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Determinants of Exercise Performance in Children and Adolescents with Repaired Tetralogy of Fallot Using Stress Echocardiography.

Pediatr Cardiol. 2019 Jan;40(1):71-78

Authors: Bhatt SM, Elci OU, Wang Y, Goldmuntz E, McBride M, Paridon S, Mercer-Rosa L

Exercise performance is variable and often impaired in patients with repaired tetralogy of Fallot (rTOF). We sought to identify factors associated with exercise performance by comparing high to low performers on cardiopulmonary exercise testing (CPET) in patients with rTOF. We conducted a cross-sectional study of subjects presenting for CPET who underwent echocardiograms at rest and peak exercise. Patients with pacemakers and arrhythmias were excluded. Right ventricular (RV) global longitudinal strain was used as a measure of systolic function. Pulmonary insufficiency (PI) was assessed with the diastolic systolic ratio and the diastolic systolic time-velocity integral ratio by Doppler interrogation of the pulmonary artery. CPET measures included percent-predicted maximum [Formula: see text][Formula: see text], percent-predicted maximum work and oxygen pulse. High versus low performers were identified as those achieving [Formula: see text] of at least 80% or falling below, respectively. Differences in echocardiographic parameters from rest to peak exercise were examined using mixed-effects regression models. Compared to the low performers (n = 17), high performers (n = 12) were younger (12.8 ± 3.3 years vs. 18.3 ± 4.8 years), had normal chronotropic response (peak heart rate > 185 bpm) with greater heart rate reserve and superior physical working capacity. High performers also had a greater reduction in PI at peak exercise, despite greater PI severity at rest. Oxygen pulse was comparable between groups. For both groups, there was no association of PI severity and RV systolic function at rest with exercise parameters. There was no group difference in the magnitude of change in RV strain and diastolic parameters from rest to peak exercise. Chronotropic response to exercise appears to be an important parameter with which to assess exercise performance in rTOF. Chronotropic health should be taken into consideration in this population, particularly given that RV function and PI severity at rest were not associated with exercise performance.

PMID: 30121867 [PubMed - indexed for MEDLINE]

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[Association between congenital heart disease and medication taken during pregnancy among women of childbearing age].

Zhonghua Liu Xing Bing Xue Za Zhi. 2018 Oct 10;39(10):1333-1338

Authors: Li MM, Guo LQ, Li SS, Zhang Q, Zhao DD, Zhang BY, Dang SN, Yan H

Objective: To analyze the relationship between medication taken during pregnancy and congenital heart disease of the newborns. Methods: A large cross-sectional survey was conducted between August and November 2013. A questionnaire survey was conducted among the childbearing aged women, selected through multistage stratified random sampling in Shaanxi from 2010 to 2013. All of the childbearing aged women under study were in pregnancy and with definite pregnancy outcomes. Multivariable Poisson regression was conducted for data analyses. Results: A total of 28 680 cases were included in this study. The proportion of medication taken at any time during pregnancy was 16.0%, and the prevalence of congenital heart disease among the newborns was 67.9/10 000. After adjustment for factors as general demographic characteristic, history of heart disease and drug allergy and the situation of disease during pregnancy of these women, results from the multivariable Poisson regression showed that, factors as taking drugs (RR=1.95, 95%CI: 1.42- 2.68), cold medicine (RR=1.68, 95%CI: 1.07-2.64), antibiotics (RR=1.90, 95%CI: 1.25-2.90), salicylates (RR=5.01, 95%CI: 1.84-13.64) and antifungal drugs (RR=10.22, 95%CI: 3.25-32.19) during pregnancy were all related to congenital heart disease, and with the history of taking cold medicine (RR=1.90, 95%CI: 1.01-3.61), antibiotics (RR=2.18, 95%CI: 1.17-4.06), salicylates (RR=6.07, 95%CI: 1.45-25.41), antifungal drugs (RR=21.01, 95%CI: 4.17-105.87) and other drugs (RR=2.31, 95%CI: 1.19-4.47) during early pregnancy. These factors were with higher risks for congenital heart disease. Conclusion: Women of childbearing age who took cold medicine, antibiotics, salicylic acid drugs, antifungal drugs and other drugs during early pregnancy would increase the risks related to congenital heart diseases.

PMID: 30453433 [PubMed - indexed for MEDLINE]

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Patient-reported outcomes of adults with congenital heart disease from eight European countries: scrutinising the association with healthcare system performance.

