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Background: A subgroup of CHDs can only be treated palliatively through a Fontan circulation. In case of a failing Fontan situation, serum proteins are lost unspecifically and can also lead to a loss of vaccine antibodies. In a failing Fontan situation, heart transplantation may be the only feasible option.

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We sought to evaluate the intracardiac morphology and associated cardiovascular anomalies in patients with double inlet right ventricle (DIRV) on multidetector CT angiography. A retrospective search of our departmental database was conducted from January 2014 to January 2023 to identify patients with a diagnosis of DIRV on CT angiography. The intracardiac anatomy and associated cardiovascular abnormalities were systematically evaluated.

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Proposal: Bold New Indications for Transcatheter Pulmonary Flow Restrictors.

Pediatr Cardiol

January 2025

Pediatric Heart Center, Johann-Wolfgang-Goethe University Clinic, Theodor-Storm-Kai 7, 60596, Frankfurt, Germany.

This proposal presents a proof of concept for the use of pulmonary flow restrictors (PFRs) based on MVP™-devices, drawing from clinical experience, and explores their potential role in the management of newborns with hypoplastic left heart syndrome (HLHS), other complex left heart lesions, and infants with end-stage dilated cardiomyopathy (DCM). At this early stage of age, manually adjusted PFRs can be tailored to patient's size and hemodynamic needs. Although currently used off-label, PFRs have substantial potential to improve outcomes in these vulnerable patient populations.

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Background Children with Down syndrome (DS) often have hypoplastic kidneys and urinary tract malformations that increase their renal dysfunction risk. They also have a higher congenital heart disease (CHD) rate, requiring cardiac surgery during infancy. Renal dysfunction in such patients may be associated with the development of cardiac surgery-associated acute kidney injury (CS-AKI), but this remains unclear.

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Cochlear nerve deficiency(CND) is a rare inner ear malformation characterized by a hypoplastic or absent cochlear nerve, resulting in variable hearing loss or total deafness, depending on the quantity of nerve fibers present. About 18% of congenital hearing loss are associated with CND. It is a disease of uncertain cause.

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