RESUMO
INTRODUCTION AND OBJECTIVES: Chronic thromboembolic disease refers to the presence of chronic thrombotic pulmonary vascular thrombosis without pulmonary hypertension (PH) at rest but with exercise limitation after pulmonary embolism (PE). Our aim was to evaluate the hemodynamic response to exercise in these patients and its correlation with the values reached in cardiopulmonary exercise testing. METHODS: We included symptomatic patients with persistent pulmonary thrombosis after PE. We excluded patients with left heart disease or significant PH (mean pulmonary arterial pressure [mPAP] >25mmHg, pulmonary vascular resistance >3 WU, and pulmonary capillary wedge pressure [PCWP] >15mmHg). Cardiopulmonary exercise testing and exercise right heart catheterization were performed. Exercise-induced precapillary PH was defined as mPAP/CO slope >3 and PCWP/CO slope <2mmHg/l/min. The hemodynamic response and the values obtained in cardiopulmonary exercise testing were compared between patients with and without exercise-induced precapillary PH. RESULTS: We studied 36 patients; 4 were excluded due to incomplete hemodynamic data. Out of the 32 patients analyzed; 3 developed a pathological increase in PCWP. Among the remaining 29 patients (mean age, 49.4±13.7 years, 34.5% women), 13 showed exercise-induced PH. Resting mPAP was higher in those who developed exercise-induced PH (23.3±5.4 vs 19.0±3.8mmHg; P=.012), although CO was similar in the 2 groups. Patients with exercise-induced PH exhibited data of ventilatory inefficiency with reduced values of end-tidal CO2 pressure at the anaerobic threshold (32.8±3.0 vs 36.2±3.3mmHg; P=.021) and a higher Ve/VCO2 slope (34.2±4.8 vs 30.7±5.0; P=.049). CONCLUSIONS: Exercise limitation and ventilatory inefficiency could be attributable to exercise-induced precapillary PH in a subgroup of patients with persistent pulmonary thrombosis and dyspnea.
Assuntos
Hipertensão Pulmonar , Embolia Pulmonar , Trombose , Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Masculino , Diagnóstico Diferencial , Cateterismo Cardíaco , Hemodinâmica/fisiologia , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Doença Crônica , Teste de Esforço , Trombose/diagnósticoRESUMO
BACKGROUND AND OBJECTIVES: Standardization of radiomic data acquisition protocols is still at a very early stage, revealing a strong need to work towards the definition of uniform image processing methodologies The aim of this study is to identify sources of variability in radiomic data derived from image discretization and resampling methodologies prior to image feature extraction. Furthermore, to identify robust potential image-based biomarkers for the early detection of cardiotoxicity. METHODS: Image post-acquisition processing, interpolation, and volume of interest (VOI) segmentation were performed. Four experiments were conducted to assess the reliability in terms of the intraclass correlation coefficient (ICC) of the radiomic features and the effects of the variation of voxel size and gray level discretization. Statistical analysis was performed separating the patients according to cardiotoxicity diagnosis. Differences of texture features were studied with Mann-Whitney U test. P-values <0.05 after multiple testing correction were considered statistically significant. Additionally, a non-supervised k-Means clustering algorithm was evaluated. RESULTS: The effect of the variation in the voxel size demonstrated a non-dependency relationship with the values of the radiomic features, regardless of the chosen discretization method. The median ICC values were 0.306 and 0.872 for absolute agreement and consistency, respectively, when varying the discretization bin number. The median ICC values were 0.678 and 0.878 for absolute agreement and consistency, respectively, when varying the discretization bin size. A total of 16 first order, 6 Gray Level Co-occurrence Matrix (GLCM), 4 Gray Level Dependence Matrix (GLDM) and 4 Gray Level Run Length Matrix (GLRLM) features demonstrated statistically significant differences between the diagnosis groups for interim scans (P<0.05) for the fixed bin size (FBS) discretization methodology. However, no statistically significant differences between diagnostic groups were found for the fixed bin number (FBN) discretization methodology. Two clusters based on the radiomic features were identified. CONCLUSIONS: Gray level discretization has a major impact on the repeatability of the radiomic features. The selection of the optimal processing methodology has led to the identification of texture-based patterns for the differentiation of early cardiac damage profiles.
