Rerouting anomalous coronary artery origin in aortopulmonary window in newborn.

Coronary artery anomalies may accompany the aortopulmonary window and, if not noticed, may cause catastrophic consequences. The repair of the aortopulmonary window is quite straightforward; however, establishing a normal coronary pattern may challenge the repair. When the anomalous origin of the coronary artery is on the defect rim, right at the location where sutures are to be placed, it may interfere with proper suture placement. A technique to overcome such a technical obstacle and reroute the anomalous right coronary in such cases is described.

Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension: extended study results.

BACKGROUND: Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term study. AIM: We have recently reported that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension in a 24-week prospective study and well tolerated without adverse events. We now aimed to evaluate clinical efficacy, safety of switch in a larger patient population, in a 24-month prospective study. METHODS: This is a single-institution, 24-month prospective study. Patients ≥12 years with idiopathic/heritable, pulmonary arterial hypertension, or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan treatment were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors/inhaled prostanoids was allowed. Outcome measures included change from baseline to 24 months, in the 6-minute walk distance, functional class, oxygen saturation at rest/after walk distance test, and natriuretic peptide levels. Safety end points included adverse events, laboratory abnormalities. RESULTS: Twenty-seven patients (19 adults/8 children, mean age: 21.1 ± 6.3 years (12-36), weight: 53.1 ± 15.7 kgs (26-87)) were included. Mean duration of macitentan treatment: 22.3 ± 3.9 months (9-24). Six-minute walk distance significantly improved from baseline (mean: 458 ± 79 m (300-620)) at 6 months (mean: 501 ± 73 m (325-616) + 43 m) (p < 0.05), at 12 months (mean: 514 ± 82 m (330-626) + 56 m) (p < 0.05), and at 24 months (mean: 532 ± 85 m (330-682) + 74 m) (p < 0.05). We observed a significant improvement during the first 6 months but no incremental improvement after 6 months (p > 0.05). Macitentan did not significantly change functional class, oxygen saturation, and natriuretic levels (p > 0.05). None of the patients had anaemia, hepatotoxicity, and peripheral edema. CONCLUSIONS: Our study is the first study which showed that switch from bosentan to macitentan improved exercise capacity in children and young adults with pulmonary arterial hypertension significantly in the first 6 months and compared to baseline in 24 months and well tolerated without adverse events.

Palivizumab compliance in congenital heart disease patients: factors related to compliance and altered lower respiratory tract infection viruses after palivizumab prophylaxis.

BACKGROUND: Lower respiratory tract infections caused by respiratory syncytial virus can be severe during infancy, which requires admission to the hospital. These infections may be more severe especially in patients with congenital heart disease. Passive immunisation with palivizumab, a monoclonal antibody, is recommended in high-risk infants. We tried to determine the compliance rates, factors affecting compliance, and also other microorganisms responsible for lower respiratory tract infections after palivizumab prophylaxis in these patients. METHODS: We evaluated patients' compliance to prophylaxis with palivizumab in two consecutive respiratory syncytial virus seasons from pharmacy records. We also investigated factors affecting compliance and the frequency of hospitalisations for lower respiratory tract infections. We investigated the causative microorganisms detected in hospitalised patients. RESULTS: In this study, 86.7% of the desired number of injections was achieved in 176 patients in two seasons. Out of these, 117 patients (66.4%) received all the doses they were prescribed. Although not statistically significant, compliance to prophylaxis was higher in male patients, cyanotic patients, those who started under 1 year old, and who lived in the city centre. Human metapneumovirus, parainfluenza type 3, and bocavirus were detected in the hospitalised patients. CONCLUSION: Patients with congenital heart disease can survive the period of infancy with less problem by making palivizumab prophylaxis more effective, and awareness about non- respiratory syncytial virus factors may be a guide for the development of new treatments.

Transcatheter closure of the patent foramen ovale in children: intermediate-term follow-up results.

The patent foramen ovale is almost a normal anatomical hole between the atria with ~30% incidence in the general population. It has been suggested that the patent foramen ovale is the cause of some neurological events, which is explained by paradoxical embolism. Transcatheter closure of the patent foramen ovale is a common procedure in adult patients with cerebral ischaemic events, but there are limited data investigating the results in children. Between January, 2005 and February, 2014, 17 patients' patent foramen ovales were closed by the transcatheter approach in our department. The indications for closure were transient ischaemic attack in 10 patients, stroke in four patients, and migraine in three patients. The mean age and mean weight at the time of the procedure were 11.1±3.7 years and 42.1±15.4 kg, respectively. We asked our patients whether their previous ailments continued. All patients responded to the study survey. In 15 patients, ailments did not continue after patent foramen ovale closure and they significantly decreased in two of them. We suggest that under the right conditions device closure of the patent foramen ovale is a safe solution for these cryptogenic ischaemic events and migraine.

Tularemia in children, Turkey, September 2009-November 2012.

Tularemia, a zoonotic disease caused by Francisella tularensis, is found throughout most of the Northern Hemisphere. It is not well known and is often misdiagnosed in children. Our aim with this study was to evaluate the diagnosis, treatment, and prognosis for 100 children with tularemia in Turkey. The mean patient age was 10.1 ± 3.5 years (range 3-18 years), and most (63%) patients were male. The most common physical signs and laboratory findings were cervical lymphadenopathy (92%) and elevated erythrocyte sedimentation rate (89%). Treatment response was higher and rate of relapse lower for children 5-10 years of age than for those in other age groups. Associated with treatment failure were female sex, treatment delay of ≥16 days, and use of doxycycline. Tularemia is endemic to Turkey, and the number of cases has been increasing among children as well as adults.

Transjugular approach for radiofrequency ablation of permanent junctional reciprocal tachycardia in a newborn with bilateral femoral vein thrombosis.

Although radiofrequency ablation is the first line therapy in some children with supraventricular tachycardia, its application in small children is still limited. Herein, we presented a premature newborn diagnosed as multidrug-resistant permanent junctional reciprocal tachycardia, and treated by radiofrequency ablation via the jugular vein approach because of bilateral femoral vein thrombosis. We think that when there is limited vascular access, the transjugular route for radiofrequency ablation might be considered as an alternative treatment in newborns with multidrug-resistant supraventricular tachycardia.

Risk adjustment and public reporting on home health care.

Risk adjustment is a critical tool in public reporting of quality measures. Its aim is to level the playing field so that providers serving different patients can be meaningfully compared. We used a theory and evidence-based approach to develop risk-adjustment models for the 10 publicly reported home health quality measures and compared their performance with current models developed using a data-driven stepwise approach. Overall, the quality ratings for most agencies were similar regardless of approach. Theory and evidence-based models have the potential to simplify risk adjustment, and thereby improve provider and consumer understanding and confidence in public reporting.

Subcutaneous defibrillator implantation in pediatric patients.

OBJECTIVE: Although sudden cardiac death is rare in children, an intracardiac defibrillator system is indicated in children with various types of cardiomyopathy, primary electrical diseases, and after surgical repair of congenital heart defects. The use of transvenous defibrillator lead systems is limited in pediatric patients because of a small body size and/or limited vascular access. Subcutaneous array leads combined with an abdominally placed generator can enable implantation. METHOD: This is a retrospective study of 13 patients who underwent subcutaneous defibrillator implantation between September 2010 and March 2015. The subcutaneous system was preferred because patients were not amenable to transvenous lead placement. RESULTS: The median patient age was 4.1 years, and the median patient weight was 12.1 kg. Diagnoses of patients were long-QT syndrome in 6, aborted cardiac arrest with left ventricular non-compaction in 3, hypertrophic cardiomyopathy with sustained ventricular tachycardia in 3, and arrythmogenic right ventricular cardiomyopathy in 1. Revision of the subcutaneous lead was required in 5 patients 2-26 months after the implantation. Appropriate shocks were observed in three patients. Inappropriate shock and lead fractures were observed in one patient during the follow-up period. The failure of therapy was observed in one patient. There were no perioperative complications and no early or late deaths. CONCLUSION: Subcutaneous defibrillator systems are safe and effective in pediatric patients when the transvenous method is risky and contraindicated. Because the high growth rate in this population leads to lead failures, a close follow-up of this population is essential.

Trends in medication use and functioning before retirement age: are they linked?

This paper explores the extent to which changes in medication use during the 1990s are linked to improvements in functioning among Americans before they reach retirement age. Using two cross-sections from a survey of Americans ages 51-61, we examined changes between 1992 and 1998 in the prevalence of functional limitations and medication use associated with five chronic conditions: hypertension, diabetes, lung disease, stroke, and arthritis. We found no evidence linking increases in medication use to improvements in functioning. Instead, increases in educational attainment offset the negative effects of increases in obesity and arthritis over this period. Findings underscore the need for caution in projecting forward improvements in old-age functioning when considering the future of Medicare, Medicaid, and other programs that serve the elderly with disabilities.

Another look at aggregate changes in severe cognitive impairment: further investigation into the cumulative effects of three survey design issues.

OBJECTIVES: This study explored whether previously reported declines in severe cognitive impairment were robust to cumulative effects of potentially confounding survey design issues. METHODS: Using the 1993 Asset and Health Dynamics of the Oldest Old study (n = 7,443) and 1998 Health and Retirement Survey (HRS; n = 7,624) the proportion of persons ages 70 and older with severe cognitive impairment was calculated under various assumptions about item nonresponse, differential loss to follow-up, and the size and composition of the nursing home population. Impairment was measured for self-respondents using a modified version of the Telephone Interview Cognitive Screen; for proxy respondents ratings of memory and judgment were used. Chi-square tests were adjusted to account for complex survey designs. RESULTS: Ignoring loss to follow-up, excluding nursing home residents, and assigning a low score to those refusing subscales yielded a statistically significant decline in severe cognitive impairment from 5.8% in 1993 to 3.8% in 1998, or an average annual decline of 6.9%. When cumulative effects of survey design issues were considered and design effects incorporated into statistical tests, statistically significant declines persisted, albeit at a reduced average annual rate, ranging from 2.5% to 6.9% per year. DISCUSSION: Previously reported improvements in severe cognitive impairment appear to be robust to a variety of specifications. Replication with future waves of the HRS and other data is warranted.

