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Purpose: Systemic-to-pulmonary collateral flow is a well-recognised phenomenon in patients with single ventricle physiology, but remains difficult to quantify. The aim was to compare the reported formula’s that have been used for calculation of systemic-to-pulmonary-collateral flow to assess their consistency and to quantify systemic-to-pulmonary collateral flow in patients with a Glenn and/or Fontan circulation using four-dimensional flow MRI (4D flow MR). Methods: Retrospective case–control study of Glenn and Fontan patients who had a 4D flow MR study. Flows were measured at the ascending aorta, left and right pulmonary arteries, left and right pulmonary veins, and both caval veins. Systemic-to-pulmonary collateral flow was calculated using two formulas: 1) pulmonary veins – pulmonary arteries and 2) ascending aorta – caval veins. Anatomical identification of collaterals was performed using the 4D MR image set. Results: Fourteen patients (n = 11 Fontan, n = 3 Glenn) were included (age 26 [22–30] years). Systemic-to-pulmonary collateral flow was significantly higher in the patients than the controls (n = 10, age 31.2 [15.1–38.4] years) with both formulas: 0.28 [0.09–0.5] versus 0.04 [−0.66–0.21] l/min/m2 (p = 0.036, formula 1) and 0.67 [0.24–0.88] versus -0.07 [−0.16–0.08] l/min/m2 (p < 0.001, formula 2). In patients, systemic-to-pulmonary collateral flow differed significantly between formulas 1 and 2 (13% versus 26% of aortic flow, p = 0.038). In seven patients, veno-venous collaterals were detected and no aortopulmonary collaterals were visualised. Conclusion: 4D flow MR is able to detect increased systemic-to-pulmonary collateral flow and visualise collaterals vessels in Glenn and Fontan patients. However, the amount of systemic-to-pulmonary collateral flow varies with the formula employed. Therefore, further research is necessary before it could be applied in clinical care.

BACKGROUND: To identify the association between comorbidities and left atrial (LA) and right atrial (RA) function in patients with paroxysmal atrial fibrillation (AF).METHODS: This is a cross-sectional study. Speckle-tracking echocardiography was performed in 344 patients with paroxysmal AF at baseline, and available in 298 patients after 1-year follow-up. The number of comorbidities (hypertension, diabetes mellitus, coronary artery disease, body mass index >25 kg/m2, age > 65 years, moderate to severe mitral valve regurgitation and kidney dysfunction (estimated glomerular filtration rate RESULTS: Mean age of the patients was 58 (SD 12) years and 137 patients were women (40%). Patients with a higher number of comorbidities had larger LA volumes (p for trend CONCLUSIONS: In patients with paroxysmal AF, individual and combined comorbidities are related to lower LA and RA strain. In patients with few comorbidities, impairment in atrial function progresses during one year of follow-up. Whether comorbidity management prevents or reverses decrease in atrial function warrants further study.

Systemic right ventricular failure is a common finding in patients with transposition of the great arteries. Some of these patients require ventricular assist device implantation. We describe the feasibility of HeartMate 3 [Abbott, Illinois, United States] implantation in a patient with transposition of the great arteries, high human leukocyte antigen sensitization, and severe pectus excavatum using a two-stage approach. Learning objectives: 1. To notice the challenges faced while implanting HeartMate 3 [Abbott, Illinois, United States] in patients with congenital heart disease and anatomical limitations. 2. To understand that despite the difficulties, HeartMate 3 implantation is possible, worthwhile, and sometimes the only choice in a patient with end-stage heart failure and congenital heart disease.

Purpose: Although cure rates in esophageal cancer (EC) have improved since the introduction of neoadjuvant chemoradiation (nCRT), evidence for treatment-related cardiac toxicity is growing, of which the exact mechanisms remain unknown. The primary objective of this study was to identify (subclinical) cardiac dysfunction in EC patients after nCRT followed by surgical resection as compared to surgery alone.Materials and Methods: EC survivors followed for 5-15 years after curative resection with (n = 20) or without (n = 20) nCRT were enrolled in this prospective cross-sectional pilot study. All patients underwent several clinical and diagnostic tests in order to objectify (sub)clinical cardiac toxicity including cardiac CT and MRI, echocardiography, ECG, 6-minutes walking test, physical examination and EORTC questionnaires.Results: We found an increased rate of myocardial fibrosis (Linear late gadolinium enhancement (LGE) 4 vs. 1; p = 0.13; mean extracellular volume (ECV) 28.4 vs. 24.0; p < 0.01), atrial fibrillation (AF) (6 vs. 2; p = 0.07) and conduction changes in ECG among patients treated with nCRT as compared to those treated with surgery alone. The results suggested an impact on quality of life in terms of worse role functioning for this patient group (95.0 vs. 88.8; p = 0.03).Conclusion: Based on our analyses we hypothesize that in EC patients, radiation-induced myocardial fibrosis plays a central role in cardiac toxicity leading to AF, conduction changes and ultimately to decreased role functioning. The results emphasize the need to verify these findings in larger cohorts of patients. (C) 2021 The Author(s). Published by Elsevier B.V.

Left atrial (LA) structure and function in heart failure with reduced (HFrEF) versus preserved ejection fraction (HFpEF) is only established in small studies. Therefore, we conducted a systematic review of LA structure and function in order to find differences between patients with HFrEF and HFpEF. English literature on LA structure and function using echocardiography was reviewed to calculate pooled prevalence and weighted mean differences (WMD). A total of 61 studies, comprising 8806 patients with HFrEF and 9928 patients with HFpEF, were included. The pooled prevalence of atrial fibrillation (AF) was 34.4% versus 42.8% in the acute inpatient setting, and 20.1% versus 33.1% in the chronic outpatient setting when comparing between HFrEF and HFpEF. LA volume index (LAVi), LA reservoir global longitudinal strain (LAGLSR), and E/e’ was 59.7 versus 52.7 ml/m2, 9.0% versus 18.9%, and 18.5 versus 14.0 in the acute inpatient setting, and 48.3 versus 38.2 ml/m2, 12.8% versus 23.4%, and 16.9 versus 13.5 in the chronic outpatient setting when comparing HFrEF versus HFpEF, respectively. The relationship between LAVi and LAGLSR was significant in HFpEF, but not in HFrEF. Also, in those studies that directly compared patients with HFrEF versus HFpEF, those with HFrEF had worse LAGLSR [WMD = 16.3% (22.05,8.61); p p

