bluuebunny/crossref_metadata_2025_split huggingface dataset:
Determinants of left ventricular hypertrophy and dysfunction in adults with coarctation of aorta
Authors: Egbe A , Vojjini R , Banala K 
Published: November 2020

Abstract

Background
Several coarctation of aorta (COA) severity indices are used for timing of COA intervention, and to define severity of residual coarctation post-intervention. However, it is unclear how many of these COA indices are required in order to recommend intervention, and what degree of residual coarctation results in suboptimal recovery of the left ventricle (LV). Our aim was to assess the correlation between different COA indices and effects of chronic LV pressure overload (LV hypertrophy, diastolic and systolic dysfunction), and to determine the effect of residual coarctation on LV reverse remodeling after COA intervention.

Methods
COA severity indices were defined as Doppler COA gradient, systolic blood pressure, upper-to-lower-extremity systolic blood pressure gradient, aortic isthmus ratio. LV remodeling indices were defined as LV mass index (LVMI), LV global longitudinal strain (LVGLS), e' and E/e'. LV reverse remodeling was defined as the difference between indices obtained pre-intervention and 5-years post-intervention (delta LVMI, e', E/e', LVGLS).

Results
Of the COA indices analyzed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β±standard error −28.3±14.1, p&lt;0.001), LVGLS (1.51±0.42, p=0.005), e' (3.11±1.10, p=0.014), and E/e' (−13.4±6.67, p=0.008). Residual aortic isthmus ratio also had the strongest correlation with LV reverse remodeling, and residual aortic isthmus ratio &lt;0.7 was predictive of suboptimal LV reverse remodeling post-intervention.

Conclusion
Considering the known prognostic implications of LV remodeling and reverse remodeling in response to pressure overload, these results support the use of aortic isthmus ratio for timing of COA intervention, and for prognostication post-intervention.

Funding Acknowledgement
Type of funding source: None
Iron status, anemia and functional capacity in adults with congenital heart disease: a single center analysis
Authors: Karathanos A , Simon I , Brockmeyer M , Lin Y , Parco C , Krieger T , Schulze V , Hellhammer K , Kelm M , Zeus T , Wolff G 
Published: November 2020

Abstract

Background
Iron is essential to the mitochondrial energy production in cardiomyocytes and its depletion is negatively associated with symptoms, functional capacity, quality of life and outcomes in patients with heart failure – independent of anemia. The relevance of iron deficiency in adults with congenital heart disease however has not been evaluated to date, and we thus aimed to evaluate it in an all-comer cohort of patients with congenital heart disease in correlation with symptoms and functional capacity.

Methods and results
527 patient cases from one referral center over 2 years were evaluated concerning their iron status, anemia, functional capacity and ejection fraction of their systemic ventricle. 264 were female, 94 had a shunt lesion, 96 had left-sided obstructive lesions, 181 right-sided lesions, while 108 were considered to have complex lesions and 28 were cyanotic. The median age was 34 years, the mean BMI was 25.2±5 kg/m2, 429 patients had a normal ejection fraction and 34 moderately and severely depressed. 35 patients were classified as NYHA III, and 56 as NYHA II, while their functional capacity was evaluated via cardiopulmonary testing with a mean VO2max/kg of 22.6±6.5 and mean 69±17% of the expected. The mean serum iron concentration was 99.4±42.3 mcg/dL, their mean transferrin saturation was 27.36±13%, the mean ferritin concentration was 130.8±185 ng/mL, the mean soluble transfer factor was 1.3±0.66 mg/l and their mean Hemoglobin 14.8±2 mg/dL, while the mean MCV was 88±5.3 and the mean MCHC 33.7±1.4.
40 patients were anemic according to the WHO definition for anemia, in 28 of those patients that was already known. Iron deficiency according to stratified according to ferritin was present in 53 patients. However, when stratified according to the heart failure guidelines definition for iron deficiency 299 patients were found affected. Using the soluble transferrin receptor (sTfR) and sTfR-ferritin index iron deficiency was suspected in 10 additional individuals. Iron deficiency was associated with the ejection fraction (p=0.0001) - patients with moderately or severely depressed systemic ventricular function more often were diagnosed with iron deficiency (p=0.007)-, while it did not correlate with functional NYHA classification (p=0.622) or functional capacity (p=0.1 and 0.057). Iron deficiency was also not found significantly different amongst congenital defects but did correlate with all laboratory iron studies.

