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1.
Crit Care Med ; 51(8): 1064-1073, 2023 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-37276353

RESUMO

OBJECTIVES: Early studies of venovenous extracorporeal membrane oxygenation (ECMO) in COVID-19 have revealed similar outcomes to historical cohorts. Changes in the disease and treatments have led to differences in the patients supported on venovenous ECMO in the first and second waves. We aimed to compare these two groups in both the acute and follow-up phase. DESIGN: Retrospective single-center cohort study comparing mortality at censoring date (November 30, 2021) and decannulation, patient characteristics, complications and lung function and quality of life (QOL-by European Quality of Life 5 Dimensions 3 Level Version) at first follow-up in patients supported on venovenous ECMO between wave 1 and wave 2 of the COVID-19 pandemic. SETTING: Critical care department of a severe acute respiratory failure service. PATIENTS: Patients supported on ECMO for COVID-19 between wave 1 (March 17, 2020, to August 31, 2020) and wave 2 (January 9, 2020, to May 25, 2021). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One hundred twenty-three patients were included in our analysis. Survival at censoring date (χ 2 , 6.35; p = 0.012) and decannulation (90.4% vs 70.0%; p < 0.001) was significantly lower in the second wave, while duration of ECMO run was longer (12.0 d [18.0-30.0 d] vs 29.5 d [15.5-58.3 d]; p = 0.005). Wave 2 patients had longer application of noninvasive ventilation (NIV) prior to ECMO and a higher frequency of barotrauma. Patient age and NIV use were independently associated with increased mortality (odds ratio 1.07 [1.01-1.14]; p = 0.025 and 3.37 [1.12-12.60]; p = 0.043, respectively). QOL and lung function apart from transfer coefficient of carbon monoxide corrected for hemoglobin was similar at follow-up across the waves. CONCLUSIONS: Most patients with COVID-19 supported on ECMO in both waves survived in the short and longer term. At follow-up patients had similar lung function and QOL across the two waves. This suggests that ECMO has an ongoing role in the management of a carefully selected group of patients with COVID-19.


Assuntos
COVID-19 , Oxigenação por Membrana Extracorpórea , Humanos , COVID-19/terapia , Oxigenação por Membrana Extracorpórea/métodos , Qualidade de Vida , Estudos de Coortes , Estudos Retrospectivos , Pandemias
2.
Echo Res Pract ; 10(1): 6, 2023 Apr 06.
Artigo em Inglês | MEDLINE | ID: mdl-37020250

RESUMO

BACKGROUND: Focused echocardiography is increasingly used in acute and emergency care, with point-of-care ultrasound integrated into several specialist training curricula (e.g. Emergency Medicine, Cardiology, Critical Care). Multiple accreditation pathways support development of this skill but there is scant empirical evidence to inform selection of teaching methods, accreditation requirements or quality assurance of education in focussed echocardiography. It has also been noted that access to in-person teaching can be a barrier to completing accreditation programmes, and that this may affect learners disproportionately depending on the location or nature of their institution. The purpose of the study was to determine whether serial image interpretation tasks as a distinct learning tool improved novice echocardiographers' ability to accurately identify potentially life-threatening pathology from focused scans. We also aimed to describe the relationship between accuracy of reporting and participants' confidence in those reports, and to assess users' satisfaction with a learning pathway that could potentially be delivered remotely. METHODS: 27 participants from a variety of healthcare roles completed a program of remote lectures and 2 in-person study days. During the program they undertook 4 'packets' of 10 focused echocardiography reporting tasks (total = 40) based on images from a standardised dataset. Participants were randomized to view the scans in varying orders. Reporting accuracy was compared with consensus reports from a panel of expert echocardiographers, and participants self-reported confidence in their image interpretation and their satisfaction with the learning experience. RESULTS: There was a stepwise improvement in reporting accuracy with each set of images reported, from an average reporting score of 66% for the 1st packet to 78% for the 4th packet. Participants felt more confident in identifying common life-threatening pathologies as they reported more echocardiograms. The correlation between report accuracy and confidence in the report was weak and did not increase during the study (rs = 0.394 for the 1st packet, rs = 0.321 for the 4th packet). Attrition during the study related primarily to logistical issues. There were high levels of satisfaction amongst participants, with most reporting that they would use and / or recommend a similar teaching package to colleagues. CONCLUSIONS: Healthcare professionals undertaking remote training with recorded lectures, followed by multiple reporting tasks were capable of interpreting focused echocardiograms. Reporting accuracy and confidence in identifying life-threatening pathology increased with the number of scans interpreted. The correlation between accuracy and confidence for any given report was weak (and this relationship should be explored further given the potential safety considerations). All components of this package could be delivered via distance learning to enhance the flexibility of echocardiography education.

