Advancing Wearable Biosensors for Congenital Heart Disease: Patient and Clinician Perspectives: A Science Advisory From the American Heart Association.

Wearable biosensors (wearables) enable continual, noninvasive physiologic and behavioral monitoring at home for those with pediatric or congenital heart disease. Wearables allow patients to access their personal data and monitor their health. Despite substantial technologic advances in recent years, issues with hardware design, data analysis, and integration into the clinical workflow prevent wearables from reaching their potential in high-risk congenital heart disease populations. This science advisory reviews the use of wearables in patients with congenital heart disease, how to improve these technologies for clinicians and patients, and ethical and regulatory considerations. Challenges related to the use of wearables are common to every clinical setting, but specific topics for consideration in congenital heart disease are highlighted.

Examining the impact of median arcuate ligament-induced celiac artery compression on target vessel patency, long-term survival and device integrity in fenestrated and branched endovascular repairs.

OBJECTIVE: To evaluate the impact of celiac artery (CA) compression by median arcuate ligament (MAL) on technical metrics and long-term CA patency in patients with complex aortic aneurysms undergoing fenestrated/branched endograft repairs (F/B-EVARs). METHODS: Single-center, retrospective review of patients undergoing fenestrated/branched endovascular aortic aneurysm repairs and requiring incorporation of the CA between 2013 and 2023. Patients were divided into two groups-those with (MAL+) and without (MAL-) CA compression-based on preoperative computed tomography angiography findings. MAL was classified in three grades (A, B, and C) based on the degree and length of stenosis. Patients with MAL grade A had ≤50% CA stenosis measuring ≤3 mm in length. Those with grade B had 50% to 80% CA stenosis measuring 3 to 8 mm long, whereas those with grade C had >80% stenosis measuring >8 mm in length. End points included device integrity, CA patency and technical success-defined as successful implantation of the fenestrated/branched device with perfusion of CA and no endoleak. RESULTS: One hundred and eighty patients with complex aortic aneurysms (pararenal, 128; thoracoabdominal, 52) required incorporation of the CA during fenestrated/branched endovascular aortic aneurysm repair. Majority (73%) were male, with a median age of 76 years (interquartile range [IQR], 69-81 years) and aneurysm size of 62 mm (IQR, 57-69 mm). Seventy-eight patients (43%) had MAL+ anatomy, including 33 patients with MAL grade A, 32 with grade B, and 13 with grade C compression. The median length of CA stenosis was 7.0 mm (IQR, 5.0-10.0 mm). CA was incorporated using fenestrations in 177 (98%) patients. Increased complexity led to failure in CA bridging stent placement in four MAL+ patients, but completion angiography showed CA perfusion and no endoleak, accounting for a technical success of 100%. MAL+ patients were more likely to require bare metal stenting in addition to covered stents (P = .004). Estimated blood loss, median operating room time, contrast volume, fluoroscopy dose and time were higher (P < .001) in MAL+ group. Thirty-day mortality was 3.3%, higher (5.1%) in MAL+ patients compared with MAL- patients (2.0 %). At a median follow-up of 770 days (IQR, 198-1525 days), endograft integrity was observed in all patients and CA events-kinking (n = 7), thrombosis (n = 1) and endoleak (n = 2) -occurred in 10 patients (5.6%). However, only two patients required reinterventions. MAL+ patients had overall lower long-term survival. CONCLUSIONS: CA compression by MAL is a predictor of increased procedural complexity during fenestrated/branched device implantation. However, technical success, long-term device integrity and CA patency are similar to that of patients with MAL- anatomy.

Impact of a multidisciplinary acute aortic dissection program: Improved outcomes with a comprehensive initial surgical repair strategy.

