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Congenital heart disease patients' and parents' perception of disease-specific knowledge: Health and impairments in everyday life.

Congenit Heart Dis. 2018 May;13(3):377-383

Authors: Helm PC, Kempert S, Körten MA, Lesch W, Specht K, Bauer UMM

BACKGROUND: Children and adolescents with congenital heart disease (CHD) and their families require qualified combined medical and psychosocial information, care, and counseling. This study aimed to analyze CHD patients' and parents' perception of disease-specific knowledge, state of health, and impairments experienced in everyday life, as well as factors influencing these perceptions.
MATERIALS AND METHODS: Analyses were based on a survey among patients/parents recruited via the German National Register for Congenital Heart Defects (NRCHD). The total sample (N = 818) was divided into four groups: "Children" (176 patients), "Adolescents" (142 patients), "Adults" (269 patients), and "Parents" (231 parents). The patients were stratified into those with simple and those with complex CHD. Descriptive and univariate analyses were performed.
RESULTS: Patients' age and CHD severity were related to self-assessed state of health (P = .04 and P = .02). In addition, CHD severity was associated with worse impairment in everyday life (P < .001). Psychosocial support was related to the self-assessed state of health (P = .01) and the reported impairment in everyday life (P < .001).
CONCLUSIONS: Patients' age, CHD severity, and psychosocial support seem to be related to self-assessed state of health and impairments in everyday life. To evaluate causality beyond associations, the development of patients' and parents' assessments and quality of life during the phase of transition from childhood to adulthood could be investigated by prospective long-term studies.

PMID: 29552750 [PubMed - indexed for MEDLINE]

Alteration in the expression of the renin-angiotensin system in the myocardium of mice conceived by in vitro fertilization.

Biol Reprod. 2018 Jul 13;:

Authors: Wang Q, Zhang Y, Le F, Wang N, Zhang F, Luo Y, Lou Y, Hu M, Wang L, Thurston LM, Xu X, Jin F

Epidemiological studies have revealed that offspring conceived by in vitro fertilization (IVF) have an elevated risk of cardiovascular malformations at birth, and are more predisposed to cardiovascular diseases in late childhood. Unfortunately, the underlying mechanisms influencing compromised cardiovascular function in IVF-conceived children remain unclear. Moreover, the continuing implications for the cardiovascular health of these children as they move into adulthood and old age, have yet to be determined. The renin-angiotensin system (RAS) plays an essential role in both the pathogenesis of congenital heart disease in fetuses and cardiovascular dysfunction in adults. This study aimed to assess the relative expression levels of genes in the RAS pathway in mice conceived using IVF, compared to natural mating with superovulation. In addition, the relative expression of candidate genes expressed in the myocardial tissue of newborn, adult and old mice was determined between the IVF and naturally mated populations. Results demonstrated that expression of the angiotensin II receptor type 1 (AGTR1), connective tissue growth factor (CTGF), and collagen 3 (COL3), in the myocardial tissue of IVF-conceived mice, was elevated at 3 weeks, 10 weeks, and 1.5 years of age, when compared to their non-IVF counterparts. These data were supported by microRNA microarray analysis of the myocardial tissue of aged IVF-conceived mice, where miR-100, miR-297, and miR-758, which interact with COL3, AGTR1 and COL1 respectively, were upregulated when compared to naturally mated mice of the same age. Interestingly, bisulfite sequencing data indicated that IVF-conceived mice exhibited decreased methylation of CpG sites in Col1. In support of our in vivo investigations, miR-297 overexpression was shown to upregulate AGTR1 and CTGF, and increased cell proliferation in cultured H9c2 cardiomyocytes. These findings indicate that the altered expression of RAS in myocardial tissue might contribute to cardiovascular malformation and/or dysfunction in IVF-conceived offspring. Furthermore, these cardiovascular abnormalities might be the result of altered DNA methylation and abnormal regulation of microRNAs.

PMID: 30010728 [PubMed - as supplied by publisher]

Prospective cohort study of C-reactive protein as a predictor of clinical events in adults with congenital heart disease: results of the Boston adult congenital heart disease biobank.