Background: Congenital Heart Disease (CHD) predisposes to Infective Endocarditis (IE), but data about characterization and prognosis of IE in CHD patients is scarce.Methods: The ESC-EORP-EURO-ENDO study is a prospective international study in IE patients (n = 3111). In this pre-specified analysis, adult CHD patients (n = 365, 11.7%) are described and compared with patients without CHD (n = 2746) in terms of baseline characteristics and mortality.Results: CHD patients (73% men, age 44.8 ± 16.6 years) were younger and had fewer comorbidities. Of the CHD patients, 14% had a dental procedure before hospitalization versus 7% in non-CHD patients (p < 0.001) and more often had positive blood cultures for Streptococcus viridans (16.4% vs 8.8%, p < 0.001). As in non-CHD patients, IE most often affected the left-sided valves. For CHD patients, in-hospital mortality was 9.0% vs 18.1% in non-CHD patients (p < 0.001), and also, during the entire follow-up of 700 days, survival was more favorable (log-rank p < 0.0001), even after adjustment for age, gender and major comorbidities (Hazard Ratio (HR) 0.68; 95%CI 0.50–0.92). Within the CHD population, multivariable Cox regression revealed the following effects (HR and [95% CI]) on mortality: fistula (HR 6.97 [3.36–14.47]), cerebral embolus (HR 4.64 [2.08–10.35]), renal insufficiency (HR 3.44 [1.48–8.02]), Staphylococcus aureus as causative agent (HR 2.06 [1.11–3.81]) and failure to undertake surgery when indicated (HR 5.93 [3.15–11.18]).Conclusions: CHD patients with IE have a better outcome in terms of all-cause mortality. The observed high incidence of dental procedures prior to IE warrants further studies about the current use, need and efficacy of antibiotic prophylaxis in CHD patients.

Aims: Improvement in left ventricular ejection fraction (LVEF) after up-titration of guideline-directed medical therapy (GDMT) has been well described in heart failure (HF) patients. Less is known about the prevalence and clinical course of right ventricular dysfunction (RVD) in patients with new-onset HF.Methods and results: From 2012 to 2018, 625 patients with a recent (Conclusion: More than one-third of patients with new-onset HF have RVD. RVD is associated with a higher risk of all-cause mortality and HF hospitalization. Recovery of RVD regularly occurs during up-titration of GDMT and is associated with improved clinical outcomes.

Different associations between epicardial adipose tissue (EAT) and cardiac function have been suggested in patients with heart failure with preserved (HFpEF) versus reduced and mildly reduced ejection fraction (HFrEF/HFmrEF). However, few studies have directly compared the association between EAT and left atrial (LA) and left ventricular (LV) function in patients with HFpEF and HFrEF/HFmrEF.We studied EAT thickness using transthoracic echocardiography in a multicentre cohort of 149 community-dwelling controls without heart failure, 99 patients with HFpEF, and 366 patients with HFrEF/HFmrEF. EAT thickness was averaged from parasternal long-axis and short-axis views, respectively, and off-line speckle tracking analysis was performed to quantify LA and LV function. Data were validated in an independent cohort of 626 controls, 243 patients with HFpEF, and 180 patients with HFrEF/HFmrEF. For LV function, LV global longitudinal strain (GLS) was measured in both derivation and validation cohorts. For LA function, LAGLS at reservoir, contractile and conduit phase were measured in the derivation cohort, and only LAGLS at reservoir phase was measured in the validation cohort. In the derivation cohort, EAT thickness was lower in HFrEF/HFmrEF (7.3 ± 2.5 mm) compared to HFpEF (8.3 ± 2.6 mm, p 0.05) and controls (7.9 ± 1.8 mm, p 0.05). Greater EAT thickness was associated with better LV and contractile LA function in HFrEF/HFmrEF, but not in HFpEF (p for interaction0.05). These findings were confirmed in the validation cohort, where EAT thickness was lower in HFrEF/HFmrEF (6.7 ± 1.4 mm) compared to HFpEF (9.6 ± 2.8 mm; p 0.05) and controls (7.7 ± 2.3 mm; p 0.05). Greater EAT thickness was associated with better LV and reservoir LA function in patients with HFrEF/HFmrEF but worse LV and reservoir LA function in patients with HFpEF (p for interaction0.05). Thickened EAT (EAT thickness10 mm) was associated with LA dysfunction (LAGLS at reservoir phase23%) in HFpEF, but not in HFrEF/HFmrEF.Epicardial adipose tissue thickness is greater in patients with HFpEF than HFrEF/HFmrEF. Increased EAT thickness is associated with worse LA and LV function in HFpEF but the opposite in HFrEF/HFmrEF.

ObjectiveThe clinical and prognostic implications of a hypertensive response to exercise after repair of coarctation of the aorta (CoA) remain controversial. We aimed to determine the prevalence of a hypertensive response to exercise, identify factors associated with peak exercise systolic blood pressure (SBP) and explore the association of peak exercise SBP with resting blood pressure and cardiovascular events during follow-up.MethodsFrom the Dutch national CONgenital CORvitia (CONCOR) registry, adults with repaired CoA who underwent exercise stress testing were included. A hypertensive response to exercise was defined as a peak exercise SBP ≥210 mm Hg in men and ≥190 mm Hg in women. Cardiovascular events consisted of coronary artery disease, stroke, aortic complications and cardiovascular death.ResultsOf the original cohort of 920 adults with repaired CoA, 675 patients (median age 24 years (range 16–72 years)) underwent exercise stress testing. Of these, 299 patients (44%) had a hypertensive response to exercise. Mean follow-up duration was 10.1 years. Male sex, absence of a bicuspid aortic valve and elevated resting SBP were independently associated with increased peak exercise SBP. Peak exercise SBP was positively predictive of office SBP (β=0.11, pConclusionsA hypertensive response to exercise was present in nearly half of the patients in this large, prospective cohort of adults with repaired CoA. Risk factors for increased peak exercise SBP were male sex, absence of a bicuspid aortic valve and elevated resting SBP. Increased peak exercise SBP independently predicted hypertension at follow-up. These results support close follow-up of patients with a hypertensive response to exercise to ensure timely diagnosis and treatment of future hypertension.

Contains fulltext : 291436.pdf (Publisher’s version ) (Open Access) BACKGROUND: Clinical factors are used to estimate late complication risk in adults after atrial switch operation (AtrSO) for transposition of the great arteries (TGA), but heterogeneity in clinical course remains. We studied whether common genetic variants are associated with outcome and add value to a clinical risk score in TGA-AtrSO patients. METHODS AND RESULTS: This multicenter study followed 133 TGA-AtrSO patients (aged 28 [IQR 24-35] years) for 13 (IQR 9-16) years and examined the association of genome-wide single-nucleotide polymorphisms (SNPs) with a composite endpoint of symptomatic ventricular arrhythmia, heart failure hospitalization, ventricular assist device implantation, heart transplantation, or mortality. Thirty-two patients (24%) reached the endpoint. The genome-wide association study yielded one genome-wide significant (p 20%) risk. Stratified by the combined score, observed 5-year event-free survival was 100%, 79% and 31% for low, intermediate, and high-risk patients, respectively. CONCLUSIONS: Common genetic variants may explain some variation in the clinical course in TGA-AtrSO and improve risk stratification over clinical factors alone, especially in patients at intermediate clinical risk. These findings support the hypothesis that including genetic variants in risk assessment may be beneficial.