Assuntos
Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Humanos , Reprodutibilidade dos Testes , Cardiotoxicidade/diagnóstico por imagem , Radiômica , Processamento de Imagem Assistida por Computador/métodosRESUMO
INTRODUCTION AND OBJECTIVES: The management of persistent moderate-severe tricuspid regurgitation (TR) in patients with chronic thromboembolic pulmonary hypertension after treatment with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA) is not well defined. This study aimed to analyze the progression and predictors of significant persistent postintervention TR and its prognostic impact. METHODS: This single-center observational study included 72 patients undergoing PEA and 20 who completed a BPA program with a previous diagnosis of chronic thromboembolic pulmonary hypertension and moderate-to-severe TR. RESULTS: The postintervention prevalence of moderate-to-severe TR was 29%, with no difference between the PEA- or BPA-treated groups (30.6% vs 25% P=.78). Compared with patients with absent-mild postprocedure TR, those with persistent TR had higher mean pulmonary arterial pressure (40.2±1.9 vs 28.5±1.3mmHg P <.001), pulmonary vascular resistance (472 [347-710] vs 282 [196-408] dyn.s/cm5; P <.001), and right atrial area (23.0 [21-31] vs 16.0 [14.0-20.0] P <.001). The variables independently associated with persistent TR were pulmonary vascular resistance> 400 dyn.s/cm5 and postprocedure right atrial area> 22cm2. No preintervention predictors were identified. The variables associated with increased 3-year mortality were residual TR and mean pulmonary arterial pressure> 30mmHg. CONCLUSIONS: Residual moderate-to-severe TR following PEA-PBA was associated with persistently high afterload and unfavorable postintervention right chamber remodeling. Moderate-to-severe TR and residual pulmonary hypertension were associated with a worse 3-year prognosis.
Assuntos
Angioplastia com Balão , Fibrilação Atrial , Hipertensão Pulmonar , Embolia Pulmonar , Insuficiência da Valva Tricúspide , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Insuficiência da Valva Tricúspide/diagnóstico , Insuficiência da Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/epidemiologia , Fibrilação Atrial/complicações , Angioplastia com Balão/métodos , Endarterectomia/métodos , Embolia Pulmonar/epidemiologia , Embolia Pulmonar/cirurgia , Embolia Pulmonar/complicações , Resultado do TratamentoRESUMO
BACKGROUND: Truncating TTN variants (TTNtv) are the main cause of dilated cardiomyopathy (DCM). The dynamic nature of this entity has previously been described. Based on own empirical observations and previous evidences, this study assessed repolarisation patterns and the possible association with morphological and functional status of TTNtv-DCM patients. METHODS: Electrocardiograms (ECGs) of index patients with TTNtv-DCM and their relatives were included and matched in time with an echocardiogram. All individuals were classified into five phenotype groups: 1) Reduced left ventricular ejection fraction (LVEF <50%); 2) Recovered LVEF: at least 10% increase and LVEF >30% after optimal medical treatment; 3) Borderline phenotype (mildly enlarged ventricle and/or hyper-trabeculation); 4) Genotype positive, phenotype negative; and 5) Non-carriers. All electrocardiograms were evaluated by two blinded observers in qualitative and quantitative terms [T index (mm)=Σ T-wave amplitude (V5, V6, II, aVF)] and these data were compared with demographic and clinical information. The Δ T-index was calculated in those individuals with more than one electrocardiogram. RESULTS: Seventy-eight (78) electrocardiograms were included (46% female, mean age 50 years). T-index and prevalence of an abnormal T-wave had significantly different results among the groups (p<0.0001). Age and haemodynamic factors were shown to be ECG-modifiers, especially in phenotype-negative patients. T-index enabled individuals with reduced LVEF (<2.5) to be identified and to differentiate patients with favourable and unfavourable responses to treatment (Δ T index >3.5 and ≤2, respectively). CONCLUSION: Repolarisation changes enabled characterisation of the spectrum of TTNtv-DCM. The T-index identified potential carriers and patients with the worst profiles of the spectrum of the disease.
