Caring for a Child with Hypoplastic Left Heart Syndrome: Parent and Medical Perspective.

This article, co-authored by parents of a child with hypoplastic left heart syndrome (HLHS) and his pediatric cardiology care team, discusses the parents' experience of having a child with HLHS from diagnosis through living day-to-day with the heart condition as well as future directions.

A profile of adult acute admissions to Lentegeur Psychiatric Hospital, South Africa.

BACKGROUND: The Western Cape province has the highest documented lifetime prevalence of common mental disorders in South Africa. To ensure the efficient, equitable and effective distribution of current resources, there is a need to determine the profile of patients requiring psychiatric admission. AIM: To describe patients admitted to the acute adult admissions unit at Lentegeur Hospital. SETTING: Lentegeur Psychiatric Hospital is situated in Mitchells Plain, Cape Town, and serves about 1 million people from nearby urban and rural areas. METHODS: This retrospective study involved an audit of all patients (18-60 years of age) admitted between 01 January 2016 and 30 June 2016. The clinical records of 573 adult patients were examined. RESULTS: The median age of the cohort was 29 years. Most patients (63%) were educated to the secondary level. Only 12% of the patients were employed, and 37% received disability grants. More than 90% of the patients presented with psychotic symptoms. Of these, 28% presented with a first-episode of psychosis. Of all patients, 20% were referred with manic symptoms and 7% with depressive symptoms. Many patients (62%) used substances concurrently in the period leading up to admission. Significantly more males (73%) used substances compared to females (38%). Cannabis was the most widely used substance (51%), followed by methamphetamine (36%). Recent violent behaviour contributed to 37% of the current admissions. A total of 70 patients (13%) tested positive for human immunodeficiency virus (HIV), and 49 (9%) tested positive for syphilis. CONCLUSION: Substance use and a history of violence contributed to admissions in this population.

A profile of adult acute admissions to Lentegeur psychiatric hospital, South Africa

Background: The Western Cape province has the highest documented lifetime prevalence of common mental disorders in South Africa. To ensure the efficient, equitable and effective distribution of current resources, there is a need to determine the profile of patients requiring psychiatric admission.Aim: To describe patients admitted to the acute adult admissions unit at Lentegeur Hospital.Setting: Lentegeur Psychiatric Hospital is situated in Mitchells Plain, Cape Town, and serves about 1 million people from nearby urban and rural areas.Methods: This retrospective study involved an audit of all patients (18­60 years of age) admitted between 01 January 2016 and 30 June 2016. The clinical records of 573 adult patients were examined.Results: The median age of the cohort was 29 years. Most patients (63%) were educated to the secondary level. Only 12% of the patients were employed, and 37% received disability grants. More than 90% of the patients presented with psychotic symptoms. Of these, 28% presented with a first-episode of psychosis. Of all patients, 20% were referred with manic symptoms and 7% with depressive symptoms. Many patients (62%) used substances concurrently in the period leading up to admission. Significantly more males (73%) used substances compared to females (38%). Cannabis was the most widely used substance (51%), followed by methamphetamine (36%). Recent violent behaviour contributed to 37% of the current admissions. A total of 70 patients (13%) tested positive for human immunodeficiency virus (HIV), and 49 (9%) tested positive for syphilis.Conclusion: Substance use and a history of violence contributed to admissions in this population

Interstage outcomes in single ventricle patients undergoing hybrid stage 1 palliation.

OBJECTIVE: Interstage readmissions are common in infants with single ventricle congenital heart disease undergoing staged surgical palliation. We retrospectively examined readmissions during the interstage period. DESIGN: Retrospective analysis. SETTING: The Heart Center at Nationwide Children's Hospital, Columbus, Ohio. PATIENTS: Newborns undergoing hybrid stage 1 palliation from January 2012 to December 2016 who survived to hospital discharge and were followed at our institution. INTERVENTIONS: All patients underwent hybrid stage 1 palliation. OUTCOME MEASURES: Outcomes included (1) reason for interstage readmission; (2) feeding modality during interstage period; (3) major interstage adverse events; and (4) interstage mortality. RESULTS: Study group comprised 57 patients. Five patients only admitted once during the interstage period for scheduled cardiac catheterization were included in the no readmission group. Therefore, 43 patients (75%) had a total of 87 interstage readmissions. Fourteen patients had 15 major interstage adverse events accounting for 17% of total readmissions. Stroke (n = 1); sepsis (n = 1); pericardial effusion requiring drainage (n = 1); mesenteric ischemia (n = 1); shock (n = 1); and cardiac catheterization requiring intervention (n = 11)-ductal stent balloon angioplasty (n = 3), enlargement of atrial septal defect/stent placement (n = 3), retrograde aortic arch stenosis (n = 4). Thirty-three readmissions were secondary to gastrointestinal/feeding issues; 15 cyanosis; 15 work of breathing; and 9 asymptomatic patients. Four patients suffered interstage deaths (7%). Five patients (9%) spent >30 days in the hospital during the interstage period. Of the 47 newborns (82%) discharged exclusively orally feeding, 74% remained all orally feeding throughout interstage period. No patient discharged with tube feedings learned to eat during the interstage period. CONCLUSION: Interstage readmissions are common in the hybrid patient population. Seventeen percent were secondary to major adverse events. Interstage mortality was 7%. Future studies to identify interventions aimed at decreasing feeding issues and viral bronchiolitis in this tenuous patient population will hopefully improve quality outcomes, reduce readmissions, and lessen health care costs.

