Update on the Treatment of Pulmonary Arterial Hypertension.

In 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.

Electrical impedance tomography in pulmonary arterial hypertension.

The characterization of pulmonary arterial hypertension (PAH) relies mainly on right heart catheterization (RHC). Electrical impedance tomography (EIT) provides a non-invasive estimation of lung perfusion that could complement the hemodynamic information from RHC. To assess the association between impedance variation of lung perfusion (ΔZQ) and hemodynamic profile, severity, and prognosis, suspected of PAH or worsening PAH patients were submitted simultaneously to RHC and EIT.

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.

Lung Cavities in Chronic Thromboembolic Pulmonary Hypertension.

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.

Extended anticoagulation after venous thromboembolism: should it be done?

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.

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.

RV Fractional Area Change and TAPSE as Predictors of Severe Right Ventricular Dysfunction in Pulmonary Hypertension: A CMR Study.

Background: The right ventricular ejection fraction (RVEF) is a surrogate marker of right ventricular function in pulmonary hypertension (PH), but its measurement is complicated and time consuming. The tricuspid annular plane systolic excursion (TAPSE) measures only the longitudinal component of RV contraction while the right ventricular fractional area change (RVFAC) takes into account both the longitudinal and the transversal components. The aim of our study was to evaluate the relationship between RVEF, RVFAC, and TAPSE according to hemodynamic severity in two groups of patients with PH: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).

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.

Usefulness of Cardiovascular Magnetic Resonance Indices to Rule In or Rule Out Precapillary Pulmonary Hypertension.

Background: Various cardiovascular magnetic resonance (CMR) imaging indices are used to assess pulmonary hypertension (PH; mean pulmonary artery pressure ≥ 25 mm Hg). We compared the value of CMR indices to diagnose precapillary PH in treatment-naive patients evaluated for the first time for known or suspected pulmonary vascular disease.

Methods: Right heart catheterization and CMR were performed within 48 hours of each other in 85 consecutive subjects.

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 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.

Quality of life as a prognostic marker in pulmonary arterial hypertension.

Background: Improvement in quality of life together with better survival are the ultimate goals in the treatment of pulmonary arterial hypertension (PAH) patients. The objective of this study was to evaluate the health-related quality of life (HRQL) of pulmonary arterial hypertension (PAH) patients with the SF-36 generic questionnaire and to identify the prognostic implication of this assessment.

Methods: Fifty-four consecutive newly diagnosed PAH patients (WHO classification group I) in a single PAH reference center were included.

Strong linear relationship between heart rate and mean pulmonary artery pressure in exercising patients with severe precapillary pulmonary hypertension.

The contribution of heart rate (HR) to pulmonary artery hemodynamics has been suggested in pulmonary hypertension (PH). Our high-fidelity pressure, retrospective study tested the hypothesis that HR explained the majority of mean pulmonary artery pressure (mPAP) and pulse pressure (PApp) variation in 12 severe precapillary PH patients who performed incremental-load cycling while in the supine position. Seven idiopathic pulmonary arterial hypertension patients and five chronic thromboembolic PH patients were studied.

Estimating right ventricular stroke work and the pulsatile work fraction in pulmonary hypertension.

Background: The mean pulmonary artery pressure (mPAP) replaces mean systolic ejection pressure (msePAP) in the classic formula of right ventricular stroke work (RVSW) = (mPAP - RAP) × stroke volume, where RAP is mean right atrial pressure. Only the steady work is thus taken into account, not the pulsatile work, whereas pulmonary circulation is highly pulsatile. Our retrospective, high-fidelity pressure study tested the hypothesis that msePAP was proportional to mPAP, and looked at the implications for RVSW.

The role of target therapies in schistosomiasis-associated pulmonary arterial hypertension.

Background: Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) may be one of the most prevalent forms of pulmonary arterial hypertension (PAH) worldwide. However, the clinical and hemodynamical response to specific PAH therapy in Sch-PAH is not known.

Methods: We retrospectively analyzed the charts of all patients with Sch-PAH who initiated specific PAH treatment between June 2003 and June 2010 in a single PAH reference center in São Paulo, Brazil.

Schistosomiasis and pulmonary hypertension.

Schistosomiasis is one of the most prevalent chronic infectious diseases in the world. One of its most severe complications, pulmonary hypertension, occurs in up to 5% of patients with hepatosplenic schistosomiasis. The prevalence of schistosomiasis is so overwhelming that schistosomiasis-associated pulmonary hypertension (Sch-PH) may be the most prevalent cause of pulmonary hypertension around the world.

The role of imaging techniques in the assessment of pulmonary circulation.

Knowledge of the structure and function of pulmonary circulation has evolved considerably in the last few decades. The use of non-invasive imaging techniques to assess the anatomy and function of the pulmonary vessels and heart has taken on added importance with the recent advent of novel therapies. Imaging findings not only constitute a diagnostic tool but have also proven to be essential for prognosis and treatment follow-up.

[Magnetic resonance imaging and pulmonary hypertension: towards an improved evaluation of right heart and pulmonary circulation].

In patients with pulmonary hypertension (PH), right ventricular (RV) function carries prognostic implications and this explains why RV function must be systematically evaluated at baseline and during follow-up. Cardiac Magnetic Resonance imaging (CMR) is nowadays considered the gold standard technique for non-invasively evaluating RV morphology and function. Pulmonary hemodynamics may be also successfully studied by CMR.

Diagnosis and treatment of pulmonary hypertension: an update.

Over the last five years, knowledge in the field of pulmonary hypertension has grown consistently and significantly. On the basis of various clinical studies showing the usefulness of new diagnostic tools, as well as the efficacy of new medications and drug combinations, new diagnostic and treatment algorithms have been developed. Likewise, in order to simplify the clinical management of patients, the classification of pulmonary hypertension has been changed in an attempt to group the various forms of pulmonary hypertension in which the diagnostic and therapeutic approaches are similar.

Survival in schistosomiasis-associated pulmonary arterial hypertension.

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.