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Enigma of pulmonary hypertension; echocardiography in the crossroads.

ABSTRACT: Pulmonary hypertension (PH) with its myriad presentations provides a challenge in its diagnosis and management. Recent WHO classification of pulmonary hypertension as defined at the 5th World Symposium held in Nice, France in 2013 tries to highlight different forms of pulmonary hypertension based on different pathophysiology, clinical presentation and therapeutic strategies [1]. These are classified in 5 groups as follows (1): PAH, (2) PH due to left heart disease, (3) PH due to chronic lung disease and/or hypoxia, (4) chronic thromboembolic PH (CTEPH), and (5) PH due to unclear or multifactorial mechanism. In clinical practice we encounter patients with pulmonary hypertension which is often very difficult to diagnose accurately and therefore presents with therapeutic challenge. Echocardiography plays a vital role in diagnosis and management of this enigmatic disease entity. Due to the complex nature of the disease, there can be some overlaps in various groups of pulmonary hypertension. This is particularly true about the two groups according to WHO classification-pulmonary arterial hypertension (WHO group 1) and chronic thromboembolic pulmonary hypertension (WHO group 3). As clinicians we are often faced with the dilemma of making the correct diagnosis and echocardiography plays a pivotal role in this crossroad. We want to present two cases of pulmonary hypertension with different etiologies as stated above and review literature to elucidate the role of echocardiogram in these cases.

SUBMITTER: Roychoudhury D

PROVIDER: S-EPMC7452039 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Enigma of pulmonary hypertension; echocardiography in the crossroads.

Roychoudhury Debasish D Sha Kenneth K Nanda Navin C NC Gustafson Gregory G

Respiratory medicine case reports 20200803

Pulmonary hypertension (PH) with its myriad presentations provides a challenge in its diagnosis and management. Recent WHO classification of pulmonary hypertension as defined at the 5th World Symposium held in Nice, France in 2013 tries to highlight different forms of pulmonary hypertension based on different pathophysiology, clinical presentation and therapeutic strategies [1]. These are classified in 5 groups as follows (1): PAH, (2) PH due to left heart disease, (3) PH due to chronic lung dis ...[more]

PMID: 32874908

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Project description:BackgroundThe world symposium on pulmonary hypertension (PH) has proposed that PH be defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg as assessed by right heart catheterisation (RHC). Transthoracic echocardiography (TTE) is an established screening tool used for suspected PH. International guidelines recommend a multi-parameter assessment of the TTE PH probability although effectiveness has not been established using real world data.Study aimsTo determine accuracy of the European Society of Cardiology (ESC) and British Society of Echocardiography (BSE) TTE probability algorithm in detecting PH in patients attending a UK PH centre. To identify echocardiographic markers and revised algorithms to improve the detection of PH in those with low/intermediate BSE/ESC TTE PH probability.MethodsTTE followed by RHC (within 4 months after) was undertaken in patients for suspected but previously unconfirmed PH. BSE/ESC PH TTE probabilities were calculated alongside additional markers of right ventricular (RV) longitudinal and radial function, and RV diastolic function. A refined IMPULSE algorithm was devised and evaluated in patients with low and/or intermediate ESC/BSE TTE PH probability.ResultsOf 310 patients assessed, 236 (76%) had RHC-confirmed PH (average mPAP 42.8 ± 11.7). Sensitivity and specificity for detecting PH using the BSE/ESC recommendations was 89% and 68%, respectively. 36% of those with low BSE/ESC TTE probability had RHC-confirmed PH and BSE/ESC PH probability parameters did not differ amongst those with and without PH in the low probability group. Conversely, RV free wall longitudinal strain (RVFWLS) was lower in patients with vs. without PH in low BSE/ESC probability group (- 20.6 ± 4.1% vs - 23.8 ± 3.9%) (P < 0.02). Incorporating RVFWLS and TTE features of RV radial and diastolic function (RVFAC and IVRT) within the IMPULSE algorithm reduced false negatives in patients with low BSE/ESC PH probability by 29%. The IMPULSE algorithm had excellent specificity and positive predictive value in those with low (93%/80%, respectively) or intermediate (82%/86%, respectively) PH probability.ConclusionExisting TTE PH probability guidelines lack sensitivity to detect patients with milder haemodynamic forms of PH. Combining additional TTE makers assessing RV radial, longitudinal and diastolic function enhance identification of milder forms of PH, particularly in those who have a low BSE/ESC TTE PH probability.

