Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 10.272
Filtrar
1.
Braz J Med Biol Res ; 52(8): e8513, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31365695

RESUMO

Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=-0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 of these patients.


Assuntos
Tolerância ao Exercício/fisiologia , Hipertensão Pulmonar/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Pulmão/fisiologia , Testes de Função Respiratória/métodos , Escleroderma Sistêmico/complicações , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/etiologia , Medidas de Volume Pulmonar/métodos , Pessoa de Meia-Idade , Ventilação Pulmonar , Doença de Raynaud/complicações , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital/fisiologia , Teste de Caminhada/métodos
2.
Methodist Debakey Cardiovasc J ; 15(2): 152-155, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384380

RESUMO

Iatrogenic aortocaval fistula is an extremely rare pathologic condition that often results in clinically significant left-to-right extracardiac shunt. In slow-progressing cases, chronic right-sided heart failure can occur and, in some patients, may persist for years. We present a patient with a long-standing aortocaval fistula that was causing high-flow left-to-right shunting, tricuspid regurgitation, severe pulmonary hypertension, and right-side heart failure. After undergoing complete endoscopic isolation of the aortocaval fistula, the patient experienced dramatic clinical improvement and continued to have excellent imaging and clinical resolution after 2 years of follow-up.


Assuntos
Aorta/fisiopatologia , Fístula Arteriovenosa/terapia , Embolização Terapêutica , Procedimentos Endovasculares , Insuficiência Cardíaca/etiologia , Hipertensão Pulmonar/etiologia , Doença Iatrogênica , Veia Cava Inferior/fisiopatologia , Aorta/diagnóstico por imagem , Aortografia/métodos , Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/etiologia , Fístula Arteriovenosa/fisiopatologia , Angiografia por Tomografia Computadorizada , Ecocardiografia Doppler em Cores , Embolização Terapêutica/instrumentação , Procedimentos Endovasculares/instrumentação , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Flebografia/métodos , Desenho de Prótese , Fluxo Sanguíneo Regional , Dispositivo para Oclusão Septal , Fatores de Tempo , Resultado do Tratamento , Veia Cava Inferior/diagnóstico por imagem
3.
Int Heart J ; 60(4): 836-844, 2019 Jul 27.
Artigo em Inglês | MEDLINE | ID: mdl-31257329

RESUMO

Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (PAP) ≥ 25 mmHg at rest as assessed by right heart catheterization (RHC), and Doppler-derived systolic PAP (sPAPECHO) or tricuspid regurgitation pressure gradient (TRPG) is widely used to screen for PH. However, the cutoff value of sPAPECHO or TRPG for detecting a mean PAP ≥ 25 mmHg that was determined invasively has not been well defined.We studied 189 patients who underwent RHC. Echocardiography was performed within 24 hours of invasive evaluation, and sPAPECHO was defined as the TRPG with right atrial pressure estimated on the basis of the current guideline.From the receiver operating characteristic (ROC) curve analysis, the optimal sPAPECHO, and TRPG cutoffs for detecting PH were 41 mmHg (sensitivity, 92%; specificity, 91%; area under the curve = 0.95) and 36 mmHg (sensitivity, 90%; specificity, 93%; area under the curve = 0.95), respectively. The area under the TRPG ROC curve was similar to the area under the sPAPECHO ROC curve.Given that Doppler echocardiography is required to accurately detect PH rather than to accurately estimate systolic PAP, our results provide useful information with regard to screening patients for PH and recommending further investigations on PH.


Assuntos
Determinação da Pressão Arterial/métodos , Pressão Sanguínea/fisiologia , Ecocardiografia Doppler/métodos , Hipertensão Pulmonar/diagnóstico , Estudos Transversais , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Pressão Propulsora Pulmonar/fisiologia , Curva ROC , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sístole
4.
Rev Med Chil ; 147(4): 426-436, 2019 Apr.
Artigo em Espanhol | MEDLINE | ID: mdl-31344203