Eur Heart J. 2018 Jul 12;:

Authors: Opotowsky AR, Valente AM, Alshawabkeh L, Cheng S, Bradley A, Rimm EB, Landzberg MJ

Aims: Despite the well-defined association of high-sensitivity hsCRP with cardiovascular outcomes in apparently healthy adults and those with acquired heart disease, the relevance of this inflammatory marker in adults with congenital heart disease (ACHD) remains unclear. We aimed to examine the clinical correlates and prognostic value of high-sensitivity C-reactive protein levels in ACHD.
Methods and results: We conducted a prospective cohort study of (n = 707) outpatient ACHD (age 39 ± 14 years, 49% women), enrolled mainly at a referral centre, who had serum hsCRP measured in conjunction with a clinical assessment between 2012 and 2016. We analysed clinical correlates of hsCRP and its association with adverse events including the primary combined outcome of all-cause mortality or non-elective cardiovascular hospitalization. Higher hsCRP was strongly associated with measures of functional status including New York Heart Association class and peak V̇O2, and with comorbidities such as atrial arrhythmia. During average follow-up of 815 ± 536 days, 114 patients (16%) experienced the primary outcome, including 29 deaths. Having elevated hsCRP, in the highest (≥2.98 mg/L) compared with the lower three quartiles, conferred increased risk for the primary outcome [30.5% vs. 11.3%, adjusted hazard ratio (HR) = 2.00, 95% confidence interval (CI) 1.35-2.97; P = 0.0006] and all-cause mortality (11.9% vs. 1.5%, adjusted HR = 4.23, 95% CI 1.87-9.59; P = 0.0006). Elevated hsCRP was associated with adverse outcomes across ACHD subgroups and other patient characteristics.
Conclusion: Adults with congenital heart disease with elevated hsCRP have not only worse functional status and exercise capacity, but also greater risk for death or non-elective cardiovascular hospitalization. Further study is warranted to characterize the role of inflammation in the pathophysiology of ACHD.

PMID: 30010900 [PubMed - as supplied by publisher]

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Transcatheter pulmonary valve implantation: will it replace surgical pulmonary valve replacement?

Expert Rev Cardiovasc Ther. 2018 Mar;16(3):197-207

Authors: Kheiwa A, Divanji P, Mahadevan VS

INTRODUCTION: Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients. Expert commentary: Transcatheter pulmonary valve implantation (TPVI) is widely accepted as an alternative to surgery to address RVOT dysfunction. However, current technology may not be able to adequately address a subset of patients with complex RVOT morphology. As the technology continues to evolve, new percutaneous valves will allow practitioners to apply the transcatheter approach in such patients. We expect that with the advancement in transcatheter technology, novel devices will be added to the TPVI armamentarium, making the transcatheter approach a feasible alternative for the majority of patients with RVOT dysfunction in the near future.

PMID: 29433351 [PubMed - indexed for MEDLINE]

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Prevalence and mortality of congenital heart disease in Korean adults.

Medicine (Baltimore). 2018 Jul;97(27):e11348

Authors: Jang SY, Seo SR, Moon JR, Cho EJ, Kim E, Chang SA, Song J, Huh J, Kang IS, Kim DK, Park SW

The aim of this study was to assess the age-standardized prevalence (prevalence) and age-standardized mortality (mortality) of congenital heart disease (CHD) by sex in Korean adults. Data were collected from the National Health Insurance Service in Korea from 2006 through 2016. The data consisted of main and secondary diagnoses related to CHD. We calculated the prevalence and mortality of CHD in adults with the direct method using the estimated Korean population in 2015 as the reference. The prevalence of CHD in Korean adults increased from 35.8 cases per 100,000 persons in 2006 to 65.6 cases in 2015. In 2015, the prevalence in the 20- to 44-year-old group, 45- to 64-year-old group, and the older than 65 years group was 54.6, 69.6, and 95.1 cases, respectively. Among women, the prevalence was 34.3 and 31.3 cases in men. The mortality of CHD in adults decreased from 3.061 persons per 100,000 persons in 2007 to 0.551 persons in 2015. The 5-year survival rate (SR) for people with CHD was 0.92 (95% confidence interval [CI] 0.91-0.93). The 5-year SR for the 20- to 44- and 45- to 64-year-old groups was more than 0.95; however, the 5-year SR of the older than 65 years group was only 0.73 (95% CI 0.72-0.74). The prevalence of CHD in Korean adults increased and the related mortality decreased during a decade. The 5-year SR of CHD for those over 65 years was lower than that for younger age groups.

PMID: 29979412 [PubMed - indexed for MEDLINE]

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Fatal heart failure caused by severe pulmonary regurgitation, tricuspid regurgitation and late-onset mitral stenosis in an adult patient with Noonan syndrome: a case report.

