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Background: Transthoracic contrast echocardiography (TTCE) can detect pulmonary right-to-left shunting (RLS) and is used to screen for pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT). We studied the prevalence and size of pulmonary RLS in HHT type 1, HHT type 2, and HHT-negative controls, and its positive predictive value (PPV) and negative predictive value (NPV) for PAVMs that can be treated by embolotherapy.Methods: In 343 consecutive persons referred for possible HHT as first-degree family members of index patients a TTCE and chest CT scan were performed. All persons were offered genetic analysis.Results: An HHT-causing mutation was confirmed in 92 (mean age 41 ± 15 y; 59% female) HHT1 relatives and in 97 (mean age 47 ± 14 y; 52% female) HHT2 relatives. TTCE showed a pulmonary RLS in 78 (85%) HHT1- and 34 (35%) HHT2-related mutation carriers, respectively (P < .0001). In HHT1 relatives, 29 of 53 (55%) PAVMs and in HHT2 relatives three of 17 (18%) PAVMS were treated, resulting in a PPV of TTCE for treatable PAVMs of 36.3% and 8.3%, respectively. The accompanying NPV was 100%. A minimal, moderate, or large shunt was present in 12 (13%), 24 (26%), and 42 (46%) HHT1-related, and in 20 (21%), 6 (6%), and 8 (8%) HHT2-related mutation carriers, respectively (P for trend < .0001). A large shunt predicted treatable PAVMs in 55.8% of HHT1 relatives and 37.5% of HHT2 relatives. TTCE was positive in four (6%) of 63 persons without HHT.Conclusions: A pulmonary shunt on TTCE is more prevalent and larger in HHT1- compared with HHT2-related mutation carriers. Shunt grading is helpful to predict treatable PAVMs, particularly in the HHT2 group. TTCE is also positive in a small fraction of persons without HHT. [ABSTRACT FROM AUTHOR]

Rationale: Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurologic complications in patients with hereditary hemorrhagic telangiectasia (HHT). Therefore, screening is warranted. Transthoracic contrast echocardiography (TTCE) can effectively detect a pulmonary right-to-left shunt (RLS).Objectives: To determine prospectively the predictive value of TTCE grading to detect PAVMs on high-resolution CT (HRCT) scans of the chest and the indication for embolotherapy.Methods: Three hundred seventeen patients, referred for possible HHT, were screened for PAVMs. Patients who underwent both chest HRCT scanning and TTCE were included in the study (n = 281). For the purposes of this study we used a 3-point grading scale, and shunt grades 3 and 4 according to the classification model of Barzilai et al were combined. Embolotherapy was performed of all PAVMs judged large enough for treatment.Results: Echocardiographic criteria for a pulmonary RLS were present in 105 patients (41%) [mean (+/- SD) age, 43.7 +/- 15.7 years; female gender, 63%]. Chest HRCT scan findings were positive in 55 patients (52%) in this group. The positive predictive value of shunt grade for the presence of PAVMs on chest HRCT scans was 22.9% for grade 1 (n = 35), 34.8% for grade 2 (n = 23), and 83.0% for grade 3 (n = 47), respectively. None of the patients with PAVMs seen on chest HRCT scans and a TTCE grade 1 (n = 8) or 2 (n = 8) were candidates for embolotherapy. Of 39 patients with TTCE grade 3 and PAVMs seen on chest HRCT scans, 26 patients (67%) underwent embolotherapy.Conclusion: An increased echocardiographic shunt grade correlates with an increased probability of PAVMs seen on chest HRCT scans. Only patients with a TTCE grade 3 displayed PAVMs on chest HRCT scans that were large enough for embolotherapy. [ABSTRACT FROM AUTHOR]

The European guidelines advocate a goal-oriented treatment approach in pulmonary arterial hypertension (PAH), based on a comprehensive risk assessment instrument, which has been validated in several PAH subgroups. We investigated its discriminatory ability and explored tricuspid annular plane systolic excursion and revised thresholds to improve its predictability within the adult congenital heart disease (CHD) population. In total, 223 adults (42 ± 16 years, 66% women, 68% Eisenmenger) were enrolled from 5 European PAH-CHD expert centers. Patients were classified as low, intermediate, or high risk at the baseline visit and at follow-up within 4 to 18 months. By the general PAH guidelines instrument, survival did not differ between the risk groups (p-value not significant), mostly because of the skewed group distribution. Reclassifying patients using revised thresholds for N-terminal pro-brain natriuretic peptide and 6-minute walk distance (i.e., low, intermediate, and high as <500, 500 to 1,400, >1,400 ng/L and >400, and 165 to 400 and <165 m, respectively) and use of tricuspid annular plane systolic