Background: Currently, patients with COPD who are evaluated for bronchoscopic treatments are routinely screened for pulmonary hypertension (PH) and systolic left ventricle dysfunction by echocardiography. Objectives: We evaluated the prevalence of PH and systolic left ventricle dysfunction in this patient group and investigated if the previously proposed CT-derived pulmonary artery to aorta (PA:A) ratio >1 and PA diameter measurements can be used as alternative screening tools for PH. Methods: Two hundred fifty-five patients were included in this retrospective analysis (FEV1 25%pred, RV 237%pred). All patients received transthoracic echocardiography and chest CT scans on which diameters of the aorta and pulmonary artery were measured at the bifurcation and proximal to the bifurcation. Results: Following echocardiography, 3 patients (1.2%) had PH and 1 (0.4%) had systolic left ventricle dysfunction. Using a PA:A ratio >1, only 10.3% of the patients with a right ventricular systolic pressure (RVSP) ≥35 mm Hg were detected and none of the patients with an RVSP >50 mm Hg were detected. Patients with an RVSP ≥35 mm Hg had significantly higher PA diameters (29.5 vs. 27.5 mm; p = 0.02) but no significantly different PA:A ratios. All patients with an RVSP >50 mm Hg had PA diameters >30 mm. Conclusions: The prevalence of PH and systolic left ventricle dysfunction is low in this preselected cohort of patients with severe COPD. In this population, a PA:A ratio >1 is not a useful cardiac screening tool for PH. A PA diameter >30 mm could substitute for routinely performed echocardiography in the screening for PH in this patient group.

BackgroundType D personality has been previously shown to increase the risk for mortality in patients with acquired heart disease.ObjectiveWe aimed to compare mortality in adult patients with congenital heart disease (CHD) with and without type D.MethodsSurvival was assessed using prospective data from the Dutch national Congenital Corvitia registry for adults with CHD. Patients were randomly selected from the registry and characterized at inclusion in 2009 for the presence of type D using the DS14 questionnaire.ResultsOne thousand fifty-five patients, with 484 (46%) males, a mean (SD) age of 41 (14) years, 613 (58%) having mild CHD, 348 (33%) having moderate CHD, and 94 (9%) having severe CHD, were included. Type D personality was present in 225 patients (21%). Type D was associated with an increased risk for all-cause mortality independent of age, sex, New York Heart Association class, number of prescribed medications, depression, employment status, and marital status (hazard ratio, 1.94; 95% confidence interval, 1.05-3.57; P = .033).ConclusionType D personality was associated with an increased risk for all-cause mortality in adult patients with CHD.

Objective:N-terminal pro-brain natriuretic peptide has an established role in the diagnosis and prognosis of heart failure. In Fontan patients, this peptide is often increased, but its diagnostic value in this particular non-physiologic, univentricular circulation is unclear. We investigated whether N-terminal pro-brain natriuretic peptide represents ventricular function or other key variables in Fontan patients.Methods and results:Ninety-five consecutive Fontan patients ≥10 years old who attended the outpatient clinic of the Center for Congenital Heart Diseases in 2012–2013 were included. Time since Fontan completion was 16 ± 9 years. Median N-terminal pro-brain natriuretic peptide was 114 (61–264) ng/l and was higher than gender-and age-dependent normal values in 54% of the patients. Peptide Z-scores were higher in patients in NYHA class III/IV compared to those in class I/II, but did not correlate with ventricular function assessed by MRI and echocardiography, nor with peak exercise capacity. Instead, peptide Z-scores significantly correlated with follow-up duration after Fontan completion (p < 0.001), right ventricular morphology (p = 0.004), indexed ventricular mass (p = 0.001), and inferior caval vein diameter (p < 0.001) (adjustedR2= 0.615).Conclusions:N-terminal pro-brain natriuretic peptide levels in Fontan patients correlate with functional class, but do not necessarily indicate ventricular dysfunction. Increased peptide levels were associated with a longer existence of the Fontan circulation, morphologic ventricular characteristics, and signs of increased systemic venous congestion. Since the latter are known to be key determinants of the performance of the Fontan circulation, these findings suggest increase in N-terminal pro-brain natriuretic peptide levels to indicate attrition of the Fontan circulation, independent of ventricular function.

Background: Coronary artery disease (CAD) will increasingly determine outcome in the aging adult congenital heart disease (CHD) population. We aimed to determine sex-specific incidence of CAD in adult CHD patients throughout adulthood, compared to the general population. Methods and results: We followed 11,723 adult CHD patients (median age 33 years; 49% male; 57% mild, 34% moderate, 9% severe CHD) from the Dutch CONCOR registry, and two age-sex-matched persons per patient from the general population for first CAD event in national registers (period 2002–2012). Incidence rates were estimated using smoothed hazard functions. CAD risk during follow-up, stratified by CHD severity, was compared using proportional subdistribution hazards regression. In ACHD patients, 103 CAD events (43 women) occurred over 60,456 person-years. Rates per 1000person-years increased from 0.3(95% confidence interval: 0.1–0.6) at age 20 to 5.8(3.7–8.9) at 70 years in female, and from 0.5(0.3–1.0) to 7.8(5.1–11.8) in male patients. Compared to the general population, relative risk was 12.0(2.5–56.3) in women and 4.6(1.7–12.1) in men aged 20 years. Relative risk declined with age, remaining significant up to age ~65 years in women and ~50 years in men. In patients with mild, moderate and severe CHD, CAD risk was 1.3(0.9–1.9), 1.6(1.0–2.5) and 2.9(1.3–6.9) times increased compared to the general population, respectively. Conclusions: We found increased CAD risk in adult CHD patients, with greater relative risk at younger age, in women and those with more severe CHD. These results underline the importance of screening for and treatment of CAD risk factors in these patients.

Background: Predicting heart failure events in patients with a systemic right ventricle (sRV) due to transposition of the great arteries (TGA) is important for timely intensification of follow-up. This study assessed the value of strain compared with currently used parameters as predictor for heart failure–free survival in patients with sRV. Methods: In participants of a multicentre trial, speckle-tracking echocardiography (STE) was performed to assess global longitudinal strain (GLS), mechanical dispersion (MD), and postsystolic shortening (PSS). Cox regression was used to determine the association of STE parameters with the combined end point of progression of heart failure and death, compared with cardiovascular magnetic resonance (CMR) and computed tomography (CT) derived parameters. Results: Echocardiograms of 60 patients were analyzed (mean age 34 ± 11 years, 65% male, 35% congenitally corrected TGA). Mean GLS was −13.5 ± 2.9%, median MD was 49 (interquartile range (IQR) 30-76) ms, and 14 patients (23%) had PSS. During a median 8 (IQR 7-9) years, 15 patients (25%) met the end point. GLS, MD, and PSS were all associated with heart failure–free survival in univariable analysis. After correction for age, only GLS (optimal cutoff > −10.5%) and CMR/CT-derived sRV ejection fraction (optimal cutoff < 30%) remained associated with heart failure–free survival: hazard ratio (HR) 8.27, 95% confidence interval (CI) 2.50-27.41 (P < 0.001), and HR 4.34, 95% CI 1.48-12.74 (P = 0.007), respectively). Combining GLS and ejection fraction improved prediction, with patients with both GLS > −10.5% and sRV ejection fraction < 30% at highest risk (HR 19.69, 95% CI 4.90-79.13; P < 0.001). Conclusions: The predictive value of GLS was similar to that of CMR/CT-derived ejection fraction. The combination of GLS and ejection fraction identified patients at highest risk of heart failure and death. Easily available STE parameters can be used to guide follow-up intensity and can be integrated into future risk prediction scores.

