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1.
BMJ ; 367: l5553, 2019 Oct 22.
Artigo em Inglês | MEDLINE | ID: mdl-31641045

RESUMO

Sarcoidosis is a highly variable granulomatous multisystem syndrome. It affects individuals in the prime years of life; both the frequency and severity of sarcoidosis are greater in economically disadvantaged populations. The diagnosis, assessment, and management of pulmonary sarcoidosis have evolved as new technologies and therapies have been adopted. Transbronchial needle aspiration guided by endobronchial ultrasound has replaced mediastinoscopy in many centers. Advanced imaging modalities, such as fluorodeoxyglucose positron emission tomography scanning, and the widespread availability of magnetic resonance imaging have led to more sensitive assessment of organ involvement and disease activity. Although several new insights about the pathogenesis of sarcoidosis exist, no new therapies have been specifically developed for use in the disease. The current or proposed use of immunosuppressive medications for sarcoidosis has been extrapolated from other disease states; various novel pathways are currently under investigation as therapeutic targets. Coupled with the growing recognition of corticosteroid toxicities for managing sarcoidosis, the use of corticosteroid sparing anti-sarcoidosis medications is likely to increase. Besides treatment of granulomatous inflammation, recognition and management of the non-granulomatous complications of pulmonary sarcoidosis are needed for optimal outcomes in patients with advanced disease.


Assuntos
Glucocorticoides/uso terapêutico , Hipertensão Pulmonar/prevenção & controle , Imunossupressores/uso terapêutico , Fibrose Pulmonar/prevenção & controle , Sarcoidose Pulmonar/diagnóstico , Biomarcadores/sangue , Broncoscopia/métodos , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Fluordesoxiglucose F18/administração & dosagem , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Incidência , Pulmão/diagnóstico por imagem , Pulmão/patologia , Tomografia por Emissão de Pósitrons/métodos , Prognóstico , Fibrose Pulmonar/etiologia , Fibrose Pulmonar/mortalidade , Sarcoidose Pulmonar/complicações , Sarcoidose Pulmonar/tratamento farmacológico , Sarcoidose Pulmonar/epidemiologia , Resultado do Tratamento
2.
Pan Afr Med J ; 33: 156, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31565118

RESUMO

Cardiac manifestations develop in the majority of patients with systemic lupus erythematosus (SLE) at some time during the course of their disease. This study was designed to assess cardiac abnormalities in patients with SLE by echocardiography and to compare the 2 groups of patients with and without cardiac manifestations. It was a transversal, descriptive study, conducted in the Internal Medicine Department at the Military Hospital of Tunis from January 2016 to June 2018. Eighty lupus patients, diagnosed on the basis of ACR (American college of rheumatology) criteria, were enrolled in the study and were evaluated by standard echocardiography with color Doppler. Out of 80 patients 42 (52%) had abnormal echocardiographic findings. Pericardial effusion was found in 55%, valvular abnormalities in 52% and 38% had pulmonary hypertension. Patients with pleural effusion (45 vs 15%) were more vulnerable to cardiac involvement as well as renal impairment (57 vs 44%). The difference, however, were not statistically significant (p>0.05) in the renal involvement. Active disease with low complement (80%) was associated with higher frequency of cardiac involvement than disease in remission (64%) but the result was not statistically significant (p=0.11). Cardiac abnormalities are very common in lupus patients even when clinically asymptomatic form. Echocardiography is an excellent non-invasive tool for cardiac evaluation. Their research must be systematic with echocardiography in order to reduce subsequent cardiac morbidity and mortality among the lupus patients.