3.
Perfusion ; 37(5): 526-529, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-34053349

RESUMO

Harlequin Syndrome (also known as North-South Syndrome) is a complication of veno-arterial extracorporeal membrane oxygenation (V-A ECMO) that can occur when left ventricular function starts to recover. While most commonly due to continued impaired gas exchange in the lungs, we present a case caused by right ventricular dysfunction, successfully managed by conversion of the ECMO circuit to a veno-veno-arterial (VV-A) configuration.


Assuntos
Oxigenação por Membrana Extracorpórea , Hipo-Hidrose , Doenças do Sistema Nervoso Autônomo , Rubor , Ventrículos do Coração , Humanos
4.
Rev Port Cardiol ; 36(9): 619-625, 2017 Sep.
Artigo em Inglês, Português | MEDLINE | ID: mdl-28826934

RESUMO

INTRODUCTION: Cardiorenal syndrome (CRS) is common in acute heart failure (AHF), and is associated with dire prognosis. Levosimendan, a positive inotrope that also has diuretic effects, may improve patients' renal profile. Published results are conflicting. OBJECTIVES: We aimed to assess the incidence of CRS in AHF patients according to the inotrope used and to determine its predictors in order to identify patients who could benefit from the most renoprotective inotrope. METHODS: In a retrospective study, 108 consecutive patients with AHF who required inotropes were divided into two groups according to the inotrope used (levosimendan vs. dobutamine). The primary endpoint was CRS incidence. Follow-up for mortality and readmission for AHF was conducted. RESULTS: Seventy-one percent of the study population were treated with levosimendan and the remainder with dobutamine. No differences were found in heart failure etiology or chronic kidney disease. At admission, the dobutamine group had lower blood pressure; there were no differences in estimated glomerular filtration rate or cystatin C levels. The levosimendan group had lower left ventricular ejection fraction. CRS incidence was higher in the dobutamine group, and they more often had incomplete recovery of renal function at discharge. In multivariate analysis, cystatin C levels predicted CRS. The dobutamine group had higher in-hospital mortality, of which CRS and the inotrope used were predictors. CONCLUSIONS: Levosimendan appears to have some renoprotective effect, as it was associated with a lower incidence of CRS and better recovery of renal function at discharge. Identification of patients at increased risk of renal dysfunction by assessing cystatin C may enable more tailored therapy, minimizing the incidence of CRS and its negative impact on outcome in AHF.


Assuntos
Síndrome Cardiorrenal/etiologia , Síndrome Cardiorrenal/prevenção & controle , Cardiotônicos/uso terapêutico , Dobutamina/uso terapêutico , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/tratamento farmacológico , Hidrazonas/uso terapêutico , Piridazinas/uso terapêutico , Doença Aguda , Idoso , Síndrome Cardiorrenal/epidemiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Simendana
6.
Rev Port Cardiol ; 35(5): 305.e1-7, 2016 May.
Artigo em Inglês, Português | MEDLINE | ID: mdl-27118142

RESUMO

The authors present a case of systemic amyloidosis with cardiac involvement. We discuss the need for a high level of suspicion to establish a diagnosis, diagnostic techniques and treatment options. Our patient was a 78-year-old man with chronic renal disease and atrial fibrillation admitted with acute decompensated heart failure of unknown cause. The transthoracic echocardiogram revealed severely impaired left ventricular function with phenotypic overlap between hypertrophic and restrictive cardiomyopathy. After an extensive diagnostic workup, which included an abdominal fat pad biopsy, the final diagnosis was amyloidosis.


