WILLIAM SALIBE FILHO
Projetos de Pesquisa
Unidades Organizacionais
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/09 - Laboratório de Pneumologia, Hospital das Clínicas, Faculdade de Medicina
LIM/09 - Laboratório de Pneumologia, Hospital das Clínicas, Faculdade de Medicina
10 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 10
- Update on pulmonary arteriovenous malformations(2023) SALIBE-FILHO, William; OLIVEIRA, Francini Rossetto de; TERRA-FILHO, MarioThis review aimed to provide an overview of pulmonary arteriovenous malformations, including the major clinical and radiological presentations, investigation, and treatment algorithm of the condition. The primary etiology of pulmonary arteriovenous malformations is hereditary hemorrhagic telangiectasia (HHT), also known as RenduOsler-Weber syndrome, with mutations in the ENG gene on chromosome 9 (HHT type 1) or in the ACVRL1/ ALK1 complex ( HHT type 2). Epistaxis should always be evaluated when repeated, when associated with anemia, and in some cases of hypoxemia. In the investigation, contrast echocardiography and chest CT are essential for evaluating this condition. Embolization is the best treatment choice, especially for correction in cases of hypoxemia or to avoid systemic infections. Finally, disease management was addressed in special conditions such as pregnancy. CT follow-up should be performed every 3- 5 years, depending on the size of the afferent and efferent vessels, and antibiotic prophylactic care should always be oriented. Ultimately, knowledge of the disease by health professionals is a crucial point for the early diagnosis of these patients in clinical practice, which can potentially modify the natural course of the disease.
- Use of medical therapies before pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension patients with severe hemodynamic impairment(2020) CASTRO, Marcela Araujo; PILOTO, Bruna; FERNANDES, Caio Julio Cesar dos Santos; JARDIM, Carlos; SALIBE FILHO, William; OLEAS, Francisca Gavilanes; ALVES, Jose Leonidas; MORINAGA, Luciana Tamie Kato; HOETTE, Susana; TERRA FILHO, Mario; FREITAS FILHO, Orival; JATENE, Fabio Biscegli; SOUZA, RogerioChronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, characterized by non-resolving fibro-thrombotic obstructions of large pulmonary arteries. Pulmonary endarterectomy (PEA) is the treatment of choice for the disease, significantly improving survival. Patients with worse hemodynamic profile have worse prognosis after surgery, raising the question of whether the use of medical therapy prior to surgery to optimize hemodynamics could improve outcomes. The aim of this study was to evaluate the role of medical therapy pre-PEA, according to the hemodynamic profile at the diagnosis. We retrospectively analyzed all patients submitted to PEA, from January 2013 to December 2017. Functional, clinical and hemodynamic data were collected to evaluate the main prognostic determinants. Patients were stratified according to the hemodynamic severity and use of targeted therapies prior to surgery. A total of 108 patients were included. Thirty-five patients (32,4%) used targeted therapy pre-PEA. The use of medical therapy delayed the surgical procedure by about 7 months. There was no difference in overall survival between patients that received targeted therapy and those treated only with supportive therapy (87.8% vs 80.3%, respectively, p = 0.426). Nevertheless, when analyzing the group of patients with severe hemodynamic impairment, defined by low cardiac output(< 3.7L/min) at baseline, patients treated with targeted therapies presented a significantly better one-year survival. In higher-risk CTEPH patients, characterized by the presence of low cardiac output, the use of targeted therapies prior to PEA was associated with better outcome, suggesting a potential role for pre-operative use of medical treatment in this particular subgroup.
- Loss of response to calcium channel blockers after long-term follow up in idiopathic pulmonary arterial hypertension(2020) PILOTO, Bruna; FERNANDES, Caio; JARDIM, Carlos; CASTRO, Marcela; SALIBE-FILHO, William; ALVES-JR, Jose Leonidas; MORINAGA, Luciana; SOBRAL-ALVES, Juliana; HOETTE, Susana; TERRA-FILHO, Mario; SOUZA, Rogerio
conferenceObject Cavities Lesions In Patients With Chronic Thromboembolic Pulmonary Hypertention(2017) OLIVEIRA, E. P.; FERNANDES, C. C.; OLEAS, F. G.; ALVES JUNIOR, J. L.; ATHANAZIO, R. A.; SALES, R. K. B.; MORAIS, I. O.; SALIBE FILHO, W.; JARDIM, C. V. P.; TERRA FILHO, M.; SOUZA, R.- The use of new anticoagulants in CTEPH(2017) GAVILANES, Francisca; ALVES JR., Jose Leonnidas; FERNANDES, Caio J. C.; PRADA, Luis F. L.; SALIBE FILHO, William; TERRA FILHO, Mario; JARDIM, Carlos; SOUZA, Rogerio
- Shear stress-exposed pulmonary artery endothelial cells fail to upregulate HSP70 in chronic thromboembolic pulmonary hypertension(2020) SALIBE-FILHO, William; ARAUJO, Thais L. S.; MELO, Everton G.; COIMBRA, Luiza B. C. T.; LAPA, Monica S.; ACENCIO, Milena M. P.; FREITAS-FILHO, Orival; CAPELOZZI, Vera Luiza; TEIXEIRA, Lisete Ribeiro; FERNANDES, Caio J. C. S.; JATENE, Fabio Biscegli; LAURINDO, Francisco R. M.; TERRA-FILHO, MarioThe pathophysiological mechanisms underlying chronic thromboembolic pulmonary hypertension (CTEPH) are still unclear. Endothelial cell (EC) remodeling is believed to contribute to this pulmonary disease triggered by thrombus and hemodynamic forces disbalance. Recently, we showed that HSP70 levels decrease by proatherogenic shear stress. Molecular chaperones play a major role in proteostasis in neurological, cancer and inflammatory/ infectious diseases. To shed light on microvascular responses in CTEPH, we characterized the expression of molecular chaperones and annexin A2, a component of the fibrinolytic system. There is no animal model that reproduces microvascular changes in CTEPH, and this fact led us to isolated endothelial cells from patients with CTEPH undergoing pulmonary endarterectomy (PEA). We exposed CTEPH-EC and control human pulmonary endothelial cells (HPAEC) to high- (15 dynes/cm(2)) or low- (5 dynes/cm(2)) shear stress. After high-magnitude shear stress HPAEC upregulated heat shock protein 70kDa (HSP70) and the HSP ER paralogs 78 and 94kDa glucose-regulated protein (GRP78 and 94), whereas CTEPH-ECs failed to exhibit this response. At static conditions, both HSP70 and HSP90 families in CTEPH-EC are decreased. Importantly, immunohistochemistry analysis showed that HSP70 expression was downregulated in vivo, and annexin A2 was upregulated. Interestingly, wound healing and angiogenesis assays revealed that HSP70 inhibition with VER-155008 further impaired CTEPH-EC migratory responses. These results implicate HSP70 as a novel master regulator of endothelial dysfunction in type 4 PH. Overall, we first show that global failure of HSP upregulation is a hallmark of CTEPH pathogenesis and propose HSP70 as a potential biomarker of this condition.
- Lung Cavities in Chronic Thromboembolic Pulmonary Hypertension(2020) FERNANDES, Caio Julio Cesar dos Santos; OLIVEIRA, Ellen Pierre de; SALIBE-FILHO, Willian; TERRA-FILHO, Mario; JARDIM, Carlos Vianna Poyares; KATO-MORINAGA, Luciana Tamie; HOETTE, Susana; SOUZA, Rogerio deOBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients. METHODS: A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016. RESULTS: Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified. CONCLUSION: Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.
- Update on the Treatment of Pulmonary Arterial Hypertension(2021) FERNANDES, Caio J.; CALDERARO, Daniela; ASSAD, Ana Paula Luppino; SALIBE-FILHO, William; KATO-MORINAGA, Luciana Tamie; HOETTE, Susana; PILOTO, Bruna; CASTRO, Marcela Araujo; LISBOA, Roberta Pontes; SILVA, Taysa Antonia Felix da; MARTINS, Murillo de Araujo; ALVES-JR, Jose L.; JARDIM, Carlos; TERRA-FILHO, Mario; SOUZA, Rogerio deIn the last decades, important advances have been made in the treatment of pulmonary arterial hypertension (PAH), a severe, progressive, incurable, and potentially fatal disease. For an adequate therapy, correct hemodynamic diagnosis and etiology classification are fundamental. Many etiologies - rheumatic disease, portal hypertension, congenital heart diseases, schistosomiasis - require specific measures, in addition to drug therapy for PAH. The specific therapy for PAH is based on medications that act on three pathophysiological pathways - prostacyclin, endothelin, and nitric oxide pathways. These drugs have multiple presentations (oral, intravenous, subcutaneous, and inhaled) and have changed the history of PAH. This review presents an overview of drug therapy strategies and different forms and peculiarities of PAH.
- Pulmonary arteriovenous malformations: diagnostic and treatment characteristics(2019) SALIBE-FILHO, William; PILOTO, Bruna Mamprim; OLIVEIRA, Ellen Pierre de; CASTRO, Marcela Araujo; AFFONSO, Breno Boueri; MOTTA-LEAL-FILHO, Joaquim Mauricio da; BORTOLINI, Edgar; TERRA-FILHO, MarioObjective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO(2) of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO(2) of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO(2) > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.
- Use of direct oral anticoagulants for chronic throm-boembolic pulmonary hypertension(2018) GAVILANES-OLEAS, Francisca Alexandra; ALVES-JR, Jose Leonidas; FERNANDES, Caio Julio Cesar; PRADA, Luis Felipe Lopes; SALIBE-FILHO, William; TERRA-FILHO, Mario; MORINAGA, Luciana; HOETTE, Susana; JARDIM, Carlos; SOUZA, RogerioOBJECTIVES: Chronic thromboembolic pulmonary hypertension is one of the most prevalent forms of pulmonary hypertension and is a major complication of acute pulmonary embolism. One mainstay of chronic thromboembolic pulmonary hypertension treatment is lifelong anticoagulation. The recent advent of direct oral anticoagulants for acute pulmonary embolism treatment has provided a viable and effective alternative for treating this condition. However, little is known about the efficacy of this new class of drugs for treating chronic thromboembolic pulmonary hypertension. We aimed to evaluate the safety and efficacy of direct oral anticoagulants in the treatment of chronic thromboembolic pulmonary hypertension. METHODS: A cohort of chronic thromboembolic pulmonary hypertension patients who initiated treatment with direct oral anticoagulants between June 2015 and November 2016 were enrolled in this study. RESULTS: Sixteen patients used rivaroxaban, three used dabigatran and one used apixaban for a mean follow-up of 20.9 months. The mean age was 51 years, and eighteen patients were classified as functional class Eight patients underwent a pulmonary endarterectomy and exhibited clinical, hemodynamic and functional improvement and currently continue to use direct oral anticoagulants. No episode of venous thromboembolism recurrence was identified during the follow-up period, but there was one episode of major bleeding after a traumatic fall. CONCLUSIONS: Although direct oral anticoagulants appear to be a safe and effective alternative for treating chronic thromboembolic pulmonary hypertension, larger studies are needed to support their routine use.