Conclusions
In this ambulatory population of adults with congenital heart disease we found an association of ejection fraction with iron deficiency, however no association of iron deficiency with functional capacity. The question arising is if a new definition of iron deficiency anemia in congenital heart disease similar to heart failure would be of clinical value.

Funding Acknowledgement
Type of funding source: None
Idiopathic pulmonary hypertension in Spanish pediatric registry: clinical characterization, management, and risk factors for survival
Authors: Playan Escribano J , Segura De La Cal T , Escribano Subias P , Mendoza Soto A , Labrandero C , Guillen Rodriguez I , Caicedo Cuenca L.M , Sabate Rotes A , Villagra Albert S , Lopez Ramon M , Conejo Munoz L , Izquierdo Riezu M.A , Inigo Martin G , Escriba Bori S , Del Cerro Marin M.J 
Published: November 2020

Abstract

Introduction
Pulmonary arterial hypertension (PAH) is a rare disease that affects the small pulmonary arteries, producing gradual obliteration of arterial lumen leading to the progressive increase in pulmonary vascular resistance and, ultimately, right ventricular failure and death. Idiopathic PH refers to patients without an identifiable underlying cause, although some of them may have a genetic cause. Our aim was to define the baseline characteristics, initial therapy and risk factors for survival of patients with 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 2007. 47 patients with idiopathic PAH were analyzed.

Results
The mean age at diagnosis was 4.5 years, and there was a similar proportion of both sexes. 64% of patients were in functional class I-II. The mean pulmonary arterial pressure was 47,3±16,3 mmHg, with mean pulmonary vascular resistance index of 12±8,3 WU·m2. Initial therapy was oral monotherapy in 67%, prostanoids monotherapy in 11% and combination therapy with prostanoids in 9%. At the end of follow up, 55% of patients had received prostanoids. The mean follow-up time was 8 years. The survival rates (free of death or transplantation) was 91% (95% confidence interval (CI): 79–97%), 82% (95% CI: 67–91%) and 70% (95% CI: 52–82%) at 1, 5 and 10 years. Lung transplantation was performed in 7 patients. The main factors influencing survival, after adjustment for pulmonary vascular resistance, were functional class III-IV (Hazard Ratio (HR) 4.6, p=0.022, 95% CI: 1.3–15.3) and diagnosis under the age of 2 (HR 4, p=0.031, 95% CI: 1.1–14.4).

Conclusions
Idiopathic Pulmonary hypertension in pediatric age affects both sexes equally and begins at a young age. Prostanoids were used in 55% of patients. The main factors influencing survival, after adjustment for pulmonary vascular resistance, were functional class III-IV and diagnosis under the age of 2.

Funding Acknowledgement
Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Janssen, Ferrer
Aetiology of sarcoidosis-associated pulmonary hypertension
Authors: Mathijssen H , Huitema M.P , Huitema M.P , Bakker A.L.M , Bakker A.L.M , Smits F , Smits F , Snijder R.J , Snijder R.J , Mager H.J , Mager H.J , Grutters J.C , Grutters J.C , Post M.C , Post M.C 
Published: November 2020

Abstract

Background
Pulmonary hypertension (PH) is a known complication of pulmonary sarcoidosis and its aetiology is unclear. The severity of PH may not correlate well with the degree of parenchymal disease; therefore other pathophysiological mechanisms have been hypothesized.

Purpose
The purpose of this study is to provide an insight in the different mechanisms of sarcoidosis-associated pulmonary hypertension (SAPH) and their characteristics.

Methods
We conducted a cohort study on all SAPH patients in our center. All patients were extensively analysed and discussed in a multidisciplinary expert team to determine the pathophysiology of PH case by case. Finally, patients were classified into different groups or a combination of groups based on the most likely mechanism of PH.

Results
Between 2010 and 2019, 41 patients with SAPH were identified. One patient was classified as isolated post-capillary PH, two patients as combined pre- and post-capillary PH and 38 patients as pre-capillary PH based on right heart catheterisation. In total, 17 patients were classified as PH solely due to chronic lung disease, one patient as suspected vasculopathy and one patient as compression of pulmonary vasculature. A combination of chronic lung disease with suspected vasculopathy (n=16), left heart disease (n=2), chronic pulmonary embolisms (n=1) or compression of pulmonary vasculature (n=5) was also found.

