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1.
Clin Endocrinol (Oxf) ; 100(6): 515-526, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38572909

RESUMO

OBJECTIVE: To investigate the metabolic, cardiovascular, and neuropsychological phenotype, quality of life (QoL), and hormonal regulation in individuals with congenital adrenal hyperplasia (CAH), a group of autosomal recessive disorders characterized by impaired synthesis of cortisol in the adrenal cortex and, if untreated compensatory hyperandrogenism. CAH is associated with an increased cardiovascular and metabolic morbidity, possibly due to overtreatment with glucocorticoids, leading to weight gain, insulin resistance, and metabolic syndrome. DESIGN, PARTICIPANTS, MEASUREMENTS: Thirty-seven individuals with CAH and 33 age- and sex-matched controls were evaluated at a single centre at Aarhus University Hospital with echocardiography, electrocardiogram, 24-h blood pressure, biochemistry, anthropometrics, and autism spectrum, anxiety, depression, personality, cognitive failures, and QoL were assessed using questionnaires. RESULTS: CAH individuals had lower height than controls (170.5 vs. 182.9 cm in males and 160.2 vs. 170.1 cm in females, p < 0.01). Compared with female controls, females with CAH had higher haemoglobin (8.8 vs. 8.2 mmol/L, p = 0.003) and BMI (29.7 vs. 25.5 kg/m2, p = 0.006), reduced insulin sensitivity (HOMA-IR): 2.7 vs. 1.9, p = 0.018), prolonged E-wave deceleration time (193 vs. 174 cm, p = 0.015), and E/é ratios (5.4 vs. 4.5, p = 0.017), and lower self-reported QoL. Males with CAH had more cognitive complaints (p = 0.034) and higher autistic scores (19.9 vs. 14.9; p = 0.068) compared with male controls. More individuals with CAH than controls reported writing problems. CONCLUSION: A sex-specific comorbidity profile is evident in CAH, with females presenting with decreased metabolic and overall self-reported health, whereas males with CAH presented with increased cognitive complaints and autistic traits.


Assuntos
Hiperplasia Suprarrenal Congênita , Qualidade de Vida , Humanos , Hiperplasia Suprarrenal Congênita/psicologia , Hiperplasia Suprarrenal Congênita/fisiopatologia , Feminino , Masculino , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Estudos de Casos e Controles
2.
Lancet Reg Health Eur ; 36: 100789, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38188272

RESUMO

Background: Guidelines recommend preoperative dental screening (PDS) prior to cardiac valve surgery, to reduce the incidence of prosthetic valve infective endocarditis (IE). However, limited data support these recommendations, particular in patients undergoing transcatheter aortic valve implantation (TAVI). We aimed to investigate the effect of mandatory PDS on risk of IE in patients undergoing TAVI. Methods: In this observational study, a total of 1133 patients undergoing TAVI in Western-Denmark from 2020 to 2022 were included. Patients were categorized based on two implemented PDS practices: mandatory PDS (MPDS group), and no referral for PDS (NPDS group). Outcome data were retrieved from Danish registries and confirmed using medical records. The primary outcome was incidence of IE. Secondary outcomes were all-cause mortality and composite outcome of all-cause mortality and IE. Findings: Of 568 patients in the MPDS group 126 (22.2%) underwent subsequent oral dental surgery, compared to 8 (1.4%) among 565 patients in the NPDS group. During a median follow-up of 1.9 years (interquartile range 1.4-2.5 years), 31 (2.7%) developed IE. The yearly incidence IE rate was 1.4% (0.8-2.3) and 1.5% (0.8-2.4) in MPDS and NPDS, respectively, p = 0.86. All-cause mortality rates were similar between groups (estimated 2-year overall mortality of 6.7% (4.8-9.2) vs. 4.7% (3.2-6.9), MPDS and NPDS, respectively, p = 0.15). Consistent findings were found in 712 propensity score-matched patients. Interpretation: Mandatory PDS did not demonstrate reduced risk of IE or all-cause mortality compared to targeted PDS in patients undergoing TAVI. Funding: The funder had no role in the study design, data management, or writing.

