Diastolic Blood Pressure Alleles Improve Congenital Heart Defect Repair Outcomes.

BACKGROUND: Congenital heart defects (CHDs) affect 40 000 US births per year, half of which require surgical intervention. Individual differences in surgical outcomes including mortality and complications are not well understood but may be due to genetic variability. We hypothesized that polygenic risk scores (PRSs) for blood pressure in adults are associated with treatments and postsurgical outcomes in children with CHD, as CHD survivors are at higher risk of negative cardiometabolic disease. METHODS: We used imputed genotype data from pediatric participants requiring surgery for CHD (median age at surgery, 201 days; nmax=2498). Base data for the systolic and diastolic blood pressure PRSs (nmax=760 226) came from published genome-wide association study. The blood pressure PRSs were tested for association with postsurgical outcomes. All effects presented are per SD increase in PRS and adjusted for age, sex, body mass index, surgical complexity score, and first 10 principal components of ancestry. RESULTS: A higher diastolic blood pressure PRS was associated with decreased in-hospital mortality risk (odds ratio, 0.57 [0.39-0.82]; P=0.0022). Additional analyses suggest an interaction between diastolic blood pressure PRS and vasopressor dose. Those with a diastolic blood pressure PRS 1 SD above the mean, receiving a vasopressor dose in the top tertile, were estimated to have 52% (32%-66%) lower risk of in-hospital mortality compared with those with a vasopressor dose in the bottom tertile. CONCLUSIONS: These results suggest a genetically determined postsurgical survival advantage for CHD patients with blood pressure increasing alleles. Further study may reveal novel mechanisms contributing to postoperative morbidity and mortality, and this approach may assist in early identification of children at risk for adverse postoperative outcomes.

Comparison of Strain-Encoding and Feature-Tracking Derived Myocardial Deformation Assessment of Left Ventricular Function in a Pediatric and Adult Congenital Heart Disease Cohort.

BACKGROUND: Cardiac magnetic resonance (CMR) strain can be assessed with feature-tracking (FT), which utilizes a post-processing algorithm to quantify myocardial deformation on routine cine images, and strain-encoding magnetic resonance imaging (SENC), which uses parallel magnetization tags combined with out-of-plane phase-encoding gradients to quantify deformation. Assessing agreement is critical to determine whether results can be translated between methods. We compared SENC to FT in the assessment of left ventricle (LV) global longitudinal strain (GLS) and global circumferential strain (GCS) in a cohort of pediatric and adult congenital heart disease (ACHD) patients. METHODS: Pediatric subjects and ACHD patients underwent CMR on 1.5 T Siemens scanners, including balanced steady-state-free precession (bSSFP) cine imaging and SENC acquisitions in apical two and four chamber, left ventricular outflow tract, and short axis views. bSSFP cine imaging FT analysis was completed with Medis QStrain. Myocardial Solutions MyoStrain was used to analyze SENC. Correlation was assessed by Spearman's rank correlation coefficient. Agreement between techniques was assessed with concordance correlation coefficient (CCC) and Bland-Altman. RESULTS: The cohort included 134 patients, 75 with congenital heart disease (56%). The median age was 16.3 years (IQR 13.7, 19.5). Median LV ejection fraction was 57% (IQR 54.4, 61.6). SENC and FT were in poor agreement for GLS (Spearman's ρ = 0.58, p < 0.001; CCC 0.24) and GCS (Spearman's ρ = 0.29, p < 0.001; CCC 0.03). CONCLUSION: There was poor agreement between SENC and FT derived GLS and GCS in a cohort of pediatric and ACHD patients, suggesting that SENC and FT cannot be used interchangeably.

Management of routine postoperative pain for children undergoing cardiac surgery: a Paediatric Acute Care Cardiology Collaborative Clinical Practice Guideline.

BACKGROUND: Pain following surgery for cardiac disease is ubiquitous, and optimal management is important. Despite this, there is large practice variation. To address this, the Paediatric Acute Care Cardiology Collaborative undertook the effort to create this clinical practice guideline. METHODS: A panel of experts consisting of paediatric cardiologists, advanced practice practitioners, pharmacists, a paediatric cardiothoracic surgeon, and a paediatric cardiac anaesthesiologist was convened. The literature was searched for relevant articles and Collaborative sites submitted centre-specific protocols for postoperative pain management. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus. RESULTS: 60 recommendations achieved consensus and are included in this guideline. They address guideline use, pain assessment, general considerations, preoperative considerations, intraoperative considerations, regional anaesthesia, opioids, opioid-sparing, non-opioid medications, non-pharmaceutical pain management, and discharge considerations. CONCLUSIONS: Postoperative pain among children following cardiac surgery is currently an area of significant practice variability despite a large body of literature and the presence of centre-specific protocols. Central to the recommendations included in this guideline is the concept that ideal pain management begins with preoperative counselling and continues through to patient discharge. Overall, the quality of evidence supporting recommendations is low. There is ongoing need for research in this area, particularly in paediatric populations.

Heart Transplantation in Children with Mitochondrial Disease.

OBJECTIVES: To compare the outcomes and comorbidities of children with mitochondrial disease undergoing heart transplantation with children without mitochondrial disease. STUDY DESIGN: Using a unique linkage between the Pediatric Health Information System and Scientific Registry of Transplant Recipients databases, pediatric heart transplantation recipients from 2002 to 2016 with a diagnosis of cardiomyopathy were included. Post heart transplantation survival and morbidities were compared between patients with and without mitochondrial disease. RESULTS: A total of 1330 patients were included, including 47 (3.5%) with mitochondrial disease. Survival after heart transplantation was similar between patients with and without mitochondrial disease over a median follow-up of 4 years. Patients with mitochondrial disease were more likely to have a stroke after heart transplantation (11% vs 3%; P = .009), require a longer duration of mechanical ventilation after heart transplantation (3 days vs 1 day; P < .001), and have a longer intensive care unit stay after heart transplantation (10 vs 6 days; P = .007). The absence of a hospital readmission within the first post-transplant year was similar among patients with and without mitochondrial disease (61.7% vs 51%; P = .14). However, patients with mitochondrial disease who were readmitted demonstrated a longer length of stay compared with those without (median, 14 days vs 8 days; P = .03). CONCLUSIONS: Patients with mitochondrial disease can successfully undergo heart transplantation with survival comparable with patients without mitochondrial disease. Patients with mitochondrial disease have greater risk for post-heart transplantation morbidities including stroke, prolonged mechanical ventilation, and longer intensive care unit and readmission length of stay. These results suggest that the presence of mitochondrial disease should not be an absolute contraindication to heart transplantation in the appropriate clinical setting.

Rehospitalization Following Pediatric Heart Transplantation: Incidence, Indications, and Risk Factors.

Rehospitalization following pediatric heart transplantation is common. However, existing data remain somewhat limited. Using a novel linkage between administrative and clinical databases, pediatric heart transplant (HT) recipients from 29 centers who survived to discharge were retrospectively reviewed to determine the frequency, timing of, and indication for all-cause rehospitalizations in the year following transplant discharge. Of 2870 pediatric HT recipients, 1835 (63.9%) were rehospitalized in the first year post-discharge (5429 total readmissions). Rehospitalization rates varied significantly across centers (46% to 100%) and were inversely correlated to center transplant volume (r2 0.25, p < 0.01). The median number of rehospitalizations per patient was 2 (IQR 1-4) and the median time to first rehospitalization was 29 days (IQR 9-99 days). Independent risk factors for rehospitalization included younger age at HT (HR 0.99, 95% CI 0.97-0.99), congenital heart disease (HR 1.2, 95% CI 1.1-1.4), listing status 1B at transplant (HR 1.3, 95% CI 1.1-1.5), and post-transplant complications including rejection prior to discharge (HR 1.5 95% CI 1.3-1.8) and chylothorax (HR 1.3, 95% CI 1.0-1.6). Cardiac diagnoses were the most common indication for rehospitalization (n = 1600, 29.5%), followed by infection (n = 1367, 25.2%). These findings may serve to guide the development of interventions aimed at reducing post-HT hospitalizations.

Designing and Conducting Scholarly Quality Improvement: A Practical Guide for Improvers Everywhere.

Successful publication of quality improvement (QI) work is predicated on the use of established QI frameworks and rigorous analytical methods that allow teams to understand the impact of interventions over time. This article is meant to help QI teams disseminate their work more broadly through publication by providing tangible methods that many journals desire in QI articles with specific examples of published works referenced throughout the article. We introduce improvement frameworks that teams should identify early and use as a foundation throughout their projects. We review vital aspects of QI projects, such as team formation, creation of a succinct and clear aim statement, defining primary, process, and balancing measures, as well as QI tools like key driver diagrams, Ishikawa (fishbone) diagrams, and Pareto charts. Finally, we highlight the importance of analyzing data over time to understand the impacts of plan-do-study-act cycles on data. Annotated run charts or, more preferably, annotated statistical process control (or Shewhart) charts are both statistically sound methods to identify significant changes over time. Deliberate planning and execution of QI projects using these concepts will lead to improved chances of QI teams finding success in their project and eventual article acceptance.

Left main coronary artery stent placement in a 7.0 kg infant with Williams Syndrome.

We report the case of a 9-month-old male with Williams syndrome who underwent patch augmentation of supravalvar aortic stenosis and pulmonary artery stenosis, and required emergent drug-eluting left coronary artery stenting on post-operative day 1 for severe left ventricular dysfunction related to myocardial ischemia.

Analysis of the relationship between diet and exercise beliefs and actual behaviors among breast cancer survivors in northwest ohio.

INTRODUCTION: Studies have shown that a diet high in fruit and vegetable intake, as well as a routine including daily exercise or physical activity, can independently affect relapse rates and survivorship in breast cancer patients. Fruits and vegetables contain powerful anti-oxidant molecules, capable of preventing tumor formation and proliferation. Exercise can lower circulating levels of estrogen, the female hormone responsible for tumor proliferation in the estrogen-sensitive form of the disease. The most beneficial results have been shown in women who exercise and consume a diet rich in fruits and vegetables. We studied the attitudes towards and behaviors related to fruit and vegetable intake and exercise in a cohort of breast cancer survivors in northwest Ohio. MATERIALS AND METHODS: Data were gathered from a survey sent out by the Northwest Ohio Branch of the Susan G. Komen For the Cure Foundation. We assessed and evaluated survivors' self-reported beliefs, attitudes, and behaviors regarding exercise and fruit and vegetable intake. RESULTS: Nearly half of the survivors (46.5%) reported being unsure or in disagreement with the statement "Eating at least 5 servings of fruits and/or vegetables per day will reduce the risk of breast cancer recurrence." Only 46.8% of those in agreement with the previous statement actually report eating at least 5 fruits and/or vegetables per day. With respect to exercise, 32.9% reported being unsure or in disagreement with the statement "Engaging in regular physical activity will reduce the risk of breast cancer recurrence." Only 68.5% of those in agreement with the previous statement report any physical activity in the past 30 days. CONCLUSIONS: Many breast cancer survivors do not appear to be aware of the benefits of diet and exercise. Further, a large proportion of those who are aware of the benefits do not adapt a healthy diet and exercise as part of their lifestyle. A majority of these survivors see a primary care physician, which we believe is the best venue to bridge this education gap. It is apparent by the pattern our data shows that more needs to be done to educate breast cancer survivors about the benefits of exercise and fruit and vegetable intake. Steps need to be taken to ensure that those who are educated also remain motivated to engage in a healthy lifestyle with the hopes of avoiding breast cancer recurrence.

Early stage breast cancer and its association with diet and exercise-related perceptions and behaviors to prevent recurrence.

BACKGROUND: The favorable prognosis for early stage breast cancer survivors may be a reason for the minimal research regarding their quality of life. Prior research has observed more long-term weight gain among early stage survivors compared to cancer-free women of a similar age. It would be useful to study survivors' perceptions and reported behaviors regarding diet and exercise to see if there is a correlation with previous studies. METHODS: A sample of 700 breast cancer survivors from Ohio and Michigan was randomly selected from the Northwest Ohio affiliate of the Susan G. Komen For the Cure mailing list and sent a survey for completion. RESULTS: 389 survivors completed the survey and among Stage 1 (50/197 = 25.4%) and Stage 2 survivors (24/105 = 22.9%), a small proportion had a positive correlation between self-reported dietary behaviors and their perceived benefits of eating fruits and vegetables. Similar correlations were observed between their self-reported exercise behaviors and their perceived benefits of exercise (Stage 1: 36/197 = 18.3%, Stage 2: 18/105 = 17.1%). CONCLUSIONS: Regardless of stage, a small proportion of survivors' self-reported dietary and exercise behaviors match their perceived benefits of diet and exercise. Factors such as access, motivation, and lack of co-morbidities among early stage survivors may prevent them from living healthier post-diagnosis. More thorough dietary and clinical measurements will provide greater certainty. Thus, innovative, sustainable programs must be accessible and provide motivation and social support from family, friends, and other survivors to truly improve quality of life.