Assuntos
Ecocardiografia/métodos , Cardiopatias/diagnóstico por imagem , Lúpus Eritematoso Sistêmico/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Cardiopatias/epidemiologia , Cardiopatias/etiologia , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/epidemiologia , Doenças das Valvas Cardíacas/etiologia , Hospitais Militares , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Masculino , Pessoa de Meia-Idade , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/epidemiologia , Derrame Pericárdico/etiologia , Derrame Pleural/diagnóstico por imagem , Derrame Pleural/epidemiologia , Derrame Pleural/etiologia , Tunísia
3.
Isr Med Assoc J ; 21(8): 528-531, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31474014

RESUMO

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, distinct pulmonary vascular disease, which is caused by chronic obstruction of major pulmonary arteries. CTEPH can be cured by pulmonary endarterectomy (PEA). PEA for CTEPH is a challenging procedure, and patient selection and the perioperative management are complex, requiring significant experience. OBJECTIVES: To describe the establishment of a national CTEPH-PEA center in Israel and present results of surgery. METHODS: In this study, we reviewed the outcomes of PEA in a national referral, multi-disciplinary center for CTEPH-PEA. The center was established by collaborating with a high-volume center in Europe. A multidisciplinary team from our hospital (pulmonary hypertension specialist, cardiac surgeon, cardiac anesthesiologist and cardiac surgery intensivist was trained under the guidance of an experienced team from the European center. RESULTS: A total of 38 PEA procedures were performed between 2008 and 2018. We included 28 cases in this analysis for which long-term follow-up data were available. There were two hospital deaths (7%). At follow-up, median New York Heart Association (NYHA) class improved from III to I (P < 0.0001), median systolic pulmonary pressure decreased from 64 mmHg to 26 mmHg (P < 0.0001), and significant improvements were seen in right ventricular function and exercise capacity. CONCLUSIONS: A national center for performance of a rare and complex surgical procedure can be successfully established by collaboration with a high-volume center and by training a dedicated multidisciplinary team.


Assuntos
Endarterectomia/métodos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/cirurgia , Embolia Pulmonar/complicações , Embolia Pulmonar/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Feminino , Humanos , Israel , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/cirurgia , Encaminhamento e Consulta , Fatores de Risco , Resultado do Tratamento , Adulto Jovem
4.
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
5.
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
6.
Lakartidningen ; 1162019 Mar 26.
Artigo em Sueco | MEDLINE | ID: mdl-31192396

RESUMO

Pulmonary hypertension is a common consequence of left heart disease, associated with poor prognosis. The pulmonary hypertension in left heart disease is initially caused by a passive congestion of the pulmonary circuit but may, if longstanding, result in endothelial dysfunction and excessive vasoconstriction. In some cases pulmonary vascular remodeling occur, further complicating the condition and worsening the prognosis. It has been debated whether these patients may benefit from pulmonary vasodilators presently used in pulmonary arterial hypertension. Several randomized controlled trials have been performed on this subject, the vast majority being negative. As maintenance therapy may be harmful, they should be avoided outside clinical trials.


Assuntos
Hipertensão Pulmonar , Vasodilatadores/efeitos adversos , Disfunção Ventricular Esquerda/complicações , Contraindicações de Medicamentos , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/fisiopatologia , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Vasodilatadores/uso terapêutico , Disfunção Ventricular Esquerda/tratamento farmacológico , Disfunção Ventricular Esquerda/fisiopatologia
7.
J Forensic Sci ; 64(6): 1916-1920, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31150105

RESUMO

Atrial septal defects (ASDs) are one of the most prevalent congenital cardiac anomalies in adults. These interatrial communications can produce changes in the right heart (remodeling and failure) and the lungs (pulmonary hypertension). Most adults with ASDs are surgically treated with excellent results. However, a small fraction of patients is at risk for postoperative complications, particularly the persistence of pulmonary hypertension. A case of a 47-year-old woman who was found unresponsive in the bathroom of her house and died despite resuscitative efforts is described. According to medical records, the woman underwent a surgical repair of an atrial septal defect at the age of 37. At the autopsy, macroscopic and microscopic signs of advanced pulmonary hypertension were detected, highlighting the importance for the forensic pathologists to recognize pulmonary hypertension as a cause of sudden death in adults with a history of late surgical closure of an atrial septal defect.