3.
Lancet Haematol ; 10(11): e922-e935, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37804848

RESUMO

Pulmonary embolism is one of the leading causes of death due to cardiovascular disease. Timely diagnosis is crucial, but challenging, as the clinical presentation of pulmonary embolism is unspecific and easily mistaken for other common medical emergencies. Clinical prediction rules and D-dimer measurement allow stratification of patients into groups of expected prevalence and are key elements in adequate selection of patients for diagnostic imaging; however, the strengths and weaknesses of the multiple proposed prediction rules, when to measure D-dimer, and which cutoff to apply might be elusive to a significant proportion of physicians. 13 international guidelines authored by medical societies or expert author groups provide recommendations on facets of the diagnostic investigations in suspected pulmonary embolism, some of which are hallmarked by pronounced heterogeneity. This Review summarises key recommendations of each guideline, considers the most recent evidence on the topic, compares guideline recommendations on each facet of the diagnosis of pulmonary embolism, and provides a synthesis on the most common recommendations.


Assuntos
Embolia Pulmonar , Humanos , Embolia Pulmonar/diagnóstico , Produtos de Degradação da Fibrina e do Fibrinogênio , Sociedades Médicas
4.
J Endocr Soc ; 7(11): bvad124, 2023 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-37822574

RESUMO

Context: Turner syndrome (TS) is a rare genetic syndrome with an increased mortality, mainly attributed to cardiovascular disease. Objective: This work aimed to investigate and correlate the lipid profile in adult women with TS to clinical characteristics. Methods: A 12-year prospective cohort study, including 4 study visits, was conducted at a specialist hospital. A total of 102 women with TS qualified for inclusion. Excluding missing variables and participants lost to follow-up, 86 women (mean age 38.1 years; range, 18.4-62.1 years) were included in this study. Fifty-three women completed the study. Repeated-measurement analysis was performed, using total cholesterol (Total-C), low-density lipoprotein (LDL), triglycerides (TGs), and high-density lipoprotein (HDL) as outcome variables and age, karyotype, body mass index (BMI), treatment with statins, antidiabetics, and hormone replacement therapy as explanatory variables. Principal component analysis (PCA) and partial least squares (PLS) analysis were performed at the first study visit. Results: Hyperlipidemia was present in 30% of the TS cohort. Total-C increased with age (0.12 mmol/L/y; P = .016). LDL (P = .08), TGs (P = .14), and HDL (P = .24) were not associated with age. BMI significantly increased total-C (0.19 mmol/L/kg/m2; P = .006), LDL (0.63 mmol/L/kg/m2; P < .001), and TGs (0.80 mmol/L/kg/m2; P < .001) and decreased HDL (-0.59 mmol/L/kg/m2; P < .001). PCA and PLS analysis found correlations between weight and BMI and total-C, LDL, and TGs. Conclusion: Hyperlipidemia is more prevalent in adult women with TS across adulthood compared to the background population. Total-C, LDL, TGs, and HDL were significantly associated with BMI characterizing the atherogenic profile in adult women with TS.

5.
Sci Rep ; 13(1): 12089, 2023 07 26.
Artigo em Inglês | MEDLINE | ID: mdl-37495650

RESUMO

The genetic architecture of the QT interval, defined as the period from onset of depolarisation to completion of repolarisation of the ventricular myocardium, is incompletely understood. Only a minor part of the QT interval variation in the general population has been linked to autosomal variant loci. Altered X chromosome dosage in humans, as seen in sex chromosome aneuploidies such as Turner syndrome (TS) and Klinefelter syndrome (KS), is associated with altered QTc interval (heart rate corrected QT), indicating that genes, located in the pseudoautosomal region 1 of the X and Y chromosomes may contribute to QT interval variation. We investigate the dosage effect of the pseudoautosomal gene SLC25A6, encoding the membrane ADP/ATP translocase 3 in the inner mitochondrial membrane, on QTc interval duration. To this end we used human participants and in vivo zebrafish models. Analyses in humans, based on 44 patients with KS, 44 patients with TS, 59 male and 22 females, revealed a significant negative correlation between SLC25A6 expression level and QTc interval duration. Similarly, downregulation of slc25a6 in zebrafish increased QTc interval duration with pharmacological inhibition of KATP channels restoring the systolic duration, whereas overexpression of SLC25A6 shortened QTc, which was normalized by pharmacological activation of KATP channels. Our study demonstrate an inverse relationship between SLC25A6 dosage and QTc interval indicating that SLC25A6 contributes to QT interval variation.