Conclusion
In sarcoidosis, different pathophysiological mechanisms contributing to the presence of PH can be identified. Extended analysis is helpful in order to differentiate between different mechanisms and could contribute to personalised therapeutic decision-making.
Venn diagram: classification of SAPH

Funding Acknowledgement
Type of funding source: None
In-hospital outcomes of catheter-directed thrombolysis in patients with pulmonary embolism
Authors: Hobohm L , Schmidt F , Gori T , Schmidtmann I , Barco S , Munzel T , Lankeit M , Konstantinides S.V , Keller K 
Published: November 2020

Abstract

Background and purpose
Catheter-directed treatment of acute pulmonary embolism (PE) is technically advancing. Recent guidelines acknowledge this treatment option for patients with overt or imminent haemodynamic decompensation, particularly when systemic thrombolysis is contraindicated or has failed. We investigated baseline characteristics and in-hospital outcomes of patients with PE who underwent catheter-directed thrombolysis (CDT) in the German nationwide inpatient cohort.

Methods
Data from hospitalizations with PE between 2005 and 2016 were collected by the Federal Office of Statistics (Statistisches Bundesamt) in Germany and included in this analysis. Patients with PE who underwent CDT were compared with patients receiving systemic thrombolysis, and those without thromboytic or other reperfusion treatment.

Results
We analyzed data from 978,094 hospitalized patients with PE. Of these, 41,903 (4.3%) patients received thrombolytic treatment (systemic thrombolysis in 4.2%, CDT in 0.1%). Among PE patients with shock, CDT was associated with lower in-hospital mortality compared to systemic thrombolysis (OR, 0.29, 95% CI 0.13–0.66, P=0.003). No intracranial bleeding occurred among PE patients with shock who received CDT. Among haemodynamically stable PE patients with right ventricular (RV) dysfunction (intermediate-risk PE), CDT also was associated with a lower risk of in-hospital mortality compared to systemic thrombolysis (OR, 0.52 [95% CI 0.38–0.70]; P&lt;0.001) or no thrombolytic treatment (0.45 [95% CI 0.33–0.62]; P&lt;0.001).

Conclusion
In the German nationwide inpatient cohort, CDT was associated with lower in-hospital mortality rates compared to systemic thrombolysis. Prospective controlled data are urgently needed to determine the true value of this treatment option in acute PE.

Funding Acknowledgement
Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study was supported by the German Federal Ministry of Education and Research (BMBF 01EO1503).
Novel procedural method for balloon pulmonary angioplasty to treat chronic thromboembolic pulmonary hypertension
Authors: Minatsuki S , Kiyosue A , Kodera S , Saito A , Maki H , Hatano M , Takimoto E , Ando J , Komuro I 
Published: November 2020

Abstract

Background
The current strategy of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH) is to dilate branches as many as possible without lung injury to normalize mean pulmonary artery pressure and oxygenation. The shape of guiding catheter is one of the important factors to achieve this strategy. However, conventional guiding catheters which are typically used for BPA are difficult to introduce into particular branches. The Ikari-curve left (IL) guiding catheter may be suitable for this purpose by adjusting its curves (Figure 1); however, its utility and effectiveness for lung injury are unclear.

Purpose
The aim of this study is to clarify utility and safety of IL guiding catheter for BPA.

Methods
We retrospectively analyzed 202 consecutive BPA sessions of 40 patients with CTEPH from November 2016 to October 2019 and divided them into two groups; IL group, in which we used IL guiding catheter; and non-IL group in which we used others. IL guiding catheter was used for branches of left lung which are difficult to introduce by conventional catheter prior Nov. 2018. After Nov.2018, we also used it for the same type branches of right lung. The occurrence of lung injury was determined by the presence of bloody sputum during the perioperative period. We compared success rate of introduction into target vessels and of occurrence of lung injury.

Results
The average age of enrolled patients was 60.3±14.4 year-old and female was 65%. There were 99 sessions in IL group. The median treated branches significantly differed between groups (IL group: 15 vs. non-IL group: 10, p&lt;0.05). The lung injury rate tended to low in the IL group (4.0% vs. 11.7%, p=0.07). The IL group had more successful insertions into branches than did the non-IL group (right lung: middle lobe*, 84.4% vs. 57.5%; medial basal branch*, 46.9% vs. 7.5%; left lung: anterior ascending and descending branches, 82.9% vs. 70.8%; lingular branches*, 90.0% vs. 62.5%; anterior basal branch, 75.7% vs. 62.5%, * p&lt;0.05).

