangiography, can now depict lymphatic anatomy and function in all major lymphatic compartments and are essential for modern therapy planning.

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Chylopericardium is a rare complication after cardiac transplantation. We report a case of a 69-year-old woman with persistent chylopericardium after a heart transplantation due to Chagas disease. Failure of conservative treatment led to dynamic contrast-enhanced magnetic resonance lymphangiography and percutaneous radiologic intervention of the lymphatic leakage and symptoms resolution.

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Article Synopsis
  • Percutaneous endovascular techniques are special procedures used in heart and blood vessel treatments.
  • These techniques can help manage problems with how the lymphatic system works, like blockages or issues in the thoracic duct.
  • This article explains how these methods are important for doctors to work together to treat difficult medical conditions.
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Article Synopsis
  • * This disease can be linked to problems with lymphatic systems, which help manage body fluids, and can be due to genetic disorders or other reasons related to heart problems.
  • * New imaging technology is helping doctors understand these issues better and create new treatments to help fix lymphatic problems in patients with congenital heart disease.
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Patients with lymphatic disorders are remarkably complex and require a wide variety of medical and surgical services. Establishing a multidisciplinary program improves the efficiency of the patients' hospital experience minimizing the compartmentalization of their care. Offering a clear intake process guarantees that patients will be seen promptly by all the required teams.

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Objective: The thoracic duct is the largest lymphatic vessel in the body, and carries fluid and nutrients absorbed in abdominal organs to the central venous circulation. Thoracic duct obstruction can cause significant failure of the lymphatic circulation (i.e.

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Lymphatic failure is a broad term that describes the lymphatic circulation's inability to adequately transport fluid and solutes out of the interstitium and into the systemic venous circulation, which can result in dysfunction and dysregulation of immune responses, dietary fat absorption, and fluid balance maintenance. Several investigations have recently elucidated the nexus between lymphatic failure and congenital heart disease, and the associated morbidity and mortality is now well-recognized. However, the precise pathophysiology and pathogenesis of lymphatic failure remains poorly understood and relatively understudied, and there are no targeted therapeutics or interventions to reliably prevent its development and progression.

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Purpose To evaluate lymphatic abnormalities before and after Fontan completion using noncontrast lymphatic imaging and relate findings with postoperative outcomes. Materials and Methods This study is a retrospective review of noncontrast T2-weighted lymphatic imaging performed at The Children's Hospital of Philadelphia from June 2012 to February 2023 in patients with single ventricle physiology. All individuals with imaging at both pre-Fontan and Fontan stages were eligible.

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Patients with central lymphatic conduction disorders commonly have recalcitrant pleural effusions and or ascites. These conditions cause a profound deterioration in the patient's quality of life. Support measures such as low-fat diet and diuretics alone hardly ever provide meaningful improvement.

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The development of new imaging techniques for the study of the central lymphatic system allows us to understand the anatomy and pathophysiology of all the disorders of the thoracic duct. With the help of catheters placed percutaneously in the thoracic duct, we can do now complex operations on the thoracic duct to restore its functionality. Advance imaging, expert percutaneous skills, and expert microsurgical skills are critical to the success of these interventions.

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Background: Maldistribution of pulmonary blood flow in patients with congenital heart disease impacts exertional performance and pulmonary artery growth. Currently, measurement of relative pulmonary perfusion can only be performed outside the catheterization laboratory. We sought to develop a tool for measuring relative lung perfusion using readily available fluoroscopy sequences.

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Background: The modified Blalock-Taussig-Thomas shunt is the gold standard palliation for securing pulmonary blood flow in infants with ductal-dependent pulmonary blood flow. Recently, the ductus arteriosus stent (DAS) has become a viable alternative.

Methods And Results: This was a retrospective multicenter study of neonates ≤30 days undergoing DAS or Blalock-Taussig-Thomas shunt placement between January 1, 2017 and December 31, 2020 at hospitals reporting to the Pediatric Health Information Systems database.

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Aim: Recent advances in lymphatic imaging allow understanding the pathophysiology of lymphatic central conduction disorders with great accuracy. This new imaging data is leading to a wide range of novel surgical interventions. We present here the state-of-the-art imaging technology and current spectrum of surgical procedures available for patients with these conditions.

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Purpose: Central lymphatic obstructions are associated with anasarca and high mortality. We hypothesized that opening dilated cutaneous lymphatic channels by creating a lymphocutaneous fistula (LCF) would decompress the lymphatic circulation and improve anasarca.

Methods: We reviewed all patients that had at least one LCF created between 9/2019 and 12/2022.

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Background Intensive monitoring has been associated with a lower death rate between the Norwood operation and superior cavopulmonary connection, possibly due to early identification and effective treatment of residual anatomic lesions like recoarctation before lasting harm occurs. Methods and Results Neonates undergoing a Norwood operation and receiving interstage care at a single center between January 1, 2005, and September 18, 2020, were studied. In those with recoarctation, we evaluated association of era ([1] preinterstage monitoring, [2] a transitional phase, [3] current era) and likelihood of hemodynamic compromise (progression to moderate or greater ventricular dysfunction/atrioventricular valve regurgitation, initiation/escalation of vasoactive/respiratory support, cardiac arrest preceding catheterization, or interstage death with recoarctation on autopsy).

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Background In the palliative pathway of single-ventricle physiology, lymphatic abnormalities on T2-weighted magnetic resonance imaging have been shown after the Glenn operation. It is believed that postsurgical hemodynamic changes contribute to the lymphatic changes.However, little is known about how early these abnormalities occur.

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Article Synopsis
  • * A study involved 356 participants, using advanced DNA sequencing techniques on cells from lymphatic fluid, which revealed genetic variations in a significant percentage of participants with primary complex lymphatic anomalies (pCLAs) and other vascular malformations.
  • * This research resulted in a molecular diagnosis for many participants, enabling new medical therapies for 63% of those affected, highlighting the potential of liquid biopsy techniques in diagnosing and treating vascular anomalies.
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Purpose Of Review: The lymphatic system was previously considered the forgotten circulation because of an absence of adequate options for imaging and intervention. However, recent advances over the last decade have improved management strategies for patients with lymphatic disease, including chylothorax, plastic bronchitis, ascites, and protein-losing enteropathy.

Recent Findings: New imaging modalities have enabled detailed visualization of lymphatic vessels to allow for a better understanding of the cause of lymphatic dysfunction in a variety of patient subsets.

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Complex lymphatic anomalies are debilitating conditions characterized by aberrant development of the lymphatic vasculature (lymphangiogenesis). Diagnosis is typically made by history, examination, radiology, and histologic findings. However, there is significant overlap between conditions, making accurate diagnosis difficult.

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Article Synopsis
  • - Central conducting lymphatic anomaly (CCLA) is a serious condition caused by improper development of the lymphatic system, and this study focused on individuals with CCLA linked to KRAS gene mutations.
  • - Researchers used human cells and zebrafish to explore how these KRAS variants lead to lymphatic issues, finding that the mutations activate a specific signaling pathway (RAS/MAPK) that contributes to lymphatic malformation.
  • - Treatment with MEK inhibitors showed promise in reducing symptoms in lab models, suggesting this approach should be further investigated for treating CCLA in patients with KRAS mutations.
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Background Congestive heart failure is a leading cause of morbidity and mortality worldwide. One of the signs of congestive heart failure is fluid overload including pulmonary edema, peripheral edema, and ascites. The cause of fluid overload remains incompletely understood, and management of these patients continues to be a challenge.

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