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Abstract: Introduction: Objective risk stratifiers are required to assess need for escalation of medical therapy or listing for transplantation in children with pulmonary arterial hypertension (PAH). We aimed to assess whether heart rate variability (HRV) predicts outcome in children with severe PAH. Patients and methods: Parameters of HRV [SDNN = standard deviation of normal-to-normal intervals, SDANN = standard deviation of mean values for normal-to-normal intervals over 5 min, and RMSSD = square root of the mean square differences of successive RR intervals] were determined from Holter electrocardiograms of 47 patients (27 male; mean age 11.4±5.5 years) with PAH (idiopathic PAH n =21; associated PAH n =26). HRV was assessed in relation to WHO Functional Class, six-minute-walk-test, echocardiographic and haemodynamic data, and as a predictor of death or lung transplantation. Results: The mean follow-up period was 19±11.5 months (1.8–49.1 months). Ten patients died and 7 underwent transplantation and these children had significantly lower values of HRV [SDANN (70.7±30.9 vs. 119.2±57.2), SDNN (79.5±30.5 vs. 129.9±63.6), and RMSSD (22.±11.7 vs. 47.7±34.5); p <0.001 for all]. On univariate Cox-proportional-hazards analysis all parameters of HRV predicted death or transplantation (p <0.05). SDANN and SDNN were also predictive of mortality alone (p <0.05). On bivariate analysis SDANN and SDNN predicted outcome independently of functional status, syncope, right ventricular function, and haemodynamic parameters. Conclusions: HRV is a useful, non-invasive means of predicting a poor outcome and hence need to escalate therapy in children with PAH. [Copyright &y& Elsevier]

Abstract: Background: We aimed to assess whether levels of B-type natriuretic peptide (BNP) – an established marker of ventricular dysfunction – relate to functional status and outcome in children with idiopathic and associated pulmonary hypertension (PH). Methods and results: BNP was measured in 50 children with PHT aged 8.4±5.1 years, all receiving PH specific therapies. Twenty-seven patients were diagnosed with idiopathic PH (IPAH), while 23 patients had associated PH [congenital heart disease (n =17), lung disease (n =4), other (n =2)]. Functional status, six-minute walk test, echocardiographic and haemodynamic data were assessed. Mean BNP value was 143.5±236.2 pg/ml (range <5–1250). BNP correlated with Functional Class II, III, and IV (50.8±61.3, 196.9±291.2 and 280.0±276.5 respectively; p =0.01), with echocardiographic assessment of right ventricular function (p <0.01), hypertrophy (p <0.01) and dilatation (p <0.01). In IPAH BNP correlated with pulmonary arterial pressure and, on inhaled nitric oxide also with vascular resistance. During a mean follow-up of 14.0±7.5 months seven patients died, five underwent transplantation and two were listed for transplantation. Using ROC analysis, a BNP value >130 pg/ml predicted death or need for transplantation (p <0.04). However, six children who died or were transplanted had a BNP value lower than this. Conclusion: BNP correlated positively with functional status in children with pulmonary hypertension, but had limited sensitivity (57%) for predicting death or need for transplantation. [Copyright &y& Elsevier]

Objectives: The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (CPET) in patients who received a Mustard and Senning (M/S) operation. Background: Patients who received an M/S operation have increased long-term risk of cardiovascular morbidity and mortality. Limited information is available on how to stratify risk in this population. Methods: Between 1996 and 2007, 274 adults (age 26.3 ± 8.9 years, range 16 to 50 years) who had received a Mustard (n = 144) or Senning (n = 130) operation in infancy were studied with CPET. During a follow-up of 3.9 ± 2.3 years (range 0.2 to 10.8 years), 12 patients died at an age of 36 ± 14 years, and 46 patients required a cardiac-related emergency (<24 h from the onset of symptom/condition) hospital admission at an age of 30 ± 11 years. Results: At multivariate Cox analysis, the slope of ventilation per unit of carbon dioxide output (VE/VCO2 slope) (hazard ratio: 1.088, p < 0.0001) and percentage of predicted peak oxygen uptake (Vo 2%) (hazard ratio: 0.979, p = 0.0136) were the strongest predictors of death/cardiac-related emergency hospital admission among demographic, clinical, and exercise variables. A VE/VCO2 slope ≥35.4 (hazard ratio: 10.7, 95% confidence interval [CI]: 7.8 to 24.6), and a peak Vo 2% ≤52.3% (hazard ratio: 3.4, 95% CI: 2.5 to 8.2) were associated with an increased 4-year risk of death/cardiac-related emergency hospital admission. Patients who had both a VE/VCO2 slope ≥35.4 and a peak Vo 2% ≤52.3% of predicted value were at highest risk (4-year event rate: 78.8%). Conclusions: CPET provides important prognostic information in adults with M/S operation. Subjects with enhanced ventilatory response to exercise or those with poor exercise capacity have a substantially higher 4-year risk of death/cardiac-related emergency hospital admission. [Copyright &y& Elsevier]

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990–2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: To evaluate the relationship between socioeconomic status (SES), access to physical activity resources, urban–rural dwelling, levels of pollution and exercise capacity in adult congenital heart disease (ACHD) patients. Background: Exercise intolerance is prevalent in ACHD and the contributing factors are poorly understood. Methods: A total of 1268 ACHD patients living in England who underwent cardiopulmonary exercise testing at our center were included. Neighborhood deprivation (English Indices of Deprivation), urban–rural dwelling, availability of green space, distance to the closest gym/fitness center and levels of pollution were estimated based on administrative data. Results: Urban–rural dwelling, availability of green space and levels of pollution were unrelated to exercise capacity. Lower SES was associated with a significantly lower peak oxygen consumption (P <0.002) and heart rate reserve (P <0.004). This association was non-linear and most pronounced in ACHD patients with cardiac defects of medium complexity living in the most socioeconomically disadvantaged communities. Low SES was associated with higher prevalence of diabetes (P =0.015) and smoking (P =0.01). Coronary artery disease was rare in this young population and low SES was found to be related to exercise capacity independently of the presence of coronary artery disease. Conclusions: Living in poorer areas is associated with exercise intolerance in contemporary ACHD patients. Although low SES is linked to traditional cardiovascular risk factors, the deleterious effects of SES on exercise capacity seem to be only partially mediated via coronary artery disease. Reducing social inequalities in ACHD patients may have a positive effect on quality of life and long-term prognostic implications. [Copyright &y& Elsevier]