OBJECTIVE: As part of a multidisciplinary aortic dissection (AD) program, a more comprehensive repair strategy for patients with acute type A aortic dissection (ATAAD) and frequent endografting for suitable patients with type B aortic dissection (ATBAD) was adopted in 2015. The aim of this study was to evaluate the impact of these changes. METHODS: This study is a retrospective review of a prospective database containing all patients treated for acute AD between 2003 and 2020. Patients were grouped based on differing repair strategies (pre 2015 vs post 2015). Clinical characteristics, procedural details, and survival data were analyzed. RESULTS: During this time, 323 patients (210 pre, 113 post) were treated for acute AD at our institution. There were 221 patients with ATAAD (149 pre, 72 post) and 102 patients with ATBAD (61 pre, 41 post). The majority (60%) were males, with a mean age of 65.9 ± 15.2 years. There were no differences in cardiovascular risk factors or demographics between the groups. After 2015, fewer patients with ATAAD underwent medical management alone (15% pre vs 4% post; P = .014), and most that underwent surgical intervention had a total arch or aggressive hemiarch repair (27% pre vs 78% post; P < .001). Seventy-four patients (73%) with ATBAD were treated medically, whereas 28 underwent medical management and endografting (23% pre, 34% post; P = .214). For all patients with AD, 30-day mortality was significantly improved (26% pre vs 10% post; P < .001) especially among patients who underwent ATAAD surgery (23% pre vs 9% post; P = .018). Three-year Kaplan-Meier survival estimates showed survival improvement among patients with ATAAD (Log rank P-value = .019); however, this improvement does not extend to type B dissections or the overall cohort. A survival analysis landmarked to 30 days after initial presentation showed no statistical difference in survival from 30 days to 3 years post-presentation. CONCLUSIONS: A more comprehensive repair strategy in the management of patients with acute AD resulted in improved overall patient outcomes and significantly decreased 30-day mortality, even though more complex repairs were performed. The long-term impact of the changes made to our program remains to be evaluated.

Video Consent for Colonoscopy Improves Knowledge Retention and Patient Satisfaction: A Randomized Controlled Study.

BACKGROUND AND AIM: Informed consent for endoscopy is variable across institutions and remains understudied in gastrointestinal endoscopy. This study aims to standardize informed consent for screening and diagnostic colonoscopies with a supplemental video tool that includes the key components of informed consent. METHODS: A video tool was developed that incorporated the key components of informed consent for colonoscopy. In addition, a 7-question survey was developed to query patients on core aspects of informed consent and satisfaction with the informed consent process. Patients undergoing elective outpatient colonoscopy with conscious sedation were randomized to traditional consent or consent with the addition of a video tool. A pilot study determined the sample size. Traditional consent was standard of practice before the procedure. Patients in the video tool group watched the video tool in the preprocedure area followed by traditional consent. Both groups had the opportunity to address questions with the attending physician before the procedure. All patients were contacted 1 to 2 days following the colonoscopy to answer the question survey. RESULTS: A total of 110 patients were eligible for participation, and 91 were included in the final data analysis. Subjects in the video tool group demonstrated significantly higher recall of key aspects of informed consent and higher satisfaction with the informed consent process versus the traditional consent group. The history of prior colonoscopy was similar between both groups. Mean endoscopy operation metrics were not negatively impacted by the inclusion of the video tool. CONCLUSION: Patients undergoing screening and diagnostic colonoscopies who received informed consent supplemented by a video tool had a higher recall of core aspects of informed consent and higher satisfaction with the process, with no impact on procedural times.

Land Cover Change Associated with Unconventional Oil and Gas Development in the Appalachian Region.

Unconventional oil and gas (UOG) wells from the Marcellus and Utica shale plays have expanded greatly across the Appalachian region of the United States (US) since the early 2000s. This region is now the single largest natural gas producing area of the US. The local and regional impacts of this industry on the landscape make it critical to understand for future planning efforts. This study investigated land cover change associated with over 21,000 unconventional wells representing 4,240 well pads permitted from 2007 to 2017 in Pennsylvania, West Virginia, and Ohio. The goal was to characterize UOG disturbance to document development patterns and extents in the region. Supervised classification was used to map land use and land-cover changes within a 25-ha buffer of well pads identified in the region. On average, disturbance related to unconventional development impacted 6.2 ha in Pennsylvania, 4.7 ha in Ohio and 4.4 ha in West Virginia and 5.6 ha over the region. Forest and grassland were found to be the most impacted cover types, with increases in impervious surface areas being a significant contributor to land-use classification change. These conversions can contribute to increased forest fragmentation and edge, which can in turn adversely impact biodiversity indicators at the regional level. Additionally, increases in impervious surface in small headwater watersheds can lead to increased sediment and runoff loads in receiving streams. Local and regional land use planning should be implemented during the well pad permit review process to help minimize environmental impacts over larger geographic scales.