Eur J Cardiovasc Nurs. 2019 Feb 26;:1474515119834484

Authors: Van Bulck L, Luyckx K, Goossens E, Apers S, Kovacs AH, Thomet C, Budts W, Sluman MA, Eriksen K, Dellborg M, Berghammer M, Johansson B, Caruana M, Soufi A, Callus E, Moons P

BACKGROUND:: Inter-country variation in patient-reported outcomes of adults with congenital heart disease has been observed. Country-specific characteristics may play a role. A previous study found an association between healthcare system performance and patient-reported outcomes. However, it remains unknown which specific components of the countries' healthcare system performance are of importance for patient-reported outcomes.
AIMS:: The aim of this study was to investigate the relationship between components of healthcare system performance and patient-reported outcomes in a large sample of adults with congenital heart disease.
METHODS:: A total of 1591 adults with congenital heart disease (median age 34 years; 51% men; 32% simple, 48% moderate and 20% complex defects) from eight European countries were included in this cross-sectional study. The following patient-reported outcomes were measured: perceived physical and mental health, psychological distress, health behaviours and quality of life. The Euro Health Consumer Index 2015 and the Euro Heart Index 2016 were used as measures of healthcare system performance. General linear mixed models were conducted, adjusting for patient-specific variables and unmeasured country differences.
RESULTS:: Health risk behaviours were associated with the Euro Health Consumer Index subdomains about patient rights and information, health outcomes and financing and access to pharmaceuticals. Perceived physical health was associated with the Euro Health Consumer Index subdomain about prevention of chronic diseases. Subscales of the Euro Heart Index were not associated with patient-reported outcomes.
CONCLUSION:: Several features of healthcare system performance are associated with perceived physical health and health risk behaviour in adults with congenital heart disease. Before recommendations for policy-makers and clinicians can be conducted, future research ought to investigate the impact of the healthcare system performance on outcomes further.

PMID: 30808198 [PubMed - as supplied by publisher]

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Reconstruction of anomalous left coronary artery from pulmonary artery in an adult patient: a case report.

J Cardiothorac Surg. 2019 Feb 26;14(1):40

Authors: Mori K, Anai H, Wada T, Shuto T, Miyamoto S

BACKGROUND: Anomalous left coronary artery from the pulmonary artery is a congenital heart disease in which myocardial ischemia occurs within 1 year of birth. Adults have been reported to survive owing to the development of collateral perfusion from right coronary artery. In these cases, however, revascularization is necessary to prevent sudden cardiac death.
CASE PRESENTATION: A 62-year-old female gradually started experiencing dyspnea during exercise. Coronary computed tomography revealed that the left coronary artery arose from the main pulmonary artery. The patient was subsequently diagnosed with adult-type, anomalous left coronary artery from the pulmonary artery. She underwent a surgery, in which a 6-mm vascular prosthesis was passed through the main pulmonary artery to anastomose the left coronary artery and ascending aorta.
CONCLUSION: Our reconstructive technique using vascular prosthesis is effective for anomalous left coronary artery from pulmonary artery in adults.

PMID: 30808374 [PubMed - in process]

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Sudden death in transposition of the great arteries with atrial switch surgery: Autopsy evidence of acute myocardial ischemia despite normal coronary arteries.

Int J Cardiol. 2019 Feb 14;:

Authors: Chaix MA, Chergui M, Leduc C, Khairy P

BACKGROUND: Sudden death is the leading cause of mortality in patients with transposition of the great arteries (TGA) and atrial switch surgery. Understanding underlying mechanisms could contribute to identifying high-risk patients and preventing such catastrophic deaths.
METHODS: A total of 144 adults (≥18 years) with TGA and atrial switch surgery were followed at our adult congenital center since 1989. Four patients were excluded: two with double-outlet right ventricles and two with subsequent arterial switch surgery in childhood.
RESULTS: Of the remaining 140 patients, age 37.6 ± 7.8 years, 37.1% female, 8 (6%) had a cardiac arrest of presumed arrhythmic etiology of whom 3 were resuscitated. The arrests occurred in 3 women and 5 men at age 30.5 ± 8.6 (range 22 to 50) years. None had established coronary artery disease, sustained ventricular arrhythmias, or syncope. Four (50%) had atrial arrhythmias and 6 (75%) had at least moderate systemic right ventricular dysfunction. For 5 patients in whom circumstances surrounding the arrests were documented, 3 occurred on exertion, 1 after consuming recreational methamphetamine, and 1 in the context of an atrial tachyarrhythmia. Autopsies were performed in 2 of 5 patients. Both revealed acute massive myocardial infarction of the hypertrophied systemic right ventricle with normal coronary arteries and chronic subendocardial ischemic lesions.
CONCLUSION: This is the first report to provide histopathological evidence in support of a myocardial ischemia hypothesis as a cause of sudden death in this patient population, despite the absence of coronary atherosclerosis.

PMID: 30808604 [PubMed - as supplied by publisher]

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Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys Dietz Syndrome.

