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1.
Kardiol Pol ; 81(10): 960-968, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37537911

RESUMO

BACKGROUND: 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. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points. RESULTS: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy). CONCLUSIONS: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Criança , Humanos , Adulto , Adolescente , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/diagnóstico , Polônia , Teste de Esforço , Eletrocardiografia , Estudos Retrospectivos
2.
J Clin Med ; 12(10)2023 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-37240571

RESUMO

BACKGROUND: Morbidity and mortality following Fontan (FO) surgery are primarily thromboembolic in nature. However, follow-up data regarding thromboembolic complications (TECs) in adult patients after FO procedure are inconsistent. In this multicenter study, we investigated the incidence of TECs in FO patients. METHODS: We studied 91 patients who underwent FO procedure. Clinical data, laboratory, and imaging investigations were collected prospectively during the scheduled medical appointments in 3 Adult Congenital Heart Disease Departments in Poland. TECs were recorded during a median follow-up of 31 months. RESULTS: Four patients (4.4%) were lost to follow-up. The mean age of patients was 25.3 (±6.0) years at enrollment, and the mean time between FO operation and investigation was 22.1 (±5.1) years. A total of 21 out of 91 patients (23.1%) had a history of 24 TECs since an FO procedure, mainly pulmonary embolism (PE; n = 12, 13.2%), including 4 (33.3%) silent PE. The mean time since FO operation to the first TEC was 17.8 (±5.1) years. During follow-up, we documented 9 TECs in 7 (8.0%) patients, mainly PE (n = 5, 5.5%). Most patients with TEC had a left type of systemic ventricle (57.1%). Three patients (42.9%) were treated with aspirin, 3 (3.4%) with Vitamin K antagonists or novel oral anticoagulants, and 1 patient had no antithrombotic treatment at the time of TEC occurrence. Supraventricular tachyarrhythmias were present in 3 patients (42.9%). CONCLUSIONS: This prospective study shows that TECs are common in FO patients, and a significant number of these events occur during adolescence and young adulthood. We also indicated how much TECs are underestimated in the growing adult FO population. The complexity of the problem requires more studies, especially to standardize the prevention of TECs in the whole FO population.

3.
Int J Cardiol ; 377: 45-50, 2023 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-36638916

RESUMO

BACKGROUND: Infective endocarditis (IE) remains major cause of morbidity and mortality in adult congenital heart disease (ACHD). Limited data exists on ACHD with IE in Central and South-Eastern European (CESEE) countries. The aim of this study is to characterize contemporary management and assess outcomes of ACHD with IE in CESEE region. METHODS: Data on ACHD patients with IE from 9 tertiary centres in 9 different CESEE countries between 2015 and 2020 was included. Baseline demographics, clinical presentation, indication for surgery, outcomes, hospital and all-cause-1-year mortality were studied. RESULTS: A total of 295 ACHD patients (mean age 40 ± 14 years) with IE were included. Median time from symptoms onset to establishing diagnosis was 25 (11-59) days. The majority of patients (203, 68.8%) received previous empiric oral antibiotic therapy. The highest incidence of IE was observed on native and left sided valves, 194(65.8%) and 204(69.2%), respectively. More than half had a vegetation size ≥10 mm (164, 55.6%); overall 138 (46.8%) had valve complications and 119 (40.3%) had heart failure. In-hospital mortality was 26 (8.8%). CONCLUSION: There is clear delay in establishing IE diagnosis amongst ACHD patients in CESEE countries. Adequate diagnosis is hampered by common prescription of empiric antibiotics before establishing formal diagnosis. Hence, patients commonly present with associated complications requiring surgery. Hospital treatment and survival are, nevertheless, comparable to other Western European countries. Improved awareness and education of patients and medical profession regarding IE preventive measures, risks, signs, and symptoms are urgently needed. Empiric antibiotic prescription before blood cultures are taken must be omitted.