Toll-like receptor 2 and 4 antagonism for the treatment of experimental autoimmune encephalomyelitis (EAE)-related pain.

Neuropathic pain is a major symptom of multiple sclerosis (MS) with up to 92% of patients reporting bodily pain, and 85% reporting pain severe enough to cause functional disability. None of the available therapeutics target MS pain. Toll-like receptors 2 and 4 (TLR2/TLR4) have emerged as targets for treating a wide array of autoimmune disorders, including MS, as well as having demonstrated success at suppressing pain in diverse animal models. The current series of studies tested systemic TLR2/TLR4 antagonists in males and females in a low-dose Myelin oligodendrocyte glycoprotein (MOG) experimental autoimmune encephalomyelitis (EAE) model, with reduced motor dysfunction to allow unconfounded testing of allodynia through 50+ days post-MOG. The data demonstrated that blocking TLR2/TLR4 suppressed EAE-related pain, equally in males and females; upregulation of dorsal spinal cord proinflammatory gene expression for TLR2, TLR4, NLRP3, interleukin-1ß, IkBα, TNF-α and interleukin-17; and upregulation of dorsal spinal cord expression of glial immunoreactivity markers. In support of these results, intrathecal interleukin-1 receptor antagonist reversed EAE-induced allodynia, both early and late after EAE induction. In contrast, blocking TLR2/TLR4 did not suppress EAE-induced motor disturbances induced by a higher MOG dose. These data suggest that blocking TLR2/TLR4 prevents the production of proinflammatory factors involved in low dose EAE pathology. Moreover, in this EAE model, TLR2/TLR4 antagonists were highly effective in reducing pain, whereas motor impairment, as seen in high dose MOG EAE, is not affected.

Machine Learning Models to Predict Major Adverse Cardiovascular Events After Orthotopic Liver Transplantation: A Cohort Study.

OBJECTIVE: To develop machine learning models that can predict post-transplantation major adverse cardiovascular events (MACE), all-cause mortality, and cardiovascular mortality in patients undergoing liver transplantation (LT). DESIGN: Retrospective cohort study. SETTING: High-volume tertiary care center. PARTICIPANTS: The study comprised 1,459 consecutive patients undergoing LT between January 2008 and December 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: MACE, all-cause mortality, and cardiovascular mortality were modeled using logistic regression, least absolute shrinkage and selection surgery regression, random forests, support vector machine, and gradient-boosted modeling (GBM). All models were built by splitting data into training and testing cohorts, and performance was assessed using five-fold cross-validation based on the area under the receiver operating characteristic curve and Harrell's C statistic. A total of 1,459 patients were included in the final cohort; 1,425 (97.7%) underwent index transplantation, 963 (66.0%) were female, the median age at transplantation was 57 (11-70) years, and the median Model for End-Stage Liver Disease score was 20 (6-40). Across all outcomes, the GBM model XGBoost achieved the highest performance, with an area under the receiver operating curve of 0.71 (95% confidence interval [CI] 0.63-0.79) for MACE, a Harrell's C statistic of 0.64 (95% CI 0.57-0.73) for overall survival, and 0.72 (95% CI 0.59-0.85) for cardiovascular mortality over a mean follow-up of 4.4 years. Examination of Shapley values for the GBM model revealed that on the cohort-wide level, the top influential factors for postoperative MACE were age at transplantation, diabetes, serum creatinine, cirrhosis caused by nonalcoholic steatohepatitis, right ventricular systolic pressure, and left ventricular ejection fraction. CONCLUSION: Machine learning models developed using data from a tertiary care transplantation center achieved good discriminant function in predicting post-LT MACE, all-cause mortality, and cardiovascular mortality. These models can support clinicians in recipient selection and help screen individuals who may be at elevated risk for post-transplantation MACE.

