Background: The development of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD) is multifactorial with a number of biomarkers serving as mediators of neurohormonal activation [B-type natriuretic peptide (BNP) and its N-terminal-pro-fragment (NT-proBNP)], endothelial dysfunction [asymmetric dimethylarginine (ADMA)] and cellular proliferation [vascular endothelial growth factor (VEGF)].
Methods: We systematically reviewed the literature for trials studying the role of these biomarkers in the clinical evaluation, prognosis and management of patients with PAH related to CHD (CHD-PAH).
Results: Twenty-six studies were included in the systematic review, involving a total of 1113 patients with CHD-PAH. These patients had higher BNP, NT-proBNP and ADMA levels and higher VEGF expression when compared with healthy controls. Baseline and serial values of plasma levels of natriuretic peptides were shown to be significant predictors of survival. ADMA concentration was elevated in patients with CHD-PAH when compared with patients with simple CHD without PAH, whereas VEGF expression was particularly high in patients with CHD and persistent PAH after corrective surgery of the underlying heart disease.
Conclusion: Right heart dysfunction, endothelial inflammation and proliferation are mirrored by plasma levels of the corresponding biomarkers among patients with CHD-PAH. There is early evidence to suggest that natriuretic peptides, in particular, may be a simple and effective tool for determining prognosis and timing for therapeutic interventions in patients with CHD-PAH.
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http://dx.doi.org/10.1016/j.ijcard.2014.04.156 | DOI Listing |
Int J Mol Sci
December 2024
Department of Experimental Immunology, Medical University of Lublin, Chodźki 4a Street, 20-093 Lublin, Poland.
Pulmonary arterial hypertension (PAH) is a disease characterized by increased pulmonary vascular resistance and right heart failure, with emerging evidence suggesting a key role for immune dysregulation in its pathogenesis. This study aimed to assess the involvement of lymphocytes, particularly regulatory T cells (Tregs), and the expression of immune checkpoint molecules PD-1 and PD-L1 on peripheral blood subpopulations in patients diagnosed with PAH. The study involved 25 patients; peripheral blood mononuclear cells were isolated and subsequently analyzed using flow cytometry to quantify the Treg cell percentage and evaluate PD-1 and PD-L1 expression across the T and B cells.
View Article and Find Full Text PDFLung
December 2024
Department of Cardiology, Affiliated Hospital of Nantong University, No. 20 Xisi Road, Nantong, 226000, China.
Objectives: This study explored the expression and diagnostic value of differentially expressed miR-3591-5p in congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH).
Methods: A total of 110 CHD patients were divided into four groups based on their mean pulmonary artery pressure (PAPm). The plasma miR-3591-5p expression was determined by reverse transcription polymerase chain reaction.
Int J Mol Sci
August 2024
Unidad de Investigación en Enfermedades Infecciosas, Hospital Infantil de México Federico Gómez-Secretaría de Salud (SS), Mexico City 06720, Mexico.
Congenital heart disease (CHD) can be complicated by pulmonary arterial hypertension (PAH). Cardiopulmonary bypass (CPB) for corrective surgery may cause endothelial dysfunction, involving endothelin-1 (ET-1), circulating endothelial cells (CECs), and endothelial progenitor cells (EPCs). These markers can gauge disease severity, but their levels in children's peripheral blood still lack consensus for prognostic value.
View Article and Find Full Text PDFKorean Circ J
October 2024
Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Background And Objectives: The transformation of pulmonary arterial hypertension (PAH) treatment in Korea, ushered by targeted therapy's advent, prompted our analysis of baseline attributes, treatment trends, and survival shifts within our single-center registry.
Methods: We examined 230 patients (72.6% female, mean age 40.
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