BMC Cardiovasc Disord. 2018 Jul 16;18(1):148

Authors: Kadoya Y, Kenzaka T, Oda Y, Matoba S

BACKGROUND: In patients with Noonan syndrome (NS), cardiac disorders such as pulmonary valve stenosis (PS) or hypertrophic cardiomyopathy (HCM) are common. While some patients can develop heart failure associated with HCM, the long-term outcome of adult patients with NS is reported to be good. Fatal outcomes of heart failure in patients with NS but without HCM are rare.
CASE PRESENTATION: We report a 25-year-old Japanese woman diagnosed with NS in adulthood. She exhibited short stature and minor facial dysmorphism and was diagnosed with PS at 1 year of age. After surgical valvuloplasty for PS at 6 years of age, her general condition became stable without specific medical treatment. She discontinued regular medical follow-up for PS. At 21 years of age, she developed acute decompensated heart failure, which was mainly right-sided heart failure due to severe pulmonary regurgitation (PR) and tricuspid regurgitation (TR). There was no evidence of HCM or PS recurrence. On the basis of the history of PS and characteristic physical features including short stature, webbed neck, and hypertelorism, she was clinically diagnosed with NS. At 25 years of age, she developed heart failure of both sides due to PR, TR and late-onset severe mitral stenosis (MS). The etiology of MS was uncertain. Owing to the patient's condition, surgical options were considered to be extremely high risk. She was treated with optimal medical treatment as well as the occasional abdominal cavity drainage for recurrent ascites; however, she died of decompensated heart failure at 27 years of age.
CONCLUSIONS: We describe an adult patient with NS without HCM who died of heart failure caused by severe PR, TR and MS. Clinicians should recognize that ongoing or late-onset cardiac disorders can develop in patients with NS, and lead to fatal heart failure. Optimal medical follow-up to monitor cardiac function and early identification of heart failure are important.

PMID: 30012103 [PubMed - in process]

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Depressive and Anxiety Symptoms in Adult Congenital Heart Disease: Prevelance, Health Impact and Treatment.

Prog Cardiovasc Dis. 2018 Jul 13;:

Authors: Jackson JL, Leslie CE, Hondorp SN

Over 1.4 million adults in the United States are living with congenital heart disease (CHD), which can vary widely in disease burden and risk for future cardiovascular complications. As a result, some CHD survivors may experience greater emotional distress, including symptoms of depression and anxiety. This review summarizes the developing research literature on the prevalence, health impact, as well as screening and treatment of depressive and anxiety symptoms among adult CHD survivors. Additionally, the review will highlight findings from the acquired heart disease literature, a well-studied area that can inform research and clinical care practices for CHD survivors with depressive and/or anxiety symptoms. The review concludes with considerations for depression and anxiety screening in medical settings and identifying referral resources for treatment.

PMID: 30012407 [PubMed - as supplied by publisher]

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Early experience of transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage.

EuroIntervention. 2018 Jul 18;:

Authors: Riahi M, Velasco Forte MN, Byrne N, Hermuzi A, Jones M, Baruteau AE, Valverde I, Qureshi SA, Rosenthal E

AIMS: Superior sinus venosus atrial septal defect (SVASD) is commonly associated with partial anomalous pulmonary venous drainage (PAPVD). We aimed to describe the first series of percutaneous SVASD and PAPVD correction using a two-step simulation for procedural planning.
METHODS AND RESULTS: Patients with SVASD and right PAPVD with a clinical indication for correction were selected. They underwent an ex vivo procedural simulation on a 3D-printed model followed by an in vivo simulation using balloon inflation in the targeted stent landing zone. The percutaneous procedure consisted in deploying a 10-zig custom-made covered stent in the SVC-RA junction. Five patients were referred for preprocedural evaluation and were deemed suitable for percutaneous correction. The procedure was successful in all patients with no residual interatrial shunt and successful redirection of the pulmonary venous drainage to the left atrium. At a median clinical follow-up of 8.1 months (2.6-19.8), no adverse events were noted, and all patients showed clinical improvement. During follow-up, transthoracic echocardiography and multidetector cardiac tomography in four patients or invasive angiography in one patient demonstrated a patent SVC stent, and no residual SVASD and unobstructed PV drainage in all patients.
CONCLUSIONS: In selected patients using a two-stage simulation strategy, percutaneous correction of SVASD with PAPVD is feasible and safe, and led to favourable short-term outcomes.

