Cancer-associated thrombosis (CAT) is a condition in which relevance has been increasingly recognised both for physicians that deal with venous thromboembolism (VTE) and for oncologists. It is currently estimated that the annual incidence of VTE in patients with cancer is 0.5% compared to 0.1% in the general population. Active cancer accounts for 20% of the overall incidence of VTE. Of note, VTE is the second most prevalent cause of death in cancer, second only to the progression of the disease, and cancer is the most prevalent cause of deaths in VTE patients. Nevertheless, CAT presents several peculiarities that distinguish it from other VTE, both in pathophysiology mechanisms, risk factors and especially in treatment, which need to be considered. CAT data will be reviewed in this review.

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Pulmonary arterial hypertension (PAH) is a life-threatening disease characterised by an elevated pulmonary vascular resistance in the absence of left ventricular disease, and increasing pulmonary artery pressure, with consequent right ventricular failure and death [1]. Several aetiologies have been associated with PAH, including connective tissue diseases, congenital heart diseases and chronic infections, such as HIV. Due to its high prevalence in developing countries, one of the most relevant forms of PAH worldwide is the one associated to schistosomiasis [2]. Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is present in 5% of patients with the hepatosplenic form of the disease [3]. Due to its histological and haemodynamic similarities to idiopathic PAH (IPAH), it is classified within group 1 (pre-capillary pulmonary hypertension) of the current pulmonary hypertension classification [4]. However, despite the similarities, a previous cohort has shown that Sch-PAH has a distinct, more benign course than IPAH, even in the absence of specific therapy [5]. Data regarding the efficacy of specific PAH therapy in Sch-PAH are scarce. Improvements in functional class, cardiac output and 6-min walking test distance (6MWT), using phosphodiesterase-5 inhibitors (PDE5i) or endothelin-1 receptor antagonists (ERA) were demonstrated in a small cohort of 12 Sch-PAH patients [6]. The effect of PAH treatment on hard end-points, such as clinical worsening or survival, has not been evaluated in this population, until this date. The aim of this study was to compare the survival of newly diagnosed Sch-PAH patients treated with PAH targeted therapies against a group of untreated patients from a historical cohort. Data from all consecutive, newly diagnosed Sch-PAH patients referred to our centre in Brazil were analysed. Sch-PAH was characterised by the presence of mean pulmonary arterial pressure ⩾25 mmHg with pulmonary artery occlusion pressure ⩽15 mmHg, in the absence of significant lung parenchymal disease, left ventricular dysfunction or chronic thromboembolic disease, associated with the presence of periportal fibrosis and/or left liver lobe enlargement associated with at least one of the following features: positive epidemiology, previous treatment of schistosomiasis or identification of eggs in stool or rectal biopsy [7]. Since specific PAH therapy became widely available in Brazil after 2010, patients diagnosed before this date received only supportive therapy and comprised our historical cohort. After 2010, Sch-PAH patients were regularly treated with PDE5i, ERA or both, at discretion of the attending physician, in accordance to the available guidelines [8, 9]. Baseline clinical, demographical, including New York Heart Association (NYHA) functional class (FC) assessment, 6MWT, brain natriuretic peptide (BNP) and haemodynamic data were collected. Analysis was performed using the SPSS 21 statistical package (SPSS, Inc.). All continuous variables are expressed as mean±SD and compared using t-test. Categorical d...

Pulmonary hypertension (PH) is a hemodynamic condition characterized by the presence of mean pulmonary artery pressure ≥ 25 mm Hg. Many different baseline conditions and/or risk factors are associated with the development of PH, increasing the complexity for the appropriate diagnosis and classification. Since it was first proposed, PH classification has been adapted to incorporate the increasing knowledge about the disease gathered along the past two decades. Current classification groups together the patients with similar pathophysiological mechanisms, clinical presentation, and management. Nevertheless, correct classification is only possible after a comprehensive diagnostic approach, assessing the most prevalent conditions associated with PH. Most importantly, left ventricular function, lung parenchyma, and the presence of chronic thromboembolic disease have to be evaluated by means of different diagnostic tools. The aim of this review is to summarize the current definitions of PH, which in turn is necessary for determination of the appropriate diagnostic approach.

OBJECTIVE: To evaluate the role of right heart catheterization in the diagnosis of pulmonary arterial hypertension (PAH). METHODS: We evaluated clinical, functional, and hemodynamic data from all patients who underwent right heart catheterization because of diagnostic suspicion of PAH-in the absence of severe left ventricular dysfunction (LVD), significant changes in pulmonary function tests, and ventilation/perfusion lung scintigraphy findings consistent with chronic pulmonary thromboembolism-between 2008 and 2013 at our facility. RESULTS: During the study period, 384 patients underwent diagnostic cardiac catheterization at our facility. Pulmonary hypertension (PH) was confirmed in 302 patients (78.6%). The mean age of those patients was 48.7 years. The patients without PH showed better hemodynamic profiles and lower levels of B-type natriuretic peptide. Nevertheless, 13.8% of the patients without PH were categorized as New York Heart Association functional class III or IV. Of the 218 patients who met the inclusion criteria, 40 (18.3%) and 178 (81.7%) were diagnosed with PH associated with LVD (PH-LVD) and with PAH, respectively. The patients in the HP-LVD group were significantly older than were those in the PAH group (p < 0.0001). CONCLUSIONS: The proportional difference between the PAH and PH-LVD groups was quite significant, considering the absence of echocardiographic signs suggestive of severe LVD during the pre-catheterization investigation. Our results highlight the fundamental role of cardiac catheterization in the diagnosis of PAH, especially in older patients, in whom the prevalence of LVD that has gone undiagnosed by non-invasive tests is particularly relevant.

OBJECTIVES: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 II/III. 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.

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.

The finding of pulmonary hypertension (PH) by echocardiography is common and of concern. However, echocardiography is just a suggestive and non-diagnostic assessment of PH. When direct involvement of pulmonary circulation is suspected, invasive hemodynamic monitoring is recommended to establish the diagnosis. This assessent provides, in addition to the diagnostic confirmation, the correct identification of the vascular territory predominantly involved (arterial pulmonary or postcapillary). Treatment with specific medication for PH (phosphodiesterase type 5 inhibitors, endothelin receptor antagonists and prostacyclin analogues) has been proven effective in patients with pulmonary arterial hypertension, but its use in patients with PH due to left heart disease can even be damaging. In this review, we discuss the diagnosis criteria, how etiological investigation should be carried out, the clinical classification and, finally, the therapeutic recommendations for PH.