ASSISTÊNCIA DE ENFERMAGEM A PORTADORES DE TETRALOGIA DE FALLOT

A tetralogia de Fallot (T4F) é uma patologia congênita considerada a mais comum das má- formações cardíacas cianóticas e se caracteriza por quatro defeitos principais: do septo interventricular, hipertrofia ventricular direita, estenose pulmonar e o posicionamento da aorta à direita. Nessas situações, o sangue de ambos os ventrículos é misturado e bombeado ao corpo, com consequente cianose sistêmica. Seu tratamento envolve diversas possibilidades de condutas, geralmente relacionadas às características clínicas e morfológicas da cardiopatia. Pode ocorrer desde um acompanhamento clínico-medi-camentoso até a reparação cirúrgica paliativa ou definitiva. O objetivo deste estudo foi identificar e analisar publicações científicas nacionais e internacionais sobre assistência de enfermagem aos pacientes portadores de T4F publicadas nos últimos cinco anos. Foi realizada uma revisão narrativa da leitura a partir de pesquisa eletrônica de artigos indexados nas bases de dados MEDLINE, Scopus, CINAHL, LILACS, BDENF, Web of Science, Embase, Cochrane e Google Acadêmico, por meio dos descritores “tetralogy of fallot” e “nursing care”. Foram incluídos estudos com acesso gratuito e na íntegra, nos idiomas português e inglês, e excluídos artigos de revisão, teses, dissertações, editoriais, debates e resenhas. Os resultados mostraram que a assistência de enferma-gem se baseou no processo de enfermagem, na implementação da sistematização da assistência de enfermagem, em práticas assistenciais contemporâneas baseadas na prática de enfermagem avançada, no uso das tecnologias da informação e comunicação (TICs) e na descrição de experiências específicas na assistência de enfermagem aos portadores de T4F em setores restritos.

Circular Network of Coregulated Sphingolipids Dictates Chronic Hypoxia Damage in Patients With Tetralogy of Fallot

Background: Tetralogy of Fallot (TOF) is the most common cyanotic heart disease. However, the association of cardiac metabolic reprogramming changes and underlying molecular mechanisms in TOF-related chronic myocardial hypoxia damage are still unclear.Methods: In this study, we combined microarray transcriptomics analysis with liquid chromatography tandem-mass spectrometry (LC–MS/MS) spectrum metabolomics analysis to establish the metabolic reprogramming that occurs in response to chronic hypoxia damage. Two Gene Expression Omnibus (GEO) datasets, GSE132176 and GSE141955, were downloaded to analyze the metabolic pathway in TOF. Then, a metabolomics analysis of the clinical samples (right atrial tissue and plasma) was performed. Additionally, an association analysis between differential metabolites and clinical phenotypes was performed. Next, four key genes related to sphingomyelin metabolism were screened and their expression was validated by real-time quantitative PCR (QT-PCR).Results: The gene set enrichment analysis (GSEA) showed that sphingolipid metabolism was downregulated in TOF and the metabolomics analysis showed that multiple sphingolipids were dysregulated. Additionally, genes related to sphingomyelin metabolism were identified. We found that four core genes, UDP-Glucose Ceramide Glucosyltransferase (UGCG), Sphingosine-1-Phosphate Phosphatase 2 (SGPP2), Fatty Acid 2-Hydroxylase (FA2H), and Sphingosine-1-Phosphate Phosphatase 1 (SGPP1), were downregulated in TOF.Conclusion: Sphingolipid metabolism was downregulated in TOF; however, the detailed mechanism needs further investigation.

A rare cause of Right ventricular outflow tract Pseudoaneurysm in Tetralogy of Fallot

Pseudoaneurysm of the right ventricular outflow tract(RVOT) is an uncommon yet catastrophic complication after intracardiac repair of Tetralogy of Fallot(TOF). We describe a patient diagnosed with RVOT pseudoaneurysm in the immediate postoperative period after complete repair for TOF with single pulmonary artery. The pseudoaneurysm was repaired successfully. This case is reported to emphasise the importance of a high degree of suspicion of this rare entity in these patients for its early diagnosis and management.

A RARE CASE OF DEXTROCARDIA AND SITUS INVERSUS WITH TETRALOGY OF FALLOT IN A 23-YEAR-OLD FEMALE: A CASE REPORT

Tetralogy of Fallot (TOF) is commonly identified cyanotic congenital cardiac anomaly; however, its affiliation with dextrocardia and situs inversus is very rare. We describe a case of dextrocardia and situs inversus associated with TOF in a 23-year old girl who arrived with central shunt at the age of 17 years and later for total surgical correction with magnificent outcomes.