excursion (low, intermediate, and high as >20, 16 to 20, and <16 mm, respectively) significantly improved the discrimination between the risk groups at baseline and follow-up (p = 0.001, receiver operating characteristic increase from 0.648 to 0.701), reclassifying 64 patients (29%). Irrespective of follow-up risk group, survival was better for patients with higher proportions of low-risk variables. Improvement to a low-risk profile at a median of 9 months of follow-up provided improved survival compared with the survival of patients who remained in the low-risk group. In conclusion, the external validity of general risk instrument for PAH appeared to be of limited discriminatory value in patients with PAH-CHD. We propose a refined risk instrument with improved discrimination for PAH-CHD., Competing Interests: Declaration of competing interest Dr. Bouma reports financial support was provided by Janssen-Cilag. The remaining authors have no competing interests to declare., (Copyright © 2024. Published by Elsevier Inc.)

Introduction: Early detection of cardiac sarcoidosis (CS) is crucial due to its association with severe complications such as ventricular arrhythmias, heart failure, and sudden cardiac death. Advanced imaging techniques like cardiac magnetic resonance imaging (CMR) and 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (FDG-PET/CT) are effective in detecting CS but not easily accessible. The optimal method for selecting patients for advanced screening remains uncertain., Methods: In this retrospective cohort study, all extracardiac sarcoidosis patients screened for CS were reviewed. CS was defined as a multidisciplinary team (MDT) consensus diagnosis. Univariate and multivariate binary logistic regressions were used to identify factors associated with CS, assessing their diagnostic performance, and integrating them into a diagnostic model., Results: Out of 354 patients (average age 51.5 years, 52.5% male), 18.4% were diagnosed with CS. In our cohort, male gender, a QRS duration > 120 ms, and nsVT on Holter monitoring were identified as significant markers associated with CS. Combining age, gender, AV-block or QRS > 120ms on ECG, and nsVT on Holter monitoring provided the highest diagnostic accuracy (AUC of 0.82). Cardiac biomarkers NT-proBNP and troponin T did not improve the diagnostic performance., Conclusion: In our cohort, male gender, a QRS duration > 120 ms, and nsVT on Holter monitoring were identified as significant markers associated with the presence of cardiac sarcoidosis. These clinical markers may aid in selecting sarcoidosis patients for screening with advanced cardiac imaging, potentially leading to earlier detection and management of the disease., Competing Interests: Declarations. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: The primary objective was to externally validate EuroSCORE I and II in surgically treated endocarditis patients. The secondary objective was to assess the predictive performance of both models across sex, redo surgery, age, and urgency., Methods: Data were retrieved from the Netherlands Heart Registration. All patients with infective endocarditis who underwent cardiac surgery between 2013 and 2021 were included. Predictive performance was assessed by discrimination (area under the curve), calibration (calibration-in-the-large and calibration plots), and a decision curve analysis., Results: Two thousand five hundred and sixty-nine cases were included. Overall postoperative 30-day mortality was 10.2%. The area under the curve was 0.73 for EuroSCORE I and 0.72 for EuroSCORE II. Both models overpredict postoperative 30-day mortality, with observed-to-expected ratios of 0.37 and 0.69. EuroSCORE I overpredicts mortality across the full range, whereas EuroSCORE II overpredicts mortality only above a 20% predicted probability. We observed no significant differences in predictive performance across sex, redo surgery, or age. Discriminative capacity of EuroSCORE II was poor in emergency surgeries., Conclusions: Both EuroSCORE models demonstrate acceptable discriminative capacity in IE patients. EuroSCORE I consistently overestimates mortality and should not be utilized in endocarditis patients. EuroSCORE II can be used in IE patients up to a predicted probability of approximately 20%, regardless of sex, redo surgery, or age. Beyond this point, the predicted mortality risk should be halved to approach the true mortality risk. EuroSCORE II should not be used for risk prediction in emergency endocarditis surgeries and patients should not be withheld from indicated surgical treatment solely based on high EuroSCOREs., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Competing Interests: Conflict of interest: None to declared.

