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1.
J Bras Pneumol ; 46(4): e20200204, 2022.
Article in English, Portuguese | MEDLINE | ID: mdl-35766678

ABSTRACT

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Brazil , Chronic Disease , Endarterectomy/adverse effects , Endarterectomy/methods , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/therapy , Pulmonary Artery/surgery , Pulmonary Embolism/complications , Pulmonary Embolism/diagnosis , Pulmonary Embolism/therapy
2.
Clinics (Sao Paulo) ; 75: e1373, 2020.
Article in English | MEDLINE | ID: mdl-31939560

ABSTRACT

OBJECTIVES: 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.


Subject(s)
Granulomatous Disease, Chronic , Hypertension, Pulmonary/diagnosis , Pulmonary Embolism/diagnosis , Thromboembolism/etiology , Angiography/methods , Anticoagulants/therapeutic use , Chronic Disease , Female , Granulomatous Disease, Chronic/pathology , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/therapy , Lung/blood supply , Male , Perfusion Imaging , Pulmonary Embolism/complications , Pulmonary Embolism/therapy , Retrospective Studies , Tomography, X-Ray Computed/methods , Treatment Outcome
3.
Clinics ; 75: e1373, 2020. tab, graf
Article in English | LILACS | ID: biblio-1055875

ABSTRACT

OBJECTIVES: 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.


Subject(s)
Humans , Male , Female , Pulmonary Embolism/diagnosis , Thromboembolism/etiology , Granulomatous Disease, Chronic/pathology , Hypertension, Pulmonary/diagnosis , Pulmonary Embolism/complications , Pulmonary Embolism/therapy , Angiography/methods , Tomography, X-Ray Computed/methods , Chronic Disease , Retrospective Studies , Treatment Outcome , Perfusion Imaging , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/therapy , Lung/blood supply , Anticoagulants/therapeutic use
4.
J. bras. pneumol ; 46(4): e20200204, 2020. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1386040

ABSTRACT

ABSTRACT Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.


RESUMO A hipertensão pulmonar tromboembólica crônica (HPTEC) é uma doença grave e debilitante, causada pela oclusão do leito arterial pulmonar por êmbolos hemáticos e por material fibroso induzido pela presença desses êmbolos. Essa oclusão eleva a resistência vascular e, por consequência, a pressão do território arterial pulmonar, caracterizando a presença de hipertensão pulmonar. Esse aumento da carga imposta ao ventrículo direito leva a progressiva insuficiência do mesmo e, finalmente, ao óbito. No entanto, ao contrário das outras formas de hipertensão pulmonar, a HPTEC possui uma particularidade muito significativa: a existência de tratamento potencialmente curativo através da tromboendarterectomia pulmonar. Dessa forma, o objetivo primordial do manejo deve ser a avaliação do potencial cirúrgico do paciente em um centro de referência em HPTEC. Entretanto, nem todos os pacientes podem ser submetidos à cirurgia. Para esses pacientes outras alternativas terapêuticas viáveis são a angioplastia de artérias pulmonares e o tratamento farmacológico. Nestas recomendações, discutir-se-ão as bases fisiopatológicas para o surgimento de HPTEC, a partir da embolia pulmonar aguda, bem como o quadro clínico apresentado pelo paciente, o algoritmo diagnóstico a ser seguido e as alternativas terapêuticas disponíveis.

5.
Ther Adv Respir Dis ; 13: 1753466619878556, 2019.
Article in English | MEDLINE | ID: mdl-31558116

ABSTRACT

Most physicians understand venous thromboembolism (VTE) to be an acute and time-limited disease. However, pathophysiological and epidemiological data suggest that in most patients VTE recurrence risk is not resolved after the first 6 months of anticoagulation. Recurrence rates are high and potentially life-threatening. In these cases, it would make sense to prolong anticoagulation for an undetermined length of time. However, what about the bleeding rates, induced by prolonged anticoagulation? Would they not outweigh the benefit of reducing the VTE recurrent risk? How long should anticoagulation be continued, and should all patients suffering from VTE be provided with extended anticoagulation? This review will address the most recent data concerning extended anticoagulation in VTE secondary prophylaxis. The reviews of this paper are available via the supplementary material section.


Subject(s)
Anticoagulants/administration & dosage , Blood Coagulation/drug effects , Secondary Prevention , Venous Thromboembolism/drug therapy , Anticoagulants/adverse effects , Clinical Decision-Making , Drug Administration Schedule , Hemorrhage/chemically induced , Humans , Patient Selection , Recurrence , Risk Assessment , Risk Factors , Time Factors , Treatment Outcome , Venous Thromboembolism/blood , Venous Thromboembolism/diagnosis
7.
J Bras Pneumol ; : 0, 2018 Jun 07.
Article in English, Portuguese | MEDLINE | ID: mdl-29898007

ABSTRACT

Acute pulmonary thromboembolism (APTE) is a highly prevalent condition (104-183 cases per 100,000 person-years) and is potentially fatal. Approximately 20% of patients with APTE are hypotensive, being considered at high risk of death. In such patients, immediate lung reperfusion is necessary in order to reduce right ventricular afterload and to restore hemodynamic stability. To reduce pulmonary vascular resistance in APTE and, consequently, to improve right ventricular function, lung reperfusion strategies have been developed over time and widely studied in recent years. In this review, we focus on advances in the indication and use of systemic thrombolytic agents, as well as lung reperfusion via endovascular and classical surgical approaches, in APTE.

8.
J. bras. pneumol ; 44(3): 237-243, May-June 2018. tab, graf
Article in English | LILACS | ID: biblio-954560

ABSTRACT

ABSTRACT Acute pulmonary thromboembolism (APTE) is a highly prevalent condition (104-183 cases per 100,000 person-years) and is potentially fatal. Approximately 20% of patients with APTE are hypotensive, being considered at high risk of death. In such patients, immediate lung reperfusion is necessary in order to reduce right ventricular afterload and to restore hemodynamic stability. To reduce pulmonary vascular resistance in APTE and, consequently, to improve right ventricular function, lung reperfusion strategies have been developed over time and widely studied in recent years. In this review, we focus on advances in the indication and use of systemic thrombolytic agents, as well as lung reperfusion via endovascular and classical surgical approaches, in APTE.


RESUMO O tromboembolismo pulmonar (TEP) agudo é uma condição altamente prevalente (104-183 por 100.000 pessoas-ano) e potencialmente fatal. Aproximadamente 20% dos pacientes com TEP agudo apresentam-se hipotensos, sendo considerados pacientes com alto risco de morte. Nesses casos, a necessidade de reperfusão pulmonar imediata é mandatória para reduzir a pós-carga do ventrículo direito e restaurar a condição hemodinâmica do paciente. Visando a redução da resistência vascular pulmonar no TEP agudo e, por consequência, a melhora na função ventricular direita, estratégias de reperfusão pulmonar foram desenvolvidas com passar do tempo e vêm sendo muito estudadas nos últimos anos. Avanços na indicação e no uso de trombolíticos sistêmicos, reperfusão pulmonar via abordagem endovascular ou abordagem cirúrgica clássica no TEP agudo são os focos desta revisão.


Subject(s)
Humans , Pulmonary Embolism/mortality , Reperfusion/rehabilitation , Ventricular Function, Right , Hemorrhage
9.
J Am Coll Cardiol ; 56(9): 715-20, 2010 Aug 24.
Article in English | MEDLINE | ID: mdl-20723801

ABSTRACT

OBJECTIVES: The objective of this study was to evaluate the natural history of untreated schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) patients as compared to idiopathic pulmonary arterial hypertension (IPAH) with respect to hemodynamics recorded at presentation and 36 months survival. BACKGROUND: Schistosomiasis (Sch) is one of the most prevalent chronic infectious diseases in the world. Nevertheless data regarding one of its most severe clinical complications, pulmonary arterial hypertension (PAH), is scarce. METHODS: We retrospectively analyzed case notes of all consecutive patients diagnosed of Sch-PAH and IPAH referred to the Heart Institute in São Paulo, Brazil, between 2004 and 2008. None of the Sch-PAH received PAH specific treatment whereas all IPAH patients did. RESULTS: Sch-PH patients (n = 54) had less severe pulmonary hypertension as evidenced by lower levels of pulmonary vascular resistance (11.3 +/- 11.3 W vs. 16.7 +/- 10.6 W; p = 0.002) and mean pulmonary artery pressure (56.7 +/- 18.7 mm Hg vs. 64.6 +/- 17.4 mm Hg; p = 0.01) and higher cardiac output (4.62 +/- 1.5 l/min vs. 3.87 +/- 1.5 l/min; p = 0.009) at presentation than IPAH patients (n = 95). None of the Sch-PAH patients demonstrated a positive response to acute vasodilator testing, whereas 16.2% of IPAH patients did (p = 0.015). Survival rates at 1, 2, and 3 years were 95.1%, 95.1%, and 85.9% and 95%, 86%, and 82%, for Sch-PAH and IPAH, respectively (p = 0.49). Both groups had a higher survival rate when compared to IPAH survival as estimated by the NIH equation (71%, 61%, and 52%, respectively). CONCLUSIONS: Sch-PAH has a more benign clinical course than IPAH despite a lack of demonstrable acute vasoreactivity at hemodynamic evaluation.


Subject(s)
Hypertension, Pulmonary/mortality , Hypertension, Pulmonary/parasitology , Schistosomiasis/mortality , Adult , Exercise Tolerance , Female , Hemodynamics , Humans , Hypertension, Pulmonary/physiopathology , Kaplan-Meier Estimate , Male , Middle Aged , Proportional Hazards Models , Retrospective Studies , Schistosomiasis/physiopathology
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