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Evolving heart transplantation across the lifespan: A growing population of adults with congenital heart disease.

Arch Cardiovasc Dis. 2016 Oct;109(10):511-513

Authors: Cohen S, Marelli A

PMID: 27595466 [PubMed - indexed for MEDLINE]

Corrigendum to 'Ventricular tachyarrhythmia during pregnancy in women with heart disease: Data from the ROPAC, a registry from the European Society of Cardiology' [Int. J. Cardiol. 220 (2016) 131-136].

Int J Cardiol. 2017 Jan 27;:

Authors: Ertekin E, van Hagen IM, Salam AM, Ruys TP, Johnson MR, Popelova J, Parsonage WA, Ashour Z, Shotan A, Oliver JM, Veldtman GR, Hall R, Roos-Hesselink JW

PMID: 28139301 [PubMed - as supplied by publisher]

How best to train doctors in adult congenital heart disease?

Arch Cardiovasc Dis. 2017 Jan 27;:

Authors: Acar P

PMID: 28139453 [PubMed - as supplied by publisher]

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Cardiac Denial and Psychological Predictors of Cardiac Care Adherence in Adults With Congenital Heart Disease.

Behav Modif. 2016 Jan;40(1-2):29-50

Authors: White KS, Pardue C, Ludbrook P, Sodhi S, Esmaeeli A, Cedars A

Abstract
The current study examined cardiac denial and psychological predictors (i.e., depression, anxiety) of health outcomes including medical nonadherence and physical health in a sample of 80 adults with congenital heart disease (ACHD). Results indicated that denial of impact was elevated in this patient group compared with reference groups, and denial was negatively associated with depression and anxiety at ps < .01. Results indicated that depression, anxiety, and denial predicted unique variance in medical nonadherence, and gender moderated the relationships between these psychological factors and nonadherence. For depression, men and women showed similar relationships between depression and nonadherence at high levels of depression; however, at low levels of depression (i.e., a more normal mood state), men were less adherent compared with women. For anxiety, men and women did not differ in adherence at low levels of anxiety; however, men experiencing high anxiety were less adherent compared with women experiencing high anxiety. Implications of this study are discussed including the role of gender and denial and the impact of denial functioning to reduce negative affect. Depression was the only significant predictor of physical functioning. Results of this study suggest that psychological interventions aimed at depression and anxiety may function differently across gender to improve patient medical adherence and improve physical functioning in ACHD.

PMID: 26538274 [PubMed - indexed for MEDLINE]

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fMRI investigation of working memory in adolescents with surgically treated congenital heart disease.

Appl Neuropsychol Child. 2017 Jan-Mar;6(1):7-21

Authors: King TZ, Smith KM, Burns TG, Sun B, Shin J, Jones RA, Drossner D, Mahle WT

Abstract
Adolescents and young adults with surgically treated congenital heart disease (CHD) have been shown to exhibit difficulties with executive functions; however, the neural underpinnings of these impairments have not been previously examined with functional magnetic resonance imaging (fMRI). The current study employed fMRI to examine the neural mechanisms during a letter n-back task of working memory compared to vigilance. Seventeen participants with CHD (Mage = 17.76 ± 1.72 years; 88% Caucasian; 30% female; mean IQ = 104.12 ± 15.15) were compared to 17 controls (Mage = 18.40 ± 1.74 years; 70% Caucasian; 30% female; mean IQ = 110.59 ± 5.28) with similar declining performance as the n-back became more challenging. Overall, both groups activated similar frontal-parietal working-memory networks as seen in previous literature; however, some significant differences were detected between the groups. Specifically, the participants with CHD demonstrated differences within the left precuneus and the right inferior frontal gyrus. Secondary analyses indicated that this difference appeared to be due to less task-induced deactivation (TID) in the CHD group during working memory and greater working-memory TID in the control group. In CHD, prefrontal fMRI deactivation on working-memory tasks correlated with improved working-memory performance. Future complementary neuroimaging research with functional connectivity is warranted to further examine the neural underpinnings of disrupted executive function in the long-term outcomes of CHD.

PMID: 26985707 [PubMed - indexed for MEDLINE]

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The importance of social media for patients and families affected by congenital anomalies: A Facebook cross-sectional analysis and user survey.

J Pediatr Surg. 2016 Nov;51(11):1766-1771

Authors: Jacobs R, Boyd L, Brennan K, Sinha CK, Giuliani S

Abstract
BACKGROUND: We aimed to define characteristics and needs of Facebook users in relation to congenital anomalies.
METHODS: Cross-sectional analysis of Facebook related to four congenital anomalies: anorectal malformation (ARM), congenital diaphragmatic hernia (CDH), congenital heart disease (CHD) and hypospadias/epispadias (HS/ES). A keyword search was performed to identify relevant Groups/Pages. An anonymous survey was posted to obtain quantitative/qualitative data on users and their healthcare needs.
RESULTS: 54 Groups and 24 Pages were identified (ARM: 10 Groups; CDH: 9 Groups, 7 Pages; CHD: 32 Groups, 17 Pages; HS/ES: 3 Groups), with 16,191 Group members and 48,766 Page likes. 868/1103 (79%) of respondents were parents. Male:female ratio was 1:10.9. 65% of the users were 26-40years old. Common reasons for joining these Groups/Pages included: seeking support, education, making friends, and providing support to others. 932/1103 (84%) would like healthcare professionals (HCPs) to actively participate in their Group. 31% of the respondents felt that they did not receive enough support from their healthcare system. 97% of the respondents would like to join a Group linked to their primary hospital.
CONCLUSIONS: Facebook Groups/Pages related to congenital anomalies are highly populated and active. There is a need for HCPs and policy makers to better understand and participate in social media to support families and improve patient care.