Background: Pulmonary arteriovenous malformations (PAVMs) are direct connections between the pulmonary artery and vein, creating a right-to-left shunt (RLS). Embolization is indicated to prevent complications. Guidelines recommend follow-up chest CT scans to confirm persistent occlusion and embolization of all treatable PAVMs. Graded transthoracic contrast echocardiography (TTCE) after PAVM embolization may offer a reliable alternative in a subgroup of patients while preventing radiation exposure., Research Question: Can TTCE predict the need for additional embolotherapy in the postembolization population as accurately as it does in the treatment-naive population?., Study Design and Methods: Since 2018, follow-up after PAVM embolization at our study institution includes both TTCE and chest CT scan after 6 to 12 months and every 3 to 5 years thereafter. Patients who underwent at least 1 follow-up TTCE and chest CT scan were included. The indication for additional embolotherapy was discussed in a multidisciplinary team meeting. The primary outcome was the indication for additional embolotherapy in each RLS grade. Additionally, the association between the RLS grade and indication for additional embolotherapy was investigated., Results: A total of 339 patients with 412 embolization procedures were included; median time to follow-up TTCE was 7.5 months. An RLS was present in 399 postembolization TTCEs (97%): RLS grade 1 in 93 patients (23%), grade 2 in 149 patients (36%) and grade 3 in 157 patients (38%). In patients with RLS grades 0 and 1, no treatable PAVMs were found on CT scan. In patients with RLS grades 2 and 3, 22 (15%) and 72 (46%) underwent additional embolization., Interpretation: This study shows chest CT scan might be forgone in patients with RLS grades 0 and 1 after PAVM embolization., Competing Interests: Financial/Nonfinancial Disclosures None declared., (Copyright © 2024. Published by Elsevier Inc.)

Therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH-specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts., Competing Interests: The authors declare no conflict of interest., (© 2024 The Author(s). Pulmonary Circulation published by Wiley Periodicals LLC on behalf of the Pulmonary Vascular Research Institute.)

