About this Research Topic
Lymphatic problems are not uncommon in congenital cardiology, particularly in the early post-operative period or in patients with a Fontan circulation. Complications such as lymphoedema, chylothorax, or chylopericardium are well-known after surgery for congenital heart disease. The medical treatment of post-operative lymphatic disorders may involve dietary recommendations, diuretics, protein infusions, steroids, heparin, octreotide, among others.
Another major entity is chronic lymphatic dysfunction in Fontan patients. This can lead to rare complications like plastic bronchitis or protein-losing enteropathy, which can have potentially life-threatening outcomes. However, diagnosing these conditions can be challenging as routine lymphatic imaging is not often performed in pediatrics. Traditional methods such as lymphangiography require lymph node or cisterna chyli puncture, but dynamic contrast-enhanced MRI is now offering new insights. In this patient population, medical treatment is often failing and several surgical and percutaneous interventions have been described, including anonymous vein rerouting, resection of lymphangiectasies, and fenestrating univentricular circuits. Recently, more targeted strategies have shown their safety and particular efficiency in chronic lymphatic impairment. Selective lymphosclerosis or lymphatic embolization of abnormal fistulae, for example in periportal or paratracheal regions, showed relief of severe protein-losing enteropathy and plastic bronchitis. Additionally, interventions on the thoracic duct using coils, glue, or covered stents have gained significant interest for the management of lymphatic diseases.
The aim of this research topic is to focus on the management of lymphatic dysfunction, from diagnosis to treatment. We are in need of a multidisciplinary approach to get a better understanding of the pathogenesis of lymphatic disorders in our patients, improve the visualization of the problem by new imaging techniques and elaborate selective treatment strategies including percutaneous interventions. We welcome research dealing with all aspects of lymphatic disease in the setting of congenital heart disease, from infancy to adult age.
Another major entity is chronic lymphatic dysfunction in Fontan patients. This can lead to rare complications like plastic bronchitis or protein-losing enteropathy, which can have potentially life-threatening outcomes. However, diagnosing these conditions can be challenging as routine lymphatic imaging is not often performed in pediatrics. Traditional methods such as lymphangiography require lymph node or cisterna chyli puncture, but dynamic contrast-enhanced MRI is now offering new insights. In this patient population, medical treatment is often failing and several surgical and percutaneous interventions have been described, including anonymous vein rerouting, resection of lymphangiectasies, and fenestrating univentricular circuits. Recently, more targeted strategies have shown their safety and particular efficiency in chronic lymphatic impairment. Selective lymphosclerosis or lymphatic embolization of abnormal fistulae, for example in periportal or paratracheal regions, showed relief of severe protein-losing enteropathy and plastic bronchitis. Additionally, interventions on the thoracic duct using coils, glue, or covered stents have gained significant interest for the management of lymphatic diseases.
The aim of this research topic is to focus on the management of lymphatic dysfunction, from diagnosis to treatment. We are in need of a multidisciplinary approach to get a better understanding of the pathogenesis of lymphatic disorders in our patients, improve the visualization of the problem by new imaging techniques and elaborate selective treatment strategies including percutaneous interventions. We welcome research dealing with all aspects of lymphatic disease in the setting of congenital heart disease, from infancy to adult age.
Keywords: Lymphatic disorders, Chylothorax, Chylopericardium, Plastic bronchitis, Lymphangiography
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