Assuntos
Endocardite Bacteriana , Endocardite , Cardiopatias Congênitas , Próteses Valvulares Cardíacas , Adulto , Humanos , Pessoa de Meia-Idade , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Próteses Valvulares Cardíacas/efeitos adversos , Endocardite Bacteriana/diagnóstico , Endocardite Bacteriana/epidemiologia , Endocardite Bacteriana/terapia , Endocardite/diagnóstico , Endocardite/epidemiologia , Endocardite/terapia , Europa (Continente)/epidemiologia , Antibacterianos/uso terapêutico , Estudos Retrospectivos
4.
Eur J Cardiothorac Surg ; 63(1)2022 12 02.
Artigo em Inglês | MEDLINE | ID: mdl-36495203

RESUMO

OBJECTIVES: The Fontan procedure is the treatment of choice in congenital cardiac malformations defined as the single ventricle. Fontan patients are at high risk of thromboembolism, but the exact mechanism of this is poorly understood. The aim of this study was to evaluate an involvement of thrombin generations and microparticles (MPs) in prothrombotic state in adults with Fontan circulation. METHODS: This study included hospitalized patients after Fontan procedure and healthy volunteers. We assessed laboratory tests including thrombin generation by calibrated automated thrombography in three variants [platelet-poor plasma (impact of coagulation factors), platelet-rich plasma (PRP) (influence of platelets) and related with MPs]. The technique allows for a comprehensive evaluation of the coagulation system. RESULTS: The study groups consisted of 81 adult Fontan patients [41 females (50.6%); median age 22 interquartile range [20-27] years] and 54 control subjects. In patients with Fontan circulation, higher values of endogenous thrombin potential and peak values were observed for both platelet-poor plasma (+17% and +33%) and MPs (+29% and 41%) compared to controls (all P < 0.05). Moreover, in the Fontan group, we found a 64.9% shorter lag time and a 70.4% time to peak for MP variant (both P < 0.001). Contrarily, analysis in the PRP showed 17.1% of reduced endogenous thrombin potential in Fontan. Furthermore, there were no differences in thrombin synthesis in PRP in Fontan patients receiving aspirin or those with thrombocytopaenia (all P > 0.05). CONCLUSIONS: This study for the first time showed that thrombin generation associated with MPs may be an important contributor to the prothrombotic state in the Fontan population.


Assuntos
Técnica de Fontan , Tromboembolia , Feminino , Humanos , Adulto , Adulto Jovem , Trombina , Técnica de Fontan/efeitos adversos , Coagulação Sanguínea , Plaquetas , Testes de Coagulação Sanguínea
5.
Nucl Med Rev Cent East Eur ; 25(2): 142-147, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35929128

RESUMO

Amyloid transthyretin cardiomyopathy is a progressive disease that confers significant mortality. While it is relatively rare, the frequency of diagnoses has risen with the increased contribution of novel diagnostic approach over the last decade. Traditionally tissue biopsy was considered to be a gold standard for amyloidosis diagnosis. However, there are significant limitations in the wide application of this approach. A noninvasive imaging-based diagnostic algorithm has been substantially developed in recent years. Establishing radionuclide imaging standards may translate into a further enhancement of disease detection and improving prognosis in the group of patients. Therefore we present in the following document current evidence on the scintigraphic diagnosis of cardiac transthyretin amyloidosis. Moreover, we present standardized protocol for the acquisition and interpretation criteria in the scintigraphic evaluation of cardiac amyloidosis.


Assuntos
Neuropatias Amiloides Familiares , Medicina Nuclear , Neuropatias Amiloides Familiares/diagnóstico por imagem , Prova Pericial , Humanos , Polônia , Cintilografia
7.
J Clin Med ; 11(1)2022 Jan 04.
Artigo em Inglês | MEDLINE | ID: mdl-35011987

RESUMO

BACKGROUND: We previously demonstrated that enhanced oxidative stress and reduced nitric oxide bioavailability are associated with unfavorable outcomes early after coronary artery bypass grafting. It is not known whether these processes may impact long-term results. We sought to assess whether during long-term follow-up, markers of oxidative stress and nitric oxide bioavailability may predict cardiovascular mortality following bypass surgery. METHODS: We studied 152 consecutive patients (118 men, age 65.2 ± 8.3 years) who underwent elective, primary, isolated on-pump bypass surgery. We measured plasma 8-iso-prostaglandin F2α and asymmetric dimethylarginine before surgery and twice after surgery (18-36 h and 5-7 days). We assessed all-cause and cardiovascular death in relation to these two biomarkers during a mean follow-up time of 11.7 years. RESULTS: The overall mortality was 44.7% (4.7 per 100 patient-years) and cardiovascular mortality was 21.0% (2.2 per 100 patient-years). Baseline 8-iso-prostaglandin F2α was associated with cardiovascular mortality (HR 1 pg/mL 1.010, 95% CI 1.001-1.021, p = 0.036) with the optimal cut-off ≤ 364 pg/mL for higher survival rate (HR 0.460, 95% CI 0.224-0.942, p = 0.030). Asymmetric dimethylarginine > 1.01 µmol/L measured 18-36 h after surgery also predicted cardiovascular death (HR 2.467, 95% CI 1.140-5.340, p = 0.020). Additionally, elevated 8-iso-prostaglandin F2α measured at the same time point associated with all-cause mortality (HR 1 pg/mL 1.007, 95% CI 1.000-1.014, p = 0.048). CONCLUSIONS: Our findings indicate that in advanced coronary disease, increased oxidative stress, reflected by 8-iso-prostaglandin F2α before bypass surgery and enhanced asymmetric dimethylarginine accumulation just after the surgery are associated with cardiovascular death during long-term follow-up.

