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1.
Eur Heart J ; 44(34): 3250-3260, 2023 09 07.
Artigo em Inglês | MEDLINE | ID: mdl-36747318

RESUMO

AIMS: To provide population-based data on the prevalence and clinical significance of immune deficiency syndromes (IDS) associated with congenital heart disease (CHD). METHODS AND RESULTS: Utilizing administrative German Health System data the prevalence of increased susceptibility to infection (ISI) or confirmed IDS was assessed in CHD patients and compared with an age-matched non-congenital control group. Furthermore, the prognostic significance of IDS was assessed using all-cause mortality and freedom from emergency hospital admission. A total of 54 449 CHD patients were included. Of these 14 998 (27.5%) had ISI and 3034 (5.6%) had a documented IDS (compared with 2.9% of the age-matched general population). During an observation period of 394 289 patient-years, 3824 CHD patients died, and 31 017 patients experienced a combined event of all-cause mortality or emergency admission. On multivariable Cox proportional-hazard analysis, the presence of ISI [hazard ratio (HR): 2.14, P < 0.001] or documented IDS (HR: 1.77, P = 0.035) emerged as independent predictors of all-cause mortality. In addition, ISI and confirmed IDS were associated with a significantly higher risk of emergency hospital admission (P = 0.01 for both on competing risk analysis) during follow-up. CONCLUSION: Limited immune competence is common in CHD patients and associated with an increased risk of morbidity and mortality. This highlights the need for structured IDS screening and collaboration with immunology specialists as immunodeficiency may be amenable to specific therapy. Furthermore, studies are required to assess whether IDS patients might benefit from intensified antibiotic shielding or tailored prophylaxis.


Assuntos
Cardiopatias Congênitas , Hospitalização , Humanos , Fatores de Risco , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Medição de Risco , Modelos de Riscos Proporcionais
2.
Eur Heart J ; 42(41): 4252-4260, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34638134

RESUMO

AIMS: The aim of this study was to provide population-based data on maternal and neonatal complications and outcome in the pregnancies of women with congenital heart disease (CHD). METHODS AND RESULTS: Based on administrative data from one of the largest German Health Insurance Companies (BARMER GEK, ∼9 million members representative for Germany), all pregnancies in women with CHD between 2005 and 2018 were analysed. In addition, an age-matched non-CHD control group was included for comparison and the association between adult CHD (ACHD) and maternal or neonatal outcomes investigated. Overall, 7512 pregnancies occurred in 4015 women with CHD. The matched non-CHD control group included 6502 women with 11 225 pregnancies. Caesarean deliveries were more common in CHD patients (40.5% vs. 31.5% in the control group; P < 0.001). There was no excess mortality. Although the maternal complication rate was low in absolute terms, women with CHD had a significantly higher rate of stroke, heart failure and cardiac arrhythmias during pregnancy (P < 0.001 for all). Neonatal mortality was low but also significantly higher in the ACHD group (0.83% vs. 0.22%; P = 0.001) and neonates to CHD mothers had low/extremely low birth weight or extreme immaturity (<0.001) or required resuscitation and mechanical ventilation more often compared to non-CHD offspring (P < 0.001 for both). On multivariate logistic regression maternal defect complexity, arterial hypertension, heart failure, prior fertility treatment, and anticoagulation with vitamin K antagonists emerged as significant predictors of adverse neonatal outcome (P < 0.05 for all). Recurrence of CHD was 6.1 times higher in infants to ACHD mothers compared to controls (P < 0.0001). CONCLUSIONS: This population-based study illustrates a reassuringly low maternal mortality rate in a highly developed healthcare system. Nevertheless, maternal morbidity and neonatal morbidity/mortality were significantly increased in women with ACHD and their offspring compared to non-ACHD controls highlighting the need of specialized care and pre-pregnancy counselling.


