Diagnostic yield of nitroglycerin-potentiated head-up tilt test in a pediatric population with suspected reflex syncope.

Background: Syncope is a prevalent issue in pediatric patients. The nitroglycerin (NTG)-potentiated head-up tilt test (HUTT) is widely used in adults for diagnosing reflex syncope; however, few and contrasting data are available in pediatric populations. The aim of our study was to evaluate the positivity rate and types of responses to NTG-potentiated HUTT in pediatric patients with suspected reflex syncope.

Outbreak of Acute Fulminant Myocarditis in Children in Campania Region, Italy: A Case Series.

Acute fulminant myocarditis is a rare event in children, accounting for about 10% of all cases of acute myocarditis. Its lack of specific onset patterns and unpredictable evolution make diagnosis and prompt treatment challenging. We observed six cases of fulminant myocarditis admitted to our Pediatric Emergency Unit (Campania region, Sothern Italy) within a very short timeframe (50 days, from July to September 2024).

Refractory Chylothorax and Ventricular Hypertrophy Treated with Trametinib in a Patient with Noonan Syndrome: 18-Month Follow-Up.

RASopathies are a group of genetic syndromes caused by germline mutations in genes involved in the RAS/Mitogen-Activated Protein Kinase signaling pathway, which regulates cellular proliferation, differentiation, and angiogenesis. Despite their involvement at different levels of this pathway, RASopathies share overlapping clinical phenotypes. Noonan syndrome is the most prevalent RASopathy, with an estimated incidence of 1 in 2500 live births, and it is typically inherited in an autosomal dominant manner, with 50% of cases involving gain-of-function mutations in the PTPN11 gene.

Incomplete Kawasaki Disease with Peripheral Facial Nerve Palsy and Lung Nodules: A Case Report and Literature Review.

The diagnosis of Kawasaki disease (KD) is challenging and often delayed mainly in case of young infants and in presence of an incomplete disease and atypical features. Facial nerve palsy is one of the rare neurologic symptoms of KD, associated with a higher incidence of coronary arteries lesions and may be an indicator of a more severe disease. Here, we describe a case of lower motor neuron facial nerve palsy complicating KD and perform an extensive literature review to better characterize clinical features and treatment of patients with KD-associated facial nerve palsy.

Ivabradine-Flecainide as Breakthrough Drug Combination for Congenital Junctional Ectopic Tachycardia: A Case Report and Literature Review.

Congenital junctional ectopic tachycardia (CJET) is a rare tachyarrhythmia that remains difficult to manage, with suboptimal control in most cases. Here, we report literature research on the use of ivabradine in the treatment of pediatric junctional ectopic tachycardia (JET), both congenital and postoperative, and describe the successful use of ivabradine-flecainide association for CJET therapy resistant to other antiarrhythmic agents. This new drug combination was effective in completely suppressing JET.

Brugada Pattern in a Child with Severe SARS-CoV-2 Related Multisystem Inflammatory Syndrome.

This report presents the first case of Brugada pattern complicated by a supraventricular arrhythmia in a child with SARS-CoV-2 related Multisystem Inflammatory Syndrome in Children (MIS-C). A 7-year-old boy came to our Emergency Department with 7 days of abdominal pain and fever. MIS-C was diagnosed on the basis of the clinical, laboratory and instrumental tests.

Invasive and Noninvasive Evaluation for the Diagnosis of Pulmonary Hypertension: How to Use and How to Combine Them.

The etiologic diagnosis of pulmonary hypertension (PH) may be very challenging. Right-heart catheterization (RHC) in isolation cannot classify a precapillary PH patient into group 1, 3, 4, or 5. Moreover, RHC may be not sufficient for reaching a definitive differential diagnosis of precapillary or postcapillary PH if hemodynamic data are not integrated in clinical context and combined with information gleaned from noninvasive imaging.

Fluid challenge predicts clinical worsening in pulmonary arterial hypertension.