Assuntos
Amiloidose/diagnóstico , Cardiomiopatias/diagnóstico , Idoso , Amiloidose/complicações , Biópsia , Cardiomiopatias/complicações , Ecocardiografia , Insuficiência Cardíaca/etiologia , Humanos , Masculino
7.
Eur Heart J Acute Cardiovasc Care ; 5(3): 223-30, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25740222

RESUMO

AIMS: Renal dysfunction is a powerful predictor of adverse outcomes in patients hospitalized for acute coronary syndrome. Three new glomerular filtration rate (GFR) estimating equations recently emerged, based on serum creatinine (CKD-EPIcreat), serum cystatin C (CKD-EPIcyst) or a combination of both (CKD-EPIcreat/cyst), and they are currently recommended to confirm the presence of renal dysfunction. Our aim was to analyse the predictive value of these new estimated GFR (eGFR) equations regarding mid-term mortality in patients with acute coronary syndrome, and compare them with the traditional Modification of Diet in Renal Disease (MDRD-4) formula. METHODS AND RESULTS: 801 patients admitted for acute coronary syndrome (age 67.3±13.3 years, 68.5% male) and followed for 23.6±9.8 months were included. For each equation, patient risk stratification was performed based on eGFR values: high-risk group (eGFR<60ml/min per 1.73m(2)) and low-risk group (eGFR⩾60ml/min per 1.73m(2)). The predictive performances of these equations were compared using area under each receiver operating characteristic curves (AUCs). Overall risk stratification improvement was assessed by the net reclassification improvement index. The incidence of the primary endpoint was 18.1%. The CKD-EPIcyst equation had the highest overall discriminate performance regarding mid-term mortality (AUC 0.782±0.20) and outperformed all other equations (ρ<0.001 in all comparisons). When compared with the MDRD-4 formula, the CKD-EPIcyst equation accurately reclassified a significant percentage of patients into more appropriate risk categories (net reclassification improvement index of 11.9% (p=0.003)). The CKD-EPIcyst equation added prognostic power to the Global Registry of Acute Coronary Events (GRACE) score in the prediction of mid-term mortality. CONCLUSION: The CKD-EPIcyst equation provides a novel and improved method for assessing the mid-term mortality risk in patients admitted for acute coronary syndrome, outperforming the most widely used formula (MDRD-4), and improving the predictive value of the GRACE score. These results reinforce the added value of cystatin C as a risk marker in these patients.


Assuntos
Síndrome Coronariana Aguda/mortalidade , Taxa de Filtração Glomerular , Nefropatias/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Feminino , Hospitalização , Humanos , Nefropatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Medição de Risco
9.
Rev Port Cardiol ; 34(5): 357.e1-5, 2015 May.
Artigo em Inglês, Português | MEDLINE | ID: mdl-25935071

RESUMO

The authors report the case of a patient diagnosed with both hypertrophic cardiomyopathy and aortic stenosis. Due to clinical deterioration, additional investigation was performed, and a high left ventricular outflow tract gradient was identified. Correct identification of the condition causing the symptoms was challenging, and involved several imaging techniques, the contribution of transesophageal echocardiography being crucial. The final diagnosis of severe aortic stenosis led to successful valve replacement surgery. The presence of these two conditions in the same patient has been documented, although it is uncommon. This association poses particular diagnostic and therapeutic challenges, which are discussed in this paper.


Assuntos
Estenose da Valva Aórtica/fisiopatologia , Cardiomiopatia Hipertrófica/fisiopatologia , Idoso , Estenose da Valva Aórtica/complicações , Cardiomiopatia Hipertrófica/complicações , Feminino , Hemodinâmica , Humanos , Função Ventricular Esquerda , Obstrução do Fluxo Ventricular Externo/etiologia
10.
Rev Port Cardiol ; 34(4): 287.e1-7, 2015 Apr.
Artigo em Inglês, Português | MEDLINE | ID: mdl-25843309