Background The long‐term burden of cardiovascular disease after repair of coarctation of the aorta (CoA) has not been elucidated. We aimed to determine the incidence of and risk factors for cardiovascular events in adult patients with repaired CoA. Additionally, mortality rates were compared between adults with repaired CoA and the general population. Methods and Results Using the Dutch Congenital Corvitia (CONCOR) registry, patients aged ≥16 years with previous surgical or transcatheter CoA repair from 5 tertiary referral centers were included. Cardiovascular events were recorded, comprising coronary artery disease, stroke/transient ischemic attack, aortic complications, arrhythmias, heart failure hospitalizations, endocarditis, and cardiovascular death. In total, 920 patients (median age, 24 years [range 16–74 years]) were included. After a mean follow‐up of 9.3±5.1 years, 191 patients (21%) experienced at least 1 cardiovascular event. A total of 270 cardiovascular events occurred, of which aortic complications and arrhythmias were most frequent. Older age at initial CoA repair (hazard ratio [HR], 1.017; 95% CI, 1.000–1.033 [ P =0.048]) and elevated left ventricular mass index (HR, 1.009; 95% CI, 1.005–1.013 [ P P Conclusions This large, prospective cohort of adults with repaired CoA showed a high burden of cardiovascular events, particularly aortic complications and arrhythmias, during long‐term follow‐up. Older age at initial CoA repair and elevated left ventricular mass index were independent risk factors for the occurrence of cardiovascular events. Mortality was 3.3‐fold higher compared with the general population. These results advocate stringent follow‐up after CoA repair and emphasize the need for improved preventive strategies.

Background This study aims to assess sex differences with regards to outcome, cardiovascular dimensions, and functional capacity in patients with a Fontan circulation. Methods In this single-center prospective, standardised cohort study of 60 Fontan patients (30 females, 50%) aged ≥10 years, the association between sex and the combined outcome variable of all-cause mortality, heart/heart-lung transplantation (including listing) and Fontan-related hospitalisation was investigated. Secondary outcome measures included functional capacity and ventricular dimensions, measured by cardiac magnetic resonance imaging (CMR), cardiopulmonary exercise testing (CPET) and concomitant clinical assessment. Results Baseline characteristics including age, diagnosis and surgical history did not differ between female and male patients. During a median follow-up time of 5.75 (5.4–6.0) years, the combined outcome variable occurred in 17 (28%) patients. Female Fontan patients had 13 (43%) adverse events and males 4 (13%). Event-free survival differed significantly (p ​= ​0.040). At baseline, the majority of males was in NYHA class I (n ​= ​19, 63%), whereas the majority of females was in NYHA class II or III (n ​= ​20, 67%) (p ​= ​0.036). Male Fontan patients had about 25% higher end-diastolic volume (p ​= ​0.001) indexed for body surface area (BSA), 28% higher indexed end-systolic volume (p ​= ​0.004) and 25% more indexed ventricular mass (p ​ Conclusions In Fontan patients, event-free survival is significantly higher in males compared to females. Relevant sex differences in ventricular dimensions as well as functional capacity exist, which exceed the differences seen in the healthy population. These differences should be considered when analysing and interpreting cut-off values to improve risk stratification.

Purpose: Radiation-induced cardiac toxicity is a potential lethal complication. The aim of this study was to assess whether there is a dose-dependent relationship between radiation dose and myocardial fibrosis in patients who received neoadjuvant chemoradiation (nCRT) for esophageal cancer (EC).Methods and Materials: Forty patients with EC treated with a transthoracic esophagectomy with (n = 20) or without (n = 20) nCRT (CROSS study regimen) were included. Cardiovascular magnetic resonance imaging (1.5 Tesla) for left ventricular (LV) function, late gadolinium enhancement, and T1 mapping were performed. Extracellular volume (ECV), as a surrogate for collagen burden, was measured for all LV segments separately. The dose-response relationship between ECV and mean radiation dose per LV myocardial segment was evaluated using a mixed-model analysis.Results: Seventeen nCRT and 16 control patients were suitable for analysis. The mean time after treatment was 67.6 +/- 8.1 (nCRT) and 122 +/- 35 (controls) months (P = .02). In nCRT patients, we found a significantly higher mean global ECV of 28.2% compared with 24.0% in the controls (P < .001). After nCRT, LV myocardial segments with elevated ECV had received significantly higher radiation doses. In addition, a linear dose-effect relation was found with a 0.136% point increase of ECV for each Gy (P < .001). There were no differences in LV function measures and late gadolinium enhancement between both groups.Conclusions: Myocardial ECV was significantly higher in long-term EC survivors after nCRT compared with surgery only. Moreover, this ECV increase was linear with the radiation dose per LV segment, indicating radiation-induced myocardial fibrosis. (C) 2021 The Author(s). Published by Elsevier Inc.

Item does not contain fulltext BACKGROUND: This study was designed to investigate the agreement of 2D transthoracic echocardiography (2D TTE) with cardiovascular magnetic resonance imaging (CMR) in a contemporary population of ST-elevation myocardial infarction (STEMI) patients. METHODS: In this subanalysis of the GIPS-III trial, a randomized controlled trial investigating the administration of metformin in STEMI patients to prevent reperfusion injury, we studied 259 patients who underwent same-day CMR and 2D TTE assessments four months after hospitalization for a first STEMI. Bland-Altman analyses were performed to assess agreement between LV end-diastolic volume (LVEDV), LV end-systolic volume (LVESV), LV ejection fraction (LVEF), and LV mass measurements. Sensitivity and specificity of 2D TTE to detect categories of LVEF (/=50%) was determined. Linear regression of absolute differences in measurements between imaging modalities was used to investigate whether patient characteristics impact measurement bias. RESULTS: Pairwise difference (bias) and 95% limits of agreement between CMR and 2D TTE measurements were +84 (37, 147) ml for LVEDV, +39 (6, 85) ml for LVESV, -1.1 +/- 13.5% for LVEF, and -75 (-154, -14) g for LV mass. Sensitivity and specificity of 2D TTE to detect subjects with moderately depressed LVEF (35-50%) as measured by CMR were 52% and 88% respectively. We observed a significant effect of enzymatic infarct size on bias between 2D TTE and CMR in measuring LVESV and LVEF (P = 0.029, P = 0.001 respectively), of age and sex on bias between 2D TTE and CMR in measuring LV mass (P = 0.027, P < 0.001) and LVEDV (P = 0.001, P = 0.039), and of heart rate on bias between 2D TTE and CMR in LV volume measurements (P = 0.004, P = 0.016). CONCLUSIONS: Wide limits of agreement, underestimation of LV volumes and overestimation of LV mass was observed when comparing 2D TTE to CMR. Enzymatic infarct size, age, sex, and heart rate are potential sources of bias between imaging modalities.