Aim: A fluid challenge with rapid saline infusion during right heart catheterization has been shown to be useful for the differential diagnosis between pre- and post-capillary pulmonary hypertension. The aim of this study was to evaluate the prognostic relevance of fluid challenge-induced changes in pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH).

Methods: Overall, 118 PAH patients (mean age 57 ± 15 years, 80 female) underwent hemodynamic measurements before and after rapid saline infusion (7 mL/kg in 10 min) and were followed up for 19 ± 4 months.

Right atrial function and prognosis in idiopathic pulmonary arterial hypertension.

Aim: To determine whether right atrial (RA) function has prognostic value in patients with idiopathic pulmonary arterial hypertension (PAH).

Methods And Results: Overall, 104 patients (70 female, mean age 58±13years) with idiopathic PAH underwent standard Doppler echocardiography and strain and strain rate (SR) analysis before right heart catheterization. At a mean follow-up of 22±7months, 30 patients (29%) had clinical worsening.

A simple echocardiographic score for the diagnosis of pulmonary vascular disease in heart failure.

Aims: A simple echocardiographic score was designed for diagnosing precapillary vs postcapillary pulmonary hypertension and for discriminating between isolated postcapillary pulmonary hypertension (Ipc-PH) and combined precapillary and postcapillary pulmonary hypertension (Cpc-PH).

Methods: The score comprised 7 points (2 for E/e' ratio ≤10, 2 for a dilated non-collapsible inferior vena cava, 1 for a left ventricular eccentricity index ≥1.2, 1 for a right-to-left heart chamber dimension ratio >1 and 1 for the right ventricle forming the heart apex) and was applied to 230 consecutive patients referred for evaluation of pulmonary hypertension.

Echocardiographic assessment of right ventricular contractile reserve in healthy subjects.

Background: Exercise-induced increase in pulmonary artery systolic pressure (PASP) as a possible measure of right ventricular (RV) contractile reserve has been shown to predict survival in severe pulmonary hypertension. However, RV contractile reserve can also be measured by changes in stroke volume (SV), tricuspid annular plane systolic excursion (TAPSE), or tricuspid annular systolic velocity (S'). The limits of normal values and the functional significance of these changes in healthy subjects are not well known.

Clinical Relevance of Fluid Challenge in Patients Evaluated for Pulmonary Hypertension.

Background: Fluid challenge may help in the differential diagnosis between pre- and postcapillary pulmonary hypertension (PH). However, the test is still in need of standardization and better defined clinical relevance.

Methods: Two hundred twelve patients referred for PH underwent a right-sided heart catheterization with measurements before and after rapid infusion of 7 mL/kg of saline.

Which is the correct management of patients with asymptomatic severe calcific aortic stenosis after symptomatic spontaneous calcium cerebral embolism?

Elective surgery cannot be recommended in every patient with asymptomatic severe aortic stenosis, and predictors identifying high-risk patients need to be identified. In guidelines we read that elective surgery, at the asymptomatic stage, can only be recommended in selected patients, at low operative risk (see guidelines of European Society of Cardiology and American Heart Association), but we have not read any reference to patients with severe calcific aortic stenosis after symptomatic spontaneous calcium cerebral embolism. Because cardioembolic stroke is associated with poor prognosis compared to other stroke subtypes, in patients with asymptomatic severe aortic stenosis and spontaneous calcific embolic stroke valve replacement appears to offer the best hope of avoiding a recidivation of stroke and should be considered in most cases.

Visceral adiposity and arterial stiffness: echocardiographic epicardial fat thickness reflects, better than waist circumference, carotid arterial stiffness in a large population of hypertensives.

Aims: Relationship between obesity and cardiovascular (CV) disease depends not only on the amount of body fat, but also on its distribution. For example, individuals with increased fat accumulation in the abdominal region have atherogenic lipid profiles and are at increased CV risk. The loss of elasticity in medium and large arteries is an early manifestation of atherosclerosis.