Assuntos
Morte Súbita/etiologia , Comunicação Interatrial/cirurgia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/patologia , Feminino , Comunicação Interatrial/complicações , Humanos , Hipertrofia Ventricular Direita/patologia , Pulmão/patologia , Pessoa de Meia-Idade , Miocárdio/patologia , Tamanho do Órgão , Placa Aterosclerótica/patologia , Artéria Pulmonar/patologia , Túnica Íntima/patologia
8.
Hosp Pract (1995) ; 47(3): 130-135, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31177873

RESUMO

Objectives: To identify predictors of pulmonary hypertension (PHT) and the predictive value of PHT for rehospitalization among patients with heart failure with reduced ejection fraction (HFrEF). Methods: A retrospective study of 351 hospitalized patients with heart failure (HF). Patients 18 years and above with HFrEF secondary to non-ischemic cardiomyopathy were reviewed. Patients with coronary artery disease, preserved ejection fraction and other secondary causes of PHT apart from HF were excluded. PHT as a predictor of 30-day and six-month re-admission was assessed as well as important possible predictors of PHT. Cox regression analysis, multiple linear regression as well as other statistical tools were employed as deemed appropriate. Results: Thirty-seven (37) and 99 patients were re-hospitalized within 30 days and 6 months after discharge for decompensated HF, respectively. After Cox regression analysis, higher hemoglobin reduced the odds of rehospitalization for decompensated HF (p = 0.015) within 30 days after discharge while higher pulmonary artery systolic pressure (PASP) (p = 0.002) and blood urea nitrogen (BUN) (p = 0.041) increased the odds of rehospitalization within 6 months of discharge. The predictors of the PHT among patients with HFrEF after multiple linear regression were low BMI (p = 0.027), increasing age (p = 0.006) and increased left atrial diameter (LAD) on echocardiography (p = 0.0001). Conclusion: Patients with HFrEF have a high predisposition to developing PHT if at admission, they have low BMI, dilated left atrium or are older. Patients with one or more of these attributes may need more intensive therapy to reduce the risk of developing PHT and in turn reduce readmission rates.


Assuntos
Insuficiência Cardíaca/complicações , Hipertensão Pulmonar/etiologia , Avaliação de Resultados (Cuidados de Saúde) , Readmissão do Paciente , Volume Sistólico , Idoso , Idoso de 80 Anos ou mais , Ecocardiografia , Feminino , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Alta do Paciente , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos
9.
J Stroke Cerebrovasc Dis ; 28(9): e135-e138, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31253482

RESUMO

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic thrombi in the pulmonary arteries, causing pulmonary hypertension and right heart failure. Early and accurate diagnosis are essential for successful treatment but are often difficult because clinical signs and symptoms can be nonspecific and risk factors, such as history of venous thromboembolism, may not always be present. Here, we report a case involving a 76-year-old woman who demonstrated paradoxical cerebral embolism as the initial manifestation of CTEPH. She developed right hemiplegia without dyspnea or edema. Brain magnetic resonance imaging revealed multiple fresh infarctions, while transesophageal echocardiography revealed a patent foramen ovale. Based on these findings, she was diagnosed as having paradoxical cerebral embolism. During the search for the embolic source, right heart catheterization showed significant pulmonary hypertension and pulmonary angiography revealed chronic thrombi in the peripheral pulmonary arteries, consistent with a diagnosis of CTEPH. To our knowledge, this is the first case of CTEPH to be diagnosed with the onset of paradoxical cerebral embolism. Because CTEPH is the only potentially curable form of pulmonary hypertension, clinicians should consider paradoxical cerebral embolism as a possible initial manifestation of CTEPH.


