Evaluation of quality of life and parental attitudes in patients who underwent atrial septal defect closure in childhood.

OBJECTIVE: In this study, we aimed to evaluate quality of life and parental attitudes in children who underwent an atrial septal defect closure procedure with a transcatheter or surgical method in childhood and whether they continued their lives with similar activities to their healthy peers by comparing the two groups. METHODS: Patient forms to define sociodemographic and clinical features, the Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents (KINDL) to measure the quality of life of children, and the Parent-Child Relationship Test (Parental Attitude Research Instrument) to measure parental attitudes were used. RESULTS: The groups were similar in terms of age and sex. The mean quality of life scale scores were high in all groups, and there was no statistically significant difference between the scores. Parents of the patients who underwent closure received higher scores from the demographic attitudes and recognition of quality subdimension compared to the parents of the healthy group. CONCLUSIONS: The quality of life of children with atrial septal defect closure was found to be similar to their healthy peers. Additionally, the effects of surgical or percutaneous closure of atrial septal defect on quality of life are similar. Children with atrial septal defect closure perceive their health status as well as their healthy peers, and this perception does not cause any difference in the attitudes and behaviours of families.

Effect of vitamin D on myocardial remodeling and inflammatory status in children with congenital heart disease.

BACKGROUND: Vitamin D insufficiency is a common public health problem that is often unrecognized in children with congenital heart disease, and is not generally evaluated at congenital heart disease (CHD) follow-up. Recent studies have suggested that inadequate vitamin D status may have an adverse effect on cardiovascular health. This study investigates the relationship between vitamin D levels and hemodynamic parameters in children with CHD. METHODS: Included in the study 40 patients (25 females, 15 males) with CHD, who were evaluated for Ross heart failure score, vitamin D, parathyroid hormone (PTH), calcium, phosphorus, alkaline phosphatase (ALP), whole blood count (WBC) and echocardiographic measurements, and all measurements were repeated in the third month of the therapy. RESULTS: The mean vitamin D level was 16.4 ± 6.6 ug/L before and 27.5 ± 9.9 µg/L in the third month of therapy, while the mean PTH level was 53.3 ± 34.9 pg/mL before and 43.8 ± 21.4 pg/mL in the third month of therapy. The mean WBC was 8084 ± 2324/µL before and 7378±1893/µL in the third month of the therapy, and the mean platelet (PLT) count was 280,897 ± 80,119/µL before and 307,179 ± 60,202/µL in the third month of the therapy. The mean ejection fraction (EF) was 64% ± 7.2% before and 66.7% ± 6.2% in the third month of therapy, while the right ventricle (RV) myocardial performance index (MPI) was 32.1% ± 6.7% before and 28.9% ± 6.5% in the third month of the therapy. IL10 level was increased in four patients in the third month of therapy. A statistically significant decrease in PTH level and WBC, and an increase in PLT number and IL-10 level were detected by the therapy. Furthermore, echocardiographic findings revealed a statistically significant increase in EF and a decrease in RVMPI attributable to the therapy. DISCUSSION: The decreased levels of PTH, which is a proinflammatory marker, the increases in IL-10, which is an antiinflammatory cytokine, and the decreases in the number of WBC resulting from vitamin D treatment demonstrate the antiinflammatory effects of vitamin D. An improvement in EF means improvement in left ventricular contractility, while a decrease in RV MPI has been shown to improve the systolic and diastolic function of the right ventricle. These results suggest that vitamin D therapy has a positive effect on the heart, and so vitamin D levels should be evaluated during the routine follow-up of congenital heart disease.

Health-related quality of life and parental depression in children with pulmonary arterial hypertension.

BACKGROUND: Impaired health-related quality of life (HRQoL) is a common problem in pulmonary arterial hypertension (PAH), but there is limited data on HRQoL in children with PAH. We aimed to investigate the QoL, determine the potential risk factors for poor HRQoL in children with PAH, and assess the depression and anxiety of their families. METHODS: We performed a prospective cross-sectional study of children with PAH, healthy peers, and their parents. HRQoL was measured by the self-reported and age-adapted KINDL questionnaire. Beck Depression Inventory (BDI) and hospital anxiety and depression scale (HADS) were used to assess the depression and anxiety of parents. RESULTS: Children with PAH had statistically lower total HRQoL scores than healthy peers (p < 0.001). There was no correlation between HRQoL and duration of disease, World Health Organization functional class, pro-B-type natriuretic peptide, 6-min walk test, and combined or single treatment. BDI and HADS scores were significantly higher in the parents of patients (p < 0.001, p = 0.023, p < 0.001, respectively). There was a negative correlation between HRQoL and BDI in patients (p = 0.016), while there was no significant correlation between HRQoL and HADS (p > 0.05). CONCLUSION: We demonstrated impairment of HRQoL of children with PAH. In addition, there was a correlation between the depression of the families and the QoL of the children.

Prognostic value of oxygen saturation and heart rate during a six-minute walk test in pediatric pulmonary hypertension

Background/aim: To evaluate the pre and post-treatment heart rate and oxygen (O2) saturation values measured before, during and after 6MWT in children who received PAH-specific treatment and to determine the effect of these variables on prognosis. Materials and methods: This retrospective study included 29 patients who were diagnosed as PAH and treated. The transcutaneous O2 saturation and heart rate levels were recorded before the test: baseline (Sat0, HR0), at the end of the test: exercise (Sat1, HR1) and 5 min after the test: recovery (Sat2, HR2). The increase in heart rate was defined as HR1-HR0 and the decrease in saturation as Sat0-Sat1. The results obtained before and after the PAH-specific treatment were analyzed and their effect on survival was assessed. Results: Fifteen of 29 patients were female (51.7%). The mean age was 127.2 ± 44.8 months and the median follow-up time was 60 (12­156) months. Only pre-treatment HR1 was found associated with survival. The mean HR1 value of survivors was 122.8 ± 18.4 and that of deceased 94.3 ± 19.1 (p = 0.03). Post-treatment 6MWT was associated with survival. It was 453.3 ± 96.5 m for survivors and 250 ± 135.2 m for deceased (p = 0.02). Conclusion: Post-treatment 6MWT, FC and proBNP had prognostic value in pediatric patients with PAH. The decrease in saturation and increase in heart rate were not found to have a prognostic value. Pre-treatment HR1 was associated with survival. Identification of these prognostic factors at the beginning and throughout the treatment may be a guide for detecting the severity of the disease and follow-up.

Decrease in Cerebral Oxygen Saturation During the 6-Minute Walk Test in Pediatric Pulmonary Arterial Hypertension.

This observational study aimed to investigate the relationship between regional cerebral oxygen saturation (rSO2) during the 6-minute walk test (6-MWT) and the demographic/clinical features of patients with pulmonary arterial hypertension (PAH). Cerebral oxygenation was evaluated during the 6-MWT in 20 pediatric patients with PAH aged ≥ 7 years [13 male, 7 female; median age 12.25 (range 7-18) years]. In all patients, regional cerebral oxygen saturation    (rSO2), arterial oxygen saturation (SpO2), and heart rate (HR) were measured using near-infrared spectroscopy (NIRS) for 2 min before the test, during the 6-MWT test, and 2 min after the test. The relationship between the changes in rSO2, heart rate, and SpO2 values and clinical and laboratory features was compared statistically. The mean pulmonary arterial pressure (mPAP) was 77 (range 25-126) mmHg, and the median 6-minute walk distance (6-MWD) was 427.5 (300-570) m. The changes in rSO2 measurements ranged between 3.41 and 21.8%, and 70% of the patients had a greater than 10% decrease in rSO2 during the test compared with baseline. Eight patients showed a decrease in rSO2 without SpO2 changes. The present study demonstrated a significant decrease in cerebral oxygenation in pediatric patients with PAH during the 6-MWT. We hypothesized that using a combination of the 6-MWT and regional cerebral oxygenation monitoring in pediatric patients with PAH in order to evaluate exercise capacity, as a reflection of reduced daily activities, would provide more precise predictive values than the 6-MWT alone.

A retrospective study on children with pulmonary arterial hypertension: A single-center experience.

OBJECTIVE: The aim of this study was to evaluate children with pulmonary arterial hypertension (PAH) regarding epidemiological characteristics, clinical status with respect to the WHO functional class (WHO-FC), prognostic factors, and efficacy of medical treatment. METHODS: A retrospective evaluation of 41 patients with PAH was made in the Pediatric Cardiology Unit, Gazi University Medical Faculty, between February 2006 and October 2015. RESULTS: Of the 41 patients included in this study, 51.2% were female. The median age was 60 months at first evaluation. The median follow-up was 60 months. At the start of the treatment, 43.9% patients were receiving combined drug therapy, and this rate increased to 60.9% by the last evaluation. The median time of adding a new medication to the therapy was 20 months. The 1- and 5-year survival rates were 94% and 86%, respectively. At the time of diagnosis, only pro-brain natriuretic peptide (proBNP) levels were associated with mortality (p=0.004), but at the last evaluation, 6-min walking test, proBNP and uric acid levels, and WHO-FC were also associated with survival (p=0.02, p=0.001, p=0.002, and p=0.05, respectively). CONCLUSION: With current treatment choices in experienced centers, positive results are obtained with respect to the functional status and survival rates of patients with PAH. At the time of diagnosis, only proBNP had a prognostic value, whereas at the last evaluation, WHO-FC, 6-min walking test, proBNP, and uric acid were reported prognostic factors. For preventing rapid progression, determination of factors that have an effect on prognosis, in particular, is extremely important.