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    Imaging risk in pulmonary arterial hypertension

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    Pulmonary arterial hypertension (PAH) is a Janus-faced entity with, on one side, the pulmonary circulation and, on the other side, the right ventricle (RV). While the disease process is turned on at the site of the pulmonary resistive vessels, the patient symptomatology and prognosis are largely determined by RV structure and function adaptation to increased afterload. Yet, this important cardiac aspect of PAH pathophysiology remains insufficiently recognised. Combined measurements of TAPSE, TR and IVC outperformed other echocardiographic measurements previously shown to be predictors of outcome in PAH, such as right atrial (RA) surface area and pericardial effusion which are part of current guidelines recommendations for risk assessment. PAH is a progressive disease with high mortality. Indeed, combination treatment strategies and the option of probably most effective parenteral prostacyclin therapies would be prescribed to patients with the highest risk of deterioration and shorter survival. Risk assessment is thus essential to optimise treatment decisions aimed at slowing progression of the disease. 14 parameters were used to categorise patients into a low risk green zone (<5% 1-year mortality), an intermediate risk yellow zone (5–10% 1-year mortality) and a high risk red zone (>10% 1-year mortality). The inherent assumption of the approach was that treatments should be titrated or combined to move patients into lower risk zones and thereby improve survival. However, the majority of PAH patients remain in intermediate risk status or with overlapping risk assessments under a double combination of oral drugs, and there is little data offering support to the difficult decision of initial or sequential addition of parenteral prostacyclins. The echocardiographic examination of PAH patients generates many measurements which have been shown to be of prognostic relevance by rigorous univariate and multivariable analysis, though most of the time in limited size mono-centric studies. TAPSE is indeed a robust though load-dependent measure of contractility and IVC diameter a reflection of increased RV and RA pressure and dimensions. The RV adapts in severe PH by increased contractility to preserve its coupling to the pulmonary circulation and it is only after this basic homeometric mechanism is exhausted that Starling’s heterometric adaptation comes into play to preserve flow output, but at the price of increased dimensions and systemic congestion. Thus, the TAPSE (corrected for TR) reflects contractility adaptation and increased IVC dimension its failure. Echocardiography also allows for measurement of other load dependent indices of pump failure, such as RV fractional area change, and more load-independent measures of RV contractility, such as the maximum velocity of isovolumic contraction or longitudinal strain, and offers exploration of regional inhomogeneity of RV contraction which, alone or in combination with CPET, may also be prognostically pertinent

    Right ventricular assessment matters for precision medicine.. reply to "Identifying parameters associated with response to switching from a PDE5i to riociguat in RESPITE"

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    We have read with great interest the article titled “Identifying parameters associated with response to switching from a PDE5i to riociguat in RESPITE”, reporting the results of a post hoc analysis performed in patients who completed RESPITE trial. This is an innovative approach in the setting of pulmonary arterial hypertension (PAH), opening the way for precision medicine. In this context, the REVEAL risk score (RRS) and NT-proBNP and growth/differentiation factor 15 (GDF-15), both indicative of right ventricular (RV) adaptation to increased afterload and RV remodelling, were identified as potential predictors of response in PAH patients switching from PDE5-i to riociguat. Interestingly, responders had lower biomarkers' levels and lower RRS than non-responders, suggestive of a less advanced RV remodelling. This finding, in line with the most recent evidence, highlights the need to integrate imaging-derived RV metrics into widely accepted risk scores, adding important information on RV function to indirect functional parameters, such as New York Heart Association functional class, six-min walk distance, eGFR and DLCO

    Pulmonary hypertension in left heart disease. the need to continue to explore

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    Pulmonary hypertension (PH) in left heart disease (LHD) is a challenge for cardiologists: in a setting of left ventricular dysfunction, the coexistence of PH and right ventricular dysfunction is known to be associated with very poor prognosis and limited efficacy of conventional medical treatments. This might explain why the off-label use of drugs specific for pulmonary arterial hypertension is quite diffuse in clinical practice in PH-LHD, despite randomized clinical trials do not provide evidence of their efficacy in such patients

    A 0-D model to predict the relationship between resistance and compliance in pulmonary arterial hypertension

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    The inverse relationship between pulmonary vascular resistance and arterial compliance plays a significant role for the treatment of pulmonary arterial hypertension. The hyperbolic relationship between pulmonary vascular resistance and arterial compliance enables to evaluate the percentage of resistance reduction, which ultimately leads to an improvement in compliance. The numerical representation of the pulmonary circulation can help clinicians evaluate these key parameters in relation to therapeutic intervention. In this paper, we present a 0-D numerical model of the pulmonary circulation. The right ventricular pressure is reproduced using a modified time-varying elastance and the pulmonary bed is modelled with RLC elements. Preliminary results obtained from clinical parameters measured in patients with pulmonary arterial hypertension are discussed in this context. The simulations performed using the 0-D model have been applied to a clinical setting to evaluate the percentage change induced on compliance by a reduction in resistance in fixed circulatory conditions

    Hemodynamic evaluation of the right heart-pulmonary circulation unit in patients candidate to transjugular intrahepatic portosystemic shunt

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    In Europe, liver cirrhosis represents the fourth-most common cause of death, being responsible for 170,000 deaths and 5500 liver transplantations per year. The main driver of its decompensation is portal hypertension, whose progression radically changes the prognosis of affected patients. Transjugular intrahepatic portosystemic shunt (TIPS) is one of the main therapeutic strategies for these patients as it reverts portal hypertension, thus improving survival. However, the coexistence of portal hypertension and pulmonary hypertension or heart failure is considered a contraindication to TIPS. Nevertheless, in the latest guidelines, the definition of heart failure has not been specified. It is unclear whether the contraindication concerns the presence of clinical signs and symptoms of heart failure or hemodynamic changes in the right heart-pulmonary circulation. Moreover, data about induced right heart volume overload after TIPS and the potential development of heart failure and pulmonary hypertension is currently scanty and controversial. In this article we revise this issue in finding predictors of cardiac performance after TIPS procedure. Performing a fluid challenge during right heart catheterization might be a promising expedient to test the adaptation of the right ventricle to a sudden increase in preload in the first few months after TIPS. This test may unmask a potential cardiac inability to sustain the hemodynamic load after TIPS, allowing for a clearer definition of heart failure and, consequently, a more robust indication to TIPS
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