Assuntos
Arteriopatias Oclusivas/complicações , Embolia Paradoxal/etiologia , Hipertensão Pulmonar/etiologia , Embolia Intracraniana/etiologia , Trombose/complicações , Idoso , Arteriopatias Oclusivas/diagnóstico por imagem , Doença Crônica , Angiografia por Tomografia Computadorizada , Imagem de Difusão por Ressonância Magnética , Ecocardiografia Doppler em Cores , Ecocardiografia Transesofagiana , Embolia Paradoxal/diagnóstico por imagem , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Embolia Intracraniana/diagnóstico por imagem , Trombose/diagnóstico por imagem
10.
Int Heart J ; 60(4): 924-937, 2019 Jul 27.
Artigo em Inglês | MEDLINE | ID: mdl-31204374

RESUMO

Our previous studies have revealed that long noncoding RNAs (lncRNAs), microRNAs (miRNAs), and genes were abnormally expressed in the pulmonary artery tissues of the chronic thromboembolic pulmonary hypertension (CTEPH) patients. We aim to establish the CTEPH-related miRNA-gene-lncRNA network for finding the core genes and associated miRNA and lncRNA in CTEPH patients.Firstly, the target genes of differential miRNAs were predicted by searching TargetScan databases, and the predicted target genes were intersected with the mRNAs from the gene chip. Secondly, the intersective genes were analyzed by the Gene Ontology function and Kyoto Encyclopedia of Genes and Genomes pathway software for obtaining differential intersective genes and then establish the miRNA-gene networks. Thirdly, the possible genes regulated by the differential lncRNAs from the gene chip were intersected with the above-screened mRNA to build the lncRNA-mRNA networks. Subsequently, the miRNA-gene-lncRNA networks were constructed according to the two networks above (miRNA-gene networks and lncRNA-mRNA networks). Finally, the core genes of the networks in the experimental group were screened according to Diffk > 0.6 and used to construct the miRNA-core gene-lncRNA networks of CTEPH.The pathway network, miRNA-mRNA network, lncRNA-mRNA networks, and miRNA-gene-lncRNA networks were successfully constructed. The core genes of the miRNA-gene-lncRNA networks (Diffk > 0.6) were the human Beta-type platelet-derived growth factor receptor (PDGFRB) and hypoxia-inducible factor-1a (HIF-1A), the miRNAs-PDGFRB-lncRNAs and miRNAs-HIF1A-lncRNAs networks were constructed. Finally, miRNA-149-5p-PDGFRB-TCONS_l2_00020587-XLOC_l2_010723 and miRNA-338-5p/miRNA-199b-5p-HIF1A- TCONS_l2_00020587-XLOC_l2_010723 were found in the analysis of the network.miRNA-149-5p-PDGFRB-lncRNA CTEPH-associated 1 (CTEPHA1) (TCONS_l2_00020587-XLOC_l2_010723) and miRNA-338-5p/miRNA-199b-5p-HIF1A-lncRNA CTEPHA1 are related to the development of CTEPH.


Assuntos
Hipertensão Pulmonar/genética , Subunidade alfa do Fator 1 Induzível por Hipóxia/genética , MicroRNAs/genética , Proteínas Proto-Oncogênicas c-sis/genética , Embolia Pulmonar/complicações , Doença Crônica , Perfilação da Expressão Gênica , Redes Reguladoras de Genes , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/metabolismo , Subunidade alfa do Fator 1 Induzível por Hipóxia/metabolismo , MicroRNAs/metabolismo , Proteínas Proto-Oncogênicas c-sis/metabolismo , Embolia Pulmonar/genética , Embolia Pulmonar/metabolismo
11.
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
12.
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
13.
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
14.
Immunology ; 157(4): 304-311, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31141166