Background and Aims: In patients with chronic heart failure (HF), the MONITOR-HF trial demonstrated the efficacy of pulmonary artery (PA)-guided HF therapy over standard of care in improving quality of life and reducing HF hospitalizations and mean PA pressure. This study aimed to evaluate the consistency of these benefits in relation to clinically relevant subgroups., Methods: The effect of PA-guided HF therapy was evaluated in the MONITOR-HF trial among predefined subgroups based on age, sex, atrial fibrillation, diabetes mellitus, left ventricular ejection fraction, HF aetiology, cardiac resynchronization therapy, and implantable cardioverter defibrillator. Outcome measures were based upon significance in the main trial and included quality of life-, clinical-, and PA pressure endpoints, and were assessed for each subgroup. Differential effects in relation to the subgroups were assessed with interaction terms. Both unadjusted and multiple testing adjusted interaction terms were presented., Results: The effects of PA monitoring on quality of life, clinical events, and PA pressure were consistent in the predefined subgroups, without any clinically relevant heterogeneity within or across all endpoint categories (all adjusted interaction P-values were non-significant). In the unadjusted analysis of the primary endpoint quality-of-life change, weak trends towards a less pronounced effect in older patients (Pinteraction = .03; adjusted Pinteraction = .33) and diabetics (Pinteraction = .01; adjusted Pinteraction = .06) were observed. However, these interaction effects did not persist after adjusting for multiple testing., Conclusions: This subgroup analysis confirmed the consistent benefits of PA-guided HF therapy observed in the MONITOR-HF trial across clinically relevant subgroups, highlighting its efficacy in improving quality of life, clinical, and PA pressure endpoints in chronic HF patients., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Aims: Heart failure (HF), a global pandemic affecting millions of individuals, calls for adequate predictive guidance for improved therapy. Congestion, a key factor in HF-related hospitalizations, further underscores the need for timely interventions. Proactive monitoring of intracardiac pressures, guided by pulmonary artery (PA) pressure, offers opportunities for efficient early-stage intervention, since haemodynamic congestion precedes clinical symptoms., Methods: The BioMEMS study, a substudy of the MONITOR-HF trial, proposes a multifaceted approach integrating blood biobank data with traditional and novel HF parameters. Two additional blood samples from 340 active participants in the MONITOR-HF trial were collected at baseline, 3-, 6-, and 12-month visits and stored for the BioMEMS biobank. The main aims are to identify the relationship between temporal biomarker patterns and PA pressures derived from the CardioMEMS-HF system, and to identify the biomarker profile(s) associated with the risk of HF events and cardiovascular death., Conclusion: Since the prognostic value of single baseline measurements of biomarkers like N-terminal pro-B-type natriuretic peptide is limited, with the BioMEMS study we advocate a dynamic, serial approach to better capture HF progression. We will substantiate this by relating repeated biomarker measurements to PA pressures. This design rationale presents a comprehensive review on cardiac biomarkers in HF, and aims to contribute valuable insights into personalized HF therapy and patient risk assessment, advancing our ability to address the evolving nature of HF effectively., (© 2024 The Author(s). European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background/Objectives: The literature reports high complication rates in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who undergo balloon pulmonary angioplasty (BPA), especially in patients with poor pulmonary hemodynamics. Here, we describe the complications of BPA based on the new definitions. Methods: All patients with CTEPH who completed BPA treatment before 15 September 2023 were selected from the CTEPH database. Peri-procedural complications were collected and classified according to the 2023 consensus paper on BPA treatment. Complications were analyzed in subgroups of patients with pulmonary vascular resistance (PVR), ≤ or >6.6 WU, and mean pulmonary artery pressure (mPAP), ≤ or >45 mmHg, at first BPA. Results: In this analysis, 87 patients (63% women; mean age 61.1 ± 14.0 years; 62% on dual PH targeted medical therapy) underwent 426 (mean 4.9 ± 1.6 per patient) BPAs. Only non-severe complications occurred in 14% of BPA treatments and in 47% of the patients; 31% patients had a thoracic complication. The thoracic complications were mild (71%) or moderate (29%). Patients with a PVR > 6.6 WU ( n = 8) underwent more BPA treatments (6.6 ± 1.5 versus 4.6 ± 1.5, p = 0.002), had more complications (88% versus 41% of patients, p = 0.020), and had more thoracic complications (17% vs. 7% of BPAs, p = 0.013) than patients with PVR ≤ 6.6 WU. Patients with mPAP > 45 mmHg ( n = 13) also had more BPA treatments (6.5 ± 1.7 versus 4.6 ± 1.4, p < 0.001), more complications (77% versus 44% of patients, p = 0.027) and more thoracic complications (14% versus 8% of BPAs, p = 0.039) than patients with mPAP ≤ 45 mmHg. Conclusions: Complications occurred in 14% of BPAs and were mostly mild. Patients with severe pulmonary hemodynamics suffered more (thoracic) complications.