Assuntos
Cardiopatias Congênitas , Complicações Cardiovasculares na Gravidez , Cesárea , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Humanos , Gravidez , Complicações Cardiovasculares na Gravidez/epidemiologia , Resultado da Gravidez/epidemiologia , Estudos Retrospectivos
3.
Eur Heart J ; 42(41): 4241-4248, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34269382

RESUMO

AIMS: The aim of this study was to provide population-based data on the healthcare provision for adults with congenital heart disease (ACHD) and the impact of cardiology care on morbidity and mortality in this vulnerable population. METHODS AND RESULTS: Based on administrative data from one of the largest German Health Insurance Companies, all insured ACHD patients (<70 years of age) were included. Patients were stratified into those followed exclusively by primary care physicians (PCPs) and those with additional cardiology follow-up between 2014 and 2016. Associations between level of care and outcome were assessed by multivariable/propensity score Cox analyses. Overall, 24 139 patients (median age 43 years, 54.8% female) were included. Of these, only 49.7% had cardiology follow-up during the 3-year period, with 49.2% of patients only being cared for by PCPs and 1.1% having no contact with either. After comprehensive multivariable and propensity score adjustment, ACHD patients under cardiology follow-up had a significantly lower risk of death [hazard ratio (HR) 0.81, 95% confidence interval (CI) 0.67-0.98; P = 0.03) or major events (HR 0.85, 95% CI 0.78-0.92; P < 0.001) compared to those only followed by PCPs. At 3-year follow-up, the absolute risk difference for mortality was 0.9% higher in ACHD patients with moderate/severe complexity lesions cared by PCPs compared to those under cardiology follow-up. CONCLUSION: Cardiology care compared with primary care is associated with superior survival and lower rates of major complications in ACHD. It is alarming that even in a high resource setting with well-established specialist ACHD care approximately 50% of contemporary ACHD patients are still not linked to regular cardiac care. Almost all patients had at least one contact with a PCP during the study period, suggesting that opportunities to refer patients to cardiac specialists were missed at PCP level. More efforts are required to alert PCPs and patients to appropriate ACHD care.


Assuntos
Cardiologia , Cardiopatias Congênitas , Adulto , Atenção à Saúde , Feminino , Cardiopatias Congênitas/terapia , Humanos , Masculino , Morbidade , Modelos de Riscos Proporcionais
4.
J Clin Med ; 13(2)2024 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-38276138

RESUMO

BACKGROUND: Coronary CT angiography (CCTA) may detect coronary artery disease (CAD) in transcatheter aortic valve implantation (TAVI) patients and may obviate invasive coronary angiography (ICA) in selected patients. We assessed the diagnostic accuracy of CCTA for detecting CAD in TAVI patients based on published data. METHODS: Meta-analysis and meta-regression were performed based on a comprehensive electronic search, including relevant studies assessing the diagnostic accuracy of CCTA in the setting of TAVI patients compared to ICA. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV), were calculated on a patient and per segment level. RESULTS: Overall, 27 studies (total of 7458 patients) were included. On the patient level, the CCTA's pooled sensitivity and NPV were 95% (95% CI: 93-97%) and 97% (95% CI: 95-98%), respectively, while the specificity and PPV were at 73% (95% CI: 62-82%) and 64% (95% CI: 57-71%), respectively. On the segmental coronary vessel level, the sensitivity and NPV were 90% (95% CI: 79-96%) and 98% (95% CI: 97-99%). CONCLUSIONS: This meta-analysis highlights CCTA's potential as a first-line diagnostic tool although its limited PPV and specificity may pose challenges when interpreting heavily calcified arteries. This study underscores the need for further research and protocol standardization in this area.

5.
J Clin Med ; 11(3)2022 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-35160148

RESUMO

INTRODUCTION: Automated echocardiography image interpretation has the potential to transform clinical practice. However, neural networks developed in general cohorts may underperform in the setting of altered cardiac anatomy. METHODS: Consecutive echocardiographic studies of patients with congenital or structural heart disease (C/SHD) were used to validate an existing convolutional neural network trained on 14,035 echocardiograms for automated view classification. In addition, a new convolutional neural network for view classification was trained and tested specifically in patients with C/SHD. RESULTS: Overall, 9793 imaging files from 262 patients with C/SHD (mean age 49 years, 60% male) and 62 normal controls (mean age 45 years, 50.0% male) were included. Congenital diagnoses included among others, tetralogy of Fallot (30), Ebstein anomaly (18) and transposition of the great arteries (TGA, 48). Assessing correct view classification based on 284,250 individual frames revealed that the non-congenital model had an overall accuracy of 48.3% for correct view classification in patients with C/SHD compared to 66.7% in patients without cardiac disease. Our newly trained convolutional network for echocardiographic view detection based on over 139,910 frames and tested on 35,614 frames from C/SHD patients achieved an accuracy of 76.1% in detecting the correct echocardiographic view. CONCLUSIONS: The current study is the first to validate view classification by neural networks in C/SHD patients. While generic models have acceptable accuracy in general cardiology patients, the quality of image classification is only modest in patients with C/SHD. In contrast, our model trained in C/SHD achieved a considerably increased accuracy in this particular cohort.