RESUMO

Pulmonary hypertension (PH) is a common but dangerous complication in chronic obstructive pulmonary disease (COPD). We hypothesized that dysregulation in the T helper type 17 (Th17) compartment could contribute to the development of COPD-associated PH (COPD-PH). To investigate this hypothesis, patients with COPD-PH and age- and sex-matched healthy controls were recruited, and their circulating CD4+ T cells were activated using anti-CD3/CD28 antibodies. The frequency of interleukin-17 (IL-17) -secreting cells was significantly higher in COPD-PH patients than in healthy controls. The secretion of IL-17 was significantly higher from COPD-PH CD4+ T cells than from control CD4+ T cells, whereas the secretion of interferon-γ and IL-4 was not significantly different. The expression of transforming growth factor-ß, on the other hand, was significantly higher in healthy controls than in COPD-PH patients. Activated CD4+ T cells from COPD-PH patients also presented significantly lower forkhead box P3 (FOXP3) and higher retinoic acid receptor-related orphan C2 (RORC2) expression than CD4+ T cells from healthy controls. In both controls and patients, a negative correlation between RORC2 and FOXP3 was found, ex vivo and after CD3/CD28 activation. The serum IL-6 level was slightly higher in COPD-PH patients than in controls, but the IL-6 transcription by monocytes was comparable in COPD-PH patients and controls. Interestingly, CD4+ T cells from COPD-PH patients presented significantly higher levels of Kirsten rat sarcoma viral oncogene homolog and neuroblastoma RAS viral oncogene homolog than CD4+ T cells from healthy controls. Inhibiting Ras-GTPases using farnesylthiosalicylic acid significantly reduced the ratio of RORC2/FOXP3 expression in CD4+ T cells. Overall, we demonstrated that an imbalance of Th17/regulatory T cells was a hallmark of COPD-PH.


Assuntos
Hipertensão Pulmonar/imunologia , Doença Pulmonar Obstrutiva Crônica/imunologia , Proteínas ras/imunologia , Idoso , Feminino , Fatores de Transcrição Forkhead/imunologia , Regulação da Expressão Gênica/imunologia , Humanos , Hipertensão Pulmonar/etiologia , Interferon gama/imunologia , Interleucina-4/imunologia , Masculino , Pessoa de Meia-Idade , Membro 3 do Grupo F da Subfamília 1 de Receptores Nucleares/imunologia , Doença Pulmonar Obstrutiva Crônica/complicações , Células Th17 , Fator de Crescimento Transformador beta
15.
Transplant Proc ; 51(5): 1435-1438, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31079941

RESUMO

Portopulmonary hypertension (PPHTN) is a rare complication of liver cirrhosis. Patients with severe PPHTN are contraindicated for liver transplant because of the associated risk of intraoperative acute right heart failure during reperfusion phase or massive volume infusion. Therefore, it has been recommended that patients with moderate to severe PPHTN undergo medical treatment to lower the pulmonary artery pressure before undergoing transplant. Herein, we report 3 patients with severe PPHTN who underwent sildenafil monotherapy before living donor liver transplant. None of the patients experienced associated adverse effects during sildenafil treatment, and the pulmonary artery pressure was effectively reduced before transplant. The first patient was diagnosed during anesthesia prior to transplant, and the mean pulmonary artery pressure was reduced by 34% after treatment. The second and third patients were followed-up with echography, and the estimated pulmonary artery systolic pressure were reduced by 34% and 47%, respectively. Pretransplant right heart catheterization also confirmed the reduction of the mean pulmonary artery pressure. Intraoperative hemodynamic parameters were stable, and the 3 patients were discharged uneventfully. After transplant, sildenafil was discontinued, and all patients remained in a stable clinical and functional status during follow-up.