8.
J Clin Med ; 10(24)2021 Dec 20.
Artigo em Inglês | MEDLINE | ID: mdl-34945271

RESUMO

OBJECTIVES: thromboembolic complications are a major cause of morbidity and mortality following Fontan (FO) surgery. It is also well established that altered FO circulation results in systemic complications, including liver and endothelium damage. We sought to evaluate whether dysfunctions of these sources of hemostatic factors may result in changes of fibrin clot properties. METHODS: a permeation coefficient (Ks) and clot lysis time (CLT) were assessed in 66 FO patients, aged 23.0 years [IQR 19.3-27.0], and 59 controls, aged 24.0 years [IQR 19.0-29.0]. Ks was determined using a pressure-driven system. CLT value was measured according to assay described by Pieters et al. Endothelium and liver-derived hemostatic factors along with liver function parameters were evaluated. The median time between FO operation and investigation was 20.5 years [IQR 16.3-22.0]. RESULTS: FO patients had lower Ks (p = 0.005) and prolonged CLT (p < 0.001) compared to that of controls. Ks correlated with CLT (r = -0.28), FVIII (r = -0.30), FIX (r = -0.38), fibrinogen (r = -0.41), ALT (r = -0.25), AST (r = -0.26), GGTP (r = -0.27) and vWF antigen (r = -0.30), (all p < 0.05). CLT correlated with the time between FO operation and investigation (r = 0.29) and FIX (r = 0.25), (all p < 0.05). After adjustment for potential cofounders, TAFI antigen and GGTP were independent predictors of reduced Ks (OR 1.041 per 1% increase, 95% CI 1.009-1.081, p = 0.011 and OR 1.025 per 1 U/L increase, 95% CI 1.005-1.053, p = 0.033, respectively). Protein C and LDL cholesterol predicted prolonged CLT (OR 1.078 per 1% increase, 95% CI 1.027-1.153, p = 0.001 and OR 6.360 per 1 µmol/L increase, 95% CI 1.492-39.894, p = 0.011, respectively). Whereas elevated tPA was associated with lower risk of prolonged CLT (OR 0.550 per 1 ng/mL, 95% CI 0.314-0.854, p = 0.004). GGTP correlated positively with time between FO surgery and investigation (r = 0.25, p = 0.045) and patients with abnormal elevated GGTP activity (n = 28, 42.4%) had decreased Ks, compared to that of the others (5.9 × 10-9 cm2 vs. 6.8 × 10-9 cm2, p = 0.042). CONCLUSION: our study shows that cellular liver damage and endothelial injury were associated with prothrombotic clot phenotype reflected by Ks and CLT.

9.
J Clin Med ; 10(19)2021 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-34640508

RESUMO

Reduction of heart rate variability (HRV) parameters may be a risk factor and precede the occurrence of arrhythmias or the development of heart failure and complications in people with postinfarct left ventricular dysfunction and after coronary artery bypass grafting. Data on this issue in adults after a Fontan operation (FO) are scarce. This study assessed the association between HRV, exercise capacity, and multiorgan complications in adults after FO. Data were obtained from 30 FO patients (mean age 24 ± 5.4 years) and 30 healthy controls matched for age and sex. HRV was investigated in all patients by clinical examination, laboratory tests, echocardiography, a cardiopulmonary exercise test, and 24-h electrocardiogram. The HRV parameters were reduced in the FO group. Reduced HRV parameters were associated with patients' age at the time of FO, time since surgery, impaired exercise capacity, chronotropic incompetence parameters, and multiorgan complications. Univariate analysis showed that saturated O2 at rest, percentage difference between adjacent NN intervals of >50 ms duration, and peak heart rate were associated with chronotropic index. Multivariable analysis revealed that all three variables were independent predictors of the chronotropic index. The results of this study suggest novel pathophysiological mechanisms that link HRV, physical performance, and organ damage in patients after FO.

