Acute vasoreactivity testing in pediatric idiopathic pulmonary arterial hypertension:an international survey on current practice

The aim of this study was to determine practice patterns and inter-institutional variability in how acute vasoreactivity testing (AVT) is performed and interpreted in pediatrics throughout the world. A survey was offered to physicians affiliated with the Pediatric & Congenital Heart Disease Taskforce of the Pulmonary Vascular Research Institute (PVRI), the Pediatric Pulmonary Hypertension Network (PPHNET), or the Spanish Registry for Pediatric Pulmonary Hypertension (REHIPED), from February to December 2016. The survey requested data about the site-specific protocol for AVT and subsequent management of pediatric patients with idiopathic pulmonary arterial hypertension (IPAH) or heritable PAH (HPAH). Twenty-eight centers from 13 countries answered the survey. AVT is performed in most centers using inhaled nitric oxide (iNO). Sitbon criteria was used in 39% of the centers, Barst criteria in 43%, and other criteria in 18%. First-line therapy for positive AVT responders in functional class (FC) I/II was calcium channel blocker (CCB) in 89%, but only in 68% as monotherapy. Most centers (71%) re-evaluated AVT-positive patients hemodynamics after 6-12 months; 29% of centers re-evaluated based only on clinical criteria. Most centers (64%) considered a good response as remaining in FC I or II, with near normalization of pulmonary arterial pressure and pulmonary vascular resistance, but a stable FC I/II alone was sufficient criteria in 25% of sites. Protocols and diagnostic criteria for AVT, and therapeutic approaches during follow-up, were highly variable across the world. Reported clinical practice is not fully congruent with current guidelines, suggesting the need for additional studies that better define the prognostic value of AVT for pediatric IPAH patients

Measurement of the Lifetime Difference Between B_s Mass Eigenstates

We present measurements of the lifetimes and polarization amplitudes for B_s --> J/psi phi and B_d --> J/psi K*0 decays. Lifetimes of the heavy (H) and light (L) mass eigenstates in the B_s system are separately measured for the first time by determining the relative contributions of amplitudes with definite CP as a function of the decay time. Using 203 +/- 15 B_s decays, we obtain tau_L = (1.05 +{0.16}/-{0.13} +/- 0.02) ps and tau_H = (2.07 +{0.58}/-{0.46} +/- 0.03) ps. Expressed in terms of the difference DeltaGamma_s and average Gamma_s, of the decay rates of the two eigenstates, the results are DeltaGamma_s/Gamma_s = (65 +{25}/-{33} +/- 1)%, and DeltaGamma_s = (0.47 +{0.19}/-{0.24} +/- 0.01) inverse ps.Comment: 8 pages, 3 figures, 2 tables; as published in Physical Review Letters on 16 March 2005; revisions are for length and typesetting only, no changes in results or conclusion

NanOpinion D6.1 – Evaluation Strategy Plan: Monitoring public opinion on Nanotechnology in Europe

Evaluation Strategy Pla

Isolated neurofibroma of the urinary bladder incidentally discovered during cystoscopy

Isolated neurofibromas of the urinary bladder are rare benign tumors typically associated with neurofibromatosis type 1 (NF-1). Herein highlights a bladder neurofibroma incidentally discovered during cystoscopy following midurethral sling removal in a 61-year-old woman without NF-1 sequela. Despite malignancy concerns due to smoking history, histology confirmed a benign neurofibroma. These tumors differ from NF-1-associated neurofibromas in origin and presentation; they are rare, often asymptomatic, and likely stem from somatic mutations. Conservative management is preferred, with surgical intervention indicated only for obstructive masses

Significance of α-Myosin Heavy Chain (<i>MYH6</i>) Variants in Hypoplastic Left Heart Syndrome and Related Cardiovascular Diseases

Hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease (CHD) with complex genetic inheritance. HLHS segregates with other left ventricular outflow tract (LVOT) malformations in families, and can present as either an isolated phenotype or as a feature of a larger genetic disorder. The multifactorial etiology of HLHS makes it difficult to interpret the clinical significance of genetic variants. Specific genes have been implicated in HLHS, including rare, predicted damaging MYH6 variants that are present in >10% of HLHS patients, and which have been shown to be associated with decreased transplant-free survival in our previous studies. MYH6 (α-myosin heavy chain, α-MHC) variants have been reported in HLHS and numerous other CHDs, including LVOT malformations, and may provide a genetic link to these disorders. In this paper, we outline the MYH6 variants that have been identified, discuss how bioinformatic and functional studies can inform clinical decision making, and highlight the importance of genetic testing in HLHS

Impact of reintervention during stage 1 palliation hospitalization: A national, multicenter study

Background: Stage 1 palliation (S1P) for hypoplastic left heart syndrome remains associated with high morbidity and mortality. Prior studies on burden of reinterventions do not include patients who remain hospitalized prior to stage 2 palliation (S2P). This study describes the rate of reintervention during S1P hospitalization and seeks to determine the impact of reintervention on outcomes. Methods: All participants enrolled in Phase II of the National Pediatric Cardiology Quality Improvement Collaborative post-S1P are included in this study. Primary outcome is rate of reintervention during hospitalization post-S1P prior to hospital discharge or S2P. Reintervention is defined as one or more unplanned interventional cardiac catheterizations and/or surgical reoperations. Results: Between 3/1/16-10/1/19, 1367 participants underwent S1P and 339 (24.8%) had a reintervention; most commonly to address the source of pulmonary blood flow. Gestational age, weight at S1P, atrioventricular septal defect, heterotaxy, pre-operative pulmonary artery bands, hybrid S1P and an additional bypass run or early extracorporeal membrane oxygenation were significantly associated with reintervention. Participants in the reintervention group experienced higher rates for nearly all postoperative complications, were less likely to be discharged prior to S2P (57.1% vs 86%, p\u3c 0.001) and more likely to suffer in-hospital mortality (17% vs 5%, p\u3c0.001). Conclusions: Unplanned reintervention during hospitalization following S1P palliation occurred in 25% of participants in a large, registry based national cohort. Participants who underwent reintervention were more likely to remain inpatient and less likely to survive to S2P. Reintervention was associated with a multitude of postoperative complications that impact survival and long-term outcome