Thyroid dysfunction in pediatric Fontan patients is associated with unfavorable hemodynamic status and severity of protein-losing enteropathy: A report from the Fontan care network.

Background: Thyroid dysfunction may have adverse effects on Fontan hemodynamics. Data on thyroid function in pediatric Fontan patients with or without protein-losing enteropathy (PLE) are limited.

Methods: This retrospective multicenter study included 67 Fontan patients (median age 10.

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry.

Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension.

Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data.

RACHS-1 score as predictive factor for postoperative ventilation time in children with congenital heart disease.

Background: Congenital heart disease is the most frequent malformation in newborns. The postoperative mortality of these patients can be assessed with the Risk Adjustment in Congenital Heart Surgery-1 (RACHS-1) score. This study evaluates whether the RACHS-1 score can also be used as a predictor for the length of postoperative ventilation and what is the influence of age.

Emerging clinical applications of strain imaging and three-dimensional echocardiography for the assessment of ventricular function in adult congenital heart disease.

Management of congenital heart disease (CHD) in adults (ACHD) remains an ongoing challenge due to the presence of residual hemodynamic lesions and development of ventricular dysfunction in a large number of patients. Echocardiographic imaging plays a central role in clinical decision-making and selection of patients who will benefit most from catheter interventions or cardiac surgery..

Successful biventricular cardiac resynchronization therapy in a failing Fontan patient: Implications of ventriculo-ventricular interdependency in single ventricle physiology.

We describe a Fontan patient with severe heart failure who was successfully treated with biventricular cardiac resynchronization therapy (CRT). Our case shows that strain imaging might play a crucial role in guiding placement of pacing leads and in characterizing the electromechanical substrate associated with a favorable CRT response. Furthermore, we demonstrate for the first time that ventriculo-ventricular interdependency seems an important mechanical concept, which can be utilized to augment cardiac performance in failing Fontan patients with a functional hypoplastic ventricle.

Stenting the Fontan pathway in paediatric patients with obstructed extracardiac conduits.

Objectives: An unobstructed extracardiac conduit (ECC) is essential for optimal Fontan haemodynamics. We aimed to evaluate the feasibility and results of percutaneous transcatheter stenting of the ECC in paediatric patients with a significant Fontan pathway obstruction.

Methods: Our institutional database was searched to identify all Fontan patients who had a stent placed in their ECC.

Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review.

Objective: A standardised diagnostic definition of protein-losing enteropathy (PLE) in Fontan patients serves both patient care and research. The present study determined whether a diagnostic definition of PLE was routinely used in published clinical Fontan studies, and to identify potentially relevant diagnostic criteria for composing a uniform PLE definition.

Methods: A systematic review was conducted in adherence to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations.

Low serum 25-hydroxyvitamin D levels and secondary hyperparathyroidism in Fontan patients.

Background: Limited data exist on the vitamin D status in Fontan patients. We determined the prevalence and potential risk factors of vitamin D deficiency in this patient subset. Methods and results Data were collected from 27 Fontan patients (55.

Morphometric parameters of the radial neck: an anatomical study.

Introduction: The purpose of this study was to describe the anatomy of the intramedullary canal of the radial neck for stem design of radial head prostheses.

Methods: Radial neck measurement was performed from the proximal edge of the radial tuberosity to the distal border of the articular surface on 40 macerated proximal radii using X-rays and Optosil imprints of the intramedullary canal. Radial neck to shaft angle was measured under image intensification.

Morphometric parameters of the radial head: an anatomical study.

Introduction: Exact anatomical description of the proximal radius is imperative for the development of radial head prostheses. The purpose of this study was to measure the anatomical parameters of the radial head.

Methods: Optosil imprints of 18 pairs of proximal radii fixed with formalin were taken.