Project description:Rationale: Genetic studies suggest that SOX17 (SRY-related HMG-box 17) deficiency increases pulmonary arterial hypertension (PAH) risk. Objectives: On the basis of pathological roles of estrogen and HIF2α (hypoxia-inducible factor 2α) signaling in pulmonary artery endothelial cells (PAECs), we hypothesized that SOX17 is a target of estrogen signaling that promotes mitochondrial function and attenuates PAH development via HIF2α inhibition. Methods: We used metabolic (Seahorse) and promoter luciferase assays in PAECs together with the chronic hypoxia murine model to test the hypothesis. Measurements and Main Results: Sox17 expression was reduced in PAH tissues (rodent models and from patients). Chronic hypoxic pulmonary hypertension was exacerbated by mice with conditional Tie2-Sox17 (Sox17EC-/-) deletion and attenuated by transgenic Tie2-Sox17 overexpression (Sox17Tg). On the basis of untargeted proteomics, metabolism was the top pathway altered by SOX17 deficiency in PAECs. Mechanistically, we found that HIF2α concentrations were increased in the lungs of Sox17EC-/- and reduced in those from Sox17Tg mice. Increased SOX17 promoted oxidative phosphorylation and mitochondrial function in PAECs, which were partly attenuated by HIF2α overexpression. Rat lungs in males displayed higher Sox17 expression versus females, suggesting repression by estrogen signaling. Supporting 16α-hydroxyestrone (16αOHE; a pathologic estrogen metabolite)-mediated repression of SOX17 promoter activity, Sox17Tg mice attenuated 16αOHE-mediated exacerbations of chronic hypoxic pulmonary hypertension. Finally, in adjusted analyses in patients with PAH, we report novel associations between a SOX17 risk variant, rs10103692, and reduced plasma citrate concentrations (n = 1,326). Conclusions: Cumulatively, SOX17 promotes mitochondrial bioenergetics and attenuates PAH, in part, via inhibition of HIF2α. 16αOHE mediates PAH development via downregulation of SOX17, linking sexual dimorphism and SOX17 genetics in PAH.

Project description:BACKGROUND:Right ventricular (RV) function is a major determinant of outcome in patients with pulmonary hypertension. Cardiac magnetic resonance (CMR) is gold standard to assess RV ejection fraction (RVEFCMR), however this is a crude measure. New CMR measures of RV function beyond RVEFCMR have emerged, such as RV lateral atrio-ventricular plane displacement (AVPDlat), maximum emptying velocity (S'CMR), RV fractional area change (FACCMR) and feature tracking of the RV free wall (FWSCMR). However, it is not fully elucidated if these CMR measures are in parity with the equivalent echocardiography-derived measurements: tricuspid annular plane systolic excursion (TAPSE), S'-wave velocity (S'echo), RV fractional area change (FACecho) and RV free wall strain (FWSecho). The aim of this study was to compare regional RV function parameters derived from CMR to their echocardiographic equivalents in patients with pulmonary hypertension and to RVEFCMR. METHODS:Fifty-five patients (37 women, 62?±?15?years) evaluated for pulmonary hypertension underwent CMR and echocardiography. AVPDlat, S'CMR, FACCMR and FWSCMR from cine 4-chamber views were compared to corresponding echocardiographic measures and to RVEFCMR delineated in cine short-axis stack. RESULTS:A strong correlation was demonstrated for FAC whereas the remaining measurements showed moderate correlation. The absolute bias for S' was 2.4?±?3.0?cm/s (relative bias 24.1?±?28.3%), TAPSE/AVPDlat 5.5?±?4.6?mm (33.2?±?25.2%), FWS 4.4?±?5.8% (20.2?±?37.5%) and for FAC 5.1?±?8.4% (18.5?±?32.5%). In correlation to RVEFCMR, FACCMR and FWSecho correlated strongly, FACecho, AVPDlat, FWSCMR and TAPSE moderately, whereas S' had only a weak correlation. CONCLUSION:This study has demonstrated a moderate to strong correlation of regional CMR measurements to corresponding echocardiographic measures. However, biases and to some extent wide limits of agreement, exist between the modalities. Consequently, the equivalent measures are not interchangeable at least in patients with pulmonary hypertension. The echocardiographic parameter that showed best correlation with RVEFCMR was FWSecho. At present, FACecho and FWSecho as well as RVEFCMR are the preferred methods to assess and follow up RV function in patients with pulmonary hypertension. Future investigations of the CMR right ventricular measures, beyond RVEF, are warranted.

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Enigma of pulmonary hypertension; echocardiography in the crossroads.

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Enigma of pulmonary hypertension; echocardiography in the crossroads.

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