Twenty six patients with tetralogy of Fallot at the age from 1 to 3 years old were examined. All examined patients required surgical correction of heart defect and were operated under conditions of artificial circulation. All patients were in a status of relative compensation or subcompensation. The level of a saturation SaO(2) among patients with tetralogy of Fallot in peripheral arterial blood was equal to 66.27 +/- 7.2%. The purpose of work was to study the dynamics of immunological parameters in patients with tetralogy of Fallot in the postoperative period. The complex dynamic study of immune status was carried out to resolve such a task. Identification of membrane markers of peripheral blood immunocompetent cells was made by flow cytofluometry with the usage of monoclonal antibodies to antigens: CD3, CD4, CD5, CD8, CD11b, CD16, CD22, CD25, CD45 RA, HLA DR, CD95. The parameters were determined in dynamics: before operation, on the 7th, the 30th day and after 3 months after operation. Stabilization of parameters of the immune status among patients with tetralogy of Fallot, has been found in the postoperative period and was maintained within 3 months. Apparently it may be due to normalization of cardiohemodynamics, and as a consequence the stabilization of metabolic processes, undoubtedly, rendering modulating effects on immune system of children. However remaining failure of functional activity of immunocompetent cells is the reason to the usage of immunocorrection therapy with the purpose of faster stabilization of immune disbalance.
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JACC Asia
December 2024
National Heart Research Institute Singapore, National Heart Centre Singapore, Singapore.
Background: Right ventricular restrictive physiology (RVRP) is a common occurrence in repaired tetralogy of Fallot (rTOF). The relationship of RVRP with biventricular blood flow components and kinetic energy (KE) from 4-dimensional (4D) flow cardiovascular magnetic resonance (CMR) is unclear.
Objectives: The purpose of this study was to investigate the association of 4D flow CMR parameters with RVRP in rTOF patients.
Cardiol Young
January 2025
Department of Pediatric Cardiology, Arnold Palmer Hospital for Children, Orlando, FL, USA.
Coronary ectasia is a very rare phenomenon seen in Noonan syndrome with only a few documented case reports. We describe a 14-year-old with Noonan syndrome and tetralogy of Fallot with described coronary artery ectasia since infancy who presented for possible transcatheter pulmonary valve placement and was found to have severe ectasia of bilateral coronary arteries.
View Article and Find Full Text PDFCardiovasc Diagn Ther
December 2024
Department of Cardiology, St. Luke's International Hospital, Tokyo, Japan.
Tetralogy of Fallot (TOF) is a condition that often leads to long-term enlargement of the aortic root in after surgery. The aortic dilation is believed to be caused by histological abnormalities of the aortic media and the hemodynamic characteristics of increased aortic flow, compared to pulmonary flow. Severe cyanosis, severe right ventricular outflow tract (RVOT) obstruction, older age at repair, a larger aortic size at the time of repair, and a history of an aortopulmonary shunt parameters related to long-standing volume overload of the aortic root were the reported risk factors.
View Article and Find Full Text PDFBiomolecules
December 2024
Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", 34137 Trieste, Italy.
Glycosylphosphatidylinositol (GPI) biosynthesis defect 11 (GPIBD11), part of the heterogeneous group of congenital disorders of glycosylation, is caused by biallelic pathogenic variants in . This rare disorder has previously been described in only 12 patients. We report four novel patients: two sib fetuses with congenital anomalies affecting several organs, including the heart; a living girl with tetralogy of Fallot, global developmental delay, behavioral abnormalities, and atypic electroencephalography (EEG) without epilepsy; a girl with early-onset, treatment-resistant seizures, developmental regression, and recurrent infections, that ultimately passed away prematurely due to pneumonia.
View Article and Find Full Text PDFAnn Noninvasive Electrocardiol
January 2025
Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Background: Electrocardiograms (EKGs) are routinely performed in pregnant patients with pre-existing cardiovascular disease. However, in pregnant patients with congenital heart disease (CHD), EKG changes during gestation have not been explored.
Methods: We performed a retrospective study of pregnant patients with CHD enrolled in the STORCC initiative.
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