RESUMO

BACKGROUND: Balloon pulmonary angioplasty (BPA) is a therapeutic alternative for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). AIM: To report the initial experience with the "refined BPA technique" with the use of intravascular images. PATIENTS AND METHODS: Between June 2015 and June 2016 we selected fourteen patients with CTEPH who were considered candidates for BPA. Lesions targeted for treatment were further analyzed using intravascular imaging with optical frequency domain imaging (OFDI). We report the immediate hemodynamic results and four weeks of follow-up of the first eight patients of this series. RESULTS: We performed 16 BPA in eight patients aged 61 ± 14 years (88% women). Mean pulmonary artery pressure (PAPm) was 48.6 ± 5.8 mmHg. Success was achieved in seven patients (88%). A mean of 2.3 segments per patient were intervened in 11 sessions (1.6 sessions/ patient). Only one patient developed lung reperfusion injury. No mortality was associated with the procedure. After the last BPA session, PAPm decreased to 37.4 ± 8.6 mmHg (p=0.02). Pulmonary vascular resistance (RVP) decreased from 858,6 ± 377,0 at baseline to 516,6 ± 323,3 Dynes/sec/cm-5 (p<0.01) and the cardiac index increased from 2.4±0.6 at baseline to 2.8±0.3 L/min/m2 (p=0.01). At 4 weeks after the last BPA, WHO functional class improved from 3.3±0.5 to 2.5±0.5 (p<0,01) and six minutes walking distance from 331±92 to 451±149 m (p=0.01). CONCLUSIONS: BPA guided by OFDI for the treatment of inoperable CTEPH patients is a safe alternative with excellent immediate hemodynamic and clinical results.


Assuntos
Angioplastia com Balão/métodos , Hipertensão Pulmonar/terapia , Embolia Pulmonar/terapia , Adulto , Idoso , Angiografia/métodos , Doença Crônica , Feminino , Hemodinâmica , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/fisiopatologia , Reprodutibilidade dos Testes , Fatores de Tempo , Tomografia de Coerência Óptica/métodos , Resultado do Tratamento
5.
Life Sci ; 232: 116546, 2019 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-31176777

RESUMO

Pulmonary arterial hypertension (PAH) is reported to contribute to right ventricular failure and death. PAH of variable degrees is often related to congenital heart disease (CHD). Galectin-3 (Gal-3) has been proven to be of great importance in PAH and CHD. Therefore, we investigated the specific mechanism of Gal-3 in CHD-PAH. Patients with CHD-PAH were enrolled to detect the changes of T-cell subsets, cytokine levels, and other related inflammatory cells in the plasma and to assess the Gal-3 levels in the serum. Next, CHD-PAH mouse models were established and treated with restored or depleted Gal-3 to evaluate the systolic pulmonary artery pressure (sPAP) and right ventricular hypertrophy index (RVHI), to determine levels of IL-4, IL-5, IL-13, AKT and p-AKT along with proliferation of pulmonary artery smooth muscle cells (PASMCs). Finally, we explored the effects of adoptive transfer of CD4+T cells on CHD-PAH in mice with Gal-3 knockdown to further investigate the role of Gal-3 in vivo. Initially, Gal-3 was up-regulated in patients with CHD-PAH. Subsequently, it was demonstrated that restored Gal-3 increased sPAP and RVHI, and promoted proliferation of PASMCs by activating the immune response with elevated levels of IL-4, IL-5, IL-13 and p-AKT. Finally, adoptive transfer of CD4+T cells promoted CD4+T cell perivascular infiltration and the progression of CHD-PAH in mice with Gal-3 knockdown. Collectively, the current study suggests a facilitating role of Gal-3 in pulmonary artery remodeling and progression of CHD-PAH via activation of Th2.


Assuntos
Galectina 3/metabolismo , Cardiopatias Congênitas/metabolismo , Hipertensão Pulmonar/metabolismo , Adulto , Idoso , Animais , Modelos Animais de Doenças , Feminino , Galectina 3/sangue , Galectina 3/imunologia , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/imunologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Hipertensão Pulmonar/imunologia , Hipertensão Pulmonar/fisiopatologia , Imunomodulação/fisiologia , Interleucina-13/metabolismo , Interleucina-4/metabolismo , Interleucina-5/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos BALB C , Pessoa de Meia-Idade , Miócitos de Músculo Liso/metabolismo , Proteínas Proto-Oncogênicas c-akt/metabolismo , Artéria Pulmonar/fisiopatologia , Remodelação Vascular/fisiologia
6.
Zhejiang Da Xue Xue Bao Yi Xue Ban ; 48(1): 102-110, 2019 May 25.
Artigo em Chinês | MEDLINE | ID: mdl-31102364

