Immune System in Single Ventricle Patients—A Complex Nexus

Kumar, S. Ram

doi:10.1177/2150135117739830

Cited by 3 publications

(1 citation statement)

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“…Intravenous immunoglobulin can aid in patients with opportunistic infections and may be administered in addition to replacing chronically low serum immunoglobulin that are commonly observed in this patient group. Interestingly, despite the alterations in immunoglobulin levels and lymphocyte counts Fontan patients usually are not prone to severe infections (66)(67)(68). Oral controlled-release budesonide has been shown to increase albumin levels in some case series (69, 70).…”

Section: Conservative Managementmentioning

confidence: 99%

Current diagnostic and therapeutic strategies for the management of lymphatic insufficiency in patients with hypoplastic left heart syndrome

Bauer

Dori²,

Scala³

et al. 2023

Front. Pediatr.

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Children with hypoplastic left heart syndrome share unique hemodynamic features that alter lymphatic integrity at all stages of palliation. Lymphatic congestion is almost universal in this patient group to some extent. It may lead to reversal of lymphatic flow, the development of abnormal lymphatic channels and ultimately decompression and loss of protein rich lymphatic fluid into extra lymphatic compartments in prone individuals. Some of the most devastating complications that are associated with single ventricle physiology, notably plastic bronchitis and protein losing enteropathy, have now been proven to be lymphatic in origin. Based on the new pathophysiologic concept new diagnostic and therapeutic strategies have recently been developed. Dynamic contrast magnetic resonance lymphangiography is now mainstay in diagnosis of lymphatic insufficiency and allows a thorough assessment of anatomy and function of the main lymphatic compartments through intranodal, intrahepatic and intramesenteric lymphatic imaging. Contrast enhanced ultrasound can evaluate thoracic duct patency and conventional fluoroscopic lymphangiography has been refined for evaluation of patients where magnetic resonance imaging cannot be performed. Novel lymphatic interventional techniques, such as thoracic duct embolization, selective lymphatic duct embolization and liver lymphatic embolization allow to seal abnormal lymphatic networks minimally invasive and have shown to resolve symptoms. Innominate vein turn-down procedures, whether surgical or interventional, have been designed to reduce lymphatic afterload and increase systemic preload effectively in the failing Fontan circulation. Outflow obstruction can now be managed with new microsurgical techniques that create lympho-venous anastomosis. Short term results for all of these new approaches are overall promising but evidence is sparse and long-term outcome still has to be defined. This review article aims to summarize current concepts of lymphatic flow disorders in single ventricle patients, discuss new emerging diagnostic and therapeutic strategies and point out lacks in evidence and needs for further research on this rapidly growing topic.

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Section: Conservative Managementmentioning

confidence: 99%

Current diagnostic and therapeutic strategies for the management of lymphatic insufficiency in patients with hypoplastic left heart syndrome

Bauer

Dori²,

Scala³

et al. 2023

Front. Pediatr.

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Stem Cell Therapy in Single-Ventricle Physiology: Recent Progress and Future Directions

Abdullah

Kegel

Gunasekaran

et al. 2021

Seminars in Thoracic and Cardiovascular Surgery: Pediatric Card

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Severe Features of Systemic Juvenile Idiopathic Arthritis in Patients With Congenital Heart Disease

Aires,

Wobma,

Samad

et al. 2024

J Rheumatol

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ObjectiveTo describe the clinical features of patients with congenital heart disease (CHD) who subsequently developed systemic juvenile idiopathic arthritis (sJIA).MethodsWe conducted a retrospective review of patients diagnosed with CHD and sJIA at our institution. Detailed clinical, laboratory and radiographic data were collected from the medical record and reviewed with each patient's primary medical team.ResultsFive patients with sJIA and CHD were identified. Each child had a unique cardiac anatomy but all of the patients required surgical repair during the first year of life. Four children had thymectomies at the time of cardiac surgery. Classic signs of sJIA such as fever (n=5), rash (n=5), and arthritis (n=4) developed after surgical intervention in all of the patients. The individuals in this cohort displayed risk factors associated with severe sJIA, including disease onset before 2 years of age (n=5), elevated IL-18 levels (n=5), baseline eosinophilia prior to initiation of biologic disease modifying anti-rheumatic drugs (bDMARDs) (n=4), and positivity for HLA-DRB1*15:01 alleles (n=4). Macrophage activation syndrome (MAS) occurred in 3 patients and sJIA-associated lung disease (sJIA-LD) was identified in 4 patients. Two children died from complications of their cardiac and/or pulmonary disease.ConclusionWe identified an association between CHD and severe forms of sJIA. While these findings will need to be confirmed in larger, multi-center cohorts, the results highlight the importance of considering a diagnosis of sJIA in children with CHD and remaining vigilant for complications such as MAS and sJIA-LD.

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Immune System in Single Ventricle Patients—A Complex Nexus

Cited by 3 publications

References 1 publication

Current diagnostic and therapeutic strategies for the management of lymphatic insufficiency in patients with hypoplastic left heart syndrome

Current diagnostic and therapeutic strategies for the management of lymphatic insufficiency in patients with hypoplastic left heart syndrome

Stem Cell Therapy in Single-Ventricle Physiology: Recent Progress and Future Directions

Severe Features of Systemic Juvenile Idiopathic Arthritis in Patients With Congenital Heart Disease

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