bluuebunny/crossref_metadata_2025_split huggingface dataset:
Proposed stages of Fabry disease: insights from multiparametric cardiac MRI and advanced ECG
Authors: Bicho Augusto JA , Johner N , Shah D , Nordin S , Knott K , Lau C , Seraphim A , Hughes R , Vijapurapu R , Bhuva A , Steeds RP , Kozor R , Hughes D , Moon JC , Namdar M 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: None.

Background
Staging of Fabry disease (FD) cardiomyopathy uses multiparametric cardiac MRI. Advanced disease is characterized by left ventricular hypertrophy (LVH), myocardial inflammation/oedema (high native T2 mapping) and/or fibrosis (late gadolinium enhancement, LGE). Pre-LVH involvement has been described and includes myocardial sphingolipid storage (low native T1 mapping), impaired LV global longitudinal strain (GLS) and microvascular disease/dysfunction (low stress myocardial blood flow, MBF, in perfusion mapping).
We aimed to define (1) the early myocardial phenotype prior to T1 lowering/pre-storage and (2) the stages of cardiac involvement in FD.

Methods
FD patients and age, sex and heart rate matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR (cines, GLS, pre-contrast T1 and T2 mapping, adenosine stress perfusion mapping [for MBF] and LGE).

Results
114 Fabry patients (46 ± 13 years, 61% female, 37% [n = 72] had LVH) and 76 controls (49 ± 15 years, 50% female) were included.

FD with vs without LVH
in brief and as expected, FD with LVH had significantly (p &lt; 0.05) lower MBF, GLS and T1, and higher T2 and %LGE.
FD pre-LVH low T1 vs pre-LVH normal T1: low T1 patients (32/72, 44%) had higher LV mass index (67 ± 14 vs 59 ± 10g/m2, P = 0.011), maximum Q wave amplitude (2[1-2] vs 1[1-2]mm, P &lt; 0.001), Sokolow-Lyon index (22[16-28] vs 17[13-23]mm, P = 0.031) and more fractionated QRS complexes (44 vs 18%, P = 0.020).
FD pre-LVH normal T1 vs healthy controls: normal T1 pre-LVH Fabry patients (40/72, 56%) had reduced GLS (-18 ± 2 vs -20 ± 2%, P &lt; 0.001), microvascular impairment (lower MBF 2.5 ± 0.7 vs 3.0 ± 0.8mL/g/min, P = 0.028), subtle T2 elevation (50 ± 4 vs 48 ± 2ms, p = 0.027) and limited LGE (%LGE 0.3 ± 1.1 vs 0%, P = 0.004) when compared to healthy controls; ECG abnormalities included shorter P wave duration (88 ± 12 vs 94 ± 15ms, P = 0.010) and T wave peak time (Tonset–Tpeak; 104 ± 28 vs 115 ± 20ms, P = 0.015), resulting in a more symmetric T wave with lower T wave time ratio (Tonset–Tpeak)/(Tpeak–Tend) (1.5 ± 0.4 vs 1.8 ± 0.4, P &lt; 0.001) compared to controls.

Conclusion
Prior staging of Fabry cardiomyopathy included a pre-LVH stage (accumulation/storage) and two LVH stages (hypertrophy and inflammation; fibrosis and impairment). Here we define an even earlier stage, pre-LVH pre-detectable storage, defined by microvascular dysfunction, impaired GLS and altered atrial depolarization and ventricular repolarization intervals (see Figure).
Abstract Figure. Proposed stages of cardiac involvement
Myocardial perfusion scintigraphy for risk stratification of patients with coronary artery disease: the AMICO registry
Authors: Pugliese NR , Buechel R , Coceani M , Clemente A , Kaufmann PA , Marzullo P , Gimelli A 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: None.

onbehalf
AMICO registry
Background. Clinical evidence promotes therapy titration based on patient risk stratification in coronary artery disease (CAD).
Purpose. We assessed the prognostic value of myocardial perfusion scintigraphy (MPS) with cadmium-zinc-telluride in addition to clinical and coronary anatomy analysis.
Methods and Results. We prospectively enrolled 1464 patients (26% females, 69.5 ± 10.4 years) referred for stress-rest MPS. All the patients underwent invasive coronary angiography (1171, 80%) or coronary computed tomography angiography (293, 20%). We defined a composite endpoint of cardiovascular death and non-fatal MI. After a median follow-up of 3.5 years (interquartile range 2 – 6 years), we observed 127 events (Table). Summed stress score (SSS) had the highest accuracy in predicting primary endpoint with a ROC-derived cut-off of SSS &gt; 8 (&gt;10% myocardium). SSS &gt; 8 portended the lowest survival probability at Kaplan–Meier analysis (p &lt; 0.0001; Figure A). The Cox-regression analysis indicated SSS as an independent predictor of the composite endpoint, along with fasting blood glucose and total cholesterol and contrary to coronary anatomy parameters. Patients with SSS &gt; 8 treated with optimal medical therapy (OMT) had the largest area of necrosis, the lower ischemic burden, the most compromised LV systo-diastolic function and the highest LV mass, but received a less aggressive treatment in comparison to early revascularized patients. Survival analysis revealed patients with SSS ≤ 8 had the greater freedom from events, irrespective of the treatment strategy, while the group with SSS &gt; 8 and OMT had the worst outcome, followed by patients with SSS &gt; 8 and early revascolarization (log-rank test: all p &lt; 0.0001). Plotting the estimates from proportional-hazard modelling against SSS (reference level: SSS = 4) shows a risk curve for the primary endpoint that increase for SSS &gt; 4 and reach a plateau for values &gt;12 (Figure B).
Conclusion. The extension of stress perfusion abnormalities constitutes a robust independent predictor of future adverse events after adjustment for multiple clinical parameters and coronary anatomy analysis. MPS could help refine risk stratification of patients with known or suspected CAD.
Primary and secondary endpoints Variable Total population (n = 1464) SSS &gt; 8 (n = 591) SSS ≤ 8 (n = 873) Hazard Ratio (95% CI)* P-value* Primary endpoint 127 (9) 85 (14) 42 (5) 3.25 (2.25 - 4.70) &lt;0.0001 Cardiovascular death 50 (3) 37 (6) 13 (1) 4.53 (2.41 - 8.51) &lt;0.0001 Non-fatal MI 84 (6) 53 (9) 31 (4) 2.71 (1.75 - 4.22) &lt;0.0001 *The hazard ratio is for the SSS &gt; 8 group as compared with the summed stress score (SSS)≤8 group, and P-values were calculated by the log-rank test and are unadjusted for multiple variables. Abstract Figure
Coronary collaterals provide more than half of normal perfusion in patients with coronary artery disease - quantification by myocardial perfusion SPECT during elective balloon occlusion
Authors: Harris B , Warren S , Persson E , Bhindi R , Ringborn M , Ugander M , Allahwala U 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: None.

BACKGROUND
The coronary collateral circulation is a preformed network of anastomotic connections acting as a "natural bypass" mechanism. Whilst the prevalence of collaterals varies between species, approximately 25% of patients have angiographically visible robust collaterals at the time of ST elevation myocardial infarction. While the presence of collaterals is associated with improved outcomes, the magnitude of myocardial perfusion which can be provided by the collateral circulation remains uncertain.

PURPOSE
The aim was to quantify collateral myocardial perfusion during experimental coronary balloon occlusion in patients with CAD.

METHODS
The study was approved by the local investigational review board, and all patients provided informed consent. Patients without prior infarction, bypass surgery, or angiographically visible collaterals undergoing elective percutaneous transluminal coronary angioplasty (PTCA) to a single epicardial vessel, underwent two scans with 99mTc-sestamibi myocardial perfusion single-photon emission computed tomography (SPECT). All subjects underwent at least three minutes of angiographically verified complete balloon occlusion, at which time an intravenous injection of the radiotracer was administered, followed by SPECT imaging. A second radiotracer injection followed by SPECT imaging was performed 24 hours after PTCA.

RESULTS
The study included 21 patients (median [interquartile range] age 70 [56-74] years, 52% male). The degree of diameter stenosis of treated vessels ranged from 60-99%, with successful PTCA performed with a mean 5-minute balloon occlusion time, resulting in ≤20% residual stenosis in all cases. The vessels undergoing PTCA were 6 in the LAD, 5 in the LCx, and 10 in the RCA. For the cohort, the size of the perfusion defect was 16 [8-30]% of the LV and the collateral perfusion at rest within the perfusion defect was 64 [58-68]% of normal perfusion within that region. Collateral perfusion was negatively correlated with perfusion defect size (R2 = 0.85, p &lt; 0.001), but did not differ by sex (p = 0.27) or age (p = 0.58). CONCLUSIONS: This is the first study to describe the magnitude of microvascular collateral perfusion in CAD. On average, despite coronary occlusion and an absence of angiographically visible collateral vessels, collaterals provide approximately 60% of the perfusion that reaches the jeopardized myocardium during coronary occlusion. This magnitude of collateral perfusion is much higher than previously speculated. A previous study using microspheres in dogs found that collateral perfusion in the setting of an occluded vessel was 6% of normal. By comparison, the current study found ten times greater collateral perfusion in patients with CAD.
Abstract Figure. Collateral perfusion during occlusion.
Prognostic value of 18 F-fluorodeoxyglucose positron emission tomography/computed tomography in infective endocarditis
Authors: San S , Ravis RE , Tessonier TL , Philip MP , Lavagna FL , Norscini GN , Oliver LO , Arregle FA , Martel HM , Torras TO , Renard RS , Ambrosi AP , Casalta CAC , Drancourt DM , Habib GH 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: None.

Background
18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) is commonly used for the diagnosis of infective endocarditis (IE), but its prognostic value remains unknown.

Objectives
This study sought to assess the prognostic value of 18F-FDG PET/CT in prosthetic valve endocarditis (PVE) and native valve endocarditis (NVE).

Methods
This study prospectively included 173 consecutive patients (109 PVE and 64 NVE) with definite left-sided IE who had an 18F-FDG PET/CT and were followed-up for 1 year. The primary endpoint was a composite of major cardiac events: death, recurrence of IE, acute cardiac failure, nonscheduled hospitalization for cardiovascular indication, and new embolic event.

Results
18F-FDG PET/CT was positive in 100 (58%) patients, 83% (n = 90 of 109) in the PVE, and 16% (n = 10 of 64) in the NVE group. At a mean follow-up of 225 days (interquartile range: 199 to 251 days), the primary endpoint occurred in 94 (54%) patients: 63 (58%) in the PVE group and 31 (48%) in the NVE group. In the PVE group, positive 18F-FDG PET/CT was significantly associated with a higher rate of primary endpoint (hazard ratio [HR]: 2.7; 95% confidence interval [CI]: 1.1 to 6.7; p = 0.04). Moderate to intense 18F-FDG valvular uptake was also associated with worse outcome (HR: 2.3; 95% CI: 1.3 to 4.5; p = 0.03) and to new embolic events in PVE (HR: 7.5; 95% CI: 1.24 to 45.2; p = 0.03) and in NVE (HR: 8.8; 95% CI: 1.1 to 69.5; p = 0.02). In the NVE group, 18F-FDG PET/CT was not associated with occurrence of the primary endpoint CONCLUSIONS: In addition to its good diagnostic performance, 18F-FDG PET/CT is predictive of major cardiac events in PVE and new embolic events within the first year following IE.
Primary Endpoint Occurrence: Univariate and Multivariate Analysis in PVE Univariate HR (95% CI) p Value Multivariate HR (95% CI) p Value Renal insufficiency at admission 2.16 (1.00-4.68) 0.05 CRP &gt;100 mg/l 2.46 (1.04-5.89) 0.02 1.90 (1.10-3.40) 0.03 Staphylococcus aureus 2.70 (1.10-6.55) 0.03 Severe valvular regurgitation 2.55 (1.01-6.41) 0.05 1.20 (0.70-2.10) 0.68 Echographic complications 1.15 (0.54-2.46) 0.72 Vegetation length &gt;10 mm 2.53 (1.19-4.60) 0.03 Positive 18F-FDG PET/CT 3.74 (1.30-10.80) 0.02 2.70 (1.10-6.70) 0.04 Moderate to intense 18FDG valvular uptake 2.70 (1.20-6.30) 0.02 2.30 (1.30-4.50) 0.03 Abstract Figure.
The strain-7 study: multimodal, multivendor, multifield strength, scan:rescan comparison of global longitudinal and circumferential strain in healthy volunteers
Authors: Nazir MS , Yazdani M , Draper JANE , Franks R , Lam S , Plein S , Kapetanakis S , Young A , Chiribiri A 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: Public Institution(s). Main funding source(s): National Insitute for Health Research
Background
There is clinical and prognostic evidence for global longitudinal strain (GLS) and circumferential strain (GCS). A range of techniques exist: 2-dimensional echocardiography (2Decho), 3-dimensional echocardiography (3Decho) and Cardiovascular Magnetic Resonance (CMR).
Purpose
To investigate inter-study repeatability and inter-method comparison of GLS and GCS techniques.
Methods

Volunteers underwent same day scan
rescan 2Decho, 3Decho, 1.5T Siemens CMR (Cine imaging and Displacement encoding with stimulated echoes [DENSE]), 3T Siemens CMR (Cine Imaging and DENSE) and 3T Philips CMR (Tagging and Fast strain-encoding [fSENC]) imaging. Strain was quantified for 2Decho (EchoPAC), 3Decho (TomTec), Feature tracking (FT) for cine imaging (CircleCVI), CIM (University of Auckland) for DENSE and Tag, and Myostrain (Myocardial solutions) for fSENC.
Results
20(6F) volunteers, mean age 33 ± 7 years, mean LVEF 62 ± 4%. All GLS and GCS methods had excellent inter-study agreement (ICC &gt; 0.75) with coefficient of variation (CoV) between 4-8% (Table 1). Median and IQR are presented in Figure 1.
Friedman’s test revealed statistically significant inter-method differences for GLS (χ2 = 66.4,p &lt; 0.0001) and GCS (χ2 = 50.9,p &lt; 0.0001). Post hoc analysis using Dunn’s test with Bonferroni correction demonstrated significant differences:
-GLS: 2Decho vs DENSE 1.5T (p = 0.001) and Myostrain 3T (p = 0.0116); 3Decho vs FT 3T (p = 0.049) and DENSE 1.5T (p &lt; 0.0001); FT 1.5T vs DENSE 1.5T (p = 0.001) and Myostrain 3T (p = 0.01); FT 3T vs Myostrain 3T (p &lt; 0.0001); DENSE 1.5T vs Tag 3T (p = 0.0008) and Myostrain 3T (p &lt; 0.0001); Tag 3T vs Myostrain (p = 0.02).
-GCS: 3Decho vs DENSE 1.5T (P = 0.0005), FT 1.5T (p &lt; 0.001), FT 3T (P &lt; 0.001) and Myostrain (p = 0.003); FT 1.5T vs Tag 3T (p = 0.001), FT 3T vs Myostrain 3T (p = 0.04).

Conclusion
There is excellent interstudy agreement for GLS and GCS methods. However, there are important inter-method differences in absolute values, that need to be considered for clinical application as a surveillance method and longitudinal studies.
Table 1 Acquisiton Post processing GLS CoV(%) GLS ICC GCS CoV(%) GCS ICC 2DEcho EchoPAC 4.88 0.80 - - 3DEcho TomTec 4.77 0.86 3.97 0.85 Siemens 1.5T cine FT CircleCVI 8.30 0.79 6.00 0.85 Siemens 3T cine FT CircleCVI 6.21 0.89 4.76 0.94 Philips 3T Tag CIM 6.15 0.89 5.86 0.88 Siemens 1.5T DENSE CIM 4.36 0.90 4.65 0.89 Philips 3T fSENC Myostrain 8.45 0.81 4.06 0.90 Interstudy agreement for the different GLS and GCS methods. Abstract Figure 1
Right ventricular apical and septal pacing: long term impacts on ventricular function
Authors: Mumin G , Celiker C 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: Public Institution(s). Main funding source(s): The costs of the study were afforded by the researchers.

Background
In patients requiring permanent pacemaker, in order to protect left and right ventricular functions the optimal pacing site has yet to be determined. Conflicting results exist about septal and apical pacing sites.

Aim
Our purpose was to evaluate the long term effects of right ventricular apical and septal pacing on left and right ventricular functions.

Methods
We scanned 378 patients from 2007 to 2012 who received a permanent pacemaker for the treatment of symptomatic bradyarrythmia. As exclusion criteria we identified the patients who did not have an echocardiography before the procedure, those who had co-morbidities which cause reduction in ventricular functions, ejection fraction &lt;%45, patients who died and those who rejected our invitation. 54 women and 66 men were eligible for our study. To determine the patients’ New York Heart Association Class (NYHA) we questioned and did the physical examination. Lead position confirmed by fluoroscopy in two planes, and electrocardiograms were obtained. Finally, we compared the pre-procedural echocardiographic data with our up-to-date findings.

Results
In sixteen patients the lead placement was inferoseptal and in one hundred and four patients apical site. Median follow up was 9 years. The mean ejection fraction before the implantation was 58,86 ± 4,08 in the apical, and 56,37 ± 8,8 in the septal group (p &lt; 0,05). The long term follow up showed that these values have been reduced, 56,66 ± 8,38 for the apical group and 51,33 ± 13,94 for the septal group, respectively (p &lt; 0,05). Placing the right ventricular lead in both septal and apical site resulted in reduced tricuspid annular plane systolic excursion (from mean 2,25 to 2,18, (p &lt; 0,05)), and in increased systolic pulmonary artery pressure (from 35,46 ± 9,93 to 39,84 ± 11,21 (p &lt; 0,05)). There were no differences regarding the mitral and tricuspid insufficiencies, and diastolic functions before the implantation and long term follow up. These findings were independent of neither the etiology of implanting the pacemaker nor the underlying diseases.

Conclusion
These two selective ventricular pacing sites caused a reduction in both left and right ventricular functions. Despite the ejection fraction declines, most of these patients have a good quality of life, without symptoms and signs of heart failure. But certainly, there is emerging need for more randomized trials in order to describe the optimal RV pacing site. The main purpose must be preserving better ventricular functions in patients requiring permanent ventricular pacing.
Prognosis of atrial fibrillation radiofrequency ablation: Iodine-123-MIBG cardiac innervation imaging
Authors: Varlamova Y , Zavadovsky K , Kisteneva I , Sazonova S , Batalov R 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: Foundation. Main funding source(s): Russian Science Foundation

BACKGROUND
To date, radiofrequency catheter ablation (RFCA) of atrial fibrillation (AF) is a valuable treatment option. So far there are no clear predictors of the effectiveness of AF ablation. Nowadays, the association of cardiac sympathetic nervous system and the development and maintenance of AF has been showed. The association between сardiac sympathetic system impairment, assessed by 123I-MIBG scintigraphy and RFCA effectiveness was shown previously. However, the is lack of data concerning to prognostic value of MIBG scintigraphy in patients with different forms of AF – persistent (PAF) and long-standing persistent (LSPAF).

PURPOSE
To study the prognostic significance of 123I-MIBG scintigraphy in RFCA efficacy in patients with persistent and long-standing persistent AF.

METHODS
The study enrolled 36 patients with drug-resistant AF (both persistent (n = 20) and long-standing persistent (n = 16) forms). All patients had comorbidity as chronic coronary syndrome and hypertension. A comparison group (n = 10) was also enrolled in the study. It includes patients with chronic coronary syndrome and arterial hypertension (without arrhythmia). All patients underwent 123I-MIBG and 99mTc-MIBI scintigraphy to evaluate the cardiac sympathetic activity and myocardial perfusion, respectively. Patients with AF performed RFCA. After of 1 year follow-up patients were examined for AF recurrence. The AF recurrence was considered to be an AF of at least 30 sec duration on a 12-lead ECG or on the Holter monitoring.

RESULTS
During the follow-up period, AF recurrences were reported in 7 (35%) PAF patients and in 8 (50%) LSPAF patients. According to multivariate analysis only a high pre-ablation washout rate of 123I-MIBG (WR) in PAF patients (OR: 1.668; 95% CI: 1.093–2.548) and large pre-ablation 123I-MIBG/99mTc-MIBI mismatch score in LSPAF patients (OR: 2.155; 95% CI: 1.192–3.897) were independent predictors of AF recurrence after RFCA. ROC analysis indicated that in PAF patients with higher WR ≥ 20.8% (AUC: 0.968; Sensitivity: 100 %; Specificity: 45%; p &lt; 0.05) and LSPAF patients with larger 123I-MIBG/99mTc-MIBI mismatch score ≥ 12.5 % (AUC: 0.942; Sensitivity: 81 %; Specificity: 28 %; p &lt; 0.05) had a higher risk of AF recurrence after RFCA.

CONCLUSION
In AF patients the incidence of arrhythmia recurrence after RFCA is associated with impaired cardiac sympathetic nervous activity. In PAF patients the values of 123I-MIBG washout rate can predict AF recurrence. In LSPAF patients innervation/perfusion mismatch has prognostic value in terms of AF recurrence.
Right ventricular stroke work index in adult patients with pulmonary arterial hypertension: - a comparison between echocardiography and right heart catheterization
Authors: Jumatate R , Ingvarsson A , Smith G , Roijer A , Ostenfeld E , Waktare J , Radegran G , Meurling C , Werther Evaldsson A 
Published: February 2021

Abstract

Funding Acknowledgements
Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Swedish Society of Pulmonary Hypertension

Background
In adult patients with pulmonary arterial hypertension (PAH), right ventricular (RV) failure may worsen rapidly, constituting a negative prognostic factor. In this population, non-invasive assessment of RV function is challenging. RV stroke work index (RVSWI) reflects right ventricular function and has been proposed to predict outcome in PAH. However, RVSWI assessed by echocardiography (ECHO) has not been thoroughly compared to measures with right heart catheterization (RHC) in adults. The aim of the present study was to therefore evaluate RVSWI derived by echocardiography (RVSWIECHO) vs. RHC (RVSWIRHC).

Methods
Fifty-four consecutive treatment naïve adult patients with PAH, were retrospectively analysed. All patients performed echocardiography and RHC with a median time of 1 day [IQR 0-1 days]. RVSWIRHC was calculated as: (mean pulmonary arterial pressure (mPAP) – mean right atrial pressure (mRAP)) x stroke volume index (SVI)RHC. Four methods for RVSWIECHO were evaluated: RVSWIECHO-1 = Tricuspid regurgitant maximum pressure gradient (TRmaxPG) x SVIECHO, RVSWIECHO-2=(TRmaxPG-mRAPECHO) x SVIECHO, RVSWIECHO-3 = TR mean gradient (TRmPG) x SVIECHO and RVSWIECHO-4=(TRmPG–mRAPECHO) x SVIECHO. Vena cava inferior diameter and its collapsibility were used for estimation of mRAPECHO.. Pearson’s correlation coefficients were used and data was expressed as mean ± standard deviation.

Results
Mean RVSWIRHC was 1132 ± 352 mmHg*mL*m-2. Mean RVSWIECHO-1-4 was: 1904 ± 568, 1732 ± 531, 1090 ± 366 and 918 ± 336 mmHg*mL*m-2, respectively. There was no significant difference between RVSWIRHC and RVSWIECHO-3 in mean values, although they exhibited the lowest correlation, but moderate (r = 0.66). The strongest correlation was demonstrated for RVSWIECHO-2 (r = 0.78, p &lt; 0.001), followed by a moderate correlation for RVSWIECHO-1 and RVSWIECHO-4 (r = 0.75 and r = 0.69, p &lt; 0.001). The absolute (relative) bias for RVSWIECHO-1 was -772 ± 385 (-50 ± 20%) mmHg*mL*m-2, RVSWIECHO-2 -600 ± 339 (-41 ± 20%) mmHg*mL*m-2, RVSWIECHO-3 42 ± 286 (5 ± 25%) mmHg*mL*m-2 and for RVSWIECHO-4 214 ± 273 (23 ± 27%) mmHg*mL*m-2.

Conclusion
RVSWIECHO-3, using the mean tricuspid gradient and SVIECHO, showed no significant difference to RVSWIRHC, albeit a moderate correlation between the methods. RVSWIECHO-1, RVSWIECHO- 2 and RVSWIECHO-4 exhibited moderate to strong correlations to RVSWIRHC, but poor concordance between absolute values. The clinical utility of RVSWIECHO and RVSWIRHC in assessing RV function in relation to PAH prognosis, risk stratification and treatment response remains to be evaluated in a larger clinical context.