PMID: 27522307 [PubMed - indexed for MEDLINE]

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Characterization of soluble N-ethylmaleimide-sensitive factor attachment protein receptor gene STX18 variations for possible roles in congenital heart diseases.

Gene. 2017 Jan 20;598:79-83

Authors: Li X, Shi S, Li FF, Cheng R, Han Y, Diao LW, Zhang Q, Zhi JX, Liu SL

Abstract
Congenital heart disease (CHD) is among the most prevalent and complex congenital anatomic malformations in newborns. Interactions of cardiac progenitor with a broad range of cellular regulatory factors play key roles in the formation of mammalian heart and pathogenesis of CHD. STX18 is a soluble N-ethylmaleimide-sensitive factor attachment protein receptor, which is involved in numeral cellular activities such as organelle assembly and the cell cycle. The aim of this work was to find evidence on whether STX18 variations might be associated with CHD in Chinese Han populations. We evaluated SNPs rs2044, rs33952588, rs61740788, rs12504020 and rs12644497, which are located within the exon or intron sequences of the STX18 gene, for 310 Chinese Han CHD patients and 400 non-CHD controls. Using SPSS software (version 19.0) and the online software OEGE, we conducted statistical analyses and Hardy-Weinberg equilibrium test, respectively. Among the five SNPs identified in the STX18 gene, rs33952588 and rs61740788 had very low genetic heterozygosity. In contrast, the genetic heterozygosity of the remaining three variations rs12504020 and rs12644497 near the 5'UTR and rs2044 within 3'UTR of the STX18 gene was considerably high. Analysis of associations of these genetic variations with the risk of CHD showed that rs12644497 (P value=0.017<0.05) was associated with the risk of CHD, specifically VSD and ASD, whereas rs12504020 (P value=0.560>0.05) and rs2044 (P value=0.972>0.05) were not. The SNP rs12644497 in the STX18 gene was associated with CHD in Chinese Han populations.

PMID: 27816473 [PubMed - indexed for MEDLINE]

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Current trends in stenting for aortic coarctation in Japan: Subanalysis of Japanese Society of Pediatric Interventional Cardiology (JPIC) stent survey.

Pediatr Int. 2016 Feb;58(2):100-4

Authors: Fujii T, Tomita H, Otsuki S, Kobayashi T, Ono Y, Yazaki S, Kim SH, Nakanishi T

Abstract
BACKGROUND: Stenting for aortic coarctation (CoA) has been accepted as an alternative to surgery for adolescents and adults, but only a few case have been reported in Japan. The purpose of this study was to provide a detailed review of Japanese national data on stenting of CoA.
METHODS: In a subanalysis of the data of the Japanese Society of Pediatric Interventional Cardiology (JPIC), we identified 35 patients with CoA who underwent stenting. We analyzed procedural characteristics including factors that may have contributed to hemodynamic effectiveness, and we compared these parameters between the patients under and over 15 years of age.
RESULTS: The mean ratio of balloon diameter/minimum lumen diameter (MLD) before stenting was 1.7 (range, 1.2-4.0), and the mean difference between the balloon diameter and the reference vessel diameter was -0.7 mm (range, -5.0 to +3.0 mm). %MLD/balloon diameter, which was defined as [(balloon diameter - MLD after dilation)/balloon diameter] × 100 predicted achievement of <10 mmHg pressure gradient after stenting. The sensitivity and the specificity of its cut-off of 7% were 93% and 47% (AUC, 0.7), respectively. There was no statistical difference between the two age groups under and over 15 years of age, in terms of selection criteria of stent size, balloon type used for deployment and immediate angiographic and hemodynamic result.
CONCLUSIONS: Stenting for CoA was clinically effective with few complications in Japan, even in patients not fully grown.

PMID: 26212515 [PubMed - indexed for MEDLINE]

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Quality of life in adults living with congenital heart disease: beyond morbidity and mortality.

J Thorac Dis. 2016 Dec;8(12):E1632-E1636

Authors: Hunter AL, Swan L

PMID: 28149600 [PubMed - in process]

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Development of Quality Metrics in Ambulatory Pediatric Cardiology.

J Am Coll Cardiol. 2017 Feb 07;69(5):541-555

Authors: Chowdhury D, Gurvitz M, Marelli A, Anderson J, Baker-Smith C, Diab KA, Edwards TC, Hougen T, Jedeikin R, Johnson JN, Karpawich P, Lai W, Lu JC, Mitchell S, Newburger JW, Penny DJ, Portman MA, Satou G, Teitel D, Villafane J, Williams R, Jenkins K, American College of Cardiology’s Adult Congenital and Pediatric Cardiology Section’s Ambulatory Pediatric Cardiology Quality Metrics Working Group

Abstract
The American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Section had attempted to create quality metrics (QM) for ambulatory pediatric practice, but limited evidence made the process difficult. The ACPC sought to develop QMs for ambulatory pediatric cardiology practice. Five areas of interest were identified, and QMs were developed in a 2-step review process. In the first step, an expert panel, using the modified RAND-UCLA methodology, rated each QM for feasibility and validity. The second step sought input from ACPC Section members; final approval was by a vote of the ACPC Council. Work groups proposed a total of 44 QMs. Thirty-one metrics passed the RAND process and, after the open comment period, the ACPC council approved 18 metrics. The project resulted in successful development of QMs in ambulatory pediatric cardiology for a range of ambulatory domains.

PMID: 28153110 [PubMed - in process]

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