Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension.

Background: Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved.

Objectives: To evaluate long-term clinical and procedural outcomes of TPS intervention.

Multifactorial pulmonary hypertension in infantile scimitar syndrome.

Background: Pulmonary hypertension in infantile scimitar syndrome is highly prevalent at diagnosis, and has a multifactorial origin.

Aims: To analyse the constellation of anatomical anomalies and initial physiology, and their contribution to pulmonary hypertension and outcome in infantile scimitar syndrome.

Methods: Pulmonary hypertension causes were classified into five categories: associated with systemic supply to the right lung; associated with left-to-right shunt; postcapillary; related to respiratory or developmental lung disease; and "idiopathic-like" pulmonary arterial hypertension.

Transcatheter patent arterial duct closure in premature infants: A new technique to ease access to the patent arterial duct, with particular benefit for the tricuspid valve.

Background: Transcatheter patent arterial duct (PAD) closure in premature infants has been shown to be feasible. Since our early transcatheter PAD closure procedures in premature infants at Hôpital Necker Enfants Malades, we have changed our technique several times to advance the guidewire through the right heart to avoid tricuspid valve damage.

Aim: To describe the technique we have been using since May 2019, to report our results with a particular focus on tricuspid leaks and to analyse the potential mechanisms of tricuspid lesion development with previous methods.

Potts anastomosis in children with severe pulmonary arterial hypertension and atrial septal defect.

Aims: Potts shunt has been proposed as a bridge or alternative to lung transplantation for children with severe and drug-refractory suprasystemic pulmonary arterial hypertension (PAH). We describe the management of the atrial shunt when a Potts shunt is planned in refractory PAH.

Methods And Results: We report a case series of children in whom a Potts shunt was done for severe PAH associated with an atrial septal defect to illustrate the different clinical and haemodynamic scenarios.

Upfront triple combination therapy in severe paediatric pulmonary arterial hypertension.

Treatment strategies in paediatric pulmonary arterial hypertension (PAH) have evolved over the last years, but survival is still poor. Recently, in adults with severe PAH, upfront triple combination therapy (uTCT) from diagnosis has been reported to show significant clinical improvement and excellent long-term outcome. This retrospective, observational study aimed to assess the efficacy of uTCT in paediatric PAH.

Pulmonary hypertension after bone marrow transplantation in children.

Introduction: Pulmonary hypertension is a rare but important cause of mortality after haematopoietic stem cell transplantation (HSCT) in children. This complication is poorly characterised in the literature. We report here a series of children who developed pulmonary hypertension after HSCT.

Cardiac catheterisation in infants weighing less than 2500 grams.

Objectives: The aim of the study was to report the outcome of cardiac catheterisation in low-weight patients.

Background: Data regarding cardiac catheterisation in infants weighing <2500 g are scarce.

Methods: We reviewed all cardiac catheterisations performed in infants weighing <2500 g between January 2000 and May 2016.

Treprostinil treatment decreases circulating platelet microvesicles and their procoagulant activity in pediatric pulmonary hypertension.

Background: Pulmonary arterial hypertension (PAH) results from pulmonary vascular disease and may eventually lead to right heart failure and death. Vasodilator therapy has greatly improved PAH prognosis. Circulating microvesicles are considered as surrogate markers of endothelial and hematopoietic cell activation.

Genetic analyses in a cohort of children with pulmonary hypertension.

The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers.The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD).

Kinetics of the utilization of dietary arginine for nitric oxide and urea synthesis: insight into the arginine-nitric oxide metabolic system in humans.

Background: The systemic availability of oral/dietary arginine and its utilization for nitric oxide (NO) synthesis remains unknown and may be related to a competitive hydrolysis of arginine into urea in the splanchnic area and systemic circulation.

Objectives: We investigated the kinetics and dose-dependency of dietary arginine utilization for NO compared with urea synthesis and studied the characteristics of the arginine-NO metabolic system in healthy humans.

Design: We traced the metabolic fate and analyzed the utilization dynamics of dietary arginine after its ingestion at 2 nutritional amounts in healthy humans (n = 9) in a crossover design by using [(15)N-(15)N-(guanido)]-arginine, isotope ratio mass spectrometry techniques, and data analysis with a compartmental modeling approach.

Endothelial-dependent vasodilation is impaired in children with sickle cell disease.

Impairment of endothelial-dependent vasodilation has been demonstrated in adults with sickle cell anemia (SCA). We enrolled 21 SCA children, mean age 10.4+/-3.

Meal amino acids with varied levels of arginine do not affect postprandial vascular endothelial function in healthy young men.

Postprandial endothelial dysfunction is a key event mediating the effects of diet on early atherogenesis. The potential effects of protein intake have been overlooked in the past, although amino acids are precursors for homocysteine and nitric oxide (NO). Our objective was to study the effect of amino acids on postprandial vascular function, in relation to the utilization of meal arginine for NO production.

Vascular remodeling after "successful" repair of coarctation: impact of aortic arch geometry.

Objectives: This study was designed to investigate the influence of aortic arch geometry on vascular remodeling after anatomically successful repair of coarctation of the aorta (CoA).

Background: Abnormalities of the precoarctation vasculature are known to occur after CoA repair and appear related to adverse outcomes. The influence of aortic arch geometry on such abnormalities is unknown.

Noninvasive assessment of arterial stiffness and risk of atherosclerotic events in children.

Noninvasive assessment of vascular dysfunction in the pediatric population has taken advantage of the development of high-resolution ultrasound techniques. The most frequently used methods are the quantification of flow-mediated endothelium-dependent dilation of the brachial artery and measurement of the intima-media thickening of the carotid artery. Both reduced flow-mediated dilation and increased intima-media thickness have been proven to correlate with late cardiovascular events and/or mortality in adults.

Arterial stiffness and endothelial dysfunction in HIV-infected children.

Background: The role of antiretroviral therapy in acceleration of atherosclerosis in HIV-infected adults is controversial, partly because of the confounding effects of the involvement of classic cardiovascular risk factors.

Objective: To study vascular function in HIV-infected children.

Design: Cross-sectional study of 49 HIV-infected children (34 receiving antiretroviral therapy and 15 never treated) and if 24 age- and sex-matched controls.