Aims: Despite clear guideline recommendations for initiating four drug classes in all patients with heart failure (HF) with reduced ejection fraction (HFrEF) and the availability of rapid titration schemes, information on real-world implementation lags behind. Closely following the 2021 ESC HF guidelines and 2023 focused update, the TITRATE-HF study started to prospectively investigate the use, sequencing, and titration of guideline-directed medical therapy (GDMT) in HF patients, including the identification of implementation barriers., Methods and Results: TITRATE-HF is an ongoing long-term HF registry conducted in the Netherlands. Overall, 4288 patients from 48 hospitals were included. Among these patients, 1732 presented with de novo, 2240 with chronic, and 316 with worsening HF. The median age was 71 years (interquartile range [IQR] 63-78), 29% were female, and median ejection fraction was 35% (IQR 25-40). In total, 44% of chronic and worsening HFrEF patients were prescribed quadruple therapy. However, only 1% of HFrEF patients achieved target dose for all drug classes. In addition, quadruple therapy was more often prescribed to patients treated in a dedicated HF outpatient clinic as compared to a general cardiology outpatient clinic. In each GDMT drug class, 19% to 36% of non-use in HFrEF patients was related to side-effects, intolerances, or contraindications. In the de novo HF cohort, 49% of patients already used one or more GDMT drug classes for other indications than HF., Conclusion: This first analysis of the TITRATE-HF study reports relatively high use of GDMT in a contemporary HF cohort, while still showing room for improvement regarding quadruple therapy. Importantly, the use and dose of GDMT were suboptimal, with the reasons often remaining unclear. This underscores the urgency for further optimization of GDMT and implementation strategies within HF management., (© 2024 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Competing Interests: Conflict of interest: Nothing to disclose.

Aims: Systemic sclerosis (SSc) is characterized by vasculopathy, inflammation, and fibrosis, and carries one of the worst prognoses if patients also develop pulmonary arterial hypertension (PAH). Although PAH is a known prognosticator, patients with SSc-PAH demonstrate disproportionately high mortality, presumably due to cardiac involvement. In this cross-sectional study, the relationship between cardiac involvement revealed by cardiovascular magnetic resonance (CMR) and systemic microvascular disease severity measured with nailfold capillaromicroscopy (NCM) in patients with SSc-PAH is evaluated and compared with patients with idiopathic PAH (IPAH)., Methods and Results: Patients with SSc-PAH and IPAH underwent CMR, echocardiography, and NCM with post-occlusive reactivity hyperaemia (PORH) testing on the same day. CMR imaging included T2 (oedema), native, and post-contrast T1 mapping to measure the extracellular volume fraction (ECV, fibrosis) and adenosine-stress-perfusion imaging measuring the relative myocardial upslope (microvascular coronary perfusion). Measures of peripheral microvascular function were related to CMR indices of oedema, fibrosis, and myocardial perfusion. SSc-PAH patients (n = 20) had higher T2 values and a trend towards a higher ECV, compared with IPAH patients (n = 5), and a lower nailfold capillary density (NCD) and reduced capillary recruitment after PORH. NCD correlated with ECV and T2 (r = -0.443 and -0.464, respectively, P < 0.05 for both) and with markers of diastolic dysfunction on echocardiography. PORH testing, but not NCD, correlated with the relative myocardial upslope (r = 0.421, P < 0.05)., Conclusion: SSc-PAH patients showed higher markers of cardiac fibrosis and inflammation, compared with IPAH patients. These markers correlated well with peripheral microvascular dysfunction, suggesting that SSc-driven inflammation and vasculopathy concurrently affect peripheral microcirculation and the heart. This may contribute to the disproportionate high mortality in SSc-PAH., Competing Interests: Conflict of interest: A.P.J.v.D. received a research grant from Janssen Pharmaceuticals and a speaker fee from Janssen. A.L.D. received a research grant from Actelion Pharmaceuticals