Conclusion
IL guiding catheter can be introduced into branches that cannot be accessed via conventional guiding catheters and has potential to reduce the occurrence of lung injury. 2228 characters including space
Figure1

Funding Acknowledgement
Type of funding source: None
Accurate and efficient non-invasive strategy for early identification of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism (InShape II study)
Authors: Klok E , Boon G.J.A.M , Ende-Verhaar Y.M , Bavalia R , Delcroix M , Dzikowska-Diduch O , Kurnicka K , Mairuhu A.T.A , Middeldorp S , Pruszczyk P , Ruigrol D , Verhamme P , Vliegen H , Vonk Noordegraaf A , Huisman M.V 
Published: November 2020

Abstract

Background
The current diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary embolism (PE) is unacceptably long exceeding 1 year, causing loss of quality-adjusted life years and excess mortality. Validated screening strategies to diagnose CTEPH earlier are lacking. Importantly, performing echocardiography in all PE patients for this purpose has a low diagnostic yield, is associated with overdiagnosis and is not cost-effective. Moreover, expertise in performing high-quality PH-dedicated echocardiograms may not be available outside expert centers.

Aim
To validate a simple screening strategy aimed at identifying CTEPH early in the course after acute PE, avoiding echocardiography if possible (Figure 1).

Methods
In this prospective, international, multicenter management study, consecutive PE survivors were managed according to the predefined algorithm starting three months after acute PE. All were followed for a total period of two years. The study protocol was approved by all local IRBs and all patients provided informed consent.

Results
424 patients were included across three European countries (Table 1). Following the algorithm, CTEPH was considered excluded in 343 (81%) patients based on clinical pre-test probability assessment by the “CTEPH prediction score”, evaluation of symptoms and application of the “CTEPH rule-out criteria” (Figure 1); only 19% was subjected to echocardiography. Only 1 of 343 patients managed without echocardiography was diagnosed with CTEPH, 10 months after initial PE, for a failure rate of 0.29% (95% CI 0–1.6%). Overall, 13 patients were diagnosed with CTEPH (incidence 3.1%), of whom 10 within 4 months after PE diagnosis.

Conclusions
The algorithm accurately ruled out CTEPH and avoided echocardiography in 81% of patients. The vast majority of CTEPH cases were identified early in the course of acute PE which is a considerable improvement compared to current clinical practice with an economic use of healthcare resources.
Figure 1. Study flowchart

Funding Acknowledgement
Type of funding source: Foundation. Main funding source(s): This study was supported by unrestricted grants from Bayer/Merck Sharp & Dohme (MSD) and Actelion Pharmaceuticals Ltd. F.A. Klok and G.J.A.M. Boon were supported by the Dutch Heart Foundation (2017T064).
Prognostic implication of sodium nitroprusside vasodilator test in pulmonary hypertension and left heart disease: insights from PUSHON registry
Authors: Boretto P , Bruno F , Bertaina M , Bocchino P , Galluzzo A , Gatti P , Peyracchia M , De Ferrari G.M 
Published: November 2020

Abstract

Background
Definition of combined post and pre-capillary pulmonary hypertension (Cpc-PH) is controversial, despite involving up to 25% of patients with heart failure with reduced ejection fraction (HFrEF). The 2019 World Symposium on PH focused on conflicting results and potential misleading interpretation of mean transpulmonary gradient (TPG) and diastolic transpulmonary gradient (DPG) in predicting outcomes in type 2 PH patients. A new Cpc-PH definition has been proposed, basing on pulmonary arterial wedge pressure (PAWP) &gt;15 mmHg and pulmonary vascular resistance (PVR) &gt;3 WU. In this setting, the role of vasodilator challenge has marginally been explored.

Purpose
This study aims to investigate whether vasodilator test increases prognostic accuracy in PH-LHD comparing to PVR and TPG.