Perception scores of siblings and parents of children with hypoplastic left heart syndrome.

OBJECTIVES: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling's quality of life as well as the caregiver's perception of this effect. STUDY DESIGN: Cross-sectional study using a web-based survey distributed via various listservs targeted towards families of children with hypoplastic left heart syndrome. Employed the Sibling Perception Questionnaire, designed to assess sibling and caregiver perceptions of adjustment to chronic illness. A Negative Adjustment Composite Score was calculated for each respondent, with higher values representing more negative adjustment. RESULTS: Thirty-five caregivers responded. Majority of caregivers were female (74%), white (86%) and college educated (54%). Thirty-two siblings participated, ranging in age from 7 to 30 years of age (12.5 ± 6.3). Most children with hypoplastic left heart syndrome (73%) had undergone the third stage of palliation. Forty-two caregiver-sibling pairs were examined. Caregiver Negative Adjustment Composite Scores were significantly higher than sibling scores, with caregivers reporting more adjustment problems (2.4 ± 0.4) than siblings (2.3 ± 0.3, P < .05). Sibling age was correlated with worse caregiver and sibling scores (r 0.35, P < .05). CONCLUSIONS: Caregivers of children with hypoplastic left heart syndrome perceive their siblings as struggling more than the children self-report. Siblings tend to report worse adjustment as they get older. These data suggest that programs should include support for the entire family through all ages to optimize quality of life.

Differences in midterm outcomes in infants with hypoplastic left heart syndrome diagnosed with necrotizing enterocolitis: NPCQIC database analysis.

INTRODUCTION: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to HLHS without NEC (HLHS-nNEC) during the interstage period. METHODS: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site, type of surgical repair, and gestational age ±1 week was performed. Baseline demographics as well as outcome data were recorded. The t tests or chi-square tests were performed as appropriate. RESULTS: There were 57 neonates in the HLHS-NEC (14 Norwood-BT, 37 Norwood-RVPA, and 6 hybrid) and 171 neonates in the HLHS-nNEC group. There were significant differences between the HLHS-NEC versus HLHS-nNEC for presence of atrioventricular valve regurgitation (7% vs 2%), use of extracorporeal membrane oxygenation (11% vs 2%), hospital stay (60.4 ± 30.0 vs 36.3 ± 33.6 days), Z-score weight at discharge (-2.1 vs -1.6), incidence of no oral intake (33% vs 14%), and use of formula only nutrition at discharge (61% vs 29%), respectively. There were no significant differences between groups in readmission rates due to adverse gastrointestinal events, use of gastrointestinal medications, interstage deaths, or Z-score weight at time of second surgery. HLHS-NEC continued to be more likely to be entirely tube dependent for enteral intake at time prior to the second procedure (39% vs 15%). CONCLUSIONS: Despite similar baseline characteristics, HLHS-NEC infants had significant differences in hospital course compared with HLHS-nNEC neonates. In addition, HLHS-NEC infants were less likely to be fed orally during the entire interstage period. Future studies are needed minimize NEC in this high risk population to possibly improve oral feeds.

Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses.