RESUMO

Pulmonary arterial hypertension (PAH) is a clinical hemodynamic syndrome characterized by elevated pulmonary arterial pressure and pulmonary vascular resistance leading to right heart failure and death. Vascular remodeling is the most prominent histopathological feature of PAH, which is regulated by many factors. Endoplasmic reticulum stress, calcium disorder and mitochondrial dysfunction are involved in the vascular cell proliferation and apoptosis by regulating intracellular calcium homeostasis and cellular metabolism. Epigenetic phenomenon such as DNA damage and abnormal expression of miRNA are also involved in the regulation of abnormal proliferation of vascular cells. Vascular cell phenotype switching including endothelial-mesenchymal transition and smooth muscle cell phenotype switching play an important role in abnormal proliferation of vascular cells. Vascular remodeling is produced by a variety of cells and molecular pathways, and aiming at multiple targets which is expected to find a new breakthrough in the treatment of PAH,and to improve abnormal vascular remodeling, delay or even reverse the progression of PAH.


Assuntos
Hipertensão Pulmonar , Artéria Pulmonar , Remodelação Vascular , Proliferação de Células , Células Cultivadas , Humanos , Hipertensão Pulmonar/fisiopatologia , MicroRNAs/genética , Miócitos de Músculo Liso/patologia , Artéria Pulmonar/patologia , Remodelação Vascular/genética
7.
Int J Cardiovasc Imaging ; 35(9): 1637-1649, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31037473

RESUMO

Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of connective tissue disease (CTD). Right atrial (RA) function is essential to maintaining adequate total right heart function in PAH. However, little is known about prognostic utility of RA function in CTD-PAH. RA longitudinal strain (LS) and strain rate (LSR) were evaluated in 53 consecutive patients (51 female, mean age 42 ± 15 years) with CTD-PAH, including systemic lupus erythematosus (SLE) (33.7%), mixed connective tissue disease (MCTD) (32.1%), primary Sjögren's syndrome (pSS) (26.4%), and systemic sclerosis (SSc) (3.8%). At a mean follow-up of 19.3 ± 10.9 months, 20 patients (37.7%) were clinically worse. The group with clinical events had worse clinical conditions and poorer RA function at baseline compared with the group that had no clinical events. RA LS independently reflected World Health Organization functional class (WHO FC) after adjusting for RA area (RAA), tricuspid regurgitation (TR) grade, right ventricular (RV) global longitudinal strain (GLS), and pulmonary vascular resistance (PVR) (P = 0.006). Receiver operator characteristic (ROC) curve analysis indicated that RA LS < 22.9% was predictive of clinical worsening during follow-up (sensitivity = 80%; specificity = 87.9%; area under the curve (AUC) = 0.858), and the Kaplan-Meier curve confirmed that RA LS ≥ 22.9% was associated with more favorable long-term outcomes compared to RA LS < 22.9% (log-rank P < 0.01). On univariate Cox proportional hazards analysis, PVR, RVGLS, RAA, and RA LS were associated with long-term outcome, while RA LS was the only independent predictor in the multivariate analysis. Our findings suggest that RA LS measurements by speckle-tracking echocardiography (STE) can independently reflect the extent of right heart failure and predict clinical outcomes in patients with CTD-PAH. RA LS < 22.9% is associated with a higher risk of clinical worsening.


Assuntos
Pressão Arterial , Função do Átrio Direito , Doenças do Tecido Conjuntivo/complicações , Ecocardiografia Doppler/métodos , Átrios do Coração/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Interpretação de Imagem Assistida por Computador/métodos , Artéria Pulmonar/fisiopatologia , Adulto , Idoso , Doenças do Tecido Conjuntivo/diagnóstico , Progressão da Doença , Feminino , Átrios do Coração/fisiopatologia , Cardiopatias/etiologia , Cardiopatias/fisiopatologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Fatores de Tempo , Função Ventricular Direita , Remodelação Ventricular
8.
Expert Rev Cardiovasc Ther ; 17(6): 461-472, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31107116