10.
J Cardiothorac Surg ; 16(1): 299, 2021 Oct 13.
Artigo em Inglês | MEDLINE | ID: mdl-34645497

RESUMO

BACKGROUND: Coronary steal phenomenon and myocardial ischemia is a complication following decompression of a hypertensive right ventricle in patients with left coronary-cameral fistulae. CASE PRESENTATION: We present a 12-year-old girl with a complex heart defect successfully operated on using a hybrid surgical-interventional approach to decompress the ventricle, embolize the fistula and reconstruct the atretic left coronary ostium. CONCLUSIONS: A novel hybrid strategy is the best solution for coronary-cameral fistulas reliant on high ventricular pressure at high risk for coronary steal phenomenon.


Assuntos
Doença da Artéria Coronariana , Anomalias dos Vasos Coronários , Cardiopatias Congênitas , Criança , Angiografia Coronária , Descompressão , Feminino , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos
13.
Kardiol Pol ; 78(4): 364-373, 2020 04 24.
Artigo em Inglês | MEDLINE | ID: mdl-32336071

RESUMO

Many cardiovascular diseases lead to heart failure, which is a progressive syndrome causing significant distress and limiting the quality of life, despite optimal cardiologic treatment. It is estimated that about 26 000 people in Poland suffer from advanced heart failure, and this number is growing. That is why palliative care (PC) dedicated to people living with end­stage cardiac diseases should be urgently implemented in Poland. Well­organized PC may not only relieve symptoms and improve quality of life in people living with cardiac diseases not responding to treatment but also support patients and their families during the dying process. Palliative care in patients with cardiac diseases should be continued during the end-of-life period. It should be implemented regardless of prognosis, and adjusted to patients' needs. Two approaches to PC are presented in this expert opinion. The first one (generic) is provided by all medical professionals incorporating PC principles into the usual patient care. The second approach, namely, specialized PC, is ensured by a multiprofessional team or at least a PC specialist who received appropriate training in PC. The model of needs-based (not prognosis-based) implementation of PC is discussed in this paper. Symptom control, support in decision-making, and sensitive, open communication are considered integral elements of PC interventions. Medical professionals developing PC in Poland should think about groups of patients with special needs like those with valvular heart disease, grown­up congenital heart disease, and pulmonary arterial hypertension, as well as elderly people. This consensus document presents main recommendations for future PC organization in Poland. Among others, we suggest changing the Polish National Health Fund reimbursement rules regarding PC and improving cardiologist education on PC.


Assuntos
Insuficiência Cardíaca , Cuidados Paliativos , Idoso , Consenso , Insuficiência Cardíaca/terapia , Humanos , Polônia , Qualidade de Vida
14.
Pol Arch Intern Med ; 130(2): 89-99, 2020 02 27.
Artigo em Inglês | MEDLINE | ID: mdl-31919335

RESUMO

INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is a heart disorder caused by autosomal dominant alterations affecting both sarcomeric genes and other nonsarcomeric loci in a minority of cases. However, in some patients, the occurrence of the causal pathogenic variant or variants in homozygosity, compound heterozygosity, or double heterozygosity has also been described. Most of the HCM pathogenic variants are missense and unique, but truncating mutations of the MYBPC3 gene have been reported as founder pathogenic variants in populations from Finland, France, Japan, Iceland, Italy, and the Netherlands. OBJECTIVES: This study aimed to assess the genetic background of HCM in a cohort of Polish patients. PATIENTS AND METHODS: Twenty­nine Polish patients were analyzed by a next generation sequencing panel including 404 cardiovascular genes. RESULTS: Pathogenic variants were found in 41% of the patients, with ultra­ rare MYBPC3 c.2541C>G (p.Tyr847Ter) mutation standing for a variant hotspot and correlating with a lower age at HCM diagnosis. Among the nonsarcomeric genes, the CSRP3 mutation was found in a single case carrying the novel c.364C>T (p.Arg122Ter) variant in homozygosity. With this finding, the total number of known HCM cases with human CSRP3 knockout cases has reached 3. CONCLUSIONS: This report expands the mutational spectrum and the inheritance pattern of HCM.