6.
J Pers Med ; 12(3)2022 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-35330460

RESUMO

Introduction: Transesophageal echocardiography (TEE) is routinely performed before catheter ablation of atrial tachyarrhythmias to rule out the presence of left atrial thrombi. However, data to support this practice are inconsistent. Methods: We analyzed consecutive pre-procedural TEE in a high-volume electrophysiology center for the presence of left atrial thrombi and a relevant flow reduction in the left atrial appendage (LAA) defined as LAA sludge or LAA emptying velocity (LAAEV) < 20 cm/s. The possible predictors of reduced flow were recorded and compared in a multivariate logistic regression analysis. Results: 1676 TEE were included (1122 before pulmonary vein isolation, 436 before atrial flutter ablation, 166 before other ablations). 543 patients (32%) were female and 991 (59%) were on DOAC. Nine patients (0.5%) had an LAA thrombus on pre-procedural TEE. Ninety-five further patients (5.7%) had a relevant reduction in LAA flow. The underlying rhythm showed a significant association with the presence of LAA thrombus or reduced LAA flow (p = 0.003). Patients in sinus rhythm and cavotricuspid isthmus-dependent atrial flutter exhibited the lowest risk. Additionally, reduced kidney function was associated with a reduction in LAA flow velocities (p = 0.04). Of note, two LAA thrombi occurred in patients in sinus rhythm and six out of nine patients with an LAA thrombus were on vitamin-K antagonists. Conclusions: LAA thrombus is a rare occurrence before an elective catheter ablation. The underlying rhythm and kidney function may serve as markers of a higher likelihood of significantly reduced LAAEV and LAA thrombus.

7.
Heart ; 107(13): 1069-1076, 2021 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-33109710

RESUMO

OBJECTIVES: Data on the clinical outcome of patients with congenital heart disease (CHD) affected by severe viral pneumonia are limited. We analysed morbidity and mortality of viral pneumonia and evaluated the association between medical conditions, medication, vaccination and outcome specifically in patients with CHD requiring hospitalisation for viral pneumonia. METHODS: Based on data from one of Germany's largest health insurers, all cases of viral pneumonia requiring hospital admission (2005-2018) were studied. Mortality, and composites of death, transplantation, mechanical circulatory support, ventilation or extracorporeal lung support served as endpoints. RESULTS: Overall, 26 262 viral pneumonia cases occurred in 24 980 patients. Of these, 1180 cases occurred in patients with CHD. Compared with patients without CHD, mortality rate was elevated in patients with CHD. As a group, patients with CHD aged 20-59 years even exceeded mortality rates in patients without CHD aged >60 years. No mortality was observed in patients with CHD with simple defects <60 years of age without associated cardiovascular risk factors. On multivariable logistic regression analysis, age, CHD complexity, chromosomal anomalies, cardiac medication, use of immunosuppressants and absence of vaccination for influenza emerged as risk factors of adverse outcome. CONCLUSIONS: We present timely data on morbidity and mortality of severe viral pneumonia requiring hospital admission in patients with CHD. Need for mechanical ventilation and risk of death in CHD increase early in life, reaching a level equivalent to non-CHD individuals >60 years of age. Our data suggest that except for patients with isolated simple defects, patients with CHD should be considered higher-risk individuals when faced with severe viral pneumonia.

