ABSTRACT
This case highlights the need for accurate and rapid testing for severe acute respiratory syndrome coronavirus 2 and also underscores the need for caregivers to remain vigilant for coronavirus disease 2019 in the postoperative setting despite negative preoperative testing.
Subject(s)
COVID-19/therapy , Heart Defects, Congenital/surgery , Postoperative Complications/therapy , SARS-CoV-2 , Extracorporeal Membrane Oxygenation , Humans , Infant , MaleABSTRACT
An 11-year-old girl with kaposiform lymphangiomatosis presented with recurrent chylous pericardial effusions that were refractory to pericardial drainage and medical therapy. Magnetic resonance imaging demonstrated a prominent lymphatic duct with anterior mediastinal extension into the left clavicular region and a region of high signal that was favored to represent a low-flow lymphatic malformation. The patient underwent direct access thoracic duct lymphangiography with thoracic duct embolization and sclerotherapy of the large left-sided neck and pericardial lymphatic malformation. After the procedure, her pericardial effusions resolved, and she has remained asymptomatic for 15 months.
Subject(s)
Embolization, Therapeutic , Lymphangiectasis/therapy , Lymphatic Abnormalities/therapy , Pericardial Effusion/therapy , Sclerotherapy , Thoracic Duct , Child , Female , Humans , Lymphangiectasis/diagnostic imaging , Lymphatic Abnormalities/diagnostic imaging , Pericardial Effusion/diagnostic imaging , Thoracic Duct/diagnostic imaging , Treatment OutcomeABSTRACT
OBJECTIVE: Fontan circulations have obligatory venous hypertension, depressed cardiac output and abnormal arterial elastance. Ventriculovascular coupling is known to be abnormal, but the underlying mechanisms are poorly defined. We aim to describe the histopathological features of vascular remodelling encountered in the central arteries and veins in the Fontan circulation as a possible underlying pathological representation of abnormal ventriculovascular coupling. METHODS: Postmortemvasculature (inferior vena cava (IVC), superior vena cava (SVC), pulmonary artery (PA), pulmonary vein (PV) and aorta) of 13 patients with a Fontan circulation (mean age 29.9 years, range 9.0-59.8 years) and 2 biventricular controls (ages 17.9 and 30.2 years) was examined. RESULTS: IVC and SVC: Eccentric and variable intimal fibromuscular proliferation occurred in 11 Fontan subjects. There was variable loss of medial smooth muscle bundles with reciprocal replacement with dense collagenous tissue.PA: Similar intimal fibromuscular proliferation was seen; however, these intimal changes were accompanied by medial thinning rather than expansion, medial myxoid degeneration and elastic alteration.PV: The PVs demonstrated intimal fibroproliferation and disorganisation of the muscular media.Aorta: The aortic lamina intima was thickened, with associated fibromuscular proliferation and elasticisation. There was also moderate lymphocytic inflammation in the aortic wall. CONCLUSIONS: Vascular architectural remodelling is common in Fontan patients. The central veins demonstrate profound changes of eccentric intimal expansion and smooth muscle replacement with collagen. The pulmonary demonstrated abnormal intimal proliferation, and aortic remodelling was characterised by intima lamina thickening and a moderate degree of aortic wall inflammation.