Aims To assess medication use in adult congenital heart disease (ACHD) patients compared to the age- and sex-matched general population, identify patterns of pharmacotherapy, and analyse associations between pharmacotherapy and adverse outcomes in ACHD. Methods and results Data of 14 138 ACHD patients from the CONCOR registry [35 (24–48) years, 49% male] and age- and sex-matched referents (1:10 ratio) were extracted from the Dutch Dispensed Drug Register for the years 2006–14. Adult congenital heart disease patients had more cardiovascular and non-cardiovascular drugs than referents (median 3 vs. 1, P Conclusion Both cardiovascular and non-cardiovascular medication use is high in ACHD with twice as much polypharmacy compared with the matched general population. Patients with polypharmacy had a four-fold increased risk of mortality and adverse drug events. Recognition of distinct medication patterns can help identify patients at highest risk. Drug regimens need repeating evaluation to assess the appropriateness of all prescriptions. More high-quality studies are needed to improve ACHD care with more evidence-based pharmacotherapy.

Funding Acknowledgements Type of funding sources: None. Background and Aim Left atrial (LA) enlargement and impaired LA function are frequently found in patients with heart failure with preserved ejection fraction (HFpEF). Whether these structural and functional LA abnormalities are a consequence of increased LA pressure or whether HFpEF patients have an intrinsic LA myopathy is unknown. We compared LA pressure, size and function between patients with HFpEF and aortic stenosis, as a comparator with LA pressure overload, as well as community-dwelling control subjects. Methods Extensive echocardiographic assessments were performed in 219 patients with HFpEF (age 68 ± 11, 48% female), 173 patients with moderate to severe AS (age 69 ± 11, 55% female, aortic valve area index 0.55 ± 0.15 cm2/m2), and 219 controls (age 65 ± 9, 48% female, 42.2% hypertensive) Results Compared to controls, both patients with HFpEF and AS had larger LV and LA size and worse LV systolic, diastolic and LA function. Compared with AS patients, HFpEF patients had smaller LA volume index (40.2 ± 19.4 vs. 44.5 ± 11.9 ml/m2 p = 0.01) but similar LV filling pressure estimated by E/e’ (13.4 ± 4.8 13.4 ± 4.8 , p = 0.12). Despite smaller LA volume index and similar LV filling pressure, HFpEF patients had remarkably poorer LA function compared to AS [reservoir GLS, 22.6 ± 10% vs 31.4 ± 10.1 (p Conclusion Patients with HFpEF had significantly worse LA function than patients with AS, despite similar LA pressure overload. These findings support the concept of an intrinsic LA myopathy in patients with HFpEF, beyond LA pressure overload. Abstract Figure.

Purpose Substantial differences between sexes exist with respect to cardiovascular diseases, including congenital heart disease. Nevertheless, clinical decisions in the long-term follow-up of patients with repaired tetralogy of Fallot (rTOF) are currently based on unisex thresholds for cardiac magnetic resonance (CMR) measurements. This study aimed to assess whether sex differences exist in cardiac adaptation to hemodynamic loading conditions in patients with rTOF. Methods and Results This cross-sectional, two-center, combined pediatric and adult cohort included 320 rTOF patients (163 males, 51%) who underwent routine CMR. Despite similar age (median and interquartile range [m + IQR] 23.4 [15.2–34.4] years), surgical history, and hemodynamic loading, males with rTOF demonstrated higher biventricular CMR-derived volumes and masses, indexed for body surface area, compared to females (e.g. m + IQR right ventricular (RV) end-diastolic volume: males 123 [100–151] mL/m2, females 114 [94–131] mL/m2, P = 0.007). Sex-specific Z-scores of biventricular volumes and masses were similar for males and females. RV volumes and masses correlated with hemodynamic loading, but these relations did not differ between sexes. Biventricular ejection fraction (EF) appeared to be lower in male patients, compared to female patients (e.g. m + IQR RVEF: males 48 [43–54]%, females 52 [46–57]%, P Conclusion Indexed ventricular volumes and masses are higher in males with rTOF, compared to females, similar to the healthy population. RV hypertrophy and dilatation correlated to loading conditions similarly for both sexes. However, under comparable loading conditions, males demonstrated more severe functional impairment. These results indicate that sex-differences should no longer be ignored in treatment strategies, including timing of pulmonary valve replacement.

No abstract available Keywords: Feature tracking; Fontan circulation; Functionally univentricular heart; Global circumferential strain; Global longitudinal strain.

Background We investigated serial serum levels of GDF‐15 (growth differentiation factor 15) in Fontan patients and their relation to outcome. Methods and Results In this single‐center prospective study of consecutive Fontan patients, serial serum GDF ‐15 measurement and clinical assessment was done at baseline (n=81) and after 2 years (n=51). The association between GDF ‐15 and the combined end point of all‐cause mortality, heart transplant listing, and Fontan‐related hospitalization was investigated. Median age at baseline was 21 years (interquartile range: 15–28 years). Median GDF ‐15 serum levels at baseline were 552 pg/mL (interquartile range: 453–729 pg/mL). GDF ‐15 serum levels correlated positively with age, age at Fontan initiation, New York Heart Association class, and serum levels of NT ‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) and ɣGT (γ‐glutamyltransferase) and negatively with exercise capacity. During a median follow‐up of 4.8 years (interquartile range: 3.3–5.5 years), the combined end point occurred in 30 patients (37%). Multivariate Cox regression showed that patients with the highest baseline GDF ‐15 (n=20, defined as the upper quartile) had a higher risk of hospitalization or death than the lowest 3 quartiles (hazard ratio [HR], 2.76; 95% CI , 1.27–6.00; P =0.011). After 2 years of follow‐up, patients in whom serum level of GDF ‐15 increased to >70 pg/mL (n=13) had a higher risk of hospitalization or death than the lowest 3 quartiles (HR, 2.69; 95% CI , 1.03–6.99; P =0.043). Conclusions In Fontan patients, elevated serum levels of GDF ‐15 are associated with worse functional status and predict Fontan‐related events. Furthermore, serial measurements showed that an increase in GDF ‐15 serum level was associated with increased risk for adverse outcome.

Background: In Fontan patients, attrition of ventricular function is well recognized, but early detection of ventricular dysfunction is difficult. The aim of this study is to longitudinally assess ventricular strain in Fontan patients using a new method for cardiac magnetic resonance (CMR) feature tracking, and to investigate the relationship between ventricular strain and cardiac systolic function. Methods and results: In this prospective, standardized follow-up study in 51 Fontan patients, age ≥ 10 years, CMR and concomitant clinical assessment was done at the start of the study and after 2 years. CMR feature tracking was done combining the dominant and hypoplastic ventricles. Global longitudinal strain (GLS) (−17.3% versus −15.9%, P = 0.041) and global circumferential strain (GCS) (−17.7 versus −16.1, P = 0.047) decreased over 2 years' time. Ejection fraction (EF) (57%), cardiac index (CI) (2.7 l/min/m2) and NYHA functional class (97% in class I/II) were preserved. The strain values of the combined dominant and hypoplastic ventricles were significantly worse compared to those of the dominant ventricle only (GLS −16.8 (−19.5 to −14.0) versus −18.8 (−21.3 to −15.3) respectively, P = 0.001, GCS −18.3 (−22.1 to −14.8) versus −22.5 (−26.3 to −19.4) respectively, P < 0.001). Conclusions: This study showed a decrease in cardiac strain over 2 years in Fontan patients without clinical signs of Fontan failure, where EF, CI and clinical status were still preserved. Cardiac strain might be a sensitive early indicator of systolic ventricular decline. Furthermore, combined strain of the hypoplastic and dominant ventricles seems a more accurate representation of cardiac strain in functionally univentricular hearts.