8.
Heart ; 106(17): 1302-1309, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32522822

RESUMO

Adults with congenital heart disease (ACHD) may be at high risk in the case of COVID-19. Due to the heterogeneity of ACHD and secondary complications, risk profiles are, however, not uniform. This document aims to give an overview of relevant data and outline our pragmatic approach to disease prevention and management. Based on anatomy and additional physiological factors including symptoms, exercise capacity, heart failure, pulmonary hypertension and cyanosis, we propose a pragmatic approach to categorising patients into low-risk, intermediate-risk and high-risk groups. We regard especially patients with complex cyanotic conditions, those with palliated univentricular hearts, heart failure, severe valvular disease or pulmonary hypertension as high-risk patients. To avoid infection, we recommend self-isolation and exemption from work for these cohorts. Infected ACHD patients with low or moderate risk and without signs of deterioration may be remotely followed and cared for at home while in self isolation. High-risk patients or those with signs of respiratory or cardiovascular impairment require admission ideally at a tertiary ACHD centre. Especially patients with complex, cyanotic disease, heart failure and arrhythmias require particular attention. Treatment in patients with cyanotic heart disease should be guided by the relative degree of desaturation compared with baseline and lactate levels rather than absolute oxygen saturation levels. Patients with right heart dilatation or dysfunction are potentially at increased risk of right heart failure as mechanical ventilation and acute respiratory distress syndrome can lead to increase in pulmonary arterial pressures.


Assuntos
Controle de Doenças Transmissíveis/métodos , Infecções por Coronavirus , Cardiopatias Congênitas , Pandemias , Administração dos Cuidados ao Paciente/métodos , Pneumonia Viral , Adulto , Betacoronavirus , COVID-19 , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Humanos , Pandemias/prevenção & controle , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Medição de Risco , SARS-CoV-2
9.
Int J Cardiovasc Imaging ; 35(12): 2189-2196, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31325067

RESUMO

Deep learning (DL) algorithms are increasingly used in cardiac imaging. We aimed to investigate the utility of DL algorithms in de-noising transthoracic echocardiographic images and removing acoustic shadowing artefacts specifically in patients with congenital heart disease (CHD). In addition, the performance of DL algorithms trained on CHD samples was compared to models trained entirely on structurally normal hearts. Deep neural network based autoencoders were built for denoising and removal of acoustic shadowing artefacts based on routine echocardiographic apical 4-chamber views and performance was assessed by visual assessment and quantifying cross entropy. 267 subjects (94 TGA and atrial switch and 39 with ccTGA, 10 Ebstein anomaly, 9 with uncorrected AVSD and 115 normal controls; 56.9% male, age 38.9 ± 15.6 years) with routine transthoracic examinations were included. The autoencoders significantly enhanced image quality across diagnostic subgroups (p < 0.005 for all). Models trained on congenital heart samples performed significantly better when exposed to examples from congenital heart disease patients. Our study demonstrates the potential of autoencoders for denoising and artefact removal in patients with congenital heart disease and structurally normal hearts. While models trained entirely on samples from structurally normal hearts perform reasonably in CHD, our data illustrates the value of dedicated image augmentation systems trained specifically on CHD samples.


Assuntos
Artefatos , Aprendizado Profundo , Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Interpretação de Imagem Assistida por Computador/métodos , Adulto , Estudos de Casos e Controles , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Razão Sinal-Ruído , Adulto Jovem
10.
Int J Cardiol ; 220: 246-50, 2016 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-27389449

RESUMO

BACKGROUND: Echocardiographic parameters of ventricular deformation of the systemic right ventricle (sRV) predict adverse clinical outcome in patients after atrial repair of transposition of the great arteries (TGA). We assessed myocardial deformation on cardiac MRI (CMR) and correlated these with clinical and conventional CMR parameters in TGA patients. METHODS: Retrospective analysis of CMR studies in 91 TGA patients (66% male; mean age 30.1±5.1years) at two tertiary adult congenital heart centers was conducted. Myocardial deformation was assessed by CMR-based feature tracking (FT), providing longitudinal (LS), radial (RS), and circumferential (CS) global strain for the sRV and the subpulmonary left ventricle. A subgroup of optimal TGA was defined (NYHA class I, NT-proBNP <300pg/ml, max. exercise work load ≥100watt, no significant clinical events) as a reference cohort. RESULTS: There was a significant correlation between FT and conventional CMR parameters. Left ventricular ejection fraction (LVEF) correlated significantly with LV LS, RS, and CS (r between 0.24 and 0.34, p values between 0.03 and 0.005). sRVEF correlated with RV CS (r=0.56, p<0.001), and RV RS (r=0.32, p=0.007). QRS duration showed a negative correlation with RV CS (r=-0.53, p<0.001), LV RS (r=-0.34, p=0.008), and LV CS (r=-0.34, p=0.006). Reference values for the novel FT method in clinically optimal TGA patients are provided. CONCLUSION: Assessment of myocardial function using CMR-based FT is feasible in TGA patients. FT measurements related to important prognostic clinical parameters. Furthermore, we provide for the first time reference values for TGA patients in an optimal clinical status.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Imagem Cinética por Ressonância Magnética/métodos , Complicações Pós-Operatórias , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Direita , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Ecocardiografia/métodos , Feminino , Alemanha , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/patologia , Átrios do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/fisiopatologia , Prognóstico , Valores de Referência , Reprodutibilidade dos Testes , Estudos Retrospectivos , Índice de Gravidade de Doença , Estatística como Assunto , Transposição dos Grandes Vasos/fisiopatologia , Resultado do Tratamento , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia
11.
Int J Cardiol ; 199: 197-203, 2015 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-26204568