Assuntos
Cardiomiopatia Hipertrófica/genética , Proteínas de Transporte/genética , Proteínas com Domínio LIM/genética , Proteínas Musculares/genética , Mutação , Adolescente , Adulto , Idoso , Cardiomiopatia Hipertrófica/metabolismo , Criança , Pré-Escolar , Análise Mutacional de DNA , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Polônia , Adulto Jovem
15.
Tex Heart Inst J ; 46(1): 14-20, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30833832

RESUMO

The right ventricle provides systemic circulation in individuals with congenitally corrected transposition of the great arteries (CCTGA) and in those with complete transposition who have had an atrial switch repair (DTGA). The aim of this study was to evaluate how the systemic right ventricle adapts to increased workload and oxygen demand during exercise. From November 2005 through December 2015, 3,358 adult patients with congenital heart disease were treated at our institution; we identified 48 (26 females, 22 males; median age, 25.4 ± 8.1 yr) who met the study criteria; 37 had DTGA and atrial switch repair, and 11 had CCTGA. We studied their echocardiographic and cardiopulmonary exercise test results. A control group consisted of 29 healthy sex- and age-matched volunteers. On exercise testing, oxygen uptake at anaerobic threshold, peak oxygen uptake, peak heart rate, and percentage of maximal heart rate were significantly lower in the group with systemic right ventricle than in the control group (all P <0.001); in contrast, the peak ventilatory equivalent for carbon dioxide was higher in the study group (P=0.013). Impaired systemic right ventricular function reduced peak oxygen uptake. The peak heart rate was lower in the CCTGA group than in the DTGA group. Our results indicate that reduced exercise capacity is related to impaired systemic right ventricular function, severe tricuspid valve regurgitation, and chronotropic incompetence. There was no correlation between cardiopulmonary exercise test results and time after surgery. Chronotropic efficiency is lower in individuals with CCTGA than in those with DTGA.


Assuntos
Transposição das Grandes Artérias/métodos , Tolerância ao Exercício/fisiologia , Ventrículos do Coração/anormalidades , Transposição dos Grandes Vasos/fisiopatologia , Função Ventricular Direita/fisiologia , Adulto , Ecocardiografia , Teste de Esforço , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Imagem Cinética por Ressonância Magnética , Masculino , Prognóstico , Estudos Retrospectivos , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/cirurgia , Adulto Jovem
16.
Pol Arch Intern Med ; 129(3): 154-159, 2019 03 29.
Artigo em Inglês | MEDLINE | ID: mdl-30778019

RESUMO

INTRODUCTION Rare cardiovascular diseases and disorders (RCDDs) constitute an important clinical problem, and their proper classification is crucial for expanding knowledge in the field of RCDDs. OBJECTIVES The aim of this paper is to provide an updated classification of rare arrhythmogenic and conduction disorders, and rare arrhythmias (RACDRAs). METHODS We performed a search for RACDRAs using the Orphanet inventory of rare diseases, which includes diseases with a prevalence of no more than 5 per 10 000 in the general population. We supplemented this with a search of PubMed and Scopus databases according to a wider definition proposed by the European Parliament and the Council of the European Union. RESULTS RACDRAs are categorized into 2 groups, primary electrical disorders of the heart and arrhythmias in specific clinical settings. The first group is further divided into subgroups of major clinical presentation: disorders predisposing to supraventricular tachyarrhythmias, ventricular tachyarrhythmias, bradyarrhythmias, and others. The second group includes iatrogenic arrhythmias or heart rhythm disturbances related to medical treatment, arrhythmias associated with metabolic disorders, and others. We provide a classification of RACDRAs and supplement them with respective RCDDs codes. CONCLUSION The clinical classification of RACDRAs may form a basis to facilitate research and progress in clinical practice, both in diagnostic and therapeutic approaches.