and consulting fees from Janssen Pharmaceuticals and Merck. M.C.P. received research grants from the St. Antonius Hospital, Janssen Pharmaceuticals, and ZonMw. M.C.V. received research grants from Boehringer Ingelheim, Grupo Ferrer Internacional, Galapagos NV (Mechelen, Belgium) (Mechelen, Belgium), and Janssen Pharmaceuticals and consulting fees from Boehringer Ingelheim, Corbus Pharmaceuticals Holdings, and Janssen Pharmaceuticals. R.N. received research grants from Biotronik and Philips and consulting fees from Sanofi Genzyme, Bayer, and Bristol Myers Squib., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objectives : Acute type A aortic dissection (ATAAD) is a life-threatening cardiovascular emergency, of which the long-term impact on health-related quality of life (HRQoL) and male-female-specific insights remain inadequately clarified. Methods : Consecutive adult ATAAD patients who underwent surgery were retrospectively included between 2007 and 2017 in four referral centers in the Netherlands, and baseline data were collected. The 36-Item Short-Form (SF-36) Health Survey was sent to all survivors between 2019 and 2021 and compared to validated SF-36 scores of the Dutch general population stratified by age group and sex. Results : In total, 324/555 surviving patients returned the SF-36 questionnaire (response rate 58%), of which 40.0% were female; the median follow-up was 6.5 years (range: 1.7-13.9, IQR: 4.0-9.4) after surgery for ATAAD. In comparison to the general population, ATAAD patients scored significantly lower on 6/8 SF-36 subdomains and higher on bodily pain. Differences in HRQoL domains compared to the sex-matched data were largely comparable between sexes, apart from bodily pain. In the age-matched subgroups impaired HRQoL was most pronounced in younger patients aged 41-60 (5/8 impaired domains). Female ATAAD patients scored significantly worse on 5/8 SF-36 subdomains and the physical component summary (PCS) scores than male patients. Age at ATAAD, female sex, hypertension, COPD, and prior thoracic aortic aneurysm were associated with worse PCS scores. Conclusions : Long-term HRQoL was impaired in both male and female ATAAD patients when compared to the general population. Further studies on the nature of this impairment and on interventions to improve HRQoL after ATAAD are clearly warranted, with special attention to females and younger patients.

Background: Acute type B aortic dissection is a cardiovascular emergency with considerable mortality and morbidity risk. Male-female differences have been observed in cardiovascular disease; however, literature on type B aortic dissection is scarce., Methods and Results: A retrospective cohort study was conducted including all consecutive patients with acute type B aortic dissection between 2007 and 2017 in 4 tertiary hospitals using patient files and questionnaires for late morbidity. In total, 384 patients were included with a follow-up of 6.1 (range, 0.02-14.8) years, of which 41% (n=156) were female. Women presented at an older age than men (67 [interquartile range (IQR), 57-73] versus 62 [IQR, 52-71]; P =0.015). Prior abdominal aortic aneurysm (6% versus 15%; P =0.009), distally extending dissections (71 versus 85%; P =0.001), and clinical malperfusion (18% versus 32%; P =0.002) were less frequently observed in women. Absolute maximal descending aortic diameters were smaller in women (36 [IQR: 33-40] mm versus 39 [IQR, 36-43] mm; P <0.001), while indexed for body surface area diameters were larger in women (20 [IQR, 18-23] mm/m 2 versus 19 [IQR, 17-21] mm/m 2 ). No male-female differences were found in treatment choice; however, indications for invasive treatment were different ( P <0.001). Early mortality rate was 9.6% in women and 11.8% in men ( P =0.60). The 5-year survival was 83% (95% CI, 77-89) for women and 84% (95% CI, 79-89) for men ( P =0.90). No male-female differences were observed in late (re)interventions., Conclusions: No male-female differences were found in management, early or late death, and morbidity in patients presenting with acute type B aortic dissection, despite distinct clinical profiles at presentation. More details on the impact of age and type of intervention are warranted in future studies.