BJOG. 2019 Feb 27;:

Authors: Cauldwell M, Steer PJ, Curtis S, Mohan AR, Dockree S, Mackillop L, Parry H, Oliver J, Sterrenburg M, Bolger A, Siddiqui F, Simpson M, Walker N, Bredaki F, Walker F, Johnson MR

OBJECTIVE: Pregnancies in women with Loeys Dietz Syndrome (LDS) are rare, and typically documented in case reports only. Early reports suggested high rates of maternal complications during pregnancy and the puerperium, including aortic dissection and uterine rupture, but information on fetal outcomes was very limited.
DESIGN: A retrospective cohort study.
SETTING: 8 specialist UK centres.
SAMPLE: Pregnant women with LDS.
METHODS: Data was collated on cardiac, obstetric and neonatal outcomes.
MAIN OUTCOME MEASURES: Maternal and perinatal outcomes in pregnancies complicated by LDS.
RESULTS: 20 pregnancies in 13 women with LDS were identified. There was one miscarriage, one termination of pregnancy and 18 livebirths. In 8 women the diagnosis was known prior to pregnancy but only one woman had preconception counselling. In 4 women the diagnosis was made during pregnancy through positive genotyping, and the other was diagnosed following delivery. Five women had a family history of aortic dissection. There were no aortic dissections in our cohort during pregnancy or postpartum. Obstetric complications were common, including postpartum haemorrhage (33%) and preterm delivery (50%). 14/18 (78%) of deliveries were by elective caesarean section, at a median gestational age at delivery of 37 weeks. Over half the infants (56%) were admitted to the neonatal unit following delivery.
CONCLUSION: Women with LDS require multidisciplinary specialist management throughout pregnancy. Women should be referred for preconception counselling to make informed decisions around pregnancy risk and outcomes. Early elective preterm delivery needs to be balanced against a high infant admission rate to the neonatal unit This article is protected by copyright. All rights reserved.

PMID: 30811810 [PubMed - as supplied by publisher]

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A parent-of-origin analysis of paternal genetic variants and increased risk of conotruncal heart defects.

Am J Med Genet A. 2018 03;176(3):609-617

Authors: Nembhard WN, Tang X, Li J, MacLeod SL, Levy J, Schaefer GB, Hobbs CA, National Birth Defects Prevention Study

The association between conotruncal heart defects (CTHDs) and maternal genetic and environmental exposures is well studied. However, little is known about paternal genetic or environmental exposures and risk of CTHDs. We assessed the effect of paternal genetic variants in the folate, homocysteine, and transsulfuration pathways on risk of CTHDs in offspring. We utilized National Birth Defects Prevention Study data to conduct a family-based case only study using 616 live-born infants with CTHDs, born October 1997-August 2008. Maternal, paternal and infant DNA was genotyped using an Illumina® Golden Gate custom single nucleotide polymorphism (SNP) panel. Relative risks (RR) and 95% confidence intervals (CI) from log-linear models determined parent of origin effects for 921 SNPs in 60 candidate genes involved in the folate, homocysteine, and transsulfuration pathways on risk of CTHDs. The risk of CTHD among children who inherited a paternally derived copy of the A allele on GLRX (rs17085159) or the T allele of GLRX (rs12109442) was 0.23 (95%CI: 0.12, 0.42; p = 1.09 × 10-6 ) and 0.27 (95%CI: 0.14, 0.50; p = 2.06 × 10-5 ) times the risk among children who inherited a maternal copy of the same allele. The paternally inherited copy of the GSR (rs7818511) A allele had a 0.31 (95%CI: 0.18, 0.53; p = 9.94 × 10-6 ] risk of CTHD compared to children with the maternal copy of the same allele. The risk of CTHD is less influenced by variants in paternal genes involved in the folate, homocysteine, or transsulfuration pathways than variants in maternal genes in those pathways.

PMID: 29399948 [PubMed - indexed for MEDLINE]

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[The change in mortality and major causes of death among Chinese adolescents from 1990 to 2016].