Background: Pulmonary valve replacement is very common among patients with congenital heart disease. The Carpentier Edwards Perimount valve (Edwards Lifesciences, Irvine, Calif), which was originally designed for the aortic position is among the most implanted valves. We aim to describe the follow-up of this valve in the pulmonary position.Methods: Patients with a Perimount valve implanted between 2003 and 2013 in the University Medical Center Groningen were followed for the primary end point reintervention, defined as surgical or transcatheter valve replacement. Secondary end point was the occurrence of valve failure, defined as significant valvular regurgitation or stenosis. Explanted valves were histologically examined.Results: Forty-five patients (median age at operation 27.8 years, 55.6% women) had a mean follow-up duration of 5.8 +/- 3.3 years. There were 7 reinterventions (5 surgical and 2 transcatheter). Freedom from reintervention was respectively 95% +/- 4% and 83% +/- 8% at 5- and 10- years of follow-up. Freedom from valve failure was 75% +/- 4% at 2 years, 65% +/- 8% at 5 years of follow-up and 57% +/- 10% at 10 years of follow-up. Morphology evaluation (n = 4) showed stiffened valves in the open position, with extensive fibrous tissue overgrowth on the leaflets and a variable proliferation of myofibroblasts.Conclusions: The Perimount valve has adequate function in the pulmonary valve position at 5 years of follow-up, although after 10 years of follow-up valve failure and reinterventions are common. Explanted valves show retraction and stiffening of the leaflets due to a fibrotic layer on both sides of the leaflet.

Aims Five echocardiographic parameters-left atrial volume index, left ventricular mass index, tricuspid regurgitation velocity, myocardial tissue velocity, and the ratio of early mitral inflow to tissue velocity of the mitral annulus (E/e')-are recommended in both the current European Society of Cardiology heart failure guidelines and the American Society of Echocardiography/European Association of Cardiovascular Imaging recommendations for the evaluation of left ventricular diastolic function. We aimed to perform a systematic review of these echocardiographic parameters at resting conditions for their correlation with left ventricular filling pressures in patients with heart failure with preserved ejection fraction (HFpEF). In addition, the prognostic value of these parameters was assessed. Methods and results Nine studies reported the correlation between echocardiography and invasive haemodynamics, and 18 papers reported on the prognostic value of echocardiography in HFpEF. Among the parameters, most data were reported for E/e'. The pooled correlation coefficient r was 0.56 for the relation between E/e' and invasively measured filling pressures. Combined weighted-mean meta-analysis of prognostic studies resulted in a hazard ratio of 1.05 (95% confidence interval 1.03-1.06) per unit increase in E/e' for the combined outcome of all-cause mortality and cardiovascular hospitalization. The other echocardiographic parameters, when taken individually, had similar or lower association with prognosis. Conclusion Only a small number of studies validated the use of echocardiographic parameters at rest in patients with HFpEF. The best established parameter appears to be E/e', but the existing data only show modest correlations of E/e' with invasive filling pressures and outcomes in HFpEF.

Hemodialysis is associated with a fall in myocardial perfusion and may induce regional left ventricular (LV) systolic dysfunction. The pathophysiology of this entity is incompletely understood, and the contribution of ultrafiltration and diffusive dialysis has not been studied. We investigated the effect of isolated ultrafiltration and isovolemic dialysis on myocardial perfusion and LV function. Eight patients (7 male, aged 55 ± 18 yr) underwent 60 min of isolated ultrafiltration and 60 min of isovolemic dialysis in randomized order. Myocardial perfusion was assessed by13N-NH3positron emission tomography before and at the end of treatment. LV systolic function was assessed by echocardiography. Regional LV systolic dysfunction was defined as an increase in wall motion score in ≥2 segments. Isolated ultrafiltration (ultrafiltration rate 13.6 ± 3.9 ml·kg−1·h−1) induced hypovolemia, whereas isovolemic dialysis did not (blood volume change −6.4 ± 2.2 vs. +1.3 ± 3.6%). Courses of blood pressure, heart rate, and tympanic temperature were comparable for both treatments. Global and regional myocardial perfusion did not change significantly during either isolated ultrafiltration or isovolemic dialysis and did not differ between treatments. LV ejection fraction and the wall motion score index did not change significantly during either treatment. Regional LV systolic dysfunction developed in one patient during isolated ultrafiltration and in three patients during isovolemic dialysis. In conclusion, global and regional myocardial perfusion was not compromised by 60 min of isolated ultrafiltration or isovolemic dialysis. Regional LV systolic dysfunction developed during isolated ultrafiltration and isovolemic dialysis, suggesting that, besides hypovolemia, dialysis-associated factors may be involved in the pathogenesis of hemodialysis-induced regional LV dysfunction.

Objectives: Supravalvar aortic stenosis (SVAS) is a rare congenital anomaly. The “single-patch technique,” “‘two sinus augmentation with an inverted Y-patch” (both nonsymmetrical corrections), “three-patch technique,” and the “slide aortoplasty” (both symmetrical corrections) are the techniques implemented by the majority of surgeons for the correction of SVAS. In the few studies that compared these techniques, no technique was shown to be superior over another. The aim of the present study is to review the 52-year experience with the surgical correction of SVAS in two of four congenital cardiothoracic surgical centers in the Netherlands. Methods: We retrospectively reviewed all patient files of those who underwent an operation to correct their SVAS, between 1962 and 2014 in our centers. Patients were divided according to their operating technique. These groups were compared using the end points freedom from reoperation and mortality. Results: A total of 49 patients were included, 23 (46.9%) patients in the nonsymmetrical group and 26 (53.1%) patients in the symmetrical group. Survival after 20 years in the nonsymmetrical group was 80% (standard error [SE]: 0.091) and in the symmetrical group was 85% (SE: 0.085; P = .163). Freedom from reoperation after 20 years in the nonsymmetrical group was 88% (SE: 0.079) and in the symmetrical group was 71% (SE: 0.107; P = 0.313). Conclusion: In this patient group, there is no significant difference in survival and freedom from reoperation between the different surgical techniques for SVAS repair. Compared to the survival in the general population, the survival of SVAS patients is remarkably low. Apparently, SVAS is not a benign disease and probably patients should be followed more closely for the rest of their lives.