Assuntos
Arritmias Cardíacas/classificação , Displasia Arritmogênica Ventricular Direita/classificação , Doença do Sistema de Condução Cardíaco/classificação , Doenças Raras , Arritmias Cardíacas/diagnóstico , Displasia Arritmogênica Ventricular Direita/diagnóstico , Doença do Sistema de Condução Cardíaco/diagnóstico , Progressão da Doença , Humanos , Índice de Gravidade de Doença
17.
Pol Arch Intern Med ; 129(3): 181-188, 2019 03 29.
Artigo em Inglês | MEDLINE | ID: mdl-30778020

RESUMO

INTRODUCTION A hemodynamic derangement in Fontan circulation causes liver pathology known as Fontan­­associated liver disease. Although liver biopsy is a standard for diagnosis of liver fibrosis, noninvasive methods are being developed, including shear wave elastography (SWE). OBJECTIVES We aimed to evaluate the degree of liver stiffness (LS) using SWE in patients with Fontan circulation in a long­­term follow­­up and to investigate a relationship between patient characteristics and LS. PATIENTS AND METHODS The study included 59 patients after the Fontan procedure. Clinical examinations and laboratory tests were performed. According to the stage of LS, patients were divided into 2 subgroups: group 1 (METAVIR stages F1 and F2) and group 2 (METAVIR stages F3 and F4). The ratio of aspartate transaminase to alanine transaminase, aspartate transaminase­­to­­platelet ratio index (APRI), fibrosis­­4 (FIB­­4) score, and Forns index were assessed. RESULTS The median LS was 9.1 kPa (interquartile range, 3.9-18.5 kPa). Five patients (9%) demonstrated LS in stage F1; 14 (26%), F2; 28 (52%), F3; and 7 (13%), F4. Group 2 had significantly higher aspartate transaminase and γ­­glutamyltranspeptidase levels, APRI, FIB­­4, and Forns index, and lower platelet count than group 1. A canonical correlation analysis indicated that LS and thrombocytopenia were related to time from the Fontan procedure, age at procedure, and single ventricular ejection fraction. CONCLUSIONS We showed that adult patients after the Fontan procedure develop liver dysfunction. Time from surgery, age at procedure, and single ventricular ejection fraction are related to the degree of LS assessed by SWE. Finally, SWE, APRI, Forns index, and FIB­4 score may help assess the degree of liver fibrosis.


Assuntos
Técnica de Fontan/efeitos adversos , Hepatopatias/diagnóstico por imagem , Hepatopatias/etiologia , Adulto , Alanina Transaminase/metabolismo , Aspartato Aminotransferases/metabolismo , Biomarcadores/metabolismo , Técnicas de Imagem por Elasticidade , Feminino , Humanos , Hepatopatias/metabolismo , Masculino , Pessoa de Meia-Idade
18.
Postepy Kardiol Interwencyjnej ; 15(4): 455-464, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31933662

RESUMO

INTRODUCTION: Despite successful repair of aortic coarctation, cardiovascular complications occur. AIM: To analyse type and frequency of late complications and their impact on exercise capacity in adults after aortic coarctation repair. MATERIAL AND METHODS: Fifty-eight adults after aortic coarctation repair, 36 male, median age 27.46 ±10.57, were compared to 30 healthy volunteers. Physical examination, transthoracic echocardiography, carotid intima-media thickness measurement, cardiopulmonary exercise test and 24-hour ambulatory blood pressure monitoring were performed. RESULTS: The main complications were: arterial hypertension 48.3%, myocardial hypertrophy in echocardiography 29.34%, recoarctation 25.86%, aortic dilation 13.79% and coronary artery disease 6.89%. Exercise tolerance was reduced in the cardiopulmonary exercise test. The VO2/kg peak was lower, 29.01 ±8.79 vs. 49.16 ±7.38 ml/kg/min, p < 0.001, VE/VCO2 peak higher 28.18 ±4.69 vs. 26.78 ±3.13, p = 0.017. The peak heart rate was reduced, 157.28 ±22.22 vs. 177.93 ±23.08 bpm, p < 0.001, peak systolic blood pressure was higher, 174.79 ±17.62 vs. 153.33 ±4.79 mm Hg, p < 0.001. Systolic blood pressure in 24-hour ambulatory monitoring correlated with left ventricle mass index, r = 0.29, p = 0.025, wall thickness, r = 0.31, p = 0.039. Age at operation was related to left ventricle wall thickness, r = 0.27, p = 0.041, and carotid intima-media thickness, r = 0.26, p = 0.046. There was no association of any cardio-pulmonary parameters with time from surgery, type of operation or echocardiography results. CONCLUSIONS: Adults after aortic coarctation repair suffer from arterial hypertension, recurrent aortic stenosis, aortic aneurysms, and coronary artery disease. Reduced exercise capacity in cardio-pulmonary exercise test is related to hypertensive reaction and chronotropic incompetence.