Assuntos
Hipertensão Portal/tratamento farmacológico , Hipertensão Pulmonar/tratamento farmacológico , Transplante de Fígado/métodos , Citrato de Sildenafila/uso terapêutico , Vasodilatadores/uso terapêutico , Anti-Hipertensivos/uso terapêutico , Feminino , Humanos , Hipertensão Portal/etiologia , Hipertensão Pulmonar/etiologia , Cirrose Hepática/complicações , Cirrose Hepática/cirurgia , Masculino , Pessoa de Meia-Idade
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.
Med Clin North Am ; 103(3): 413-423, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30955510

RESUMO

Pulmonary hypertension (PH) is a chronic and progressive disease that presents like many other lung diseases, often leading to a delay in diagnosis, and therefore a delay in optimal therapy. This article provides a review of PH for internists, covering clinical presentation, diagnostic algorithm, different types of PH, and overview of treatments. In addition, it emphasizes the importance of early referral to, and partnership between, PH specialists and physicians on the front lines to improve early diagnosis and optimize management of these complex patients.


Assuntos
Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/terapia , Humanos , Hipertensão Pulmonar/classificação , Hipertensão Pulmonar/etiologia , Encaminhamento e Consulta , Medição de Risco , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/terapia
18.
Br J Anaesth ; 122(4): 437-447, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30857600

RESUMO

BACKGROUND: A pulmonary hypertensive crisis (PHC) can be a life-threatening condition. We established a PHC model by exposing rats with monocrotaline (MCT)-induced pulmonary hypertension to acute hypoxia, and investigated the effects of vasopressin, phenylephrine, and norepinephrine on the PHC. METHODS: Four weeks after MCT 60 mg kg-1 administration i.v., right ventricular systolic pressure (RVSP), systolic BP (SBP), mean BP (MBP), cardiac index (CI), and pulmonary vascular resistance index (PVRI) were measured. PHC defined as an RVSP exceeding or equal to SBP was induced by changing the fraction of inspiratory oxygen to 0.1. Rats were subsequently treated by vasopressin, phenylephrine, or norepinephrine, followed by assessment of systemic haemodynamics, isometric tension of femoral and pulmonary arteries, cardiac function, blood gas composition, and survival. RESULTS: PHC was associated with increased RV dilatation and paradoxical septal motion. Vasopressin increased MBP [mean (standard error)] from 52.6 (3.8) to 125.0 (8.9) mm Hg and CI from 25.4 (2.3) to 40.6 (1.8) ml min-1 100 g-1 while decreasing PVRI. Vasopressin also improved RV dilatation, oxygenation, and survival in PHC. In contrast, phenylephrine increased MBP from 54.8 (2.3) to 96.8 (3.2) mm Hg without improving cardiac pump function. Norepinephrine did not alter MBP. Vasopressin contracted femoral but not pulmonary arteries, whereas phenylephrine contracted both arterial beds. Hence, improvements with vasopressin in PHC might be associated with decreased PVRI and selective systemic vasoconstriction. CONCLUSIONS: In this rat model of a PHC, vasopressin, but not phenylephrine or norepinephrine, resulted in better haemodynamic and vascular recovery.


Assuntos
Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Hipóxia/complicações , Vasoconstritores/uso terapêutico , Vasopressinas/uso terapêutico , Doença Aguda , Animais , Avaliação Pré-Clínica de Medicamentos/métodos , Ecocardiografia , Hemodinâmica/efeitos dos fármacos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Hipóxia/fisiopatologia , Masculino , Monocrotalina , Nordefrin/farmacologia , Oxigênio/sangue , Pressão Parcial , Fenilefrina/farmacologia , Ratos Sprague-Dawley , Vasoconstrição/efeitos dos fármacos , Vasoconstritores/farmacologia , Vasopressinas/farmacologia
19.
Int J Mol Sci ; 20(6)2019 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-30909527

RESUMO

Pulmonary arterial hypertension (PAH) is characterized by pulmonary arterial proliferation and remodeling, resulting in a specific increase in right ventricle systolic pressure (RVSP) and, ultimately right ventricular failure. Recent studies have demonstrated that caffeic acid phenethyl ester (CAPE) exerts a protective role in NF-κB-mediated inflammatory diseases. However, the effect of CAPE on PAH remains to be elucidated. In this study, monocrotaline (MCT) was used to establish PAH in rats. Two weeks after the induction of PAH by MCT, CAPE was administrated by intraperitoneal injection once a day for two weeks. Pulmonary hemodynamic measurements and pulmonary artery morphological assessments were examined. Our results showed that administration of CAPE significantly suppressed MCT-induced vascular remodeling by decreasing the HIF-1α expression and PDGF-BB production, and improved in vivo RV systolic performance in rats. Furthermore, CAPE inhibits hypoxia- and PDGF-BB-induced HIF-1α expression by decreasing the activation of the AKT/ERK pathway, which results in the inhibition of human pulmonary artery smooth muscle cells (hPASMCs) proliferation and prevention of cells resistant to apoptosis. Overall, our data suggest that HIF-1α is regarded as an alternative target for CAPE in addition to NF-κB, and may represent a promising therapeutic agent for the treatment of PAH diseases.


Assuntos
Ácidos Cafeicos/farmacologia , MAP Quinases Reguladas por Sinal Extracelular/metabolismo , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/metabolismo , Subunidade alfa do Fator 1 Induzível por Hipóxia/metabolismo , Álcool Feniletílico/análogos & derivados , Fator de Crescimento Derivado de Plaquetas/metabolismo , Proteínas Proto-Oncogênicas c-akt/metabolismo , Animais , Apoptose/efeitos dos fármacos , Linhagem Celular , Proliferação de Células/efeitos dos fármacos , Modelos Animais de Doenças , Expressão Gênica , Hemodinâmica/efeitos dos fármacos , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Hipertrofia Ventricular Direita/tratamento farmacológico , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/metabolismo , Hipertrofia Ventricular Direita/fisiopatologia , Imuno-Histoquímica , Álcool Feniletílico/farmacologia , Fator de Crescimento Derivado de Plaquetas/genética , Artéria Pulmonar/efeitos dos fármacos , Artéria Pulmonar/metabolismo , Artéria Pulmonar/fisiopatologia , Ratos , Transdução de Sinais/efeitos dos fármacos , Remodelação Vascular/efeitos dos fármacos
20.
Medicine (Baltimore) ; 98(12): e14594, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30896614

RESUMO

RATIONALE: Pulmonary hypertension (PH) is a complicated disease which has complex causes and poor outcome. Many factors are involved in the increase of pulmonary artery pressure. It is often difficult to identify the specific cause of a particular patient. However, identifying the etiology is of great importance for specifying treatment strategies and improving the prognosis of patients. PATIENT CONCERNS: A 58-year-old male was admitted because of fatigue, breath shortness for 6 months, which got worse in the last 3 months. The ultrasound cardiogram (UCG) indicated a remarkably elevated pulmonary artery systolic pressure (PASP = 82 mm Hg). He had hypertension for 15 years. Besides, his spleen was found to be enlarged since 15 years ago. Bone marrow biopsy of the patient revealed myeloproliferative neoplasm (MPN) with severe myelofibrosis (MF). DIAGNOSIS: Myeloproliferative neoplasm (MPN) with severe myelofibrosis (MF) which in turn caused PH and portal vein hypertension (PVH). INTERVENTIONS: We treated the patient with diuretics and fosinopril, and also steroids and thalidomide for his MPN/MF. OUTCOMES: Two weeks later, the pulmonary artery pressure (PAP) was remarkably decreased (PASP = 53.1 mm Hg by UCG, mean PAP = 21 mm Hg by right cardiac catheterization). Within 2 years' follow-up, his circulatory state and hematological state remained stable. LESSONS: It is often difficult to define the cause of PH, but it is important for making the appropriate treatment at the same time.


Assuntos
Hipertensão Portal/etiologia , Hipertensão Pulmonar/etiologia , Transtornos Mieloproliferativos/complicações , Mielofibrose Primária/complicações , Esplenomegalia/etiologia , Humanos , Janus Quinase 2/genética , Masculino , Pessoa de Meia-Idade , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/genética
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