RESUMO

Introduction: Pulmonary hypertension (PH) secondary to left-sided heart disease (Group 2 PH) is a frequent complication of heart failure (HF) and is a heterogeneous phenotypic disorder that worsens exercise capacity, increases risk for hospitalization and survival independent of left ventricular ejection fraction (LVEF) or stage of HF. Areas covered: In this review, an update of the current knowledge and some potential challenges about the pathophysiology and treatments of group 2 PH in patients with HF of either preserved or reduced ejection fraction are provided. Also, this review discusses the epidemiology and provides hints for the optimal evaluation and diagnosis of these patients to prevent misclassification of their pulmonary hypertension. Expert opinion: There are many of areas lacking knowledge and understanding in the field of pulmonary hypertension associated to left heart disease (PH-LHD) that should be addressed in the future. Further research should be performed, in terms of pathobiology, and understanding the predisposition (genetic susceptibility and contributing factors) of the different phenotypes of this disorder. More clinical trials targeting new therapeutic options and specific PH therapies are warranted to help this increasing important patient group as the current guidelines recommend to only treat the underlying left-sided heart disease.


Assuntos
Insuficiência Cardíaca/complicações , Hipertensão Pulmonar/fisiopatologia , Função Ventricular Esquerda/fisiologia , Insuficiência Cardíaca/terapia , Hospitalização , Humanos , Volume Sistólico/fisiologia , Disfunção Ventricular Esquerda/diagnóstico
9.
Expert Rev Cardiovasc Ther ; 17(6): 449-459, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31120797

RESUMO

Introduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. Areas covered: In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers is described, with an emphasis on co-morbidities and complications. Expert opinion: PAH-CHD describes a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.


Assuntos
Complexo de Eisenmenger/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Hipertensão Pulmonar/fisiopatologia , Adulto , Humanos , Qualidade de Vida
10.
Intern Med ; 58(9): 1301-1305, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31061260

RESUMO

This case report concerns a 22-year-old woman with large patent ductus arteriosus and atrial septal defect. She was referred to our hospital because of exertional dyspnea and was revealed to have advanced pulmonary arterial hypertension (PAH) with a mean pulmonary arterial pressure (PAP) of 79 mmHg. Although both shunts had bidirectional flow, based on the results of acute pulmonary vasoreactive testing, one-stage surgical closure was performed followed by up-front combination therapy for post-operative pulmonary hypertensive crisis and residual PAH. At 14 months after the surgery, her symptoms were markedly improved, and her mean PAP had dramatically decreased to 13 mmHg.


Assuntos
Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/patologia , Comunicação Interatrial/complicações , Comunicação Interatrial/patologia , Hipertensão Pulmonar/etiologia , Terapia Combinada , Dispneia/etiologia , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Encefalopatia Hipertensiva/complicações , Complicações Pós-Operatórias , Pressão Propulsora Pulmonar , Adulto Jovem
11.
Int J Cardiovasc Imaging ; 35(8): 1499-1508, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31037475

RESUMO

Resting two-dimensional speckle tracking echocardiography (2D-STE) identified right ventricular (RV) systolic function were reported to predict exercise capacity in pulmonary hypertension (PH) patients, but little attention had been payed to 2D-STE detected RV diastolic function. Therefore, we aim to elucidate and compare the relations between 2D-STE identified RV diastolic/systolic functions and peak oxygen consumption (PVO2) determined by cardiopulmonary exercise testing (CPET) in pre-capillary PH. 2D-STE was performed in 66 pre-capillary PH patients and 28 healthy controls. Linear correlation and multivariate regression analyses were performed to evaluate and compare the relations between RV 2D-STE parameters and PVO2. Receiver operating characteristic curves were used to compare the predictive value of 2D-STE parameters in predicting the cut-off-PVO2 < 11 ml/min/kg. There were significant differences of all the 2D-STE parameters between PH patients and healthy controls. In patients, RV-peak global longitudinal strain (GLS, rs = - 0.498, P < 0.001), RV- peak systolic strain rate (GSRs, rs = - 0.537, P < 0.001) and RV- peak early diastolic strain rate (GSRe, rs = 0.527, P < 0.001) significantly correlated with PVO2, but no significant correlation was observed between RV- peak late diastolic strain rate (GSRa, rs = 0.208, P = 0.093) and PVO2. The first multivariate regression analysis of clinical data without echocardiographic parameters identified WHO functional class, NT-proBNP and BMI as independent predictors of PVO2 (Model-1, adjusted r2 = 0.421, P < 0.001); Then we added conventional echocardiographic parameters and 2D-STE parameters to the clinical data, identified S,(Model-2,adjusted r2 = 0.502, P < 0.001), RV-GLS (Model-3, adjusted r2 = 0.491, P < 0.001), RV-GSRe (Model-4, adjusted r2 = 0.500, P < 0.001) and RV-GSRs (Model-5, adjusted r2 = 0.519, P < 0.001) as independent predictors of PVO2, respectively. The predictive power was increased, and Model-5 including RV-GSRs showed the highest predictive capability. ROC curves found RV-GSRs expressed the strongest predictive value (AUC = 0.88, P < 0.001), and RV-GSRs > - 0.65/s had a 88.2% sensibility and 82.2% specificity to predict PVO2 < 11 ml/min/kg. 2D-STE assessed RV function improves the prediction of exercise capacity represented by PVO2 in pre-capillary PH.