PMID: 30012542 [PubMed - as supplied by publisher]

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Response by Brida et al to Letter Regarding Article, "Systemic Right Ventricle in Adults With Congenital Heart Disease: Anatomic and Phenotypic Spectrum and Current Approach to Management".

Circulation. 2018 Jul 17;138(3):326-327

Authors: Brida M, Diller GP, Gatzoulis MA

PMID: 30012708 [PubMed - in process]

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Advanced diffusion imaging for assessing normal white matter development in neonates and characterizing aberrant development in congenital heart disease.

Neuroimage Clin. 2018;19:360-373

Authors: Karmacharya S, Gagoski B, Ning L, Vyas R, Cheng HH, Soul J, Newberger JW, Shenton ME, Rathi Y, Grant PE

Background: Elucidating developmental trajectories of white matter (WM) microstructure is critically important for understanding normal development and regional vulnerabilities in several brain disorders. Diffusion Weighted Imaging (DWI) is currently the method of choice for in-vivo white matter assessment. A majority of neonatal studies use the standard Diffusion Tensor Imaging (DTI) model although more advanced models such as the Neurite Orientation Dispersion and Density Imaging (NODDI) model and the Gaussian Mixture Model (GMM) have been used in adult population. In this study, we compare the ability of these three diffusion models to detect regional white matter maturation in typically developing control (TDC) neonates and regional abnormalities in neonates with congenital heart disease (CHD).
Methods: Multiple b-value diffusion Magnetic Resonance Imaging (dMRI) data were acquired from TDC neonates (N = 16) at 38 to 47 gestational weeks (GW) and CHD neonates (N = 19) aged 37 weeks to 41 weeks. Measures calculated from the diffusion signal included not only Mean Diffusivity (MD) and Fractional Anisotropy (FA) derived from the standard DTI model, but also three advanced diffusion measures, namely, the fiber Orientation Dispersion Index (ODI), the isotropic volume fraction (Viso), and the intracellular volume fraction (Vic) derived from the NODDI model. Further, we used two novel measures from a non-parametric GMM, namely the Return-to-Origin Probability (RTOP) and Return-to-Axis Probability (RTAP), which are sensitive to axonal/cellular volume and density respectively. Using atlas-based registration, 22 white matter regions (6 projection, 4 association, and 1 callosal pathways bilaterally in each hemisphere) were selected and the mean value of all 7 measures were calculated in each region. These values were used as dependent variables, with GW as the independent variable in a linear regression model. Finally, we compared CHD and TDC groups on these measures in each ROI after removing age-related trends from both the groups.
Results: Linear analysis in the TDC population revealed significant correlations with GW (age) in 12 projection pathways for MD, Vic, RTAP, and 11 pathways for RTOP. Several association pathways were also significantly correlated with GW for MD, Vic, RTAP, and RTOP. The right callosal pathway was significantly correlated with GW for Vic. Consistent with the pathophysiology of altered development in CHD, diffusion measures demonstrated differences in the association pathways involved in language systems, namely the Uncinate Fasciculus (UF), the Inferior Fronto-occipital Fasciculus (IFOF), and the Superior Longitudinal Fasciculus (SLF). Overall, the group comparison between CHD and TDC revealed lower FA, Vic, RTAP, and RTOP for CHD bilaterally in the a) UF, b) Corpus Callosum (CC), and c) Superior Fronto-Occipital Fasciculus (SFOF). Moreover, FA was lower for CHD in the a) left SLF, b) bilateral Anterior Corona Radiata (ACR) and left Retrolenticular part of the Internal Capsule (RIC). Vic was also lower for CHD in the left Posterior Limb of the Internal Capsule (PLIC). ODI was higher for CHD in the left CC. RTAP was lower for CHD in the left IFOF, while RTOP was lower in CHD in the: a) left ACR, b) left IFOF and c) right Anterior Limb of the Internal Capsule (ALIC).
Conclusion: In this study, all three methods revealed the expected changes in the WM regions during the early postnatal weeks; however, GMM outperformed DTI and NODDI as it showed significantly larger effect sizes while detecting differences between the TDC and CHD neonates. Future studies based on a larger sample are needed to confirm these results and to explore clinical correlates.

PMID: 30013919 [PubMed - in process]