Assuntos
Síndrome de Klinefelter , Síndrome do QT Longo , Síndrome de Turner , Animais , Feminino , Humanos , Masculino , Trifosfato de Adenosina , Eletrocardiografia , Síndrome do QT Longo/genética , Cromossomo X , Peixe-Zebra/genética , Translocador 3 do Nucleotídeo Adenina
6.
Clin Endocrinol (Oxf) ; 99(6): 545-551, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-36890688

RESUMO

OBJECTIVE: Cardiovascular complications and congenital malformations are known traits in Turner syndrome (TS), which increases mortality. Women with TS have varying phenotype and cardiovascular risks. A biomarker assessing the risk for cardiovascular complications could potentially reduce mortality in high-risk TS and reduce screening in TS participants with low cardiovascular risk. DESIGN, PATIENTS, PARTICIPANTS AND MEASUREMENTS: As part of a study initiated in 2002, 87 TS participants and 64 controls were invited to magnetic resonance imaging of the aorta, anthropometry, and biochemical markers. TS participants were re-examined thrice lastly in 2016. The focus of this paper is the additional measurements of transforming growth factor beta (TGFß), matrix metalloproteinase (MMP's), tissue inhibitor of matrix metalloproteinase (TIMP), peripheral blood DNA and their associations with TS and the cardiovascular risk and congenital heart disease. RESULTS: TS participants had lower TGFß1 and TGFß2 values compared to controls. snp11547635 heterozygosity was not associated with any biomarkers but was associated with increased risk of aortic regurgitation. TIMP4 and TGFß1 were correlated with the aortic diameter at several measuring positions. During follow-up, the antihypertensive treatment decreased the descending aortic diameter and increased TGFß1 and TGFß2 levels in TS. CONCLUSION: TGFß and TIMP's are altered in TS and may play a role in the development of coarctation and dilated aorta. snp11547635 heterozygosity was not found to impact biochemical markers. Further studies should investigate these biomarkers to further unravel the pathogenesis of the increased cardiovascular risk in TS participants.


Assuntos
Síndrome de Turner , Humanos , Feminino , Síndrome de Turner/complicações , Síndrome de Turner/genética , Fator de Crescimento Transformador beta/genética , Aorta , Genótipo , Biomarcadores , Metaloproteinases da Matriz/genética , Inibidor Tecidual de Metaloproteinase-1/genética
8.
Eur Heart J Imaging Methods Pract ; 1(2): qyad045, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39045065

RESUMO

Aims: Currently, electrical rather than mechanical parameters of delayed left ventricular (LV) activation are used for patient selection for cardiac resynchronization therapy (CRT). However, despite adhering to current guideline-based criteria, about one-third of heart failure (HF) patients fail to derive benefit from CRT. This study sought to investigate the prognostic survival significance of a recently introduced index of contractile asymmetry (ICA) based on the deformation of entire opposing LV walls in the context of selecting patients with HF and left bundle branch abnormality (LBBB) for CRT. Methods and results: We analysed 367 patients with HF and LBBB undergoing CRT (31.6% females, 69 ± 9 years, ischaemic aetiology in 50.7%, LV ejection fraction 27 ± 6%). ICA was calculated using LV strain rate values from curved anatomical M-mode plots of apical 2D echocardiography images. The predictive value of ICA was assessed using Kaplan-Meier analysis and Cox proportional hazards models. During a median follow-up time of 5.54 years, death or cardiac transplantation occurred in 105 (28.6%) cases. Higher baseline ICA values in all apical views, particularly in the two-chamber view (ICA-2ch), were associated with increased event-free survival, and the unadjusted hazard ratio was 0.28 (95% confidence interval 0.18-0.46). Higher ICA-2ch (>0.319 s-1) consistently predicted survival across clinical subgroups and remained significant after covariate adjustment, while the event rate sharply increased in low ICA-2ch cases. Additionally, including ICA-2ch improved the predictive value of the multivariate risk model containing the typical LBBB pattern. Conclusion: Pre-implant ICA suggests a quantitative prognostic threshold for both long-term survival and adverse outcomes following CRT implantation.

9.
Open Heart ; 9(2)2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36270714

RESUMO

In patients with atrial fibrillation and previous episodes of bleeding on oral anticoagulant treatment, left atrial appendage occlusion (LAAO) has emerged as an alternative way to decrease the risk of stroke.The use of the procedure has been on the rise, and the news coverage has been dominated by an uncritical acceptance of the benefit of this procedure, which probably have contributed to the increasing number of procedures.This commentary is a presentation and critical appraisal of the available evidence on the efficacy and safety of left atrial appendage closure as stroke prophylaxis.We illustrate that LAAO is supported by limited randomised data risk of serious complications, which we do not believe supports the current widespread use.


Assuntos
Apêndice Atrial , Fibrilação Atrial , Procedimentos Cirúrgicos Cardíacos , Acidente Vascular Cerebral , Humanos , Anticoagulantes/efeitos adversos , Apêndice Atrial/diagnóstico por imagem , Apêndice Atrial/cirurgia , Fibrilação Atrial/complicações , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/terapia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/prevenção & controle , Ensaios Clínicos Controlados Aleatórios como Assunto
10.
Ugeskr Laeger ; 184(28)2022 07 11.
Artigo em Dinamarquês | MEDLINE | ID: mdl-35959815

RESUMO

Dyspnoea and chest pain are common causes of referral to emergency departments. Both symptoms are non-specific and may be caused by ST elevation myocardial infarction, aortic dissection, pulmonary embolism, or infection. Fibrin D-dimer is often used for ruling out suspicion of acute diseases, but the clinical utility is limited by a modest specificity which lowers with age to approximately ten per cent at the age of 80 years. This review summarises the recommended approaches and highlights potential pitfalls when utilizing and interpreting fibrin D-dimer in the diagnostic work-up of commonly suspected medical conditions.


Assuntos
Dissecção Aórtica , Embolia Pulmonar , Idoso de 80 Anos ou mais , Dissecção Aórtica/diagnóstico , Dor no Peito/diagnóstico , Dor no Peito/etiologia , Produtos de Degradação da Fibrina e do Fibrinogênio , Humanos , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico
11.
Endocrine ; 78(3): 418-428, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36018537

RESUMO

PURPOSE: This study aims to review all published cases on the association between thyrotoxicosis and Takutsubo Syndrome by describing clinical characteristics, diagnostic work-up, treatment, and outcome. METHODS: We searched PubMed and Embase databases from inception to the 17th of February 2022 for case reports or series reporting the above-mentioned association. We extracted data on demographic characteristics, clinical features, diagnostic work-up, treatment, and clinical outcomes. Cases were stratified into groups based on the presumed cause of the thyrotoxicosis (iatrogenic vs non-iatrogenic and Graves' diseases vs non-Graves' disease, respectively). RESULTS: We identified 25 cases from 24 articles. The mean age was 61.7 years (+/- SD 14.5). Most patients were women (88%). Graves' disease (52%) was the leading cause of thyrotoxicosis. Previous cancer was significantly more common in patients with iatrogenic thyrotoxicosis (P = 0.03). The most common symptoms were respiratory symptoms (68%), chest pain (56%), and palpitations (40%). The most common ECG characteristics were T-wave abnormalities (48%) and ST-elevations (36%). Elevated troponin levels were found in 92% of the cases. Patients with Graves's disease and Takutsubo Syndrome had higher plasma levels of serum thyroxine (P = 0.03) and were more often treated with beta-blockers (P = 0.01) compared to patients with thyrotoxicosis of other origins. Notably, 40% of cases experienced in-hospital complications. No deaths were reported. All patients had improved cardiac function within a median follow-up of 42 days. CONCLUSION: Evidence-based on current case reports suggests an increased risk of Takutsubo Syndrome and subsequently increased risk of in-hospital complications in patients with thyrotoxicosis.


Assuntos
Doença de Graves , Cardiomiopatia de Takotsubo , Tireotoxicose , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Cardiomiopatia de Takotsubo/etiologia , Tireotoxicose/complicações , Tireotoxicose/epidemiologia , Doença de Graves/diagnóstico
12.
Am J Cardiol ; 176: 1-7, 2022 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-35606174

RESUMO

The long-term cardiovascular risk for patients examined with coronary computed tomography angiography (CCTA) to rule out coronary heart disease compared with population controls remains unexplored. A nationwide register-based study including first-time CCTA-examined patients between 2007 and 2017 in Denmark alive 180 days post-CCTA was conducted. We evaluated 5-year outcomes of myocardial infarction (MI) or revascularization and all-cause mortality in 3 distinct CCTA-groups: (1) no post-CCTA preventive pharmacotherapy use (cholesterol-lowering drugs, antiplatelets, or anticoagulants); (2) post-CCTA preventive pharmacotherapy use; and (3) revascularization or MI within 180 days post-CCTA. For each patient group, population controls were matched on age, gender, and calendar year. Absolute risks standardized to the age, gender, selected co-morbidity, and anti-anginal pharmacotherapy distributions of the specific CCTA-examined patients and respective controls were obtained from multivariable Cox regression. Of 110,599 CCTA-examined patients, (1) 48,231 patients were not prescribed preventive pharmacotherapy 180 days post-CCTA; (2) 42,798 patients were prescribed preventive pharmacotherapy within 180 days post-CCTA; and (3) 19,570 patients were diagnosed with MI or revascularized within 180 days post-CCTA. For patient groups 1 to 3 versus respective controls, 5-year MI or revascularization risks were <0.1% versus 2.0%, <0.1% versus 3.8%, and 19.0% versus 2.5%, all p<0.001. Five-year all-cause mortality were 2.8% versus 4.2%, 5.5% versus 8.8%, and 6.7% versus 8.5%, all p <0.001. In conclusion, the 5-year MI or revascularization risk can be considered very low for CCTA-examined patients without ischemic events within 180 days post-CCTA. Conversely, CCTA-examined patients with MI or revascularization events within 180 days post-CCTA have significantly elevated 5-year MI or revascularization risk.


Assuntos
Doença da Artéria Coronariana , Infarto do Miocárdio , Angiografia por Tomografia Computadorizada , Angiografia Coronária/métodos , Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/epidemiologia , Dinamarca/epidemiologia , Seguimentos , Humanos , Infarto do Miocárdio/epidemiologia , Revascularização Miocárdica
14.
Open Heart ; 8(2)2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34675133

RESUMO

OBJECTIVE: Incidence and severity of acute myocarditis vary significantly in previous reports and there is a lack of epidemiological studies on the short-term risks of mortality, heart failure and ventricular arrhythmias in patients with acute myocarditis. Therefore, study aims were to examine 90-day risks of mortality, heart failure (HF) and ventricular arrhythmias in patients with acute myocarditis in comparison to age-matched and sex-matched background population controls. METHODS: In this nationwide register-based follow-up study of patients hospitalised with myocarditis between 2002 and 2018 in Denmark, 90-day risks of all-cause mortality, HF, ventricular arrhythmias (ventricular tachycardia, ventricular fibrillation (VF)), cardiac arrest and implantable cardioverter-defibrillator (ICD) implantation were compared with age-matched and sex-matched controls from the background population (1:5 matching). Absolute risks standardised to the age, sex and comorbidity distribution of the entire study population were derived from multivariable Cox regression. RESULTS: A total of 2523 patients hospitalised with myocarditis were included. Median age was 48 years (Q1-Q3: 30-69) and 67.7% were men. Comorbidity burden was more pronounced among patients with myocarditis relative to controls. Standardised 90-day all-cause mortality risk was 4.9% for patients with acute myocarditis versus 0.3% for controls (p<0.001). Ninety-day standardised risks for other endpoints were 7.5% versus 0.1% for HF, 1.9% versus <0.1% for VF/VF/arrest risk and 1.6% versus <0.1% for ICD implantation (all p<0.001). CONCLUSIONS: In this large nationwide register-based follow-up study, patients hospitalised with myocarditis had significantly higher 90-day risks of all-cause mortality, HF, ventricular arrhythmias, cardiac arrest and ICD implantation compared with background population controls.