Assuntos
Ecocardiografia Doppler/métodos , Tolerância ao Exercício , Hipertensão Pulmonar/diagnóstico por imagem , Função Ventricular Direita , Adulto , Estudos de Casos e Controles , Estudos Transversais , Teste de Esforço , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Adulto Jovem
12.
Int J Cardiovasc Imaging ; 35(8): 1509-1524, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31049753

RESUMO

Multi-energy computed tomography (MECT) refers to acquisition of CT data at multiple energy levels (typically two levels) using different technologies such as dual-source, dual-layer and rapid tube voltage switching. In addition to conventional/routine diagnostic images, MECT provides additional image sets including iodine maps, virtual non-contrast images, and virtual monoenergetic images. These image sets provide tissue/material characterization beyond what is possible with conventional CT. MECT provides invaluable additional information in the evaluation of pulmonary vasculature, primarily by the assessment of pulmonary perfusion. This functional information provided by the MECT is complementary to the morphological information from a conventional CT angiography. In this article, we review the technique and applications of MECT in the evaluation of pulmonary vasculature.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Hemodinâmica , Hipertensão Pulmonar/diagnóstico por imagem , Tomografia Computadorizada Multidetectores/métodos , Imagem de Perfusão/métodos , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar , Embolia Pulmonar/diagnóstico por imagem , Humanos , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/terapia , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar/fisiopatologia , Embolia Pulmonar/terapia , Interpretação de Imagem Radiográfica Assistida por Computador
13.
Int J Cardiovasc Imaging ; 35(8): 1465-1472, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31123846

RESUMO

An accurate distinction between isolated post-capillary pulmonary hypertension (Ipc-PH) and combined post- and pre-capillary pulmonary hypertension (Cpc-PH) is integral to therapy and prognosis in heart failure (HF). This study aimed to compare the ability of four previously validated Doppler estimates of pulmonary vascular resistance (PVRDoppler) to distinguish Ipc-PH from Cpc-PH in a well-defined HF population. Consecutive subjects referred for HF assessment underwent standard echocardiography immediately followed by right heart catheterization (RHC). Subjects with atrial fibrillation, acute coronary syndrome, significant valvular disease or poor image quality were excluded. PVRDoppler estimates were correlated with invasive PVR and agreement was studied using Bland-Altman analysis. Receiver operating characteristics analyses were performed to determine the ability of PVRDoppler methods to identify PVR > 3WU. 55 HF subjects (58 ± 16 years, 55% Ipc-PH) were analyzed. PVRDoppler estimates demonstrated weak to modest associations with invasive PVR. The Doppler method proposed by Abbas et al. demonstrated relatively strong discriminatory ability to distinguish Ipc-PH from Cpc-PH (AUC = 0.79; 95% CI 0.63-0.96; p = 0.001). However, Bland-Altman analysis revealed wide limits of agreement (bias = 0; SD = 1.83WU) and greater variability at higher mean PVR. Conclusions: PVRDoppler estimates demonstrate reasonable ability to distinguish Ipc-PH from Cpc-PH but may not be reliable independent PH distinguishers in HF.