RESUMO

BACKGROUND: As a consequence of heterogeneous results of relatively small individual trials, the impact of congenital heart defects (CHD) and the effect of disease severity on patient reported outcome measures (PROs) of quality of life (QoL) remains uncertain. We aimed to systematically summarize QoL data in CHD patients using meta-analytic methods. METHODS AND RESULTS: We performed a systematic review of the literature focusing on QoL in CHD. The search yielded 234 publications meeting the inclusion criteria, with a median of 88 patients per study (46% females, average age 24years). In total, QoL was reported using PROs in 47,471 CHD-patients. More than 95 different PROs were used to evaluate QoL. The most commonly used tool was the SF36 form (69 publications). Analysis of available quantitative QoL data from SF36 publications (n=4217 CHD patients) showed that QoL was reduced in patients with moderate or complex cardiac disease (e.g. relative physical functioning scores 0.96 [0.93-0.99] and 0.91 [0.88-0.95] compared with controls), while no such effect was evident in those patients with simple cardiac lesions. Similar results were found for the general health domain of the SF36 domain. CONCLUSIONS: Despite the proliferation of QoL-studies in CHD no standardized approach for measuring and reporting QoL has emerged and the published results are heterogeneous. In aggregation, however, the results of this study suggest that QoL is impaired in moderate or complex CHD, while no such impact of CHD on QoL could be established--on average--in patients with simple defects.


Assuntos
Cardiopatias Congênitas/psicologia , Qualidade de Vida , Resultado do Tratamento , Adulto , Feminino , Cardiopatias Congênitas/complicações , Humanos , Masculino , Satisfação Pessoal
12.
Heart ; 100(24): 1933-8, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25060755

RESUMO

OBJECTIVE: Aortic regurgitation (AR) after transcatheter aortic valve implantation (TAVI) is associated with a poor clinical outcome and its assessment therefore crucial. Quantification of AR by transthoracic echocardiography (TTE), however, remains challenging in this setting. The present study used quantitative flow measurement by cardiac MRI (CMR) with calculation of regurgitant fraction (RF) for the assessment of AR and compared the results with TTE. METHODS AND RESULTS: We included 65 patients with a mean age of 82.2±8.1 years (38 women) who underwent successful TAVI with Edwards SAPIEN valves (52 transfemoral, 13 transapical). The post-interventional degree of AR was assessed by CMR and by TTE. There was agreement between CMR and TTE with regards to the absence of severe AR. However, TTE significantly underestimated the presence of moderate AR classifying it to be mild in 38 and moderate in only 5 patients, whereas CMR found mild AR in 23 and moderate in 16 patients. Overall, there was only fair agreement between CMR and TTE regarding the grading of AR with a weighted κ of 0.33. The rate of detection of TTE for more than mild AR was only 19%. CONCLUSIONS: Using CMR for the quantification of AR in a sizeable group of TAVI patients, we demonstrate a strong tendency of TTE to underestimate AR compared with CMR. Since higher AR severity on echocardiography has been associated with worse patient outcome, the potential incremental prognostic value of CMR should be studied prospectively in this setting.