Assuntos
Miocardite/mortalidade , Vigilância da População , Sistema de Registros , Taquicardia Ventricular/etiologia , Doença Aguda , Dinamarca/epidemiologia , Seguimentos , Incidência , Miocardite/complicações , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Taquicardia Ventricular/mortalidade , Fatores de Tempo
15.
J Bone Miner Res ; 36(5): 901-909, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33567127

RESUMO

Marfan syndrome (MFS), a rare genetic disease, has a prevalence of 6.5 in 100,000. Studies show that patients with MFS have reduced areal bone mineral density (BMD) compared with non-MFS individuals. We have previously shown that patients with MFS have reduced volumetric BMD and compromised trabecular and cortical bone microarchitecture. The present study was a registry-based, nationwide, population-based, cohort study using register data, aimed to evaluate fracture risk and fracture rates in MFS. We included 406 (196 women) patients with MFS through the Danish National Patient Register and 40,724 (19,327 women) persons, randomly selected and matched from the Civil Registry System. A total of 21.9% of the MFS and 18.9% of the reference population had experienced at least one fracture from 1995 to 2018. The fracture incidence rate was 27.5 per 1000 person-years in the MFS cohort (highest in young men and old women with MFS), and 20.3 per 1000 person-years in the reference population. The overall incidence rate ratio between the MFS and the reference population was 1.35 (95% confidence interval [CI ] 1.18-1.55) for all fractures. When evaluating the risk of being registered with an osteoporosis diagnosis in the Danish National Patient Register, starting relevant treatment for osteoporosis or experiencing a hip or spine fracture, 10.3% of the MFS cohort and 3.3% of the reference population could be classified as being osteoporotic. The between-group subhazard ratio was 3.97 (95% CI 2.56-6.25). Patients with MFS started treatment with an antiosteoporotic drug at a younger age than the reference population (57 [interquartile range 55-67] versus 71 [63-73]) years. The life expectancy in MFS is increasing, resulting in more patients facing diseases that are related to old age, such as age-related bone loss and increased risk of fractures. Our data suggest that bone health and fracture prevention needs to be part of the standard care for patients with MFS. © 2021 American Society for Bone and Mineral Research (ASBMR).


Assuntos
Fraturas Ósseas , Síndrome de Marfan , Osteoporose , Idoso , Densidade Óssea , Estudos de Coortes , Feminino , Fraturas Ósseas/epidemiologia , Humanos , Masculino , Síndrome de Marfan/complicações , Síndrome de Marfan/epidemiologia , Pessoa de Meia-Idade
16.
Eur J Pediatr ; 180(3): 717-723, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32772155

RESUMO

Kawasaki disease has well-described cardiovascular complications. However, the association to autoimmunity and cancer in the long term is not well described. We investigated theses associations using a registry-based matched cohort follow-up study of patients diagnosed with Kawasaki disease. Patients with Kawasaki disease were included and matched 1:5 to a population control group, matched by birth year, sex and incident month of the Kawasaki disease diagnosis. A total of 820 cases < 21 years of age were identified. Median age at diagnosis was 3 years. Median follow-up time was 12 years. Patients with KD were at higher risk of being diagnosed with ischaemic heart disease at 10 years (HR 39.94 (95% CI 5.00-319.28)) and 30 years (HR 8.33 (95% CI 3.03-22.91)). The 10-, 20- and 30-year risks of developing autoimmune disorders were HR 4.23 (95% CI 3.01-5.94), HR 3.23 (95% CI 2.44-4.29) and 2.83 (95% CI, 2.17-3.68), all p < 0.001. Cancer risk was increased after 30 years (HR 2.42 (95% CI, 1.09-5.34)). All-cause mortality after 35 years was also significantly increased (HR 3.14 (95% CI, 1.03-9.60)). Children with KD have increased long-term risks of ischaemic heart disease also of autoimmune disease and cancer, as well as an increased all-cause mortality. The surprisingly increased risk of autoimmunity must be investigated further. What is known: • Kawasaki disease is characterized by acute vasculitis and inflammation that can affect the coronary arteries. • Anti-inflammatory medicine is effective in the acute stages of the disease. What is new: • Children with Kawasaki disease have an increased risk of developing autoimmune disease in the long term. • Kawasaki disease is associated with a slightly increased mortality rate driven by non-cardiovascular causes.