Objective: Acute type B aortic dissection (ATBD) is a rare yet serious cardiovascular event that potentially has an impact on health related quality of life (HRQoL). However, long term follow up data on this topic are scarce. This study aimed to review the long term HRQoL among patients treated for ATBD., Methods: In this multicentre, cross sectional survey study, consecutive treated patients with ATBD between 2007 and 2017 in four referral centres in the Netherlands were retrospectively included and baseline data were collected. Between 2019 and 2021 the 36 Item Short Form Survey (SF-36) was sent to all surviving patients (n = 263) and was compared with validated SF-36 scores in the Dutch general population stratified by age and sex., Results: In total, 144 of 263 surviving patients completed the SF-36 (response rate 55%). Median (IQR) age was 68 (61, 76) years at completion of the questionnaire, and 40% (n = 58) were female. Initial treatment was medical in 55% (n = 79), endovascular in 41% (n = 59), and surgical in 4% (n = 6) of ATBD patients. Median follow up time was 6.1 (range 1.7-13.9; IQR 4.0, 9.0) years. Compared with the general population, patients scored significantly worse on six of eight SF-36 subdomains, particularly physical domains. Apart from bodily pain, there were no substantial differences in HRQoL between male and female ATBD patients. Compared with sex matched normative data, females scored significantly worse on five of eight subdomains, whereas males scored significantly lower on six subdomains. Younger patients aged 41-60 years seemed more severely impaired in HRQoL compared with the age matched general population. Treatment strategy did not influence HRQoL outcomes. Follow up time was associated with better Physical and Mental Component Summary scores., Conclusion: Long term HRQoL was impaired in ATBD patients compared with the Dutch general population, especially regarding physical status. This warrants more attention for HRQoL during clinical follow up. Rehabilitation programmes including exercise and physical support might improve HRQoL and increase patients' health understanding., (Copyright © 2023 The Author(s). Published by Elsevier B.V. All rights reserved.)

Purpose of Review: In recent years new recommendations have been published about organ assessment in the diagnosis of sarcoidosis., Recent Findings: Screening for pulmonary, cardiac, ocular, neurologic and renal involvement and hypercalcemia is recommended in the work-up for sarcoidosis, additionally, screening for hypercalciuria at the time of the diagnosis might be beneficial., Summary: One of the goals in the work-up of sarcoidosis is to assess the extent and severity of organ involvement. Timely and accurate assessment leads to determination of treatment indication. Screening for pulmonary involvement should include pulmonary imaging and pulmonary function tests. Screening for cardiac involvement should include a clear history including palpitations and collapse and a baseline electrocardiogram or 24-h Holter monitoring. At diagnosis, ophthalmological assessment is recommended. Furthermore, serum calcium level and serum creatinine level should be obtained. Although routine 24-h urinary calcium excretion is not included in the guidelines, performing this test routinely can be considered. On indication, neurologic, rheumatologic or dermatologic assessment can be performed., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important. In light of this development, the ESC Working Group on Pulmonary Circulation and Right Ventricular Function has decided to publish a document that helps standardize BPA to meet the need of uniformity in patient selection, procedural planning, technical approach, materials and devices, treatment goals, complications including their management, and patient follow-up, thus complementing the guidelines. Delphi methodology was utilized for statements that were not evidence based. First, an anatomical nomenclature and a description of vascular lesions are provided. Second, treatment goals and definitions of complete BPA are outlined. Third, definitions of complications are presented which may be the basis for a standardized reporting in studies involving BPA. The document is intended to serve as a companion to the official ESC/ERS guidelines., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Side effects limit the long-term use of glucocorticoids in cardiac sarcoidosis (CS), and methotrexate has gained attention as steroid sparing agent although the supporting evidence is poor. This study compared prednisone monotherapy, methotrexate monotherapy or a combination of both, in the reduction of myocardial Fluorine-18 fluorodeoxyglucose (FDG) uptake and clinical stabilization of CS patients., Methods and Results: In this retrospective cohort study, 61 newly diagnosed and treatment naïve CS patients commenced treatment with prednisone (N = 21), methotrexate (N = 30) or prednisone and methotrexate (N = 10) between January 2010 and December 2017. Primary outcome was metabolic response on FDG PET/CT and secondary outcomes were treatment patterns, major adverse cardiovascular events, left ventricular ejection fraction, biomarkers and side effects. At a median treatment duration of 6.2 [5.7-7.2] months, 71.4% of patients were FDG PET/CT responders, and the overall myocardial maximum standardized uptake value decreased from 6.9 [5.0-10.1] to 3.4 [2.1-4.7] (P < 0.001), with no significant differences between treatment groups. During 24 months of follow-up, 7 patients (33.3%; prednisone), 6 patients (20.0%; methotrexate) and 1 patient (10.0%; combination group) experienced at least one major adverse cardiovascular event (P = 0.292). Left ventricular ejection fraction was preserved in all treatment groups., Conclusions: Significant suppression of cardiac FDG uptake occurred in CS patients after 6 months of prednisone, methotrexate or combination therapy. There were no significant differences in clinical outcomes during follow-up. These results warrant further investigation of methotrexate treatment in CS patients., (© 2023. The Author(s) under exclusive licence to American Society of Nuclear Cardiology.)

Importance: It is suggested that patients with Cutibacterium acnes endocarditis often present without fever or abnormal inflammatory markers. However, no study has yet confirmed this statement., Objective: To assess the clinical characteristics and outcomes of patients with C acnes endocarditis., Design, Setting, and Participants: A case series of 105 patients presenting to 7 hospitals in the Netherlands and France (4 university hospitals and 3 teaching hospitals) with definite endocarditis according to the modified Duke criteria between January 1, 2010, and December 31, 2020, was performed. Clinical characteristics and outcomes were retrieved from medical records. Cases were identified by blood or valve and prosthesis cultures positive for C acnes, retrieved from the medical microbiology databases. Infected pacemaker or internal cardioverter defibrillator lead cases were excluded. Statistical analysis was performed in November 2022., Main Outcomes and Measures: Main outcomes included symptoms at presentation, presence of prosthetic valve endocarditis, laboratory test results at presentation, time to positive results of blood cultures, 30-day and 1-year mortality rates, type of treatment (conservative or surgical), and endocarditis relapse rates., Results: A total of 105 patients (mean [SD] age, 61.1 [13.9] years; 96 men [91.4%]; 93 patients [88.6%] with prosthetic valve endocarditis) were identified and included. Seventy patients (66.7%) did not experience fever prior to hospital admission, nor was it present at hospitalization. The median C-reactive protein level was 3.6 mg/dL (IQR, 1.2-7.5 mg/dL), and the median leukocyte count was 10.0 × 103/µL (IQR, 8.2-12.2 × 103/µL). The median time to positive blood culture results was 7 days (IQR, 6-9 days). Surgery or reoperation was indicated for 88 patients and performed for 80 patients. Not performing the indicated surgical procedure was associated with high mortality rates. Seventeen patients were treated conservatively, in accordance with the European Society of Cardiology guideline; these patients showed relatively high rates of endocarditis recurrence (5 of 17 [29.4%])., Conclusions and Relevance: This case series suggests that C acnes endocarditis was seen predominantly among male patients with prosthetic heart valves. Diagnosing C acnes endocarditis is difficult due to its atypical presentation, with frequent absence of fever and inflammatory markers. The prolonged time to positivity of blood culture results further delays the diagnostic process. Not performing a surgical procedure when indicated seems to be associated with higher mortality rates. For prosthetic valve endocarditis with small vegetations, there should be a low threshold for surgery because this group seems prone to endocarditis recurrence.