Methods
125 patients with a first diagnosis of PH-LHD were selected within the PUlmonary hypertenSion in rigHt heart catheterization (PUSHON) registry. All patients underwent right heart catheterization and sodium nitroprusside vasodilator infusion. Hemodynamic response was defined as decrease of mean pulmonary arterial pressure (mPAP) &lt;25 mmHg with maintenance of systemic systolic arterial pressure &gt;85 mmHg. Primary endpoint was a composite of cardiac death, heart transplantation and urgent LVAD implant at 2 years.

Results
34 (27%) patients were sodium nitroprusside responders. Normalization of mPAP during acute vasodilator challenge was associated with higher event-free survival at 2 years (HR 2.46, 95% C.I. 1.29–4.69, p=0.006). Responders to nitroprusside showed lower baseline pressure regimens in pulmonary artery (sPAP 56.4±12.9 vs 63.2±12.4 mmHg, p=0.009; mPAP 38.1±6.9 vs 44.7±8.8 mmHg, p=0.001) and lower Wedge Pressure (25.1±6.6 vs 29.8±6.6 mmHg, p=0.001). Also, patients with positive vasodilator test showed better right ventricular function expressed by TAPSE (17.2±4.3 vs 15.4±3.8 mm, p=0.05) and higher EF (34.6±15.3% vs 28.1±13.1%, p=0.023). Interestingly, nitroprusside responders exhibited lower DPG (−0.11±5.30 vs 2.04±5.21 p=0.04). At multivariate analysis PVR and TPG ≥12 mmHg fail to independently predict primary endpoint, while positive vasodilator response was independently associated with better outcome (OR 0.22, 95% C.I. 0.09–0.52, p=0.001). The same results were confirmed in the Cpc-PH subgroup (OR 0.54, 95% C.I. 0.08–0.78, p=0.018).

Conclusions
Nitroprusside vasodilator response was associated with a higher event-free survival at 2 years follow-up in our cohort of patients with PH-LHD. Larger studies with prospective hemodynamic evaluations are needed to support our hypothesis.
Figure 1

Funding Acknowledgement
Type of funding source: None
Effect of macitentan on left ventricular (LV) function in pulmonary arterial hypertension (PAH): results from REPAIR
Authors: Rosenkranz S , Channick R , Cottreel E , Galie N , Kiely D.G , Marcus J.T , Swift A.J , Tawakol A , Torbicki A , Vonk Noordegraaf A , Wetherill G , Peacock A 
Published: November 2020

Abstract

Introduction
PAH impacts right ventricular (RV) structure and function but also leads to changes in the LV due to RV/LV interaction and underfilling. REPAIR, the first PAH study to use a primary endpoint assessed by cardiac MRI (cMRI), reported that RV stroke volume (RVSV) increased by 12 mL and pulmonary vascular resistance (PVR) decreased by 38% from baseline (BL) to Week 26 with macitentan.

Purpose
To assess the effect of macitentan on LV function in patients with PAH.

Methods
REPAIR (NCT02310672) was a 52-week, multicentre, open-label, single-arm, phase 4 study assessing the effect of macitentan primarily on RV structure and function, determined by cMRI and right heart catheterisation. Macitentan 10 mg was initiated in treatment-naïve patients, in patients receiving stable background phosphodiesterase type-5 inhibitor (PDE5i) at BL, or in initial combination with PDE5i. Exploratory LV endpoints were assessed by cMRI at Weeks 26 and 52. Safety was assessed up to end of study treatment +30 days in all patients who received ≥1 dose of macitentan (N=87). Patients with BL and Week 26 assessments for both PVR and RVSV were included in the modified Full Analysis Set (mFAS; N=71).

Results
In the mFAS, 57 (80%) patients were female. At BL, median age was 45 years; median (Q1, Q3) six-minute walk distance was 395 (323, 483) m; 48%/51% of patients were WHO functional class II/III; 59% had idiopathic PAH. Compared to BL, at Weeks 26 and 52 there were significant changes in LV cMRI parameters (table). The most common AEs were peripheral oedema (22%), headache (21%) and dizziness (14%).

Conclusions
Macitentan led to improvements in LV mass, volume and function, including clinically-relevant increases in LV stroke volume, at both 26 and 52 weeks in patients with PAH. Safety was consistent with other macitentan clinical trial data.

Funding Acknowledgement
Type of funding source: Other. Main funding source(s): Actelion Pharmaceuticals Ltd