RESUMO

Brugada syndrome, first described over 20 years ago, is characterized by a typical electrocardiographic pattern with coved-type ST-segment elevation in the right precordial leads and a high risk of sudden death in otherwise healthy young adults. The electrocardiographic pattern is sometimes intermittent, and fever is a possible trigger. The authors present the case of a 68-year-old woman who came to the emergency department with fever and syncope. A diagnosis of community-acquired pneumonia was made. The electrocardiogram performed when the patient had fever revealed a type 1 Brugada pattern, which disappeared after the fever subsided. After other causes of Brugada-like pattern were excluded, Brugada syndrome was diagnosed and a cardioverter-defibrillator was implanted. This case demonstrates that this entity can be diagnosed at more advanced ages and highlights the usefulness of electrocardiography in a febrile state.


Assuntos
Síndrome de Brugada/etiologia , Febre/complicações , Idoso , Síndrome de Brugada/fisiopatologia , Eletrocardiografia , Feminino , Humanos
11.
Rev Port Cardiol ; 34(2): 95-102, 2015 Feb.
Artigo em Inglês, Português | MEDLINE | ID: mdl-25662471

RESUMO

INTRODUCTION AND OBJECTIVES: Intravenous loop diuretics are an essential part of acute heart failure management; however, data to guide their use is sparse. Our aim was to compare continuous intravenous infusion of loop diuretics with intravenous bolus administration in terms of efficacy and adverse events in patients admitted with severe acute heart failure. METHODS: Over a period of three years, 110 patients were admitted to our cardiac intensive care unit with acute heart failure. Clinical, laboratory and prognostic parameters were compared according to the diuretic strategy used and mortality and readmission for acute heart failure during follow-up were analyzed. RESULTS: Previous medical history was similar in the two groups. At admission, the continuous infusion group met criteria for worse prognosis: lower systolic blood pressure (p=0.011), more severe renal injury (p=0.008), lower left ventricular ejection fraction (p=0.016) and higher incidence of restrictive pattern of diastolic dysfunction (p=0.032). They were more often treated with vasopressors (p=0.003), inotropes (p=0.010), renal support therapy (p=0.003) and non-invasive ventilation (p<0.001). They had longer hospitalizations (p=0.014) and a higher incidence of cardiorenal syndrome (p=0.009); however, at discharge, there were no differences in renal function between the groups. In-hospital mortality was similar, and during follow-up there were no differences in mortality or readmission for acute heart failure. CONCLUSIONS: Continuous infusion was preferred in patients presenting with worse clinical status, in whom renal dysfunction was transiently worse. However, in-hospital mortality and creatinine at discharge were similar. Continuous infusion thus appears to counteract the initial dire prognosis of more unstable patients.


Assuntos
Insuficiência Cardíaca/tratamento farmacológico , Inibidores de Simportadores de Cloreto de Sódio e Potássio/administração & dosagem , Doença Aguda , Idoso , Feminino , Hospitalização , Humanos , Infusões Intravenosas , Injeções Intravenosas , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença
13.
Artigo em Inglês | Index Psicologia - Periódicos | ID: psi-65072

RESUMO

INTRODUCTION: adolescence is a phase that entails biopsychosocial changes with specific emotional and behavioral impact on sexual and reproductive health of both sexes. The World Health Organization considers adolescents to be the ages from 10 to 19 years. Chronologically this is important for epidemiological research, for development of public health policies , to define programs and specific actions for this audience. The concern with the sexual and reproductive health of adolescents is due to early onset of sexual activity, which contributes to expose these young people to the risks of unplanned pregnancy and sexually transmitted diseases OBJECTIVE: to identify the sources of information used by adolescents in public schools about sexuality and reproduction METHODS: descriptive quantitative study. There were 90 Semi-structured interviews applied to adolescents with predominant age between 16 and 19 years old from the second year of high school in a Public School located in the urban periphery of a capital in the northeast of Brazil RESULTS: it was observed that the adolescents of this study has a relatively high accessibility to the educational activities in the area of sexual and reproductive health. The school was the main area cited by the participants of the study. As for the search for information on the subject, friends are the most sought after by adolescents. Afterwards, the most often cited are family members. The majority of the adolescents said that the source of information most sought was television (35.5%), followed by the internet, books and magazines. Contraception was practiced by only 14 (36.9%) of the adolescents CONCLUSION: this study points to the school as the main area of participation in educational activities on sexual and reproductive health among adolescents, in addition to friends, television and the internet.(AU)


