Use of medical therapies before pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension patients with severe hemodynamic impairment.

Chronic 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.

Reperfusion in acute pulmonary thromboembolism.

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.

Use of direct oral anticoagulants for chronic thromboembolic pulmonary hypertension.

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.

Pulmonary artery enlargement in schistosomiasis associated pulmonary arterial hypertension.

BACKGROUND: Schistosomiasis associated pulmonary arterial hypertension (Sch-PAH) might represent the most prevalent form of PAH worldwide. In Sch-PAH, the presence of aneurismal dilation of the pulmonary artery has been described, although it is still a matter of debate whether on average the pulmonary artery is more enlarged in Sc-PAH than IPAH. METHODS: We retrospectively evaluated patients with IPAH (n = 25) and Sch-PAH (n = 22) who underwent computed tomography pulmonary angiogram and right heart catheterization. RESULTS: Sch-PAH patients were older and presented less severe hemodynamic profiles. Main pulmonary artery diameter (MPAD) was greater in Sch-PAH than IPAH (4.5 ± 1.8 vs 3.7 ± 1.1 cm, p = 0.018). For the same level of mean pulmonary artery pressure, the MPAD in Sch-PAH was 0.89 cm larger than in IPAH (Covariance model p = 0.02). CONCLUSION: This study demonstrated that pulmonary artery enlargement is more pronounced in Sch-PAH than IPAH, independently of mean pulmonary artery pressure level, suggesting that this is more likely a feature of Sch-PAH.

Pulmonary arterial hypertension in the southern hemisphere: results from a registry of incident Brazilian cases.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare and ultimately fatal disorder of the pulmonary vasculature. There is increasing interest in the worldwide characteristics of patients with PAH, although data coming from the Southern Hemisphere remain scarce. The objective of this study was to describe a cohort of incident patients with PAH from a large reference center in Brazil. METHODS: All consecutive patients who received a diagnosis of PAH by right-sided heart catheterization between 2008 and 2013 were included in the study. RESULTS: A total of 178 patients with newly diagnosed PAH were enrolled in the study (mean age, 46 years; female/male ratio, 3.3:1; 45.5% in New York Heart Association functional class III or IV). Idiopathic PAH (IPAH), connective tissue disease (CTD), and schistosomiasis-associated PAH (Sch-PAH) accounted for 28.7%, 25.8%, and 19.7% of all cases, respectively. The patients were treated with phosphodiesterase type 5 inhibitors (66%), endothelin receptor antagonists (27%), or a combination of both (5%). For the PAH group as a whole, the estimated survival rate 3 years after diagnosis was 73.9%. The prognosis for the patients with CTD was worse than that for the patients with IPAH and Sch-PAH (P = .03). CONCLUSIONS: The distribution of PAH causes and the baseline characteristics in our registry clearly differ from the previously published European and US-based registries. These differences highlight the importance of regional registries and also raise questions regarding the need to better account for such differences in future clinical trials.

Left ventricular dysfunction in patients with suspected pulmonary arterial hypertension.

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.

OBJETIVO: Avaliar o papel do cateterismo de câmaras cardíacas direitas no diagnóstico de hipertensão arterial pulmonar (HAP). MÉTODOS: Entre 2008 e 2013, foram avaliadas as características clínicas, funcionais e hemodinâmicas de todos os pacientes que realizaram cateterismo cardíaco direito por suspeita de HAP em nosso laboratório, depois de afastada a presença de disfunção ventricular esquerda (DVE) grave, de alterações significativas nos testes de função pulmonar ou de resultados de cintilografia pulmonar de inalação/perfusão compatíveis com tromboembolismo pulmonar crônico. RESULTADOS: Durante o período de estudo, 384 pacientes foram submetidos a cateterismo cardíaco diagnóstico. A hipertensão pulmonar (HP) foi confirmada em 302 pacientes (78,6%). A média de idade desses pacientes foi de 48,7 anos. Os pacientes sem HP apresentaram melhor perfil hemodinâmico e menores níveis de peptídio natriurético do tipo B que aqueles diagnosticados com HP. No entanto, 13,8% dos pacientes sem HP apresentavam-se em classe funcional III/IV do New York Heart Association. Dos 218 pacientes que cumpriam os critérios de inclusão, 40 (18,3%) e 178 (81,7%) foram diagnosticados como portadores de HP associada à DVE (HP-DVE) e HAP, respectivamente. O grupo HP-DVE tinha idade significativamente mais avançada que aqueles com HAP (p < 0,0001). CONCLUSÕES: A diferença proporcional entre os grupos HAP e HP-DVE foi bastante significativa, considerando a inexistência de sinais ecocardiográficos sugestivos de DVE importante como parte da investigação que antecedeu o cateterismo. Nossos resultados reforçam o papel fundamental do cateterismo cardíaco no diagnóstico da HAP, ainda mais em faixas etárias mais avançadas, nas quais a prevalência de DVE não diagnosticada através de exames não invasivos é particularmente importante.