Right ventricular function has been identified as an important prognostic factor in children with pulmonary arterial hypertension. The aim of the study was to assess the deformation pattern and prognostic value of right ventricular longitudinal strain in children with pulmonary arterial hypertension. We prospectively evaluated 46 children (25 with pulmonary arterial hyperetension and 21 age and sex matched controls) using conventional and speckle-tracking echocardiography, brain natriuretic peptide levels and clinical status expressed by WHO functional class and 6-min walking test. According to the clinical status after 12 months of follow-up, the pulmonary arterial hypertension patients were divided in two groups: non-worsening (13) and worsening (12). Right ventricular free wall longitudinal strain and strain rate were significantly lower in children with PAH compared with controls (−24.72 ± 3.48 vs −15.60 ± 3.40, p = 0.0001 and −1.44 ± 0.09 vs −1.09 ± 0.15, p = 0.0001, respectively). There was a more expressed decrease of basal than apical region of right ventricular free wall longitudinal strain/strain rate in pulmonary arterial hypertension patients compared with controls (strain: p = 0.0001 vs p = 0.07 and strain rate: p = 0.0001 vs p = 0.002). Comparing worsening and non-worsening pulmonary arterial hypertension patients there was a significant difference in Mid right ventricular free wall longitudinal strain (−14.00 ± 3.13 vs. −20.76 ± 4.62, p = 0.0001). In multivariable logistic regression analysis Mid right ventricular free wall longitudinal strain was an independent predictor of worsening in pulmonary arterial hypertension children (OR 0.45; 95 % CI: 0.21–0.96, p = 0.041). In ROC curve analysis a cut-off value of Mid right ventricular free wall longitudinal strain of −18.50 % predicted clinical worsening in pulmonary arterial hypertension children, with a sensitivity and specificity of 91.7 and 30.8 %, respectively (area under curve = 0.88 ± 0.06, 95 % CI: 0.75–1.00, p = 0.001). Two-dimensional speckle-tracking echocardiography is a complementary non-invasive tool for assessment of right ventricular function in children with severe pulmonary arterial hypertension, allowing also clinical prediction and segmental analysis of right ventricular myocardial performance in these patients.

Scleroderma, known also as systemic sclerosis (SSc), is a severe disease associated with high mortality rates, and right ventricular (RV) remodeling and dysfunction, along with pulmonary artery hypertension (PAH), are among the most important internal organ manifestations of this disease. PAH has a higher prevalence in patients with SSc compared to the general population and represents a significant predictor of mortality in SSc. In patients with SSc, the morphological remodeling and alteration of RV function begin even before the setting of PAH and lead to development of a specific adaptive pattern of the RV which is different from the one recorded in patients with IAPH. These alterations cause worse outcomes and increased mortality rates in SSc patients. Early detection of RV dysfunction and remodeling is possible using modern imaging tools currently available and can indicate the initiation of specific therapeutic measures before installation of PAH. The aim of this review is to summarize the current knowledge related to mechanisms involved in the remodeling and functional alteration of the RV in SSc patients.

Background: Several studies in recent years have shown the association between vitamin D levels and heart failure. Vitamin D deficiency is related to increased cardiovascular morbidity and mortality, with a higher risk of developing heart failure. In this systematic review, we aimed to assess recent studies that analyzed vitamin D deficiency and heart failure in adult and pediatric populations. (2) Methods: We conducted a systematic search for studies published in the following databases: PubMed and Scopus from January 2012 to October 2022. (3) Results: Most observational studies that were included found a significant association between hypovitaminosis D and heart failure. However, the beneficial role of vitamin D supplementation is still controversial due to the lack of randomized controlled trials. (4) Conclusions: Vitamin D may play an important role as a cardiovascular marker in heart failure patients. More well-designed studies are needed to investigate the relationship between vitamin D and heart failure and to determine if vitamin D supplementation could improve long-term outcomes.

The occurrence of an haematoma in the interventricular septum is very rare. We report a case of a giant interventricular septal haematoma after surgical correction of a critical pulmonary valve stenosis in a newborn with intact ventricular septum. Control echocardiography showed complete resolution of the haematoma at 6-month follow-up.

Supraventricular tachyarrhythmia represents the most frequent fetal dysrhythmia. In the lack of diagnosis and treatment these fetuses may develop hydrops and even death. For the therapeutic approach it is important to establish the diagnosis of the type of supraventricular tachycardia. In this paper we report 29 cases with different types of supraventricular tachycardia in which the diagnosis was established using our own protocol, which allowed us to make the difference between the types of tachycardia (atrioventricular reentry tachycardia due to the accessory pathway, atrial ectopic tachycardia and permanent junctional reciprocal tachycardia). We acquired the data by a series of recordings in M mode and pulsed Doppler by simultaneous recording of an artery and a vein flow. First of all, we diagnosed the supraventricular tachycardia type, with short or long ventriculoarterial interval, and afterwards, we made the difference between atrial ectopic tachycardia and permanent junctional reciprocal tachycardia using methods to decrease the atrioventricular conduction.

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. Methods and Results: A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty‐one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178 ‐ 313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein‐losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1 ‐ 4) procedures per year and 12 (interquartile range, 5 ‐ 15) interventions in total per center. Conclusions: This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence‐based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.