INTRODUÇÃO: a adolescência é uma fase que acarreta mudanças biopsicossociais, com especificidades emocionais e comportamentais que repercutem na saúde sexual e reprodutiva de ambos os sexos. A Organização Mundial da Saúde considera como adolescentes a faixa etária dos 10 aos 19 anos. Cronologicamente isso é importante para a investigação epidemiológica, para elaboração de políticas públicas e para definição de programas e ações específicas para esse público. A preocupação com a saúde sexual e reprodutiva dos adolescentes se deve a precocidade do início da atividade sexual, a qual contribui para expor esses jovens aos riscos da gravidez não planejada e das doenças sexualmente transmissíveis OBJETIVO: identificar as fontes de informações utilizadas pelos adolescentes da escola pública sobre sexualidade e reprodução MÉTODO: estudo descritivo de natureza quantitativa. 90 entrevistas semiestrutu-radas foram aplicadas em adolescentes do segundo ano do Ensino Médio de uma Escola Pública da periferia urbana de uma capital do Nordeste Brasileiro, cuja faixa etária predomina entre 16 e 19 anos RESULTADOS: observou-se que os adolescentes da pesquisa têm uma acessibilidade relativamente elevada a atividades educativas na área de saúde sexual e reprodutiva. A escola foi o principal espaço citado pelos participantes do estudo. Quanto à busca por informações sobre o assunto, os amigos são os mais procurados pelos adolescentes. Posteriormente os mais citados são os familiares. A maioria dos adolescentes disse que a fonte de informação mais buscada era a televisão (35,5%), seguido da internet e livros e revistas. A anticoncepção era praticada por somente 14 (36,9%) dos adolescentes CONCLUSÃO: esse estudo aponta para a escola como principal espaço de participação em atividades educativas sobre saúde sexual e reprodutiva entre os adolescentes, além de os amigos, televisão e internet.(AU)


Assuntos
Adolescente , Adolescente , Sexualidade , Saúde Sexual , Saúde Reprodutiva
14.
Artigo em Inglês | LILACS | ID: lil-747945

RESUMO

INTRODUÇÃO: a adolescência é uma fase que acarreta mudanças biopsicossociais, com especificidades emocionais e comportamentais que repercutem na saúde sexual e reprodutiva de ambos os sexos. A Organização Mundial da Saúde considera como adolescentes a faixa etária dos 10 aos 19 anos. Cronologicamente isso é importante para a investigação epidemiológica, para elaboração de políticas públicas e para definição de programas e ações específicas para esse público. A preocupação com a saúde sexual e reprodutiva dos adolescentes se deve a precocidade do início da atividade sexual, a qual contribui para expor esses jovens aos riscos da gravidez não planejada e das doenças sexualmente transmissíveis OBJETIVO: identificar as fontes de informações utilizadas pelos adolescentes da escola pública sobre sexualidade e reprodução MÉTODO: estudo descritivo de natureza quantitativa. 90 entrevistas semiestruturadas foram aplicadas em adolescentes do segundo ano do Ensino Médio de uma Escola Pública da periferia urbana de uma capital do Nordeste Brasileiro, cuja faixa etária predomina entre 16 e 19 anos RESULTADOS: observou-se que os adolescentes da pesquisa têm uma acessibilidade relativamente elevada a atividades educativas na área de saúde sexual e reprodutiva. A escola foi o principal espaço citado pelos participantes do estudo. Quanto à busca por informações sobre o assunto, os amigos são os mais procurados pelos adolescentes. Posteriormente os mais citados são os familiares. A maioria dos adolescentes disse que a fonte de informação mais buscada era a televisão (35,5%), seguido da internet e livros e revistas. A anticoncepção era praticada por somente 14 (36,9%) dos adolescentes CONCLUSÃO: esse estudo aponta para a escola como principal espaço de participação em atividades educativas sobre saúde sexual e reprodutiva entre os adolescentes, além de os amigos, televisão e internet.