Assuntos
Ecocardiografia Doppler de Pulso , Insuficiência Cardíaca/complicações , Hipertensão Pulmonar/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar , Resistência Vascular , Adulto , Idoso , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Valor Preditivo dos Testes , Artéria Pulmonar/fisiopatologia , Reprodutibilidade dos Testes
14.
Life Sci ; 227: 64-73, 2019 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-31004656

RESUMO

AIM: Growing evidence suggests that endothelial-mesenchymal transition (EndMT) play key roles in pulmonary arterial remodeling during pulmonary arterial hypertension (PAH), but the underlying mechanisms have yet to be fully understood. miR-27a has been shown to promote proliferation of pulmonary arterial cells during PAH, but its role in EndMT remains unexplored. This study was designed to investigate the role and underlying mechanism of miR-27a in EndMT during PAH. MAIN METHODS: Rats were exposed in hypoxia (10% O2) for 3 weeks to induce PAH, and human pulmonary artery endothelial cells (HPAECs) were exposed in hypoxia (1% O2) for 48 h to induce EndMT. Immunohistochemistry, in situ hybridization, immunofluorescence, real-time PCR and Western blot were conducted to detect the expressions of RNAs and proteins, and luciferase assay was used to verify the putative binding site of miR-27a. KEY FINDINGS: We found that hypoxia up-regulated miR-27a in the tunica intima of rat pulmonary arteries and HPAECs, and that inhibition of miR-27a suppressed hypoxia-induced EndMT. Furthermore, elevated expression of miR-27a suppressed bone morphogenetic protein (BMP) signaling by targeting Smad5, thereby lessening Id2-mediated repression of the 2 critical mediators of EndMT (Snail and Twist). SIGNIFICANCE: Our data unveiled a novel role of miR-27a in EndMT during hypoxia-induced PAH. Thus, targeting of miR-27a-related pathway may be therapeutically harnessed to treat PAH.


Assuntos
Transição Epitelial-Mesenquimal/genética , Hipertensão Pulmonar/genética , MicroRNAs/fisiologia , Animais , Técnicas de Cultura de Células , Proliferação de Células , Células Endoteliais/metabolismo , Endotélio/metabolismo , Regulação da Expressão Gênica , Humanos , Hipertensão Pulmonar/fisiopatologia , Hipóxia/genética , Masculino , MicroRNAs/genética , MicroRNAs/metabolismo , Artéria Pulmonar/metabolismo , Ratos , Ratos Sprague-Dawley , Transdução de Sinais , Regulação para Cima , Remodelação Vascular/genética
15.
Nagoya J Med Sci ; 81(1): 19-30, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30962652

RESUMO

Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms of PH are pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), PH caused by left-heart disease, and PH due to lung disease. Previously regarded as untreatable, the treatment of PAH has dramatically advanced since the introduction of the drug epoprostenol in 1999, with three-year survival rates improving from 30%-40% to over 85%. Drugs available for the specific treatment of PAH include endothelin-receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogs, and prostacyclin-receptor agonists. In the past decade, management and treatment of CTEPH have also improved. While pulmonary endarterectomy used to be the only option for the treatment of CTEPH, newer treatments include a soluble guanylate cyclase stimulator, which has proven to be an efficacious targeted therapy. Other cases benefit from balloon pulmonary angioplasty.


Assuntos
Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/fisiopatologia , Animais , Endarterectomia , Antagonistas dos Receptores de Endotelina/uso terapêutico , Humanos , Hipertensão Pulmonar/cirurgia , Artéria Pulmonar/efeitos dos fármacos , Artéria Pulmonar/patologia , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar
16.
Pediatr Cardiol ; 40(5): 973-979, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30937503

RESUMO

Through this study, we aimed to assess the ability of routine neonatal screening at time of bronchopulmonary dysplasia (BPD) diagnosis to predict the development of late pulmonary hypertension (PHTN). This is a retrospective longitudinal cohort study of 37 premature infants with BPD assessing the utility of screening serum brain natriuretic peptide (BNP) and echocardiograms performed at the time of BPD diagnosis ('early PHTN') to predict 'late PHTN' at the last follow-up. Screening evaluation demonstrated early PHTN in 9/37 patients. At an average follow-up interval of 52.7 ± 38.7 weeks, 4/9 had late PHTN; one patient without early PHT had late PHT. At initial screening, infants with late PHTN were significantly more likely to have demonstrated elevated BNP values (p = 0.003), and echocardiographic evidence of right atrial dilatation (p = 0.01), right ventricular hypertrophy (p = 0.01), lower right ventricular area change percentage (p = 0.03), and larger main pulmonary artery Z-scores (p = 0.02). Serum BNP and echocardiographic evaluation performed at the time of BPD diagnosis can detect patients at increased risk of late PHTN. Large, prospective studies are necessary to further address this question.