Disability and home care dynamics among older unmarried Americans.

OBJECTIVES: We describe how paid and unpaid home care hours received by older unmarried Americans change in response to disability dynamics. We test whether responses to disability declines and improvements are symmetric; that is, we test whether reductions in care hours that are due to disability improvements are of similar magnitude to increases in care hours that are due to disability declines. METHODS: Using a national sample of older unmarried Americans, we examine changes in total hours, paid hours, and unpaid hours of care in response to declines and improvements in personal care (activities of daily living, or ADLs) and routine care (instrumental ADLs, or IADLs) disability. We model changes in the total hours of care received in the past month and jointly model changes in unpaid and paid hours, using Tobit models. RESULTS: Changes in the total hours of care received respond to both increases and decreases in the count of ADL limitations and appear close to symmetric. In contrast, responses to IADL disability dynamics appear to be far less symmetric: Although increases in the count of IADL limitations are met with substantial increases in the total hours of care, decreases are not met with correspondingly large declines in care. The same general pattern is found for unpaid and paid care, and for Medicaid-funded home care. DISCUSSION: Disability and care are not static constructs in old age. Older unmarried persons experience worsening, stabilizing, and recovery of function, and their care hours change accordingly. Evaluations of home care programs must be cognizant of such dynamic realities.

Asking neutral versus leading questions: implications for functional limitation measurement.

UNLABELLED: National surveys of older Americans routinely have included functional limitation items using either a leading approach ("how much difficulty do you have...") or a neutral approach ("do you have any difficulty..."). This article evaluates the performance of scales based on these two approaches. METHODS: Using responses from 595 randomly selected participants to the 1994 Health and Retirement Study, the authors compared prevalences and evaluated scales based on each approach with respect to the extent of missing data, face validity, reliability, predictive validity, convergent validity, and robustness of odds ratios in predictive models. RESULTS: The authors found that leading questions provided higher estimates of functional limitations than neutral questions, but both approaches yielded scales with similar validity and reliability. However, for both approaches, scales incorporating degree of difficulty had better validity and reliability than those based on counts of tasks. All four approaches yielded substantially similar coefficients in a model predicting disability onset. DISCUSSION: The authors conclude that, because they minimize survey time without compromising validity and reliability, items that explicitly capture degree of difficulty by asking "How much difficulty do you have..." may be the optimal approach for survey designers.

Complexity in geriatric home healthcare.

The aging population and the associated rise in the prevalence of chronic conditions suggest that the home health population is increasingly complex and challenging to manage. The purpose of this study was to use national administrative data (Outcome and Assessment Information Set assessments of persons discharged in 2004 and 2005) to examine the clinical complexity of older adults admitted to home healthcare. Our descriptive analyses confirm that multiple chronic conditions and cognitive impairment are common and result in longer lengths of stay. The findings support the need for geriatric home healthcare practices that effectively address multiple morbidities and cognitive function.

Effect of childlessness on nursing home and home health care use.

This study examines the likelihood of nursing home and home health care use for childless older Americans. Four research questions are addressed: (1) Are the childless elderly at a greater risk of nursing home and home health care use? (2) Is it childlessness per se or not having children with particular characteristics that affects the likelihood of using these formal long-term care services? (3) Does having additional children beyond the first one have a significant effect on the use of these services? (4) Are the effects of childlessness different on the likelihood of nursing home and home health care use? Longitudinal data from the first (1993) and second (1995) waves of the Asset and Health Dynamics Among the Oldest Old Survey (AHEAD) and multinomial logistic regression models are used for the analyses. Separate models are developed for women and men, each controlling for a variety of demographic, socioeconomic, and health-related characteristics of sample persons. Findings indicate childlessness as an important risk factor, especially for older women's use of nursing home services. Implications of findings for planning for long-term care needs of the baby boom generation are discussed.

Resolving inconsistencies in trends in old-age disability: report from a technical working group.

In September 2002, a technical working group met to resolve previously published inconsistencies across national surveys in trends in activity limitations among the older population. The 12-person panel prepared estimates from five national data sets and investigated methodological sources of the inconsistencies among the population aged 70 and older from the early 1980s to 2001. Although the evidence was mixed for the 1980s and it is difficult to pinpoint when in the 1990s the decline began, during the mid- and late 1990s, the panel found consistent declines on the order of 1%-2.5% per year for two commonly used measures in the disability literature: difficulty with daily activities and help with daily activities. Mixed evidence was found for a third measure: the use of help or equipment with daily activities. The panel also found agreement across surveys that the proportion of older persons who receive help with bathing has declined at the same time as the proportion who use only equipment (but not personal care) to bathe has increased. In comparing findings across surveys, the panel found that the period, definition of disability, treatment of the institutionalized population, and age standardizing of results were important to consider. The implications of the findings for policy, national survey efforts, and further research are discussed.