Evaluation of the impact of patient input in health technology assessments at NICE.

OBJECTIVE: Accounts of patient experiences are increasingly used in health technology assessment (HTA) processes. However, we know little about their impact on the decision-making process. This study aims to assess the level and the type of impact of patient input to highly specialised technologies (HSTs) and interventional procedures (IPs) guidance at the National Institute for Health and Care Excellence (NICE). METHODS: A questionnaire was developed to capture quantitative and qualitative data on the amount and type of impact of patient input into NICE HTAs. It was completed by committee members of the guidance-producing programs after a discussion of the considered topics. The data were analyzed by topic and overall, for each program, and compared across programs. RESULTS: Patient input was assessed on ten pieces of HST guidance published between January 2015 and November 2019, and on twenty-six pieces of IP guidance scoped between February 2016 and October 2018. A total of 96 responses were collected for HST and 440 for IP. The level of impact of patient input was higher for HST than for IP. For HST, no respondents stated that it had no impact, whereas in IP, 35 percent of respondents did. The most common types of impact found for HST and IP were that it helped interpret the other evidence and that it provided new evidence. CONCLUSIONS: The impact of patient input is not necessarily explicit in changing recommendations, but it provides context, reassurance, and new information to the committee for the decision-making process in HTAs.

Evaluation of conventional troponin I testing for the detection of myocardial dysfunction in children.

Objectives: Troponin is a marker of myocardial injury but is not well studied in children. Our primary objective was to ascertain the sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of conventional troponin I for the detection of acute myocardial dysfunction in previously healthy children. Our secondary objective was to identify clinical predictors of myocardial dysfunction in the setting of elevated troponin. Study Design: This was a retrospective chart review in a single, paediatric, tertiary care centre of troponin tests performed in all admitted children over a 4-year period. Demographics, symptoms, signs, chest x-ray, ECG, and echocardiogram abnormalities were documented. Myocardial dysfunction was presumed to be absent when the patient had a normal cardiac assessment, with or without echocardiography, and did not re-present. Results: From January 2014 through December 2017, 566 patients had troponin tested as a screen for myocardial injury. Troponin was positive in 38 of 566 cases (6.7%). Myocardial dysfunction was detected in 9 of 566 cases (1.6%). Troponin was elevated in six of nine cases of myocardial dysfunction. The sensitivity of conventional troponin I for detecting acute myocardial dysfunction was 66% (95% confidence interval [CI] 30 to 93%). The specificity was 94% (95% CI 92 to 96%). PPV was 16% (95% CI 6 to 31%) and NPV 99% (95% CI 98 to 100%). An abnormal ECG was more prevalent in patients with a true positive versus a false-positive troponin result (P=0.03). Conclusion: Troponin testing identified few cases of myocardial dysfunction. We found the test to have only 66% sensitivity. Troponin testing as a screen for myocardial injury in children has limited utility.

Ambulatory blood pressure and carotid intima media thickness in children with type 1 diabetes.

BACKGROUND/OBJECTIVE: Blood pressure abnormalities may play an important role in macrovascular damage in type 1 diabetes. Little is known about blood pressure abnormalities and macrovascular damage in children with type 1 diabetes. METHODS: Children with type 1 diabetes (n = 57) for a short (3 months-2 years; n = 24) or long duration (≥5 years; n = 33) and a group of control children without diabetes (n = 29) completed 24-h ambulatory blood pressure monitoring (ABPM). Carotid intima media thickness (cIMT), a subclinical indicator of atherosclerosis, was assessed by carotid ultrasound. RESULTS: ABPM abnormalities were more prevalent (57% vs 24%, respectively), and daytime, nighttime and 24-h systolic, diastolic, and mean arterial blood pressure indices were higher in children with type 1 diabetes compared to control children. The odds estimate of an ABPM abnormality was 6.68 (95% confidence interval: 1.95, 22.9; P = .003) in children with type 1 diabetes compared to controls after adjusting for age, sex, and BMI standardized for age and sex (zBMI). An interaction between ABPM and zBMI on cIMT was observed. In children with type 1 diabetes and ABPM abnormalities, every 1 SD increase in zBMI was associated with a 0.030 mm increase in cIMT (95% confidence interval: 0.002, 0.041; P = .031). This was not observed in control children with ABPM abnormalities or in children with normal ABPM, regardless of type 1 diabetes status. CONCLUSIONS: Children with type 1 diabetes have a high prevalence of ABPM abnormalities independent of disease duration and this is related to early indicators of cardiovascular damage.