OBJECTIVE: Previous studies suggest that children with congenital cardiac diagnoses report lower quality of life when compared with healthy norms. A few studies have evaluated quality of life specifically in children born with hypoplastic left heart syndrome, a condition requiring several surgeries before age three. The aim of this study was to use an empirically validated and standardised measure - the Pediatric Quality of Life Inventory - to evaluate quality of life in children with hypoplastic left heart syndrome and compare the findings with similar, medically complicated samples. METHODS: The parent-report Pediatric Quality of Life Inventory was administered, and demographic information was collected through an internet portal. A total of 121 caregivers of children with hypoplastic left heart syndrome responded. The sample included children aged 2-18 years (M=10.81 years). Independent sample t-tests were used to compare our sample with published norms of healthy children and children with acute or chronic illnesses. RESULTS: Children with hypoplastic left heart syndrome were rated as having significantly lower overall quality-of-life scores (M=59.69) compared with published norms of children without medical diagnoses (M=83.00) and those with acute (M=78.70) or chronic (M=77.19) illnesses (p<0.001). Children with hypoplastic left heart syndrome complicated by a stroke or seizure (15%) reported the lowest quality of life. The results held for all subscales (p<0.001). CONCLUSIONS: Children with hypoplastic left heart syndrome appear to be a significantly vulnerable population with difficulties in functioning across psychosocial domains and across the age span. Further research is required to facilitate early identification of the need for resources for these children and families, especially for children who experience additional medical complications.

Interstage Survival for Patients with Hypoplastic Left Heart Syndrome After ECMO.

There is a reported 5-20 % incidence of extracorporeal membrane oxygenation (ECMO) following stage I palliation for hypoplastic left heart syndrome (HLHS). This study compares the interstage mortality of HLHS patients supported with ECMO (HLHS-ECMO) to those who were not supported with ECMO (HLHS-nECMO) using the National Pediatric Cardiology Quality Improvement Initiative database. Patients with HLHS who survived to hospital discharge after stage I palliation were analyzed. HLHS-ECMO patients were compared to HLHS-non-ECMO patients with respect to demographics, surgical variables, and interstage survival. A total of 931 patients were identified in the database. Sixty-six (7.1 %) patients were supported with ECMO during their stage I palliation admission. There were no statistically significant differences between the groups with respect to demographics or anatomic subtype. HLHS-ECMO patients were more likely to have a preoperative risk factor identified (62 vs. 48 %, p = 0.03) or require ECMO prior to stage I palliation (3 vs. 0.5 %, p = 0.03). HLHS-ECMO patients had a significantly higher incidence of death or transplant versus the HLHS-nECMO group (18 vs. 9 %, p = 0.03). Despite survival to discharge, patients with HLHS requiring ECMO after their palliation continue to have an increased risk of death/cardiac transplant versus patients that do not require ECMO. ECMO use is likely a marker for a high-risk patient group. These patients may benefit from closer follow-up during the interstage period.

Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome.

OBJECTIVES: Caring for children with congenital heart disease places significant stress on caregivers. Minimal data exist evaluating stress levels in caregivers of children with hypoplastic left heart syndrome (HLHS). The goal of this study was to obtain baseline stress scores for caregivers of children with HLHS and determine if associations exist between scores and specific caregiver factors. STUDY DESIGN: A cross-sectional study using a web-based survey targeted towards caregivers of children with HLHS was performed. Baseline demographics of the caregiver and child with HLHS were obtained. Caregivers completed three validated questionnaires including the Pediatric Quality of Life Inventory (PedsQL), Parenting Stress Index- Short Form (PSI-SF) and the Pediatric Inventory for Parents (PIP). RESULTS: Four hundred fifty-nine caregivers completed at least one questionnaire. PSI-SF total score was 80.5 ± 23.1 (> 86 = significant stress), PIP frequency total score was 119.0 ± 37.2, and PIP difficulty total score was 118.1 ± 35.7. Lower quality of life was significantly correlated with higher scores on the PSI-SF (r = -0.6), the presence of a developmental issue in the child (r = 0.3) as well as higher scores on the PIP frequency (r = -0.5) and difficulty scales (r = 0.4). Other demographic values for the caregiver and child did not significantly correlate with PSI or PIP total scores. CONCLUSIONS: Anxiety/stress scores of caregivers with children with HLHS are correlated with how well the child is perceived to be doing physically and developmentally by the caregivers. Caregivers with physical and/or developmental concerns may need added psychosocial support.

Caregiver anxiety due to interstage feeding concerns.

INTRODUCTION: Improved weight gain during the interstage (IS) period has been shown to improve overall outcomes in patients with single ventricle physiology (SVP). This emphasis on nutrition may have untoward effects, such as increasing anxiety/stress levels in caregivers, who are already known to be at risk for increased anxiety/stress levels. The goal of this study was to evaluate anxiety/stress levels of caregivers as it pertains to feeding during the IS period and to determine if certain characteristics were associated with higher anxiety/stress scores. METHODS: Caregivers of children with SVP who completed the IS period, defined as the time between the first and second cardiac surgeries, were recruited. Baseline demographics were obtained. Anxiety/stress levels were measured via eight questions using a 0- to 10-point scale. Correlations were performed between demographic variables and anxiety/stress level scores. RESULTS: Fifty-six surveys were completed (39 males, 27 females) on 43 children. Fourteen children required tube feeds during the IS period. There were significant correlations between anxiety/stress scores and caregiver's gender, caregiver's age, caregiver's level of education, percent of time a caregiver spent feeding the child, if caregivers were taking medications for anxiety, and if the child was seen in the emergency room during the IS period. There were no correlation of anxiety/stress scores with caregiver's race, child's underlying cardiac diagnosis, age of child, route of feeding during the IS period, birth order of the child or number of children in the family, relationship status, or distance from the hospital. CONCLUSION: In general, caregivers of children with SVP experience anxiety/stress during the IS period specifically due to feeding concerns. Certain intrinsic and extrinsic characteristics were associated with higher anxiety/stress levels. Future studies are needed to determine how to minimize anxiety/stress levels during this stressful time period.

Neuropsychological profiles of victims of financial elder exploitation at the los angeles county elder abuse forensic center.

The current article examines neuropsychological correlates of financial elder exploitation in a sample of older adults who have been documented victims of financial elder exploitation. The purpose of this exploratory study was twofold. First, a subsample of the referrals at the Los Angeles County Elder Abuse Forensic Center (LACEAFC) was compared to community dwelling adults in terms of the specific cognitive domains linked to financial capacity including memory, calculation, and executive functioning. Next, the correlation between presence of neuropsychological data and the likelihood of filing a case with the LA County's District Attorney office was examined. Twenty-seven LACEAFC cases and 32 controls were assessed. Overall, the forensic center group performed worse than a community-based age-matched control group on the MMSE, calculation, and executive functioning (ps < .01). The presence of neuropsychological data was significantly correlated to an increased likelihood of a case being filed.

Interstage weight gain for patients with hypoplastic left heart syndrome undergoing the hybrid procedure.

BACKGROUND: Weight gain during the interstage (IS) period for hypoplastic left heart (HLHS) patients has been associated with improved outcomes. IS home monitoring has been shown to improve mortality. No data exist on IS weight gain and home monitoring effects on weight gain for HLHS patients undergoing the hybrid procedure. OBJECTIVE: Goal of this study was to describe the weight gain of patients with HLHS undergoing the hybrid procedure during the IS period, to determine if weight parameters were associated with mortality, and to determine if home monitoring improved weight gain. METHODS: Retrospective review was performed. Patients were included if they had the diagnosis of HLHS and underwent hybrid procedure. Baseline demographics, surgical dates, and all IS weights were recorded. RESULTS: Forty-four patients met inclusion criteria, 24 patients had IS monitoring. Time period evaluated was from April 2006 to June 2011. Mean birth weight of the total population was 3.13 ± 0.61 kg, age at hybrid was 5.84 ± 4.10 days, weight z-score at hybrid discharge was -1.66 ± 1.01, age at pre-Stage II was 6.12 ± 1.37 months, IS weight gain was 16.85 ± 5.94 g/day, and weight z-score pre-Stage II was -2.25 ± 1.28. Monitored patients had significantly higher weight z-score pre-Stage II (-1.67 ± 0.98 vs. -2.82 ± 1.28) and lower change in weight z-score (-0.26 ± 0.97 vs. -1.24 ± 1.06). Eight patients died IS. There was a significant difference in weight gain per day in those that survived the IS period (17.87 ± 4.75 g/day vs. 12.28 ± 8.65 g/day). There were no significant differences in weight characteristics in patients that survived the Stage II procedure (n = 28) vs. those that did not (n = 7). CONCLUSION: Home monitoring improved IS weight gain in patients undergoing the hybrid procedure. Decreased weight gain per day was associated with IS mortality.

Effects of cocoa flavanols on risk factors for cardiovascular disease.