19.
Kardiochir Torakochirurgia Pol ; 15(2): 107-113, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30069191

RESUMO

INTRODUCTION: Observations of patients after repair of tetralogy of Fallot (ToF) indicate good correction results and long-term survival. Few papers have been published in which the quality of life (QoL) of this population has been assessed. AIM: To evaluate QoL in adults with repaired ToF.. MATERIAL AND METHODS: We included 39 patients with repaired ToF and 40 age- and sex-matched healthy volunteers. Information recorded included echocardiography, cardiac magnetic resonance, cardiopulmonary exercise test, and self-reported health-related QoL questionnaire (SF-36).. RESULTS: The perceived physical and mental domains of health were signi cantly poorer in ToF patients than in controls. A positive correlation between VO2 peak and physical domains was observed: (VO2 peak vs. physical domains (r = 0.6, p ≤ 0.001), general health (r = 0.36, p = 0.03), and physical complex status (r = 0.51, p = 0.001). VO2 peak % correlated with physical functioning (r = 0.43, p = 0.007), general health (r = 0.39, p = 0.015) and physical complex status (r = 0.49, p = 0.002). Right ventricle ejection fraction, determined with cardiac magnetic resonance, positively correlated with role physical (r = 0.38, p = 0.04). In echocardiography, pressure half time was posi- tively correlated with physical functioning (r = 0.48, p = 0.004) and role physical (r = 0.4, p = 0.02).. CONCLUSIONS: The QoL in adults after repair of ToF and healthy control subjects was compared directly. The self-perceived physical and mental domains of health were significantly poorer in ToF patients than in controls. Strong associations were found between objective exercise capacity and physical aspects of quality of life. Complex assessment and quality of life instruments should be used together to obtain an accurate view of health status of patients with repaired ToF.

20.
Pol Merkur Lekarski ; 44(263): 219-222, 2018 May 25.
Artigo em Polonês | MEDLINE | ID: mdl-29813038

RESUMO

The coarctation of the aorta (CoA) is a congenital condition of the thoracic aorta. AIM: The aim of the study was assessment of atherosclerosis risk factors in adult patients after surgical treatment of aortic coarctation. MATERIALS AND METHODS: 58 patients (36 male, 22 female) at median age of 27.46 ±10.57 were compared with 30 healthy, age and sex matched volunteers. The arterial blood pressure, lipid profile, fasting glucose, inflammation markers and the lifestyle factors were analyzed. RESULTS: CoA patients have higher systolic blood pressure 136.55±16.27 vs 123.47±10.34 mmHg, p<0.001, fasting glucose 4.95±0.5 vs 4.65±0.46 mmol/l p=0.002, hsCRP 1.03±0.12 vs 0.89±0.14 mg/l p=0.025 and fibrinogen 2.55±0.34 vs 1.98±0.28 g/l p<0.001. Hyperlipidemia is more common 44.8% vs 23.3% p=0.048, treated with statin. When comparing hypertensive patients (N=28) with normotensive ones (N=30), the patients with arterial hypertension are older 33.5±12.23 vs 25.73±7.12 p=0.004, have higher body weight 78.03±14.58 vs 68.7±14.29 p=0.017, in this group more common are: hypoplastic aortic arch 28.6% vs 6.7% p=0.027, recoarctation 39.3% vs 13.3% p=0.024, cardiovascular disease 14.3% vs 0% p=0.032 and family history 21.4% vs 3.3% p=0.034. CONCLUSIONS: The coarctation of aorta is related to higher cardiovascular risk due to arterial hypertension, hyperlipidemia, higher glucose and inflammation markers levels when comparing with healthy population.


Assuntos
Coartação Aórtica/cirurgia , Doenças Cardiovasculares/epidemiologia , Adolescente , Adulto , Aterosclerose , Glicemia , Doenças Cardiovasculares/etiologia , Feminino , Humanos , Hiperlipidemias , Hipertensão , Inflamação , Masculino , Fatores de Risco , Procedimentos Cirúrgicos Vasculares , Adulto Jovem
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