Students as catalysts for curricular innovation: A change management framework.

Introduction: The role of medical students in catalyzing and leading curricular change in US medical schools is not well described. Here, American Medical Association student and physician leaders in the Accelerating Change in Medical Education initiative use qualitative methods to better define student leadership in curricular change.Methods: The authors developed case studies describing student leadership in curricular change efforts. Case studies were presented at a national medical education workshop; participants provided worksheet reflections and were surveyed, and responses were transcribed. Kotter's change management framework was used to categorize reported student roles in curricular change. Thematic analysis was used to identify barriers to student engagement and activators to overcome these barriers.Results: Student roles spanned all eight steps of Kotter's change management framework. Barriers to student engagement were related to faculty (e.g. view student roles narrowly), students (e.g. fear change or expect faculty-led curricula), or both (e.g. lack leadership training). Activators were: (1) recruiting collaborative faculty, staff, and students; (2) broadening student leadership roles; (3) empowering student leaders; and (4) recognizing student successes.Conclusions: By applying these activators, medical schools can build robust student-faculty partnerships that maximize collaboration, moving students beyond passive educational consumption to change agency and curricular co-creation.

The relative incidence of cardiogenic and septic shock in neonates.

OBJECTIVE: To evaluate the relative incidence of cardiogenic and septic shock in term neonates and identify findings that help differentiate the two entities. STUDY DESIGN: We conducted a retrospective chart review of term neonates presenting to British Columbia Children's Hospital (BCCH) with decompensated shock of an undiagnosed etiology between January 1, 2008 and January 1, 2013. Charts were reviewed to determine the underlying diagnoses of all neonates meeting our inclusion criteria. Patients were categorized as having septic, cardiogenic, or other etiologies of shock. We then evaluated potential demographic, clinical, and biochemical parameters that could help differentiate between septic and cardiogenic shock. RESULTS: Cardiogenic shock was more common than septic shock (relative risk=1.53). A history of cyanosis was suggestive of cardiogenic shock (positive likelihood ratio, LR+=3.2 and negative likelihood ratio, LR-=0.4). Presence of a murmur or gallop (LR+=5.4, LR-=0.3), or decreased femoral pulses (LR+=5.1, LR-=0.5) on physical exam were also suggestive of cardiogenic shock as was cardiomegaly on chest x-ray (LR+=4.9, LR-=0.5). Notably, temperature instability (LR+=0.7, LR-=1.8) and white blood cell count elevation or depression (LR+=0.8, LR-=1.1) were all poor predictors of septic shock. CONCLUSION: Cardiogenic shock is a more common cause of decompensated shock than septic shock. A history of cyanosis, murmur or gallop, or decreased femoral pulses on exam and cardiomegaly on chest x-ray are useful indicators of cardiogenic shock. In evaluating the neonate with decompensated shock, early consideration for Cardiology consultation and interventions to treat the underlying condition is warranted.

Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research.

Acute aortic dissection (AAD) is a life-threatening condition associated with high morbidity and mortality rates, and it remains a challenge to diagnose and treat. The International Registry of Acute Aortic Dissection was established in 1996 with the mission to raise awareness of this condition and provide insights to guide diagnosis and treatment. Since then, >7300 cases have been included from >51 sites in 12 countries. Although presenting symptoms and physical findings have not changed significantly over this period, the use of computed tomography in the diagnosis has increased, and more patients are managed with interventional procedures: surgery in type A AAD and endovascular therapy in type B AAD; with these changes in care, there has been a significant decrease in overall in-hospital mortality in type A AAD but not in type B AAD. Herein, we summarized the key lessons learned from this international registry of patients with AAD over the past 20 years.

Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades.

BACKGROUND: We aimed to characterize the clinical and pathologic findings of aortic dissection (AD) over a nearly 60-year period. METHODS: The Jesse E. Edwards Registry of Cardiovascular Disease database was queried for cardiac specimens from autopsies with AD as a diagnosis and compared 2 cohorts: early (1956-1992) and current (1993-2015). RESULTS: From 1956 to 2015, 338 cases (166 early, 170 current) with AD were included (mean age: 60; 62% male). The AD was 86% type A and 14% type B. Sixty-two percent of cases were under medical care at time of death (61% early, 62% current, P = not significant). Of those under medical care, 63% were not diagnosed prior to death (64% early, 62% current, P = not significant). Risks for dissection did not differ between time intervals and include left ventricular hypertrophy, suggestive of hypertension (84%), prior cardiovascular surgery (38%), bicuspid valve (14%), and connective tissue disease (9%). An intimal tear was identified in the ascending aorta in the majority (68%), followed by descending (14%), root (9.5%), and arch (7%). Aortic rupture occurred in 58%, most frequently in the ascending aorta (41%). CONCLUSIONS: In a large cardiovascular registry, >60% of cases of AD were not detected clinically and first identified at autopsy. Although diagnostic techniques have significantly improved over the time interval, the percentage of AD discovered at autopsy did not differ from the early to the current era. The most prevalent risk factors for dissection including hypertension and prior cardiovascular surgery remain similar in both time periods. AD death is related to rupture of the aorta in the majority of cases.

Rivaroxaban, a direct Factor Xa inhibitor, versus acetylsalicylic acid as thromboprophylaxis in children post-Fontan procedure: Rationale and design of a prospective, randomized trial (the UNIVERSE study).

BACKGROUND: The Fontan procedure is the final step of the 3-stage palliative procedure commonly performed in children with single ventricle physiology. Thrombosis remains an important complication in children after this procedure. To date, guideline recommendations for the type and duration of thromboprophylaxis after Fontan surgery are mainly based on extrapolation of knowledge gained from adults at risk for thrombosis in other clinical settings. Warfarin is being used off-label, and because of its multiple interactions with other drugs and food, a new alternative is highly desirable. Rivaroxaban, a direct Factor Xa inhibitor with a predictable pharmacokinetic profile, is a candidate to address this medical need. STUDY DESIGN: The UNIVERSE study is a prospective, open-label, active-controlled, multicenter study in children 2 to 8 years of age who have single ventricle physiology and had the Fontan procedure within the 4 months preceding enrollment. This study consists of 2 parts. In Part A, rivaroxaban pharmacokinetics, pharmacodynamics, safety, and tolerability are assessed to validate the pediatric dosing selected. In Part B, safety and efficacy of rivaroxaban versus acetylsalicylic acid are evaluated for thromboprophylaxis in children post-Fontan procedure. Children in each part will receive study drug for 12 months. Part A has been completed with 12 children enrolled. Enrollment into Part B is currently ongoing. CONCLUSIONS: The UNIVERSE study aims to provide dosing, pharmacokinetics/pharmacodynamics, safety, and efficacy information on the use of rivaroxaban, an oral anticoagulant, versus acetylsalicylic acid, an antiplatelet agent, in children with single ventricle physiology after the Fontan procedure.

Use of sildenafil in an infant with persistent pulmonary hypertension secondary to lung and renal hypoplasia - a case report.