Diabetes mellitus is associated with left-sided myocardial remodeling in heart failure with preserved ejection fraction (HFpEF). Little is known about the impact of diabetes mellitus on right ventricular (RV) function in HFpEF. We therefore studied the relation between diabetes mellitus and RV dysfunction in HFpEF. We have examined patients with HFpEF who underwent simultaneous right-sided cardiac catheterization and echocardiography. RV systolic function was assessed using multiple established echocardiographic parameters, and systolic dysfunction was present if ≥2 parameters were outside the normal range. RV diastolic function was assessed using the peak diastolic tissue velocity of the lateral tricuspid annulus (RV e') and was present if8.0 cm/s. Diabetes mellitus was defined as a documented history of diabetes, a fasting glucose level of ≥7.0 mmol/L, a positive glucose intolerance test result, or a glycated hemoglobin level of ≥6.5%. A total of 91 patients were studied (mean age 74 ± 9 years, 69% women). A total of 37% had RV systolic dysfunction and 23% RV diastolic dysfunction. Thirty-seven percent of the patients had type 2 diabetes mellitus. These patients had higher pulmonary artery pressure (34 mm Hg vs 29 mm Hg, p = 0.004), more RV systolic dysfunction (57% vs 29%, p = 0.009), more RV diastolic dysfunction (46% vs 12%, p = 0.001), and lower RV e' (8.7 cm/s vs 11.5 cm/s, p = 0.006). The presence of diabetes mellitus was independently associated with RV systolic dysfunction (odds ratio 2.84, 95% confidence interval 1.09 to 7.40, p = 0.03) and with RV diastolic dysfunction (odds ratio 4.33, 95% confidence interval 1.25 to 15.07, p = 0.02), after adjustment for age, gender, and pulmonary pressures. In conclusion, diabetes mellitus is strongly associated with RV systolic and diastolic dysfunctions in patients with HFpEF, independent of RV afterload.

OBJECTIVE: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research.METHODS: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients.RESULTS: Fontan patients scored significantly lower on general health than their healthy peers (t(19)=-3.0, P = .008), whereas their scores on other QOL domains and sexual well-being were comparable to normal values. During childhood, most patients experienced physical limitations and the feeling of being an outsider, and frequently faced bullying. Regarding sexual well-being, large interindividual differences were noted. Four interviewed patients (25-30 years) reported a good sexual well-being, whereas the other interviewed patients (33-47 years) reported erectile dysfunction, low self-esteem and avoidance of sexual intercourse. Both the QOL domains mental health and role restrictions due to emotional problems were associated with female avoidance (P = .083, respectively, P = .089) and dyspareunia (P = ns respectively P = .094). In males, role restrictions due to physical problems and health change were related to sexual dissatisfaction (P = .056) respectively nonsensuality (P = .025).CONCLUSIONS: Overall, Fontan patients have a relatively preserved quality of life and sexual wellbeing but face more social isolation and bullying during childhood/adolescence than their healthy peers. Sexual problems were mainly associated with physical limitations in males and with psychosocial limitations in females. Finally, sexual dysfunction was more common in older Fontan patients, and future research has to clarify whether progressive attrition of the Fontan circulation affects the patients' QOL and sexual well-being.

OBJECTIVES: Pulmonary arterial hypertension (PAH) is a progressive and often fatal disease characterized by increased pulmonary vascular resistance (PVR) and right ventricular (RV) failure. End-stage PAH is often an indication for a lung transplant (LTX). Our goal was to study ventricular recovery using cardiac magnetic resonance imaging late after LTX.METHODS: We studied 10 patients with PAH who underwent isolated bilateral LTX. RV and left ventricular (LV) volumes, function and mass were measured. In addition, the RV stroke volume/end-systolic ratio (SV/ESV), the LV eccentricity index, the RV/LV volume ratio, the area of the tricuspid valve annulus and the severity of tricuspid regurgitation (TR) were calculated.RESULTS: The median age was 44 [30-54] years and the mean PVR was 1020 +/- 435 dynes.s.cm(-5). Six patients had >= moderate TR. After LTX, the RV ejection fraction increased from 32 to 64% (P = mild TR post-LTX.CONCLUSIONS: Cardiac magnetic resonance imaging confirms ventricular recovery after isolated bilateral LTX for end-stage PAH.

Aims Many patients with heart failure with preserved ejection fraction (HFpEF) develop post-capillary pulmonary hypertension (PH) due to increased left-sided filling pressures. However, a subset of patients develops combined postand pre-capillary PH. We studied the value of echocardiographic right-sided characterization for the discrimination between pre- vs. post-capillary PH in HFpEF, using invasive haemodynamics as gold standard.Methods and results 102 consecutive HFpEF patients with simultaneous right heart catheterization and echocardiography were identified. Patients were divided into: 'no PH', 'isolated post-capillary PH', and 'post-and pre-capillary PH'. Systolic pulmonary arterial pressure (SPAP), tricuspid valve annular plane systolic excursion (TAPSE), right ventricular-vascular coupling (TAPSE/SPAP), and VO2-max were assessed. Primary endpoint was all-cause mortality. A total of 97 patients were included: 22% no PH, 47% isolated post-capillary PH, and 31% post- and pre-capillary PH. Patients with post-and pre-capillary PH had more often diabetes mellitus (47 vs. 24%, P = 0.04), had more heart failure hospitalizations (57 vs. 26%, P = 0.007) and lower VO2-max (10 vs. 13 mL/min/kg, P = 0.008), compared with those with isolated post-capillary PH. Patients with post-and pre-capillary PH also had more reduced TAPSE (17 vs. 21 mm, P = 0.001) and TAPSE/SPAP (0.3 vs. 0.5, P Conclusion Abnormal right ventricular-vascular coupling identifies patients with HFpEF and additional pre-capillary PH, and predicts poor outcome in HFpEF.

Quantification of pulmonary regurgitation (PR) is essential in the management of patients with repaired tetralogy of Fallot (TOF). We sought to evaluate the accuracy of first-line Doppler echocardiography in comparison with cardiac magnetic resonance imaging (MRI) to identify hemodynamic significant PR. Paired cardiac MRI and echocardiographic studies (n = 97) in patients with repaired TOF were retrospectively analyzed. Pressure half time (PHT) and pulmonary regurgitation index (PRi) were measured using continuous wave Doppler. The ratio of the color flow Doppler regurgitation jet width to pulmonary valve (PV) annulus (jet/annulus ratio) and diastolic to systolic time velocity integral (DSTVI; pulsed wave Doppler) were assessed. Accuracy of echocardiographic measurements was tested to identify significant PR as determined by phase-contrast MRI (PR fraction [PRF] ≥ 20%). Mean PRF was 29.4 ± 15.7%. PHT

Sensitivity and specificity of the modified Duke criteria for native valve endocarditis are both suboptimal, at approximately 80%. Diagnostic accuracy for intracardiac prosthetic material-related infection is even lower. Non-invasive imaging modalities could potentially improve diagnosis of infective endocarditis; however, their diagnostic value is unclear. We did a systematic literature review to critically appraise the evidence for the diagnostic performance of these imaging modalities, according to PRISMA and GRADE criteria. We searched PubMed, Embase, and Cochrane databases. 31 studies were included that presented original data on the performance of electrocardiogram (ECG)-gated multidetector CT angiography (MDCTA), ECG-gated MRI, (18)F-fluorodeoxyglucose ((18)F-FDG) PET/CT, and leucocyte scintigraphy in diagnosis of native valve endocarditis, intracardiac prosthetic material-related infection, and extracardiac foci in adults. We consistently found positive albeit weak evidence for the diagnostic benefit of (18)F-FDG PET/CT and MDCTA. We conclude that additional imaging techniques should be considered if infective endocarditis is suspected. We propose an evidence-based diagnostic work-up for infective endocarditis including these non-invasive techniques.