Assuntos
Insuficiência da Valva Aórtica/diagnóstico , Complicações Pós-Operatórias/diagnóstico , Substituição da Valva Aórtica Transcateter/métodos , Idoso de 80 Anos ou mais , Insuficiência da Valva Aórtica/etiologia , Insuficiência da Valva Aórtica/fisiopatologia , Ecocardiografia , Ecocardiografia Doppler em Cores/métodos , Feminino , Humanos , Angiografia por Ressonância Magnética , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Sensibilidade e Especificidade
13.
Kardiol Pol ; 72(4): 363-71, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24293146

RESUMO

BACKGROUND: Left ventricular longitudinal strain (LV-LS) and strain rate (SR) are sensitive markers of early systolic dysfunction. AIM: To evaluate the feasibility of a novel, cardiac magnetic resonance (CMR) based method known as feature tracking (FT) for the assessment of strain and SR, and to compare the CMR based results to those obtained on standard transthoracic echocardiography (TTE) in healthy volunteers and in patients with left ventricular hypertrophy cardiomyopathy (HCM). METHODS: Overall, 20 healthy volunteers (ten male, mean age 24 ± 3 years) and 20 consecutive patients with HCM (12 male, mean age 47 ± 19 years) were included. Longitudinal and circumferential strain and SR of the left ventricle were measured on CMR at 1.5 Tesla and TTE and interobserver variability was assessed. RESULTS: FT measurements were feasible in all subjects. A good agreement between global LV-LS measured on CMR (controls: 20.8 ± 3.0; HCM: 17.6 ± 3.8) and TTE (controls: 19.4 ± 2.1; HCM: 16.6 ± 2.9) was found, while the agreement was worse for circumferential strain and all SR measurements. For the left and right ventricles, interobserver reproducibility was higher for strain measurements compared to SR. Coefficients of variation were lowest for LV-LS (13.2%) by CMR. CONCLUSIONS: FT analysis is a novel CMR based method for the analysis of myocardial strain and SR that is simple and correlates well with the echocardiographic measurements. Since CMR is unaffected by inadequate acoustic windows, FT may represent an attractive alternative to echocardiography in assessing the increasingly important parameters of myocardial deformation.


Assuntos
Ecocardiografia/métodos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/diagnóstico , Espectroscopia de Ressonância Magnética/métodos , Contração Miocárdica/fisiologia , Volume Sistólico/fisiologia , Adulto , Idoso , Feminino , Voluntários Saudáveis , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes
14.
Orphanet J Rare Dis ; 9: 203, 2014 Dec 10.
Artigo em Inglês | MEDLINE | ID: mdl-25491897

RESUMO

BACKGROUND: Echocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults. Accordingly, frequency of MPA dilatation based on echocardiography remains to be assessed in adults with Marfan syndrome (MFS). METHODS: We enrolled 123 normal adults (72 men, 52 women aged 42 ± 14 years) and 98 patients with MFS (42 men, 56 women aged 39 ± 14 years) in a retrospective cross-sectional observational controlled study in four tertiary care centers. We defined outcome measures including upper normal limits of MPA-d and MPA-r as 95 quantile of normal persons, MPA dilatation as diameters > upper normal limits, MPA aneurysm as diameters >4 cm, and indication for surgery as MPA diameters >6 cm. RESULTS: MPA diameters revealed normal distribution without correlation to age, sex, body weight, body height, body mass index and body surface area. The upper normal limit was 2.6 cm (95% confidence interval (CI) =2.44-2.76 cm) for MPA-d, and 1.05 (95% CI = .86-1.24) for MPA-r. MPA dilatation presented in 6 normal persons (4.9%) and in 68 MFS patients (69.4%; P < .001), MPA aneurysm presented only in MFS (15 patients; 15.3%; P < .001), and no patient required surgery. Mean MPA-r were increased in MFS (P < .001), but ratios >1.05 were equally frequent in 7 normal persons (5%) and in 8 MFS patients (10.5%; P = .161). MPA-r related to aortic root diameters (P = .042), reduced left ventricular ejection fraction (P = .006), and increased pulmonary artery systolic pressures (P = .040). No clinical manifestations of MFS and no FBN1 mutation characteristics related to MPA diameters. CONCLUSIONS: We established 2.6 cm for MPA-d and 1.05 for MPA-r as upper normal limits. MFS exhibits a high prevalence of MPA dilatation and aneurysm. However, patients may require MPA surgery only in scarce circumstances, most likely because formation of marked MPA aneurysm may require LV dysfunction and increased PASP.