BACKGROUND: Premature preterm rupture of membranes (PPROM) is reported to be associated with high rates of neonatal mortality and morbidity. Sildenafil has been used in infants with persistent pulmonary hypertension of newborn (PPHN) due to congenital diaphragmatic hernia (CDH) and bronchopulmonary dysplasia (BPD). Recently, Sildenafil has been evaluated as an alternative or adjunctive pulmonary vasodilator. This case report illustrates the use of early sildenafil for PPHN and right ventricular dysfunction in an unusual setting of lung and renal hypoplasia. CASE PRESENTATION: A male infant was born at 37 weeks with a birth weight of 2840 g. Rupture of membranes developed at approximately 24 weeks of gestational age (GA). Bilateral small kidneys (< 2 standard deviations below average) were detected on ultrasound (US) examination at 30 weeks of gestation. The baby developed pneumothorax and pulmonary hypertensive crisis towards the end of the first day. An echocardiogram showed a dilated right ventricle, moderate right ventricular systolic dysfunction, hypoplastic pulmonary arteries and a large patent ductus arteriosus with bidirectional flow. The patient was sedated, paralyzed, and inhaled nitric oxide was administered to decrease the pulmonary resistance. In anticipation of persistent pulmonary hypertension due to the hypoplastic lungs and small calibre of pulmonary arteries, sildenafil was started on day of life (DOL) 5 at a dosage of 0.25 mg/kg/dose Q8H and gradually increased to 2 mg/kg/dose Q8H on DOL 9. The patient was finally extubated on DOL 7 and weaned off of non-invasive respiratory support on DOL 26. Sildenafil was gradually weaned beginning on DOL 21 and discontinued on DOL 48. Repeat echocardiogram assessment at 3 months showed complete resolution of PHT and right ventricular dilatation. CONCLUSIONS: We describe the early use of sildenafil in treating pulmonary hypertension associated with lung and renal hypoplasia in a non-CDH patient. Following this treatment the patient made a full recovery from right ventricular dysfunction.

Pulmonary artery wall thickness in children with Fontan physiology: an optical coherence tomography case control study.

INTRODUCTION: Failure of the Fontan circulation is not a well-understood clinical phenomena.For some patients, a gradual increase in pulmonary vascular resistance (PVR) and structural changes in the pulmonary artery may be an important causative factor. To further investigate this issue, we employed optical coherence tomography (OCT) to evaluate structural changes within the pulmonary arteries of Fontan patients and compared to those with a normal pulmonary circulation. MATERIALS AND METHODS: Pulmonary artery OCT was performed, without complications, in 12 Fontan and 11 control patients. Wall thickness and wall:vessel cross-sectional area (CSA) ratio were calculated after image acquisition, using digital planimetry. RESULTS: There was no difference in wall thickness between both groups. Median wall thickness for Fontan patients was 0.12 mm (IQR, 0.10-0.14) and for controls was 0.11 mm (IQR, 0.10-0.12; p = 0.62). Wall:vessel CSA ratio for Fontan patients was 0.13 (IQR, 0.12-0.16) and for controls was 0.13 (IQR, 0.11-0.15) (p = 0.73). There was no association between wall thickness and ventricle morphology, age at catheterisation, age at Fontan, years since Fontan completion, pulmonary artery pressure, and PVR. The vessel media was more readily visualised in control patients. DISCUSSION: OCT of the pulmonary arteries in Fontan patients is safe and feasible. Our OCT findings suggest that during childhood, pulmonary artery wall dimensions are normal in Fontan children with reassuring hemodynamics. Further evaluation of Fontan patients with abnormal hemodynamics and serial evaluation into adulthood are required to conclude on the utility of OCT for identifying early pulmonary artery structural changes.

Intimal thickening at coronary bifurcations in pediatric heart transplant recipients.

Heart transplant recipients are at increased risk for atherosclerosis and cardiac allograft vasculopathy, both initially presenting as intimal thickening. We aimed to determine the presence, extent, and anatomical characteristics of intimal thickness at coronary bifurcations in children using OCT. We measured the intimal thickness of coronary arteries in pediatric transplant recipients using OCT during routine cardiac catheterization. Intimal thickening was defined as (i) a percent change in contralateral intimal thickness greater than 50% when comparing the thickness at the bifurcation to the baseline thickness, and (ii) greater than 0.1 mm. We evaluated 153 unique coronary bifurcations in 31 children (58% boys, median 12.7 years). Intimal thickening was almost exclusively observed in the left coronary system (22 of 67 bifurcations) and rare in the right coronary system (2 of 86 bifurcations; P < .001). There was a positive association between the relative size of the side branch and contralateral intimal thickening at coronary bifurcations (P = .009). Intimal thickening at coronary bifurcations is already present in the left coronary system in many pediatric transplant recipients. The correlation between intimal thickening and side branch size suggests that low shear stress and oscillating shear stress may have an important role in the development of intimal thickening at coronary bifurcations.