Zhonghua Yu Fang Yi Xue Za Zhi. 2018 Aug 06;52(8):802-808

Authors: Xu RB, Wen B, Song Y, Luo DM, Dong YH, Dong B, Ma J

Objective: To analyze the change in mortality and major causes of death among Chinese adolescents aged 10-19 years from 1990 to 2016. Methods: Data of death for Chinese adolescents aged 10-19 years were extracted from the Global Burden of Disease Study 2016 (GBD 2016). The data was used to describe the change in mortality of Chinese adolescents from 1990 to 2016.Top 15 causes of death were compared between 1990 and 2016. Results: From 1990 to 2016, the all-cause mortality of Chinese adolescents aged 10-19 years old dropped from 102.5/100 000 to 41.2/100 000, reaching about a half of the global average (78.6/100 000) but still almost twice as high as the average of high-income countries (24.3/100 000) by 2016; the reduction in mortality was higher among females than that among males (decreased 68.7% vs. 54.7%), and among adolescents aged 10-14 years than that among those aged 15-19 years (decreased 62.1% vs. 57.1%). During the 26-year period, the cause-specific mortality of all top 15 causes saw sharp decreases. Among them, communicable, maternal, and nutritional diseases saw the most significant decrease (78.1%), and its proportion in all adolescent deaths fell from 11.1% to 6.1%. Meanwhile, non-communicable diseases and injuries saw relatively small decreases (58.2% and 57.3% respectively), and their proportions rose from 33.4% and 55.6% to 34.8% and 59.1%, respectively. The top 5 causes of death changed from drowning (17.39/100 000 in mortality, 17.0% in proportion), road injuries (14.77/100 000, 14.4%), self-harm (11.44/100 000, 11.2%), leukemia (5.48/100 000, 5.4%) and interpersonal violence (3.12/100 000, 3.0%) in1990 into road injuries (9.27/100 000, 22.5%), drowning (6.83/100 000, 16.6%), leukemia(2.73/100 000, 6.6%), self-harm (2.53/100 000, 6.2%) and congenital birth defects (1.76/100 000, 4.3%) in 2016, and tuberculosis, poisonings and rheumatic heart disease had dropped out of the top 15 in 2016. Conclusion: The mortality of Chinese adolescents aged 10-19 years has decreased significantly, but still higher than developed countries. Since 1990, injuries,especially for road injuries and drowning, have always been the leading causes of death among Chinese adolescents aged 10-19 years, followed by non-communicable diseases.

PMID: 30107713 [PubMed - indexed for MEDLINE]

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Substantial Cardiovascular Morbidity in Adults with Lower-Complexity Congenital Heart Disease.

Circulation. 2019 Feb 28;:

Authors: Saha P, Potiny P, Rigdon J, Morello M, Tcheandjieu C, Romfh A, Fernandes SM, McElhinney DB, Bernstein D, Lui GK, Shaw GM, Ingelsson E, Priest JR

BACKGROUND: Although lower-complexity cardiac malformations constitute the majority of adult congenital heart disease (ACHD), the long-term risks of adverse cardiovascular events and relationship with conventional risk factors in this population are poorly understood. We aimed to quantify the risk of adverse cardiovascular events associated with lower-complexity ACHD that is unmeasured by conventional risk factors.
METHODS: A multi-tiered classification algorithm was used to select individuals with lower-complexity ACHD and individuals without ACHD for comparison amongst >500,000 British adults in the UK Biobank (UKB). ACHD diagnoses were sub-classified as "isolated aortic valve (AoV)" and "non-complex" defects. Time-to-event analyses were conducted for primary endpoints of fatal or non-fatal acute coronary syndrome (ACS), ischemic stroke, heart failure (HF), and atrial fibrillation, and a secondary combined endpoint for major adverse cardiovascular event (MACE). Maximum follow-up time for the study period was 22 years using retrospectively and prospectively collected data from the UKB.
RESULTS: We identified 2,006 individuals with lower-complexity ACHD and 497,983 unexposed individuals in the UKB (median [IQR] age at enrollment 58 [51,63]). Of the ACHD-exposed group, 59% were male; 51% were current or former smokers; 30% were obese; 69%, 41%, and 7% were diagnosed or treated for hypertension, hyperlipidemia, and diabetes respectively. After adjustment for 12 measured cardiovascular risk factors, ACHD remained strongly associated with the primary endpoints, with hazard ratios (HR) ranging from 2.0 (95% confidence interval [CI] 1.5-2.8, p<0.001) for ACS to 13.0 (95% CI 9.4-18.1, p<0.001) for HF. ACHD-exposed individuals with ≤2 cardiovascular risk factors had a 29% age-adjusted incidence rate of MACE in contrast to 13% in non-ACHD individuals with ≥5 risk factors.
CONCLUSIONS: Individuals with lower-complexity ACHD had higher burden of adverse cardiovascular events relative to the general population that was unaccounted for by conventional cardiovascular risk factors. These findings highlight the need for closer surveillance of patients with mild to moderate ACHD and further investigation into management and mechanisms of cardiovascular risk unique to this growing population of high-risk adults.

PMID: 30813762 [PubMed - as supplied by publisher]

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Left Atrial Wall Trauma Causing Intracardiac Thrombus After Device Closure of Patent Foramen Ovale.

Circ Cardiovasc Imaging. 2019 Mar;12(3):e008720

Authors: Small AJ, Denton KL, Aboulhosn JA

PMID: 30813771 [PubMed - in process]