Assuntos
Aneurisma Aórtico/diagnóstico por imagem , Síndrome de Marfan/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Vasodilatação , Adolescente , Adulto , Idoso , Aneurisma Aórtico/fisiopatologia , Estudos Transversais , Ecocardiografia/normas , Feminino , Humanos , Masculino , Síndrome de Marfan/fisiopatologia , Pessoa de Meia-Idade , Artéria Pulmonar/fisiopatologia , Valores de Referência , Estudos Retrospectivos , Adulto Jovem
15.
Expert Rev Cardiovasc Ther ; 11(7): 919-31, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23895034

RESUMO

A significant number of adult patients with congenital heart disease suffer from pulmonary arterial hypertension leading to a markedly increased morbidity and mortality. Some defects may be eligible for operative or interventional repair in adulthood but careful selection of candidates is crucial. With the emergence of disease-targeting therapies, symptomatic improvement and stabilization have become possible while the impact on survival currently remains unclear.


Assuntos
Cardiopatias Congênitas/fisiopatologia , Hipertensão Pulmonar/terapia , Seleção de Pacientes , Adulto , Fatores Etários , Hipertensão Pulmonar Primária Familiar , Humanos , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/cirurgia , Sobrevida
16.
BMJ Case Rep ; 20102010 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-22778206

RESUMO

A 30-year-old female experienced severe acute respiratory distress in her apartment assumed to be due to an allergic asthma. Upon arrival of the emergency physician at the scene the patient was unconscious and cyanotic. Auscultation yielded no respiratory sounds despite visible efforts of the patient. Mask ventilation was virtually impossible. Endotracheal intubation was performed but complicated by a distinct resistance. Ventilation remained difficult, despite antiobstructive medication and deep general anaesthesia. Fiberoptic bronchoscopy in the hospital finally showed a bulk of granulomatous tissue located just above the tracheal bifurcation. Here, the authors report a rare case of acute-onset respiratory distress due to Wegener's granulomatosis.


Assuntos
Granulomatose com Poliangiite/complicações , Síndrome Respiratória Aguda Grave/etiologia , Adulto , Obstrução das Vias Respiratórias/complicações , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia , Broncoscopia , Feminino , Granulomatose com Poliangiite/cirurgia , Humanos , Respiração Artificial , Síndrome Respiratória Aguda Grave/cirurgia , Síndrome Respiratória Aguda Grave/terapia
17.
J Investig Med ; 58(8): 977-81, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-20926957

RESUMO

PURPOSE: Percutaneous dilatational tracheotomy (PDT) is a well-established procedure in intensive care medicine. Several reports confirm a low acute and long-term complication rate. However, dilatational tracheotomy is still often postponed even in patients that are under ventilator support for more than 2 weeks. We present a retrospective study analyzing the subjective long-term results after percutaneous tracheotomy performed at our medical intensive care unit between 2002 and 2008. METHODS: We used a modified method described by Ciaglia for tracheotomy. We selected 649 patients eligible for the survey among those who had received PDT between 2002 and 2008 and had been dismissed from hospital alive. RESULTS: The return rate was 38.5% with 29.8% of the questionnaires being completed by the patients; in 32 cases, relatives reported of the patients' death. No patient experienced wound infection; none needed surgical wound revision. Dysarthria was reversible in all 16 cases; 2 patients reported a persistent hoarseness that was preexisting in both cases. One patient experienced a tracheal stenosis and received a tracheal stent; one other required a permanent tracheotomy because of a nonresectable granulation tissue. The overall rate of long-term complications associated with the long-term ventilation and the PDT was 1.8%. CONCLUSIONS: Percutaneous dilatational tracheotomy is an easily performed, cost-saving method for long-term ventilated patients with a low rate of acute and long-term complications even in old and multimorbid internal medicine patients.


Assuntos
Dilatação/instrumentação , Intubação Intratraqueal/métodos , Respiração Artificial/métodos , Insuficiência Respiratória/cirurgia , Traqueotomia/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Coleta de Dados , Feminino , Humanos , Unidades de Terapia Intensiva , Intubação Intratraqueal/efeitos adversos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Inquéritos e Questionários , Traqueotomia/efeitos adversos , Resultado do Tratamento , Adulto Jovem
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