Evaluation of ventricular function and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography

Aim: The left and right ventricular dysfunction are important clinical course indicators in patients with repaired tetralogy of Fallot. This study aimed to evaluate ventricular volumes, functions, and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography and compared with healthy children. It also aimed to investigate the relationships between ventricular volumes, functions, and myocardial deformation parameters in the patients. Materials and methods: In this cross-sectional study, 35 patients (mean age 15.1 ± 2.8 years, 54% male) and 35 healthy controls of similar age, gender, and body measurements underwent echocardiography. End-diastolic volume index, end-systolic volume index, and ejection fractions of both ventricles; global longitudinal, circumferential, radial strain, twist, and torsion of the left ventricle; the longitudinal strain of the right ventricle free wall and septum were measured. Results: Left ventricular ejection fraction, global circumferential and radial strain, twist and torsion were significantly lower in patients compared with controls. Left ventricular ejection fraction correlated with global circumferential (r = −0.446, p < 0.001) and radial strain (r = −0.433, p < 0.001) in the patients. Right ventricular volumes were significantly higher, and ejection fraction was significantly lower in patients compared with controls. All right ventricular parameters correlated with each other in the patients. Conclusion: Left ventricular contraction pattern was changed, circumferential and radial fibres were most affected in the patients. Right ventricular dilatation and dysfunction were detected, and right ventricular ejection fraction correlated well with strain measurements of the right ventricle.

Accelerated Growth, Differentiation, and Ploidy with Reduced Proliferation of Right Ventricular Cardiomyocytes in Children with Congenital Heart Defect Tetralogy of Fallot

The myocardium of children with tetralogy of Fallot (TF) undergoes hemodynamic overload and hypoxemia immediately after birth. Comparative analysis of changes in the ploidy and morphology of the right ventricular cardiomyocytes in children with TF in the first years of life demonstrated their significant increase compared with the control group. In children with TF, there was a predominantly diffuse distribution of Connexin43-containing gap junctions over the cardiomyocytes sarcolemma, which redistributed into the intercalated discs as cardiomyocytes differentiation increased. The number of Ki67-positive cardiomyocytes varied greatly and amounted to 7.0–1025.5/106 cardiomyocytes and also were decreased with increased myocytes differentiation. Ultrastructural signs of immaturity and proliferative activity of cardiomyocytes in children with TF were demonstrated. The proportion of interstitial tissue did not differ significantly from the control group. The myocardium of children with TF under six months of age was most sensitive to hypoxemia, it was manifested by a delay in the intercalated discs and myofibril assembly and the appearance of ultrastructural signs of dystrophic changes in the cardiomyocytes. Thus, the acceleration of ontogenetic growth and differentiation of the cardiomyocytes, but not the reactivation of their proliferation, was an adaptation of the immature myocardium of children with TF to hemodynamic overload and hypoxemia.

COMPARISON OF TRANS-RIGHT ATRIAL VERSUS COMBINED (TRANS-RIGHT ATRIAL, TRANS-RIGHT VENTRICULAR) APPROACH FOR INTRA-CARDIAC REPAIR OF TETRALOGY OF FALLOT: SINGLE-CENTER EXPERIENCE

Background and Objective: We compared trans-right atrial (t-RA) versus combined (trans-right-atrial and trans-ventricular (t-RA/RV) approaches for intra-cardiac repair of Tetralogy of Fallot (TOF) for the pre-operative and post-operative right ventricular (RV) function. The RV function was calculated using a tricuspid annular plane systolic excursion (TAPSE) using two-dimensional (2-D) echocardiography. Materials and Methods: This was a retrospective study. Fifty-three patients operated for the intra-cardiac repair of TOF between August 2019 and March 2021 were included in the study and divided into two groups based on the approach for repair as follows: t-RA or combined (t-RA/RV) approach. The first group (t-RA) had twenty-one patients, and the second group (combined t-RA/RV approach) had thirty-two patients. The assessment of pre-operative and post-operative RV function was done using TAPSE. Records of follow-up at 1 month and 3 months were evaluated. Results: Age, body surface area (BSA), preoperative saturation, cardiopulmonary bypass time, aortic cross?clamp time, postoperative intensive care unit (ICU) stay, and hospital stay were similar in both groups. However, t?RA/RV group had more pleural effusions (9 vs. 1 patients, P < 0.05), but had more improvements in Right Ventricular outflow tract (RVOT) gradients. There were no differences in arrhythmias in either group. Pre-operative TAPSE for both groups was similar (1.46 ± 0.27 vs. 1.61 ± 0.31, P > 0.05) and so was the post?operative TAPSE at discharge (1.54 ± 0.31 vs. 1.49 ± 0.33, P > 0.05), at 1 months (1.64 ± 0.25 vs. 1.48 ± 0.32, P > 0.05) and 3months (1.75 ± 0.19 vs. 1.7 ± 0.15, P > 0.05). Conclusion: Both approaches provide adequate palliation with effective improvements in RVOT gradients for patients with TOF. A limited right ventriculotomy does not adversely affect early RV function or increase the incidence of arrhythmias at the immediate post-operative period and early follow-up. More extensive studies with prospective randomized design and longer follow-ups are needed to address these issues further. Keywords: Tetralogy of Fallot, transatrial approach, intracardiac repair.