Interventional Treatment of Acute Right Ventricular Outflow Tract Infectious Endocarditis: A Bridge to Surgical or Percutaneous Pulmonary Valve Replacement.

Background: Infective endocarditis (IE) poses significant risks following percutaneous pulmonary valve implantation (PPVI) or surgical replacement (PVR).

Aims: This study evaluates the effectiveness of emergency percutaneous treatment in stabilizing patients with severe right ventricular dysfunction or obstructive cardiac shock, allowing for delayed surgical or percutaneous valve replacement.

Methods: This retrospective study examines 16 patients (age 19.

Active learning for extracting rare adverse events from electronic health records: A study in pediatric cardiology.

Objective: Automate the extraction of adverse events from the text of electronic medical records of patients hospitalized for cardiac catheterization.

Methods: We focused on events related to cardiac catheterization as defined by the NCDR-IMPACT registry. These events were extracted from the Necker Children's Hospital data warehouse.

An exceptional anomaly of the coronary venous drainage: anatomic description.

Background: Anomalies of the coronary sinus are rare in the general population but are more frequent in patients with congenital heart defects. Whatever the cardiac anatomy, the coronary sinus is invariably located in the left atrioventricular sulcus, inferior to the wall of the morphologically left atrium.

Methods: A complete morphological examination of a fetal cardiac specimen of the M3C collection, according to segmental analysis, was performed by two observers.

Pulmonary hypertension in patients carrying FLNA loss-of-function variants.

Background: Pulmonary hypertension (PH) is an unusual complication of X-linked disease caused by loss-of-function (LOF) variants in the () gene. Patients with LOF may also present dysmorphic facial features, aortic dilation, thrombopenia, and periventricular nodular heterotopia (PVNH).

Methods: We reported clinical, functional, radiologic, and hemodynamic characteristics of patients with LOF variants and PH from the French PH Network.

Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations.

Background: Lymphatic disorders such as protein-losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers.

Myocardial infarction in a 17-year-old patient diagnosed with MPOD II syndrome.

Introduction: Microcephalic osteodysplastic primordial dwarfism (MOPD) syndrome type 2, caused by a mutation in the PCNT gene (21q22.3), is a rare autosomal recessive disorder. Patients present with bone dysplasia, insulin resistance, kidney diseases, and cardiac malformations, making them prone to vascular diseases.

Sacubitril/Valsartan in Pediatric Heart Failure (PANORAMA-HF): A Randomized, Multicenter, Double-Blind Trial.

Background: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD).

Device Closure of Hemodynamically Significant Patent Ductus Arteriosus in Premature Infants.

The patent ductus arteriosus is a very common condition in preterm infants, and a hemodynamically significant patent ductus arteriosus increases morbidity and mortality in these vulnerable patients. However, despite numerous randomized controlled trials, there is no consensus regarding management. Medical therapy is typically offered as first-line treatment, although it yields limited success and carries the potential for severe adverse events.

Embracing the challenges of neonatal and paediatric pulmonary hypertension.

Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps.

Pulmonary Hypertension Induced by Right Pulmonary Artery Occlusion: Hemodynamic Consequences of Mutation.

Background: The primary genetic risk factor for heritable pulmonary arterial hypertension is the presence of monoallelic mutations in the gene. The incomplete penetrance of mutations implies that additional triggers are necessary for pulmonary arterial hypertension occurrence. Pulmonary artery stenosis directly raises pulmonary artery pressure, and the redirection of blood flow to unobstructed arteries leads to endothelial dysfunction and vascular remodeling.

Prophylactic Pulmonary Artery Banding in Pediatric Dilated Cardiomyopathy: An Additional Therapeutic Option.

Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy and is associated with considerable early mortality. Heart transplantation is often the only viable life-saving option. Pulmonary artery banding (PAB) has been recently proposed as a bridge or alternative to transplantation for DCM.

Pulmonary vascular phenotype identified in patients with () or variants: an international multicentre study.

Background: Bone morphogenetic proteins 9 and 10 (BMP9 and BMP10), encoded by and , respectively, play a pivotal role in pulmonary vascular regulation. variants have been reported in pulmonary arterial hypertension (PAH) and hereditary haemorrhagic telangiectasia (HHT). However, the phenotype of and carriers remains largely unexplored.

Increased aortic pressures and pulsatile afterload components promote concentric left ventricular remodeling in adults with transposition of the great arteries and arterial switch operation.

Background: Functional abnormalities of the ascending aorta (AA) have been mainly reported in young patients who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA).

Objectives: To compare systolic, diastolic brachial and central blood pressures (bSBP, bDBP, cSBP, cDBP), aortic biomechanical parameters, and left ventricular (LV) afterload criteria in adult ASO patients with healthy controls and to assess their relationships with LV remodeling and aortic size.

Materials And Methods: Forty-one prospectively enrolled patients (16.

Early hybrid cardiac rehabilitation in congenital heart disease: the QUALIREHAB trial.

Background And Aims: Cardiopulmonary fitness in congenital heart disease (CHD) decreases faster than in the general population resulting in impaired health-related quality of life (HRQoL). As the standard of care seems insufficient to encourage and maintain fitness, an early hybrid cardiac rehabilitation programme could improve HRQoL in CHD.

Methods: The QUALIREHAB multicentre, randomized, controlled trial evaluated and implemented a 12-week centre- and home-based hybrid cardiac rehabilitation programme, including multidisciplinary care and physical activity sessions.

Postoperative catheter thrombaspiration to open an occluded right ventricle-to-pulmonary artery conduit in child.

We report on a 6-year-old girl (18 kg/120 cm) who was diagnosed on day 6 postoperative with an occlusive thrombosis of a right ventricle-to-pulmonary artery conduit in the setting of a Ross procedure that was performed for severe native aortic valve insufficiency secondary to late diagnosis of bacterial endocarditis. We applied the Indigo® aspiration system from Penumbra® (Alameda, USA) to mechanically dissolve and remove the thrombus, restore flow, gradually wean from extracorporeal support, and replace the conduit after 4 days. The patient experiences good surgical outcomes at 3 months of follow-up.

Neurodevelopmental outcomes of preterm and growth-restricted neonate with congenital heart defect: a systematic review and meta-analysis.

Unlabelled: The purpose of the study is to assess the risks of neurodevelopmental morbidity among preterm and growth restricted youth with congenital heart defects (CHD). This systematic review and meta-analysis included observational studies assessing neurodevelopmental outcomes among children with CHD born preterm (i.e.