Background: Turner Syndrome (TS) is a chromosomal disorder frequently associated with congenital cardiovascular abnormalities, particularly bicuspid aortic valve (BAV), coarctation of the aorta (CoA), and aortic dilatation. These conditions substantially increase the risk of aortic dissection. Although echocardiography (ECHO) is commonly used for cardiac monitoring, its limitations in evaluating aortic morphology require cardiac magnetic resonance imaging (CMR), as recommended by recent guidelines.
View Article and Find Full Text PDF: Management of complex congenital heart defects with functionally single ventricle remains one of the greatest challenges of pediatric cardiology. The multistage surgical treatment completed with Fontan procedure is related to multiple complications. Due to non-pulsatile continuous pulmonary flow and chronic hypoxia, Fontan circulation may induce pulmonary endothelial dysfunction.
View Article and Find Full Text PDFGalectin-3 (Gal-3), a β-galactoside-binding lectin, has emerged as a potential diagnostic and prognostic biomarker for various diseases, including certain heart and kidney diseases, as well as cancer. Its significance is particularly notable in the context of congenital heart defects (CHD), which are the most prevalent congenital malformations, occurring in 6 to 8 out of every 1000 live births. Symptoms of heart failure (HF) in patients with congenital heart defects can manifest early in life, but in some cases, the disease progresses gradually, leading to a gradual decline in quality of life and the development of various complications.
View Article and Find Full Text PDFInt J Mol Sci
August 2024
The clinical significance of B-type natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) in pediatric patients remains an area of evolving understanding, particularly regarding their utility in the presence or absence of pre-existing heart conditions. While clear cutoff values and established roles in heart failure are understood in adult patients, pediatric norms vary with age, complicating interpretation. Notably, the emergence of multi-system inflammatory syndrome in children (MIS-C) has highlighted the importance of these markers not only in the detection of acute heart failure but also as a marker of disease severity and even as a differential diagnosis tool.
View Article and Find Full Text PDFChildren (Basel)
March 2024
In September 2023, the European Society of Cardiology (ESC) published new guidelines for the management of endocarditis. Infective endocarditis (IE) remains a significant life-threatening disease, concerning an increasingly younger age group, especially children with congenital heart disease (CHD) and young adults after multiple cardiac surgeries. This study's aim was to alert the medical community to the problem of increasing IE case numbers and IE course complexity.
View Article and Find Full Text PDFBackground: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers.
Aims: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey.
Methods: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents.
J Interv Card Electrophysiol
August 2023
Background: Radiofrequency catheter ablation (RFCA) of the slow pathway in atrioventricular nodal reentry tachycardia (AVNRT) is associated with high efficacy and low risk of total perioperative or late atrioventricular block. This study aimed to evaluate the efficacy, safety, and feasibility of slow-pathway RFCA for AVNRT using a zero-fluoroscopy approach.
Methods: Data were obtained from a prospective multicenter registry of catheter ablation from January 2012 to February 2018.
Riociguat, a soluble guanylate cyclase stimulator, is approved for treatment of adults with pulmonary arterial hypertension (PAH). The safety, tolerability, and pharmacokinetics (PK) of oral riociguat in a pediatric population with PAH was assessed in PATENT-CHILD (NCT02562235), a multicenter, single-arm, 24-week, open-label, Phase 3 study. Patients aged 6-17 years in World Health Organization functional class (WHO-FC) I-III treated with stable endothelin receptor antagonists and/or prostacyclin analogs received riociguat equivalent to 0.
View Article and Find Full Text PDFCases of severe heart damage in patients presenting with multisystem inflammatory syndrome in children are one of the most intriguing phenomena during the coronavirus disease of 2019 pandemic. The pathophysiology of myocardial changes in the course of this syndrome has not been fully understood yet. We present a case of a child with multisystem inflammatory syndrome in children and with cardiac changes corresponding to Takotsubo syndrome.
View Article and Find Full Text PDFDuring the winter months of 2020/2021 a wave of multisystem inflammatory syndrome in children (MIS-C) emerged in Poland. We present the results of a nationwide register aiming to capture and characterise MIS-C with a focus on severity determinants. The first MIS-C wave in Poland was notably high, hence our analysis involved 274 children.
View Article and Find Full Text PDFPostepy Kardiol Interwencyjnej
September 2021
Postepy Kardiol Interwencyjnej
June 2021
J Card Surg
August 2021
Hypoplastic left heart syndrome (HLHS) was first described by Lev in 1952, but it was not until 1958 that it received a name from Noonan and Nadas. For the next several decades, the defect was considered untreatable. In 1979, William Norwood and his colleagues from Boston initiated a program to evaluate staged surgical management for infants with HLHS.
View Article and Find Full Text PDFJ Clin Med
April 2021
The COVID-19 pandemic has impacted healthcare systems worldwide. Little is known about the impact of the pandemic on medical and psycho-social aspects of children with rare diseases such as pulmonary arterial hypertension and their parents. The study is based on children registered in The Database of Pulmonary Hypertension in the Polish Population and a parent-reported survey deployed during the first 6 months of the pandemic.
View Article and Find Full Text PDFKardiol Pol
October 2020
Atrial septal defect is the most common congenital heart lesion in adults. Although atrial septal defect closure is recommended in those with right heart enlargement or paradoxical embolism, data informing such indications in adults are quite limited. This population has many unique characteristics and needs.
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