Epidemiologic investigations support the hypothesis that regular consumption of flavonoid-containing foods can reduce the risk of cardiovascular diseases (CVD). While flavonoids are ubiquitous in plants, cocoa can be particularly rich in a sub-class of flavonoids known as flavanols. A number of human dietary intervention trials with flavanol-containing cocoa products have demonstrated improvements in endothelial and platelet function, as well as blood pressure. These studies provide direct evidence for the potential cardiovascular benefits of flavanol-containing foods and help to substantiate the epidemiological data. In this review, results from selective published trials with cocoa and chocolate focused on risk for CVD will be discussed along with a study we recently completed evaluating the effects of the daily consumption of flavanol-containing dark chocolate (CocoaVia?) with and without plant sterol esters on CVD markers in a normotensive population with mild hypercholesterolemia. In this study, the daily consumption of flavanol-containing dark chocolate was associated with a significant mean reduction of 5.8 mmHg in systolic blood pressure. Together the results of these human dietary intervention trials provide scientific evidence of the vascular effects of cocoa flavanols and suggest that the regular consumption of cocoa products containing flavanols may reduce risk of CVD.

Daily consumption of a dark chocolate containing flavanols and added sterol esters affects cardiovascular risk factors in a normotensive population with elevated cholesterol.

Previous studies with plant sterols (PS) and cocoa flavanols (CF) provide support for their dietary use in maintaining cardiovascular health. This double-blind, placebo-controlled, cross-over study evaluated the efficacy of daily consumption of a cocoa flavanol-containing dark chocolate bar with added PS on serum lipids, blood pressure, and other circulating cardiovascular health markers in a population with elevated serum cholesterol. We recruited 49 adults (32 women, 17 men) with serum total cholesterol concentrations of 5.20-7.28 mmol/L and blood pressure of < or = 159/99 mm Hg. Following a 2-wk lead-in utilizing the AHA style diet, participants were randomized into 2 groups and instructed to consume 2 cocoa flavanol-containing dark chocolate bars per day with (1.1 g sterol esters per bar) or without PS. Each 419-kJ bar was nutrient-matched and contained approximately 180 mg CF. Participants consumed 1 bar 2 times per day for 4 wk then switched to the other bar for an additional 4 wk. Serum lipids and other cardiovascular markers were measured at baseline and after 4 and 8 wk. Blood pressure was measured every 2 wk. Regular consumption of the PS-containing chocolate bar resulted in reductions of 2.0 and 5.3% in serum total and LDL cholesterol (P < 0.05), respectively. Consumption of CF also reduced systolic blood pressure at 8 wk (-5.8 mm Hg; P < 0.05). Results indicate that regular consumption of chocolate bars containing PS and CF as part of a low-fat diet may support cardiovascular health by lowering cholesterol and improving blood pressure.

Importance of pretest probability score and D-dimer assay before sonography for lower limb deep venous thrombosis.

OBJECTIVE: The purpose of our study was to develop and validate a clinical score (the Hamilton score) for the assessment of lower limb deep venous thrombosis (DVT) and to determine the usefulness of this score and a D-dimer assay before a complete lower limb sonographic examination. SUBJECTS AND METHODS: Five hundred forty-two consecutive ambulatory patients presenting to the emergency department were prospectively recruited, of whom 16 patients were excluded from the study. Eighteen history and examination variables were collected by the emergency department physicians. The Simplify D-dimer assay and a complete, single lower limb sonographic examination were performed in all patients. All patients with a negative sonographic examination for DVT were followed up for 3 months, and all those with a positive sonographic examination were given anticoagulation therapy. The Hamilton score was developed using the data from the first 214 patients and was prospectively validated in the next 312 patients. RESULTS: The most significant factors associated with a diagnosis of DVT were immobilization of the lower limb, active malignancy, and a strong clinical suspicion of DVT without other diagnostic possibilities by the emergency department physicians. Other factors were bed rest or recent surgery, male sex, calf circumference difference greater than 3 cm, and erythema. The Hamilton score was developed with the following weights: immobilization of the lower limb (2 points), active malignancy (2 points), strong clinical suspicion of DVT without other diagnostic possibilities by the emergency physicians (2 points), bed rest or recent surgery (1 point), male sex (1 point), calf circumference difference greater than 3 cm (1 point), and erythema (1 point). A score of 3 or greater indicates a likely probability for DVT, and a score of 2 or less represents an unlikely probability for DVT. Of the 103 patients with an unlikely probability Hamilton score and a negative D-dimer assay, only one patient had isolated calf DVT. A combined diagnostic strategy of unlikely-probability Hamilton score and a negative D-dimer would have a negative predictive value of 99% (95% confidence interval, 94.7-100%). CONCLUSION: An unlikely-probability Hamilton score and a negative Simplify D-dimer assay effectively exclude lower limb DVT, and a sonographic examination is unnecessary in this group of ambulatory emergency department patients.