Assuntos
Doenças Autoimunes , Síndrome de Linfonodos Mucocutâneos , Neoplasias , Doenças Autoimunes/complicações , Doenças Autoimunes/epidemiologia , Criança , Estudos de Coortes , Seguimentos , Humanos , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Neoplasias/epidemiologia , Neoplasias/etiologia
17.
Acta Cardiol ; 76(7): 760-768, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32594904

RESUMO

OBJECTIVE: The objective of the current study is to determine the characteristics of myocardial infarction with non-obstructive coronary arteries (MINOCA) patients with and without cardiac magnetic resonance (CMR) abnormalities. METHODS: We evaluated patients admitted with a presentation of acute myocardial infarction (MI) with no coronary obstruction on invasive angiography in our institution between 2012 and 2017. Patients with prior cardiac disease, myocarditis, Takotsubo cardiomyopathy and type 2 myocardial infarction were excluded. Myocardial fibrosis was determined by late gadolinium enhancement (LGE). Patients were divided into two groups based on the presence or absence of CMR abnormalities (LGE or oedema). Major adverse cardiovascular events (MACE) were defined as non-fatal MI, all-cause mortality, ventricular arrythmias or heart failure hospitalisation at follow-up. RESULTS: Thirty-four patients fulfilling the inclusion criteria were identified. Myocardial changes with CMR were observed in 20 (59%) patients with signs of subendocardial infarct by LGE in 13 (38%) patients, transmural infarct by LGE in 6 (18%) patients and one patient had myocardial oedema. ECG and echocardiographic features were similar between patients with and without CMR abnormalities. Troponin T was significantly higher among patients with CMR abnormalities. The median duration of follow-up was 702 (IQR 456-1394) days. Two patients had MACE (both heart failure). One of them had LGE changes. CONCLUSIONS: A significant number of patients with MINOCA have ischaemic LGE changes or myocardial wall oedema. The patients with CMR abnormalities have similar ECG and echocardiographic features except higher biomarker, highlighting the role of CMR in patients with MINOCA.


Assuntos
Vasos Coronários , Infarto do Miocárdio , Meios de Contraste , Vasos Coronários/diagnóstico por imagem , Gadolínio , Humanos , Imageamento por Ressonância Magnética , Imagem Cinética por Ressonância Magnética , Infarto do Miocárdio/complicações , Infarto do Miocárdio/diagnóstico , Valor Preditivo dos Testes
19.
Clin Case Rep ; 7(9): 1680-1684, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31534726

RESUMO

Traditionally sustained monomorphic ventricular tachycardia (SMVT) is associated with areas of myocardial scar such as that of chronic coronary artery disease. We present a case of SMVT in the initial setting of an acute myocardial infarction in a previously healthy individual suggesting that acute ischemia can give rise to SMVT.

20.
Cardiol Young ; 29(1): 1-10, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30375310

RESUMO

A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal function throughout life and never need treatment. Numerous genetic mechanisms are involved in the abnormal cellular functions that may cause abnormal development of the aortic valve during early foetal life. As several chromosomal disorders are also associated with a bicuspid valve, there does not appear to be an apparent common trigger to the abnormal development of the aortic valve. The clinical care of the bicuspid aortic valve patient has been changed by a significant body of evidence that has improved the understanding of the natural history of the disease, including when to best intervene with valve replacement and when to provide prophylactic aortic root surgery. Moreover, as bicuspid valve disease is also part of various syndromes, we can identify high-risk patients in whom a bicuspid valve is much more unfavourable than in the normal population. This review provides an overview of all aspects of the bicuspid aortic valve condition and gives an updated perspective on issues from pathophysiology to clinical care of bicuspid aortic valve disease and associated aortic disease in asymptomatic, symptomatic, and pregnant patients, as well as our viewpoint on population screening.


Assuntos
Valva Aórtica/anormalidades , Valva Aórtica/fisiopatologia , Cardiopatias Congênitas/complicações , Doenças das Valvas Cardíacas/genética , Valva Mitral/fisiopatologia , Dissecção Aórtica/etiologia , Valva Aórtica/patologia , Cardiopatias Congênitas/patologia , Doenças das Valvas Cardíacas/fisiopatologia , Implante de Prótese de Valva Cardíaca , Humanos , Valva Mitral/patologia
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