Assessing the quality of health technology registers for national guidance development.

Background: The National Institute of Health and Care Excellence makes use of registers to collect data for technologies that require more evidence to inform future decision-making. This is particularly so for the Interventional Procedures Programme, which since 2003 has produced guidance for procedures that are typically not well established, meaning that named registers are often recommended for future data collection. Methods: We constructed a questionnaire based on quality standards for recommended registers defined by the Interventional Procedures Programme. All guidance from 2003 to 2016 were reviewed to identify recommended registers and compile a list of corresponding registries. We made a maximum of four attempts to contact each registry. Each register was scored on seven quality standards: accessibility, responsiveness, data publication, data coverage, data validity, independent oversight and data protection, with a maximum of 14 points. Results: We obtained responses from 17 out of 24 eligible registries, a response rate of 70.8%. The mean total score was 8.5 (standard deviation 2.9, range 4-14). Overall, the quality of recommended registers was disappointing, with a split between large registries that scored highly across all standards and smaller registries that scored poorly. Conclusions: This the first study to our knowledge to assess the quality of registers recommended by health technology assessment agencies. Only a limited number of registers were mature enough to deliver evidence of sufficiently high quality to inform funding decisions. A standardised quality assessment tool is needed to evaluate registers before their recommendation for observational data gathering by decision-making bodies.

Death and Cardiac Arrest in U.S. Triathlon Participants, 1985 to 2016: A Case Series.

BACKGROUND: Reports of race-related triathlon fatalities have raised questions regarding athlete safety. OBJECTIVE: To describe death and cardiac arrest among triathlon participants. DESIGN: Case series. SETTING: United States. PARTICIPANTS: Participants in U.S. triathlon races from 1985 to 2016. MEASUREMENTS: Data on deaths and cardiac arrests were assembled from such sources as the U.S. National Registry of Sudden Death in Athletes (which uses news media, Internet searches, LexisNexis archival databases, and news clipping services) and USA Triathlon (USAT) records. Incidence of death or cardiac arrest in USAT-sanctioned races from 2006 to 2016 was calculated. RESULTS: A total of 135 sudden deaths, resuscitated cardiac arrests, and trauma-related deaths were compiled; mean (±SE) age of victims was 46.7 ± 12.4 years, and 85% were male. Most sudden deaths and cardiac arrests occurred in the swim segment (n = 90); the others occurred during bicycling (n = 7), running (n = 15), and postrace recovery (n = 8). Fifteen trauma-related deaths occurred during the bike segment. Incidence of death or cardiac arrest among USAT participants (n = 4 776 443) was 1.74 per 100 000 (2.40 in men and 0.74 in women per 100 000; P < 0.001). In men, risk increased substantially with age and was much greater for those aged 60 years and older (18.6 per 100 000 participants). Death or cardiac arrest risk was similar for short, intermediate, and long races (1.61 vs. 1.41 vs. 1.92 per 100 000 participants). At autopsy, 27 of 61 decedents (44%) had clinically relevant cardiovascular abnormalities, most frequently atherosclerotic coronary disease or cardiomyopathy. LIMITATIONS: Case identification may be incomplete and may underestimate events, particularly in the early study period. In addition, prerace medical history is unknown in most cases. CONCLUSION: Deaths and cardiac arrests during the triathlon are not rare; most have occurred in middle-aged and older men. Most sudden deaths in triathletes happened during the swim segment, and clinically silent cardiovascular disease was present in an unexpected proportion of decedents. PRIMARY FUNDING SOURCE: Minneapolis Heart Institute Foundation.