Background: Myocardial strain has been shown to predict outcome in various cardiovascular diseases, including congenital heart diseases. The aim of this study was to evaluate the predictive value of cardiac magnetic resonance (CMR) feature-tracking derived strain parameters in repaired tetralogy of Fallot (rTOF) patients for developing ventricular tachycardia (VT) and deterioration of ventricular function. Methods: Patients with rTOF who underwent CMR investigation were included. Strain and strain-rate of both ventricles were assessed using CMR feature tracking. The primary outcome was a composite of the occurrence of sustained VT or non-sustained VT requiring invasive therapy. The secondary outcome was analyzed in patients that underwent a second CMR after 1.5 to 3.5 years. Deterioration was defined as reduction (≥10%) in right ventricular (RV) ejection fraction, reduction (≥10%) in left ventricular (LV) ejection fraction or increase (≥30 mL/m2) in indexed RV end-diastolic volume compared to baseline. Results: 172 patients (median age 24.3 years, 54 patients

Background: Currently, all patients evaluated for bronchoscopic lung volume reduction are screened for pulmonary hypertension (PH) and congestive heart failure (CHF), since these can increase procedural risks and might affect patient outcomes. Objectives: To evaluate the prevalence of PH and CHF in this specific patient group, and investigate if CT-derived pulmonary artery (PA) measurements can be used as alternative screening tool. Methods: In this retrospective analysis, all patients screened for a bronchoscopic intervention in our hospital were included. The 6-Minute Walking Distance (6MWD) was used to assess patient outcome. PA:Aorta (PA:A) ratio and perpendicular PA diameter were measured on CT. Results: 274 Patients were included (FEV1 25%pred and RV 235%pred). 39 Patients had measurable PH with a right ventricle systolic pressure (RVSP) >35 mmHg and 1 patient had an LVEF 1, 3 patients with a RVSP >35mm Hg were detected (7.7%). Patients with an RVSP >35 mmHg had significantly higher perpendicular PA diameters (29.5 vs. 27.5 mm, p=0.020), but no significantly different PA:A ratios. Higher RVSP and worse diastolic function were stronger associated with a lower 6MWD (r=-0.27, p=0.015 and r=-0.25, p=0.006, respectively) than PA:A ratio (r=-0.16, p=0.012). Conclusion: The prevalence of PH with a RVSP >35 mmHg or CHF with an LVEF 1 is not a useful cardiac screening tool for PH. Perpendicular PA measurements may be helpful in preselecting patients for echocardiography, but needs to be evaluated in larger numbers of patients with PH.

IntroductionChronic non-invasive ventilation (NIV) has become evidence-based care for stable hypercapnic COPD patients. While the number of patients increases, home initiation of NIV would greatly alleviate the healthcare burden. We hypothesise that home initiation of NIV with the use of telemedicine in stable hypercapnic COPD is non-inferior to in-hospital NIV initiation.MethodsSixty-seven stable hypercapnic COPD patients were randomised to initiation of NIV in the hospital or at home using telemedicine. Primary outcome was daytime arterial carbon dioxide pressure (PaCO2) reduction after 6 months NIV, with a non-inferiority margin of 0.4 kPa. Secondary outcomes were health-related quality of life (HRQoL) and costs.ResultsHome NIV initiation was non-inferior to in-hospital initiation (adjusted mean difference in PaCO2change home vs in-hospital: 0.04 kPa (95% CI −0.31 to 0.38 kPa), with both groups showing a PaCO2reduction at 6 months compared with baseline (home: from 7.3±0.9 to 6.4±0.8 kPa (pDiscussionThis is the first study showing that home initiation of chronic NIV in stable hypercapnic COPD patients, with the use of telemedicine, is non-inferior to in-hospital initiation, safe and reduces costs by over 50%.Trial registration numberNCT02652559.

Cardiac tachyarrhythmias are the leading cause of morbidity and mortality in patients with repaired tetralogy of Fallot (TOF). We evaluated risk factors for sustained ventricular tachyarrhythmia (VT) and atrial tachyarrhythmia (ATA) in these patients. Patients (n = 319) who underwent cardiac magnetic resonance (CMR) imaging at two tertiary centers between 2007 and 2016 were assessed. Potential risk markers, based on history, cardiac magnetic resonance imaging (CMR), electrocardiography (ECG) and echocardiography, were analyzed for prediction of the primary endpoint of VT, and the secondary endpoint of ATA. During a follow-up of 3.5 (0.9–6.1) years, 20 (6.3%) patients reached the primary endpoint, and 30 (9.4%) the secondary endpoint. Multivariable cox hazards regression identified right ventricular (RV) end-diastolic volume (Hazard ratio [HR] 2.03, per 10 ml/m2 increase; p = 0.02), RV end-systolic volume (HR 3.04, per 10 ml/m2 increase; p = 0.04), RV mass (HR 1.88, per 10 g/m2 increase; p = 0.02), and RV ejection fraction (HR 6.06, per 10% decrease; p = 0.02) derived from CMR to be independent risk factors of VT. In addition, QRS-duration (HR 1.70, per 10 ms increase; p = 0.001) and body mass index (BMI: HR 1.8, per 5 kg/m2 increase; p = 0.02) were independent markers of VT. Older age at TOF repair (HR 1.33, per 2 months increase; p = 0.03) and BMI (HR 1.76, per 5 kg/m2 increase; p

Objective: Hinge washing is a crucial factor in the prevention of mechanical prosthetic valvar thrombosis, especially in the pulmonary valve position. The aim of this laboratory study was to determine the relationship between pressure difference and the amount of hinge washing in the closed position, using the pressures that are normal for the right ventricle and pulmonary artery. Methods: In an in vitro setting, four different bileaflet mechanical valves were tested for hinge washing in closed position. Based on similarity in inner diameter (range: 20.5-21.4 mm), the following valves were tested: Abbott SJM Regent size 23, Cryolife On-X size 23, LivaNova Carbomedics-R size 25, Medtronic Open Pivot (M-OP)-A size 25. Tests were carried out in a range between 3 and 100 mm Hg pressure difference, using water as a test fluid. The amount of leakage per minute through the closed valve was measured. Results: All four valves showed an increase in leakage with increasing transvalvar gradient, and the relationship between pressure and leakage behaves in logarithmic fashion. Leakage under normal pulmonary diastolic pressure conditions (10 mm Hg) was between 23.3% and 29.3% of the leakage under aortic diastolic pressure conditions (80 mm Hg). The Cryolife On-X valve showed the highest closed leakage volume under pulmonary conditions (10 mm Hg) 0.254 ± 0.01 (L/min), where the Medtronic M-OP showed the lowest leakage volume with 0.125 ± 0.014 (mL/min). Conclusion: Hinge washing is related to transvalvar pressure difference in closed position. Valve brands differed significantly from each other in the amount of hinge washing.