INTRODUCTION: adolescence is a phase that entails biopsychosocial changes with specific emotional and behavioral impact on sexual and reproductive health of both sexes. The World Health Organization considers adolescents to be the ages from 10 to 19 years. Chronologically this is important for epidemiological research, for development of public health policies , to define programs and specific actions for this audience. The concern with the sexual and reproductive health of adolescents is due to early onset of sexual activity, which contributes to expose these young people to the risks of unplanned pregnancy and sexually transmitted diseases OBJECTIVE: to identify the sources of information used by adolescents in public schools about sexuality and reproduction METHODS: descriptive quantitative study. There were 90 Semi-structured interviews applied to adolescents with predominant age between 16 and 19 years old from the second year of high school in a Public School located in the urban periphery of a capital in the northeast of Brazil RESULTS: it was observed that the adolescents of this study has a relatively high accessibility to the educational activities in the area of sexual and reproductive health. The school was the main area cited by the participants of the study. As for the search for information on the subject, friends are the most sought after by adolescents. Afterwards, the most often cited are family members. The majority of the adolescents said that the source of information most sought was television (35.5%), followed by the internet, books and magazines. Contraception was practiced by only 14 (36.9%) of the adolescents CONCLUSION: this study points to the school as the main area of participation in educational activities on sexual and reproductive health among adolescents, in addition to friends, television and the internet.


Assuntos
Humanos , Masculino , Feminino , Adolescente , Saúde do Adolescente , Promoção da Saúde , Gravidez não Desejada , Atenção Primária à Saúde , Fatores de Risco , Educação Sexual , Saúde Reprodutiva , Sexualidade , Infecções Sexualmente Transmissíveis , Ensino Fundamental e Médio , Estratégias de Saúde Nacionais , Política Pública , Instituições Acadêmicas
16.
Rev Port Cardiol ; 33(7-8): 471.e1-6, 2014.
Artigo em Inglês, Português | MEDLINE | ID: mdl-25108561

RESUMO

The authors report the case of a 53-year-old man, with a long-standing history of mild mitral stenosis, admitted for worsening fatigue. Transthoracic echocardiography (limited by poor image quality) showed mitral annular calcification, leaflets that were difficult to visualize and an estimated mitral valve area of 1.8 cm(2) by the pressure half-time method. However, elevated mean transmitral and right ventricle/right atrium gradients were identified (39 and 117 mmHg, respectively). This puzzling discrepancy in the echocardiographic findings prompted investigation by transesophageal echocardiography, which revealed an echogenic structure adjacent to the mitral annulus, causing severe obstruction (effective orifice area 0.7 cm(2)). The suspicion of supravalvular mitral ring was confirmed during surgery. Following ring resection and mitral valve replacement there was significant improvement in the patient's clinical condition and normalization of the left atrium/left ventricle gradient. Supravalvular mitral ring is an unusual cause of congenital mitral stenosis, characterized by an abnormal ridge of connective tissue on the atrial side of the mitral valve, which often obstructs mitral valve inflow. Few cases have been reported, most of them in children with concomitant congenital abnormalities. Diagnosis of a supravalvular mitral ring is challenging, since it is very difficult to visualize in most diagnostic tests. It was the combination of clinical and various echocardiographic findings that led us to suspect this very rare condition, enabling appropriate treatment, with excellent long-term results.


Assuntos
Estenose da Valva Mitral/diagnóstico , Estenose da Valva Mitral/etiologia , Valva Mitral/anormalidades , Erros de Diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Valva Mitral/cirurgia
17.
Rev Port Cardiol ; 33(5): 261-7, 2014 May.
Artigo em Inglês, Português | MEDLINE | ID: mdl-24909443

RESUMO

INTRODUCTION AND AIM: The prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients. METHODS: From a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow-up were analyzed. RESULTS: Patients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/É ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p<0.001) and lower left ventricular ejection fraction (p=0.056). Patients with LGE more often had an implantable cardioverter-defibrillator (ICD) (p=0.015). At follow-up, no differences were found in the frequency of ventricular arrhythmias, appropriate ICD therapies or mortality. CONCLUSIONS: The presence of LGE emerges as a risk marker, associated with the classical predictors of sudden cardiac death in this population. However, larger studies are required to confirm its independent association with clinical events.