Assuntos
Cardiomiopatia Dilatada , Cardiomiopatia Dilatada/diagnóstico , Conectina , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Volume Sistólico , Função Ventricular EsquerdaAssuntos
COVID-19 , Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Humanos , Incidência , SARS-CoV-2Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Hipertensão Pulmonar/etiologia , Pneumonia Viral/complicações , Embolia Pulmonar/complicações , Adulto , Idoso , COVID-19 , Teste para COVID-19 , Doença Crônica , Técnicas de Laboratório Clínico/métodos , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/tratamento farmacológico , Feminino , Humanos , Hipertensão Pulmonar/terapia , Masculino , Pessoa de Meia-Idade , Oxigenoterapia , Pandemias , Pneumonia Viral/diagnóstico , Pneumonia Viral/tratamento farmacológico , SARS-CoV-2 , Resultado do Tratamento , Tratamento Farmacológico da COVID-19RESUMO
The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) could represent an especially vulnerable population because of the high mortality rates reported for respiratory infections. However, the number of COVID-19 cases reported among PAH and CTEPH patients is surprisingly low. Furthermore, the clinical picture that has been described in these patients is far from the severity that experts would expect. Endothelial dysfunction is a common feature between patients with PAH/CTEPH and COVID-19, leading to ventilation/perfusion mismatch, vasoconstriction, thrombosis and inflammation. In this picture, the angiotensin-converting enzyme 2 plays an essential role, being directly involved in the pathophysiology of both clinical entities. Some of these common characteristics could explain the good adaptation of PAH and CTEPH patients to COVID-19, who could also have obtained a benefit from the disease's specific treatments (anticoagulant and pulmonary vasodilators), probably due to its protective effect on the endothelium. Additionally, these common features could also lead to PAH/CTEPH as a potential sequelae of COVID-19. Throughout this comprehensive review, we describe the similarities and differences between both conditions and the possible pathophysiological and therapeutic-based mechanisms leading to the low incidence and severity of COVID-19 reported in PAH/CTEPH patients to date. Nevertheless, international registries should look carefully into this population for better understanding and management.
Assuntos
Cardiopatias Congênitas , Pericárdio , Coração , Humanos , Pericárdio/diagnóstico por imagemAssuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Pneumonia Viral/complicações , Hipertensão Arterial Pulmonar/complicações , Hipertensão Arterial Pulmonar/mortalidade , Adulto , Idoso , COVID-19 , Estudos de Coortes , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/diagnóstico , Pneumonia Viral/mortalidade , Hipertensão Arterial Pulmonar/patologia , SARS-CoV-2 , Taxa de SobrevidaRESUMO
Pulmonary artery aneurysm (PAA) is a common finding in patients with long-term pulmonary arterial hypertension (PAH). The influence of PAH severity in the development of PAA remains unclear. We sought to determine whether PAA development is related to PAH severity and whether treatment optimization based on risk profile estimation is effective to stop pulmonary artery (PA) enlargement. This is a retrospective study of 125 PAH patients who underwent an imaging test (computed tomography or magnetic resonance) combined with a right heart catheterization within a six-month period. A multivariate analysis was performed to identify independent risk factors for PAA. Patients who underwent an additional imaging-test and RHC during follow-up were analyzed to evaluate changes on PA dimensions. PAA was diagnosed in 42 (34%) patients. PAA was more frequent in patients with congenital heart disease and toxic oil syndrome. PAH time-course showed to be an independent risk factor for PAA (HR 1.051, 95% CI 1.013-1.091, p = 0.008) whereas PAH severity did not. Twenty-six patients underwent a follow-up imaging-test and catheterization. After treatment optimization, a non-significant reduction of mean PA pressure was observed (58.5 mmHg [43.5-70.8] vs. 55.5 mmHg [47.5-66.3], p = 0.115) and a higher proportion of patients achieved a low-risk profile (19% vs. 35%, p = 0.157). However, the PA diameter significantly increased (40.4 ± 10.1 mm vs. 42.1 ± 9.6 mm; p = 0.003). PAA is a common condition in long-standing PAH but its development is not necessarily related to PAH severity. Despite stabilization after treatment optimization, a progressive PA dilatation was observed.
Assuntos
Aneurisma/etiologia , Pressão Arterial , Hipertensão Arterial Pulmonar/complicações , Artéria Pulmonar/fisiopatologia , Remodelação Vascular , Adulto , Aneurisma/diagnóstico por imagem , Aneurisma/fisiopatologia , Cateterismo Cardíaco , Angiografia por Tomografia Computadorizada , Dilatação Patológica , Feminino , Humanos , Angiografia por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/fisiopatologia , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Fatores de TempoAssuntos
Displasia Arritmogênica Ventricular Direita/genética , DNA/genética , Desmoplaquinas/genética , Eletrocardiografia , Predisposição Genética para Doença , Ventrículos do Coração/fisiopatologia , Adulto , Displasia Arritmogênica Ventricular Direita/diagnóstico , Desmoplaquinas/metabolismo , Ventrículos do Coração/diagnóstico por imagem , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Tomografia Computadorizada com Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
Detection of pulmonary artery aneurysms (PAA) in pulmonary arterial hypertension (PAH) is increasing. We sought to determine the frequency of PAA in a PAH cohort, variables related to its development and its prognostic impact. We conducted a retrospective analysis of PAH patients who underwent a computed tomography or magnetic resonance. PAA was defined as a pulmonary artery >40 mm. Baseline, echocardiographic, and hemodynamic findings at PAH diagnosis were compared. Freedom from death or lung transplant was estimated by Kaplan-Meier method and compared by log-rank test. Predictors of PAA development were analyzed with multivariate models. Two-hundred patients underwent a computed tomography and/or magnetic resonance. In 77 (38%), a PAA (48.3 ± 7.2 mm) was detected. Time-course (months) of PAH was an independent risk factor for PAA (hazard ratio 1.01; 95% confidence interval 1.002 to 1.019; pâ¯=â¯0.016) whilst connective tissue disease was associated with a lower risk (hazard ratio 0.236; 95% confidence interval 0.060 to 0.920; pâ¯=â¯0.037). PAA patients showed lower rates of death and lung transplant from PAH diagnosis (pâ¯=â¯0.005), but no differences appeared when survival analysis was performed from first imaging test (pâ¯=â¯0.269). PAA patients presented a nonsignificant higher rate of sudden death (5% PAA vs 1% no-PAA; pâ¯=â¯0.073). In conclusion, the frequency of PAA was 38%. PAH time-course was an independent risk factor for PAA development whereas connective tissue disease -related PAH patients showed a lower risk. PAA patients showed lower rates of death or lung transplant from PAH diagnosis but no differences were found from imaging test. PAA patients had a nonsignificant higher rate of sudden death.
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Aneurisma/diagnóstico por imagem , Aneurisma/mortalidade , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/mortalidade , Artéria Pulmonar/diagnóstico por imagem , Adulto , Aneurisma/complicações , Doenças Assintomáticas , Estudos de Coortes , Morte Súbita/epidemiologia , Feminino , Insuficiência Cardíaca/complicações , Humanos , Transplante de Pulmão/estatística & dados numéricos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X , Insuficiência da Valva Tricúspide/complicaçõesAssuntos
Antibacterianos/administração & dosagem , Ecocardiografia/métodos , Enfisema/diagnóstico por imagem , Gastrite/diagnóstico por imagem , Dor Abdominal/diagnóstico , Dor Abdominal/etiologia , Tratamento Conservador , Serviço Hospitalar de Emergência , Enfisema/tratamento farmacológico , Enfisema/patologia , Gastrite/tratamento farmacológico , Gastrite/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Doenças Raras , Resultado do TratamentoRESUMO
INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. METHODS: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration. RESULTS: Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) CONCLUSIONS: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques.