bluuebunny/crossref_metadata_2025_split huggingface dataset:
Potential benefits of ambulatory blood pressure monitoring in coarctation of aorta
Authors: Egbe A , Banala K , Vojjini R 
Published: November 2020

Abstract

Background
Ambulatory blood pressure monitoring (ABPM) is more sensitive than resting BP assessment in detecting hypertension in patients with coarctation of aorta (COA). However, it is not known whether ABPM provides a better measure of left ventricular (LV) pressure load and LV remodeling as compared to resting BP. LV pressure overload is the lynchpin in the pathogenesis of cardiovascular mortality in COA patients, because it leads to LV remodeling, LV dysfunction and heart failure, which sets-the-stage for cardiovascular death. Therefore identifying a sensitive clinical metric that reflects LV pressure load and LV remodeling, is an important step towards optimizing antihypertensive therapy to prevent LV dysfunction and mortality. We hypothesized that ambulatory systolic BP (SBP) has better correlation with arterial load and LV remodeling indices as compared to resting SBP.

Methods
Our study cohort comprised of adults with isolated COA without significant residual coarctation (Doppler peak velocity ≤2.5 m/sec), aortic or mitral valve disease. All patients underwent BP measurement at rest, followed by ABPM. Arterial load (LV pressure load) was assessed using Doppler-derived effective arterial elastance index (EAI). LV remodeling was assessed using: LV mass index (LVMI), and E/e'. We divided the cohorts into 4 categories (normotensive, masked, white-coat, and overt hypertension).

Results
Of the 106 patients (male 69 [65%); age 39±11 years), 98 (93%) had prior COA intervention at 6±5 years. Resting SBP was 132±17, daytime ambulatory SBP was 142±16, and nighttime ambulatory SBP was 128±12 mmHg. The mean arterial load and LV remodeling indices were: EAI 3.4±0.8 mmHg/ml·m2, LVMI 106±21 g/m2, and E/e'11±4. Compared to resting SBP, ambulatory SBP had better correlation with EAI, LVMI, and E/e'. EAI, LVMI and E/e' were significantly different between hypertension categories (Figure 1).

Conclusions
In this study, we demonstrated that ambulatory SBP had better correlations with arterial load and LV remodeling indices as compared to resting SBP. This suggests that ambulatory SBP was a more robust metric of LV pressure overload and remodeling, and can improve detection of vascular stiffness, LV hypertrophy and elevated LV filling pressure as compared to resting SBP. The results of the current study have important clinical implications with regards to initiation and titration of antihypertensive therapy. If ABPM is used as the gold standard for diagnosis of hypertension, the masked hypertension group (who would have been misclassified as normotensive based on resting SBP alone) will qualify for antihypertensive therapy. Additionally, since ambulatory SBP had better correlation with LV remodeling indices, perhaps titration of antihypertensive therapy using ABPM will potentially reduce the risk of LV hypertrophy and dysfunction due to hypertension.
Figure 1

Funding Acknowledgement
Type of funding source: None
Pulmonary arterial hypertension in Spanish pediatric registry age: clinical characterization, management and survival
Authors: Playan Escribano J , Segura De La Cal T , Escribano Subias P , Labrandero C , Rodriguez Ogando A , Moreno Galdo A , Lozano Balseiro M , Moya Bonora A , Gomez Guzman E , Espin Lopez J.M , Perin F , Plata Izquierdo B , Viadero Ubierna M.T , Zapata Sanchez M , Del Cerro Marin M.J 
Published: November 2020

Abstract

Background
Pulmonary arterial hypertension (PAH) includes idiopathic PAH and congenital heart disease (CHD) related PAH. A wide variety of CHD can develop PAH, but their clinical characteristics define four large groups: Eisenmenger, PAH associated with non-restrictive shunt, severe PAH associated with restrictive shunt and postoperative PAH. Our aim was to study the clinical and prognostic differences between these groups and idiopathic PAH.

Methods
The REHIPED registry is a Spanish, multicenter, observational and prospective registry on pulmonary hypertension in the pediatric population (&lt;18 years of age) that began in 2008. 183 patients were analyzed: baseline characteristics, functional class, right catheterization data, treatment and survival were compared

Results
183 patients were analyzed, characteristics are detailed in the table. In patients with idiopathic PAH, treatment with prostanoids was more frequently used as first line therapy and during follow up. The average follow-up time was 9 years. There was not a statistically significant difference in survival among the 4 clinical groups of CHD related PAH. There was a borderline significant difference (logrank p 0.05) in survival between the group of CHD related PAH and idiopathic PAH.

Conclusion
Idiopathic PAH patients have worse outcome than CHD related PAH patients although they have less comorbidities, less severe hemodynamics and are treated more aggressively.

Funding Acknowledgement
Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Janssen, Ferrer
Shift in transcriptional landscape of human right ventricle in chronic thromboembolic pulmonary arterial hypertension
Authors: Jafari L , Doerr O , Chelladurai P , Pullamsetti S.S , Troidl C , Keller T , Guenther S , Gruen D , Keranov S , Kriechbaum S , Liebetrau C , Mayer E , Seeger W , Hamm C.W , Nef H.M 
Published: November 2020

Abstract

Background
Chronic thromboembolic pulmonary hypertension (CTEPH) is a sub group of pulmonary hypertension (PH). CTEPH is characterized by the existence of thromboemboli and vascular remodeling in pulmonary vessels. The effect of increase in pulmonary artery pressures causes right ventricle (RV) hypertrophy and dilatation and finally leads to right heart failure and death. Surgical intervention in operable patients makes the CTEPH as an only curable and unique form of ph. Pulmonary endarterectomy (PEA) is the surgical procedure to remove the thromboembolic clots from the pulmonary vasculature, which restores RV function back to normal with significant improvements in cardiovascular magnetic resonance.

Purpose
The aim of this study is to use transcriptomic profiling to identify signaling pathways, master regulators, and potentially new biomarkers that specifically indicate the effect of PEA on the RV of patients with chronic thromboembolic pulmonary hypertension.

Results
RNA -sequencing (RNA-seq) was performed on RV biopsies obtained from CTEPH patients at PEA baseline (before PEA surgery) and the results were compared with those from RV biopsies obtained during follow-up evaluation. Bioinformatic analysis of RNA-seq data identified 2799 genes (n=14, −0.585 ≤ Log2 fold change ≥0.585, FDR ≤0.05) differentially regulated between the PEA baseline and follow-up sample groups. The great number of genes (2799) differentially expressed after PEA surgery in CTEPH patients confirms a major shift in the transcriptional landscape of RV in these patients. To further identify potential biomarker candidates from the large pool of 2799 differentially expressed genes (DEGs), extensive bioinformatic analysis of different data sets shortlisted 250 DEGs that were functionally associated with cardiovascular development or disease. The findings of this study reveal prominent transcriptional changes that occur in response to PEA. Gene ontology enrichment and pathway analysis confirmed altered regulation of hypoxia-inducible factor 1 (HIF-1) signaling, advanced glycation end products and their receptors (AGE-RAGE), mitogen-activated protein kinase (MAPK) signaling, hippo signaling, the Janus kinase/ signal transducers and activators of transcription (Jak-STAT) signaling pathway, and proteoglycans after PEA compared with before PEA.

Conclusion
Comparison of the results of RNA-seq analysis of RV biopsies of CTEPH patients, pre and post PEA, revealed a major shift in the transcriptional landscape of these patients after reducing the pressure overload of the RV by PEA.

Funding Acknowledgement
Type of funding source: Foundation. Main funding source(s): German Research Foundation (DFG)
Additional role of echocardiogram in pulmonary arterial hypertension risk stratification according to current ESC/ERS guidelines
Authors: Rotunno M , Dardi F , De Lorenzis A , Palazzini M , Zuffa E , Pasca F , Guarino D , Magnani I , Ballerini A , Manes A , Galie N 
Published: November 2020

Abstract

Background
Current pulmonary hypertension (PH) guidelines stratify the risk of patients with pulmonary arterial hypertension (PAH) using a multiparametric approach. A simplified risk table has been recently proposed and validated without including echocardiographic parameters.

Purpose
We evaluate the additional role of echocardiogram in PAH risk stratification using the recently proposed simplified risk table in patients with idiopathic/heritable (I/H) PAH and PAH associated with connective tissue disease (CTD) and congenital heart disease (CHD).

Methods
All patients with I/H-PAH, CTD-PAH and CHD-PAH referred to a single centre were included from 2003 to 2017. All patients were treated according to PH guidelines. The simplified risk assessment considered the following criteria: WHO functional class, 6-min walking distance, right atrial (RA) pressure or brain natriuretic peptide (BNP) plasma levels and cardiac index (CI) or mixed venous oxygen saturation (SvO2). For the last 2 criteria the worst parameter was chosen. Risk strata were defined as: Low risk= at least 3 low risk and no high-risk criteria; High risk= at least 2 high risk criteria including CI or SvO2; Intermediate risk= definitions of low or high risk not fulfilled. Then we performed a Cox analysis to evaluate the independent echocardiographic predictors of survival that were subsequently added to the simplified risk table to test their additional role in ameliorating risk stratification.

Results
461 treatment-naïve patients were enrolled. Echocardiographic independent predictors of prognosis were the severity of tricuspid regurgitation [HR (95% CI) = 1.013 (1.006–1.021); p-value = 0.001], right atrial area [HR (95% CI) = 1.028 (1.012–1.045); p-value = 0.001] and the presence of pericardial effusion [HR (95% CI) = 1.533 (1.142–2.057); p-value= 0.004]. Only RA area significantly ameliorate the risk stratification power of the recently validated simplified PAH risk table (likelihood ratio chi2 increased from 63.8 to 68.1, likelihood ratio test = 0.039). Due to the significant correlation between RA area and both RA pressure (r=0.470; p&lt;0.001) and BNP (r=0.372; p=0.004), we elaborate a second risk table in which RA area (utilizing the cut-offs proposed by the current PH guidelines) was considered together within the criteria including RA pressure and BNP (the worst parameter of the 3 was considered). Considering the second risk table including RA area the risk stratification power significantly improved (likelihood ratio chi2 increased from 63.8 to 72.7, AIC/BIC decreased from 1956/1964 to 1947/1955).

Conclusions
Echocardiographic RA area significantly improve the risk discrimination power of the recently proposed simplified risk table for patients with PAH.

Funding Acknowledgement
Type of funding source: None
Could we suspect pulmonary veno-oclussive disease with a cardiopulmonary exercise test?
Authors: Perez Olivares Delgado C , Segura De La Cal T , Flox A , Prudencio V , Cristo M.J , Coronel L , Arribas F , Escribano P 
Published: November 2020

Abstract

Background
Cardiopulmonary exercise test (CPET) provides information about the pathophysiology of the exercise limitation. CPET parameters in pulmonary arterial hypertension (PAH) exhibit distinctive features, such as low peak oxygen uptake (pVO2), low end tidal carbon dioxide tension (PetCO2) and high ventilation-carbon dioxide slope (VE/VCO2).Pulmonary veno-oclussive disease (PVOD) is a PAH subgroup with particular underlying physiology, difficult diagnosis and poor prognosis.

Purpose
We hypothesized that different CPET patterns could be identified in PAH subgroups, and this may contribute to their diagnosis workup.

Methods
CPETs performed on the following PAH subgroups between September 2019 and January 2020 were prospectively included: Idiopathic PAH (IPAH), Heritable PAH (HPAH), and PAH responders to calcium channel blockers (CCB-R). Due to its low prevalence and its clinical relevance, CPET data on PVOD patients was retrospectively included.

Results
57 CPET were included: 20 IPAH (35%); 13 HPAH (22.8%); 9 CCB-R (15.8%),15 PVOD (26.3%). All patients presented impaired functional capacity with reduced peakVO2 and increased VE/VCO2). Remarkably, PVOD patients,in spite of a younger age, exhibit the worst functional capacity and ventilatory efficiency even showing downward PetCO2 kinetics. CCB-R group achieved the highest peakVO2 and had better ventilatory parameters.(Table 1)

Conclusion
CPET is a useful tool to classify patients by PAH subgroups. Very low PetCO2 at rest and a downward PetCO2 kinetic strongly suggest PVOD, which has significant implications on treatment.

Funding Acknowledgement
Type of funding source: None
Thermodilution vs indirect fick cardiac output measurement in clinical practice: insights from a tertiary centre
Authors: Pereira A , Santos J.G , Loureiro M.J , Ferreira F , Almeida A.R , Cale R , Repolho D , Vitorino S , Morgado J , Pereira H 
Published: November 2020

Abstract

Introduction
Thermodilution (TD) and indirect Fick (IF) methods are widely used to measure cardiac output (CO). They are often used interchangeably to make critical clinical decisions, yet few studies have compared these approaches concerning agreement and comparative prognostic value as applied in medical practice.

Purpose
To assess agreement between TD and IF methods and to compare how well these methods predict mortality.

Methods
Retrospective cohort study including all consecutive right heart catheterizations performed in a referral pulmonary hypertension (PH) centre from 2010 to 2018. Cardiac index (CI) was calculated by indexed CO to body surface area. PH was classified according to the new definition of the 6st World Symposium on Pulmonary Hypertension 2018 [mean pulmonary arterial pressure (mPAP) &gt;20 mmHg]. Patients with cardiac or extra-cardiac shunts or significant (moderate to severe or severe) tricuspid regurgitation were excluded. All-cause mortality over 1 year after right heart catheterization was recorded. Logistic regression was used to identify predictors of the adverse event.

Results
From a total of 569 procedures, 424 fulfilled the inclusion criteria: mean age 56.7±15.4 years, 67.3% female. Haemodynamic parameters were diagnosed of PH in 86.2% of cases: mPAP 35.3±15.3 mmHg, 83.6% pre-capillary subtype, 42.9% belonging to group 4 (chronic thromboembolic pulmonary hypertension) and 26.6% to group 1 (pulmonary arterial hypertension). Mean values of CO and CI were, respectively, 4.5±2.8 L/min and 2.5±0.8 L/min/m2 measured by TD and 4.6±2.4 L/min and 2.6±1.3 L/min/m2 measured by IF method. There was a median difference (IF minus TD) of - 0.03 / min to CO and - 0.05 L/min/m2 to CI but both meausres correlated only modestly (r=0.6 to TD and r=0.5 to IF). One-year all-cause mortality rate was 5.4% (median time to death was 50.5 days). Lower values of CO and CI assessed by TD were significantly associated with all-cause mortality occurrence (CO TD: 4.5±1.3 L/min versus 3.6±1.0 L/min, p&lt;0.01; CI TD: 2.6±0.7 L/min/m2 versus 2.1±0.4 L/min/m2, p&lt;0.01). No association was observed between CO (p=0.31) and CI (p=0.42) measured by IF method and the adverse event. Logistic regression identified 2 independent predictors of all-cause mortality: TD CO (OR 0.55, 95% CI 0.38–0.79, p&lt;0.01) and TD CI (OR 0.34, 95% CI 0.17–0.67, p&lt;0.01). Similar results were obtained when patients diagnosed with PH were independently analyzed.

Conclusions
There is only modest agreement between TD and IF CO and CI estimates. Despite being more time-consuming, TD measurements were predictors of all-cause mortality and present a highest prognostic value. These findings favored their used over IF in clinical practice.

Funding Acknowledgement
Type of funding source: None
Efficacy and outcomes of balloon pulmonary angioplasty in elderly vs non-elderly chronic thromboembolic pulmonary hypertension patients
Authors: Shinya Y , Kimura M , Kawakami T , Hiraide T , Moriyama H , Kataoka M , Endo J , Itabashi Y , Murata M , Kohno T , Fukuda K 
Published: November 2020

Abstract

Introduction
Balloon pulmonary angioplasty (BPA) has been reported as an effective and safe treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, its safety and efficacy in elderly patients remains unknown.

Purpose
We investigated the effect of BPA on hemodynamics and respiratory parameters, functional capacity, and short- and long-term outcome in elderly patients.

Methods
From November 2012 to May 2018, 141 consecutive CTEPH patients who underwent BPA in a single university hospital were enrolled (age: 65 [54.5–74] years old, WHO functional class [WHO-FC] II/III/IV; 35/96/10). Patients were divided into two groups according to the age; elderly (≥75 years, N=32) and young groups (&lt;75 years, N=109). Hemodynamics (right-sided heart catheterization), biomarkers (brain natriuretic peptide), respiratory function (spirometry and diffusion capacity measurement), and functional capacity (6-minute walk distance [6MWD] and WHO-FC) were evaluated at baseline and 1-year post BPA. Procedure-related complications (in hospital death, use of percutaneous cardiopulmonary support [PCPS], and pulmonary injury) and all cause death during the follow up period were also assessed.

Results
At baseline, although elderly group had less severe hemodynamics (mPAP: 33.1±6.7 vs 39.0±11.8 mmHg, p&lt;0.05), they had poor exercise capacity and reduced pulmonary diffusion capacity, compared with young group (6MWD: 264.6±101.3 vs 369.7±105.2 m, %DLco: 42.0±12.0 vs 50.2±12.7%, all p&lt;0.05). BPA improved hemodynamics, biomarkers, exercise capacity, and pulmonary diffusion capacity in both elderly and young groups (all p&lt;0.05). There was no in-hospital death or use of PCPS in both groups, although the incidence of pulmonary injury was higher in elderly group (14.3% vs 5.3%, p&lt;0.01). Under the normalized hemodynamics 1-year after BPA in both groups, exercise capacity and pulmonary diffusion capacity were worse in the elderly group than young groups (p&lt;0.01). The incidence of all-cause death in the follow up period was higher in elderly group, all of which were due to non-pulmonary hypertension (PH)-related death (p&lt;0.01).

Conclusion
BPA was effective in improving hemodynamics and respiratory parameters and functional capacity, in associated with no critical complication, regardless of the age. Elderly patients who were treated with BPA were associated with higher incidence of non-PH-related death.
Changes of mean PAP in the two groups

Funding Acknowledgement
Type of funding source: None
Treatment with macitentan for pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD): real-world experience from the combined OPUS and OrPHeUS data sets
Authors: McLaughlin V , Chin K , Kim N.H , Flynn M , Ong R , Wetherill G , Channick R 
Published: November 2020

Abstract

Introduction
Restrictive inclusion criteria can exclude some CHD-PAH patients from clinical trials. The OPsumit® USers (OPUS) Registry and the OPsumit® Historical USers (OrPHeUS) data sets provide real-world data in PAH patients newly started on macitentan, including patients with CHD-PAH regardless of defect type.

Purpose
To describe the characteristics, safety and clinical outcomes of CHD-PAH patients newly treated with macitentan.

Methods
OPUS is a prospective, US, multicentre, observational drug registry ongoing since April 2014. OrPHeUS was a retrospective, US, multicentre chart review; observation period Oct 2013–Mar 2017. This analysis reports information on CHD-PAH patients in the combined OPUS/OrPHeUS data set, descriptively compared with idiopathic/heritable PAH (I/HPAH) patients.

Results
As of Sept 2019, there were 4268 PAH patients with follow-up data, of whom 264 (6%) had CHD-PAH and 2396 (56%) had I/HPAH. For CHD-PAH and I/HPAH patients respectively at macitentan initiation: median age (Q1, Q3) was 48 (36, 62) and 65 (53, 73) years; 199 (75%) and 1748 (73%) were female; 67/114 (59%) and 802/1301 (62%) were WHO functional class III/IV; median (Q1, Q3) 6-minute walk distances were 350 (274, 420) and 289 (195, 375) m for the 82 and 840 patients with measurements; median (Q1, Q3) time from PAH diagnosis to macitentan initiation was 37.3 (4.5, 113.1) and 7.4 (1.4, 38.3) months; and 99 (38%) and 1056 (44%) initiated macitentan as monotherapy. The number of patients with ≥1 hepatic adverse event (HAE) was similar for CHD-PAH and I/HPAH (22 [8%] and 184 [8%]), as were the adverse event (AE) profiles (collected from OPUS only). Exposure, discontinuations, outcomes and most common AEs are shown in the table.

Conclusions
In general, compared with I/HPAH patients, CHD-PAH patients were younger and a greater proportion had prevalent disease than I/HPAH patients. Safety and outcomes were similar between the groups.

Funding Acknowledgement
Type of funding source: Other. Main funding source(s): Actelion Pharmaceuticals Ltd
Impact of timing on major complications after thoracic endovascular aortic repair for uncomplicated type B aortic dissection
Authors: Xie E.M , Liu J.T , Yang F , Ding H.Y , Hu B.Q , Chen L.F , Yang X.Y , Luo J.F 
Published: November 2020

Abstract

Background
This study aimed to analyze the impact of the timing of intervention from presentation of symptoms to TEVAR and its relation to major complications for uncomplicated TBAD.

Methods
We retrospectively analyzed the data of 390 patients who underwent TEVAR for acute, subacute and chronic uncomplicated type B aortic dissection from January, 2010 to December 2017. Standard univariate and survival methods were used. The primary outcome of interest was in-hospital mortality. The Secondary outcomes of interest was long-term death and in-hospital adverse events, including postoperative coma, postoperative endoleak, postoperative stroke, postoperative spinal cord ischemia, postoperative limb ischemia, postoperative mesenteric ischemia/infraction.

Results
Of the 390 TEVARs for uncomplicated TBAD, 145 were performed within 14 days of presentation of symptoms (acute), 172 between 14 hours and 90 days (sub-acute), and 73 were beyond 90 days (chronic). Demographic characteristics were similar among groups (Table 1). A slight increase of in-hospital death, post-operative endoleak and limb ischemia, were observed in the acute intervention group compared with the sub-acute group (4.8% vs 2.3%, 11.7% vs 9.3%, 2.8% vs 2.3%, respectively). The chronic group patients had a highest rates of post-operative endoleak. However, there are no significantly differences between the three groups (Table 2). During long-term follow-up (median duration 36.9 months, interquartile range, 22.5–67.7 months), overall survival was similar among groups (log-rank test, P=0.280) (Figure).

Conclusion
TEVER performed for uncomplicated TBAD within 14 days to the presentation of symptoms, as compared with TEVAR between 14 hours and 90 days or beyond 90 days, neither appear to increase the risk of in-hospital complications nor long-term mortality.
Long-term All-cause Mortality

Funding Acknowledgement
Type of funding source: Public hospital(s). Main funding source(s): This research was supported by grant DFJH201807 from High-level Hospital Construction Project of Guangdong Provincial People's Hospital.