Assuntos
Técnicas de Imagem Cardíaca/métodos , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/genética , Meios de Contraste , Gadolínio , Imageamento por Ressonância Magnética/métodos , Fenótipo , Cardiomiopatia Hipertrófica/complicações , Morte Súbita Cardíaca/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco
19.
Rev Port Cardiol ; 33(3): 183.e1-5, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24661928

RESUMO

Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by abnormalities of the GLA gene, which encodes the enzyme α-galactosidase A. A deficiency of this enzyme leads to the lysosomal accumulation of glycosphingolipids, which may cause left ventricular hypertrophy that is typically concentric and symmetric. We present the case of a 60-year-old woman with symptoms of dyspnea, atypical chest pain and palpitations, in whom a transthoracic echocardiogram revealed an apical variant of hypertrophic cardiomyopathy. Analysis of specific sarcomeric genetic mutations was negative. The patient underwent a screening protocol for Anderson-Fabry disease, using a dried blood spot test, which was standard at our institution for patients with left ventricular hypertrophy. The enzymatic activity assay revealed reduced α-galactosidase A enzymatic activity. Molecular analysis identified a missense point mutation in the GLA gene (p.R118C). This case report shows that Anderson-Fabry disease may cause an apical form of left ventricular hypertrophy. The diagnosis was only achieved because of systematic screening, which highlights the importance of screening for Anderson-Fabry disease in patients with unexplained left ventricular hypertrophy, including those presenting with more unusual patterns, such as apical variants of left ventricular hypertrophy. This case also supports the idea that the missense mutation R118C is indeed a true pathogenic mutation of Anderson-Fabry disease.


Assuntos
Doença de Fabry/complicações , Doença de Fabry/genética , Hipertrofia Ventricular Esquerda/etiologia , Mutação de Sentido Incorreto , alfa-Galactosidase/genética , Doença de Fabry/diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade
20.
Rev Port Cardiol ; 33(3): 139-46, 2014 Mar.
Artigo em Inglês, Português | MEDLINE | ID: mdl-24642129

RESUMO

INTRODUCTION AND OBJECTIVE: Worsening renal function has an unquestionably negative impact on prognosis in patients with acute heart failure (HF). In Portugal there is little information about the importance of this entity in HF patients admitted to hospital. The objective of this work was to assess the prevalence of cardiorenal syndrome and to identify its key predictors and consequences in patients admitted for acute HF. METHODS: This was a retrospective study of 155 patients admitted for acute HF. Cardiorenal syndrome was defined as an increase in serum creatinine of ≥26.5 µmol/l. Clinical, laboratory and echocardiographic parameters were analyzed and compared. Mortality was assessed at 30 and 90 days. RESULTS: Cardiorenal syndrome occurred in 46 patients (29.7%), 5.4 ± 4.4 days after admission; 66.7% (n=24) did not recover baseline creatinine levels. The factors associated with cardiorenal syndrome were older age, chronic renal failure, moderate to severe mitral regurgitation, higher admission blood urea nitrogen, creatinine and troponin I, and lower glomerular filtration rate. Patients who developed cardiorenal syndrome had longer hospital stay, were treated with higher daily doses of intravenous furosemide, and more often required inotropic support and renal replacement therapy. They had higher in-hospital and 30-day mortality, and multivariate analysis identified cardiorenal syndrome as an independent predictor of in-hospital mortality. CONCLUSIONS: Renal dysfunction is common in acute HF patients, with a negative impact on prognosis, which highlights the importance of preventing kidney damage through the use of new therapeutic strategies and identification of novel biomarkers.


Assuntos
Síndrome Cardiorrenal/epidemiologia , Síndrome Cardiorrenal/etiologia , Insuficiência Cardíaca/complicações , Doença Aguda , Idoso , Feminino , Hospitalização , Humanos , Incidência , Masculino , Prevalência , Prognóstico , Estudos Retrospectivos
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