Assuntos
Displasia Broncopulmonar , Ecocardiografia/métodos , Hipertensão Pulmonar , Peptídeo Natriurético Encefálico/sangue , Triagem Neonatal/métodos , Biomarcadores/sangue , Displasia Broncopulmonar/sangue , Displasia Broncopulmonar/complicações , Displasia Broncopulmonar/diagnóstico por imagem , Estudos de Casos e Controles , Feminino , Idade Gestacional , Humanos , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Estudos Longitudinais , Masculino , Estudos Prospectivos , Estudos Retrospectivos
17.
Int J Cardiovasc Imaging ; 35(9): 1581-1586, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30968263

RESUMO

A growing body of evidence has demonstrated that pulmonary arterial capacitance (PAC) is the strongest hemodynamic predictor of clinical outcomes across a wide spectrum of cardiovascular disease, including pulmonary hypertension and heart failure. We hypothesized that a ratio of right ventricular stroke volume (RVOT VTI) to the associated peak arterial systolic pressure (PASP) could function as a reliable non-invasive surrogate for PAC. We performed a prospective study of patients undergoing simultaneous transthoracic echocardiography and right heart catheterization (RHC) for various clinical indications. Measurements of the RVOT VTI/PASP ratio from echocardiographic measurements were compared against PAC calculated from RHC measurements. Correlation coefficients and Bland-Altman analysis compared the RVOT VTI/PASP ratio with PAC. Forty-five subjects were enrolled, 38% were female and mean age was 54 years (SD 13 years). The reason for referral to RHC was most commonly post-heart transplant surveillance (40%), followed by heart failure (22%), and pulmonary hypertension (18%). Pre-capillary pulmonary hypertension was present in 18%, isolated post-capillary pulmonary hypertension was present in 13%, and combined pre-and post-capillary pulmonary hypertension was present in 29%. The RVOT VTI/PASP ratio was obtainable in the majority of patients (78%), and Pearson's correlation demonstrated moderately-strong association between PAC and the RVOT VTI/PASP ratio, r = 0.75 (P < 0.001). Bland-Altman analysis demonstrated good agreement between measurements without suggestion of systematic bias and a mean difference in standardized units of - 0.133. In a diverse population of patients and hemodynamic profiles, we validated that the ratio of RVOT VTI/PASP to be a reliably-obtained non-invasive marker associated with PAC.


Assuntos
Pressão Arterial , Ecocardiografia Doppler , Hipertensão Pulmonar/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar , Volume Sistólico , Capacitância Vascular , Função Ventricular Direita , Adulto , Idoso , Cateterismo Cardíaco , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Artéria Pulmonar/fisiopatologia
18.
Eur J Radiol ; 114: 137-145, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31005164

RESUMO

PURPOSE: Cardiac magnetic resonance (CMR) is a substitute technique for noninvasively assessing pulmonary hemodynamics. Some preliminary studies have shown that CMR has the potential to quantify pulmonary vascular resistance (PVR). However, the evaluative value has not been well established. The purpose of the systematic review is to assess the feasibility of CMR in the measurement of PVR in patients with pulmonary hypertension (PH). METHODS: Studies were retrieved from multiple databases. Methodological evaluation of CMR and right heart catheterization (RHC) in estimating PVR were obtained from included studies. The Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool was used to assess the quality of studies. The results of comparisons of continuous variables are reported as weighted mean difference (WMD), together with the corresponding 95% confidence intervals (CIs). Summary correlation coefficient (r) values were extracted from each study, and 95% CIs were calculated after Fisher's z transformation. Sensitivity analysis was conducted to investigate potential heterogeneity. RESULTS: A total of 15 studies were included in the systematic review, and 6 of these studies were included in the meta-analysis. The pooled WMD with fixed-effects analysis revealed no statistical significance between PVR-CMR and PVR-RHC in patients with PH (WMD = 0.278 WU; 95% CI: -0.415 to 0.972; p = 0.431). The pooled r value for all studies was 0.85 (95% CI: 0.81, 0.89), and notable heterogeneity was evident. The pooled r value after the exclusion of one heterogeneous article was 0.81 (95% CI: 0.74, 0.87) and was not significantly heterogeneous. CONCLUSIONS: CMR and RHC have good consistency in the testing of PVR in the meta-analysis. The systematic review shows that completely noninvasive evaluation of PVR with CMR in patients with pH is feasible.


Assuntos
Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Espectroscopia de Ressonância Magnética , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Resistência Vascular/fisiologia , Cateterismo Cardíaco , Débito Cardíaco/fisiologia , Estudos de Viabilidade , Humanos , Monitorização Fisiológica
19.
Pediatr Cardiol ; 40(4): 820-826, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30830279

RESUMO

Red cell width distribution (RDW) is known to be a prognostic marker in adults with pulmonary hypertension. The value of this test in the pulmonary arterial hypertension (PAH) pediatric population was not yet established. The aim of the study was to evaluate the prognostic value of RDW in children with PAH and utility of this parameter in the management. Data were collected retrospectively in 61 patients with PAH confirmed by right heart catheterization. RDW was measured at diagnosis, 3 and 12 months after initial therapy, during and after deterioration if occurred. Results were compared with NTproBNP, WHO-FC and oxygen blood saturation. Mean RDW at baseline was 15.3 ± 2.4% (12.1-24.4, median 14.7%) and was elevated in 29 patients (47%). There were no significant difference in clinical status, NTproBNP and hemodynamic parameters among patient with normal and elevated RDW at diagnosis. Poor negative correlation with SaO2 and SvO2 was shown. After 3 and 12 months of treatment no significant change of RDW level was found despite of statistically significant improvement of WHO-FC and decrease of NTproBNP level (NS). Episodes of clinical deterioration weren't connected with change of RDW level (16 vs. 15.6% NS). Kaplan-Meier analysis did not show differences in prognosis between patients with normal and elevated RDW. Elevation of RDW was not associated with any measured parameters. Prognostic value of RDW in the pediatric PAH population was not confirmed. Usefulness of RDW in management in PAH pediatric population is limited and required further studies.


Assuntos
Índices de Eritrócitos , Hipertensão Pulmonar/sangue , Biomarcadores/sangue , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/métodos , Criança , Pré-Escolar , Feminino , Hemodinâmica/fisiologia , Humanos , Hipertensão Pulmonar/mortalidade , Hipertensão Pulmonar/fisiopatologia , Estimativa de Kaplan-Meier , Masculino , Peptídeo Natriurético Encefálico , Fragmentos de Peptídeos , Prognóstico , Estudos Retrospectivos
20.
Pediatr Cardiol ; 40(4): 841-847, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30830280

RESUMO

This study aimed to clarify the characteristics of pulmonary arterial resistance (Rp)-compliance (Cp) coupling in individuals with Down syndrome (DS), who have increased risks of pulmonary arterial hypertension (PAH). We performed cardiac catheterization before and after corrective surgery in 85 DS infants and 85 controls with congenital heart disease and PAH. We retrospectively collected hemodynamic data and compared Rp and Cp between the groups. Age at surgery was 3.5 (2.6-4.6) months. The first and second catheterizations were performed 1 month before and after corrective surgery in both groups. Preoperative Cp in DS patients was significantly lower than that in controls [2.27 (1.62-3.0) vs. 2.50 (1.86-3.31) mL/mmHg/m2, p = 0.039], although there was no significant difference in mean pulmonary arterial pressure and Rp between the groups. Analysis of covariance revealed that the slopes of the preoperative regression lines for the logarithmic transformations of Rp and Cp were identical in DS patients and controls (p = 0.299). However, the postoperative regression line was shifted downward in DS patients after corrective surgery. Postoperative home oxygen therapy (HOT) was performed in 39 patients (36 DS patients) and multivariate logistic regression analysis revealed that postoperative HOT was significantly related to low preoperative Cp (p = 0.039) and DS (p = 0.0001). Individuals with DS have the unique pulmonary vasculature characterized with low Cp that is related to postoperative HOT.


Assuntos
Síndrome de Down/complicações , Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Cateterismo Cardíaco/métodos , Estudos de Casos e Controles , Feminino , Cardiopatias Congênitas/cirurgia , Hemodinâmica/fisiologia , Humanos , Lactente , Masculino , Estudos Retrospectivos
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA