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Risk factors for congenital heart disease: The Baby Hearts Study, a population-based case-control study.

PLoS One. 2020;15(2):e0227908

Authors: Dolk H, McCullough N, Callaghan S, Casey F, Craig B, Given J, Loane M, Lagan BM, Bunting B, Boyle B, Dabir T

We investigated the role of maternal environmental factors in the aetiology of congenital heart disease (CHD). A population-based case-control study (242 CHD cases, 966 controls) was conducted using an iPad questionnaire for mother with linkage to maternity and first trimester prescription records. Risk of CHD was associated with low maternal education (OR adjusted for confounders 1.59; 95% confidence interval [CI], 1.02-2.49), pregestational diabetes (OR 4.04; 95% CI 1.00-16.28), self-reported maternal clotting disorders (adjOR 8.55, 95%CI 1.51-48.44), prescriptions for the anticlotting medication enoxaparin (adjOR 3.22, 95%CI 1.01-10.22) and self-reported vaginal infections (adjOR 1.69, 95%CI 1.01-2.80). There was no strong support for the hypothesis that periconceptional folic acid supplements have a protective effect, but there was a protective effect of frequent consumption of folate rich fruits (adjOR 0.64, 95%CI 0.47-0.89). Compared to the most common pre-pregnancy dietary pattern, CHD risk was associated with a poor diet low in fruit and vegetables (adjOR 1.56, 95%CI 1.05-2.34). Mothers of cases reported more pregnancy related stress (adjOR 1.69; 95% CI 1.22-2.34) and multiple stressors (adjOR 1.94, 95%CI 0.83-4.53). We found no supportive evidence for CHD risk being associated with obesity, smoking, depression or antidepressant use in this population. Our findings add to the previous evidence base to show potential for public health approaches to help prevent CHD in future by modifying environmental factors. Independent confirmation should be sought regarding elevated CHD risk associated with maternal blood clotting disorders and their treatment, since we are the first to report this.

PMID: 32092068 [PubMed - indexed for MEDLINE]

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Tissue oxygen saturation assessment of microvascular perfusion in adults with Fontan palliation and comparator groups using vascular optical spectrophotometry: a pilot study.

Physiol Meas. 2019 07 01;40(6):06NT01

Authors: Agarwal R, Chaudhry B, Jansen K, O'Sullivan J, Hudson M, Allen J, Coats L

OBJECTIVE: The Fontan operation greatly improves survival for single ventricle congenital heart disease patients but creates a physiology that leads to long-term multi-organ dysfunction. A non-invasive screening tool that can identify impending decline is sought. The objective of this pilot study was to assess the microcirculation in Fontan-palliated patients by measuring tissue oxygen saturation (StO2) in superficial and deeper tissues.
APPROACH: Three patient cohorts were studied: Fontan group (n  =  8) and two patient control groups, liver disease group (n  =  8) and tetralogy of Fallot group (n  =  9). 22 healthy controls were also examined. Superficial and deeper StO2 was measured at the forearm, thenar eminence, index and ring fingers of both arms using the LEA O2C spectrophotometry device.
MAIN RESULTS: Superficial StO2 was reduced in Fontan patients compared to healthy controls (p   =  0.002) and tetralogy patients (p   =  0.016), but not compared to the liver group (p   =  0.313). Deeper StO2 was similar between groups (p   =  0.112). The gap between deeper and superficial StO2 was raised in Fontan patients compared to healthy controls (p   =  0.001) and tetralogy patients (p   =  0.037), but not compared to the liver group (p   =  0.504). There was no clinically relevant difference in StO2 between the left and right arms, and the variation in StO2 according to measurement site was similar between the four groups.
SIGNIFICANCE: Vascular optical spectrophotometry is a feasible non-invasive measure of micro-circulatory function that can easily be performed in the clinic setting and may have utility in patients with Fontan circulations. Further, we provide important normal range data in the healthy control population which can be used to design future studies.

PMID: 31051474 [PubMed - indexed for MEDLINE]

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Commentary: Shunting Between the Left Ventricle and Right Atrium Can Be Produced by Straddling Tricuspid Valve With Dual Orifices.

Semin Thorac Cardiovasc Surg. 2020;32(1):143-144

Authors: Lopes BS, Coats L, Anderson RH

PMID: 31586467 [PubMed - indexed for MEDLINE]

The globe on the spotlight: Coronavirus disease 2019 (Covid-19).

Int J Cardiol. 2020 Apr 03;:

Authors: Brida M, Chessa M, Gu H, Gatzoulis MA

PMID: 32321654 [PubMed - as supplied by publisher]

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Potential Value of Native T1 Mapping in Symptomatic Adults with Congenital Heart Disease: A Preliminary Study of 3.0 Tesla Cardiac Magnetic Resonance Imaging.

Pediatr Cardiol. 2020 Jan;41(1):94-100

Authors: Shiina Y, Inai K, Taniguchi K, Takahashi T, Nagao M

The native T1 value at 3.0 Tesla is a sensitive marker of diffuse myocardial damage. We evaluated the clinical usefulness of native T1 mapping in symptomatic adults with congenital heart disease (CHD), particularly in the systemic right ventricle (RV). Prospectively, 45 consecutive symptomatic adults with CHD were enrolled: 20 with systemic RV and 25 with tetralogy of Fallot underwent cardiac magnetic resonance (CMR) imaging at 3.0 Tesla. The Modified Look-Locker Inversion recovery sequence was used for T1 mapping. Cardiovascular events in the systemic RV were defined as heart failure and tachyarrhythmia. Brain natriuretic peptide (BNP) and indexed systemic ventricular end-diastolic volume were significantly higher in the systemic RV group. The native T1 value and extracellular volume (ECV) of the septal and lateral walls were higher in the systemic RV group, suggesting high impairment of the myocardium in the systemic RV group. There was a strong correlation between the native T1 value and ECV of the septum (r = 0.58, P = 0.03) and lateral wall (r = 0.56, P = 0.046) in the systemic RV group. Seven patients with systemic RV had cardiovascular events. In univariate logistic regression analysis, BNP and native T1 values of the insertion point were important for predicting cardiovascular events. The native T1 value at 3.0 Tesla may be a sensitive, contrast-free, and non-invasive adjunct marker of myocardial damage in CHD and predictive of cardiovascular events in the systemic RV.

PMID: 31654097 [PubMed - indexed for MEDLINE]

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Factors associated with long-term cardiac dysfunction in neonatal lupus.

Ann Rheum Dis. 2020 02;79(2):217-224

Authors: Saxena A, Izmirly PM, Bomar RP, Golpanian RS, Friedman DM, Eisenberg R, Kim MY, Buyon JP

OBJECTIVES: Cardiac manifestations of neonatal lupus (NL) have been associated with significant morbidity and mortality; however, there is minimal information on long-term outcomes of affected individuals. This study was initiated to evaluate the presence of and the risk factors associated with cardiac dysfunction in NL after birth in multiple age groups to improve counselling, to further understand pathogenesis and to provide potential preventative strategies.
METHODS: Echocardiogram reports were evaluated in 239 individuals with cardiac NL: 143 from age 0-1 year, 176 from age >1-17 years and 64 from age >17 years. Logistic regression analyses evaluated associations of cardiac dysfunction at each age group with demographic, fetal and postnatal factors, using imputation to address missing data.
RESULTS: Cardiac dysfunction was identified in 22.4% at age 0-1 year, 14.8% at age >1-17 years and 28.1% at age >17 years. Dysfunction in various age groups was significantly associated with male sex, black race, lower fetal heart rates, fetal extranodal cardiac disease and length of time paced. In 106 children with echocardiograms at ages 0-1 year and >1-17 years, 43.8% with dysfunction at age 0-1 year were also affected at age >1-17 years, while the others reverted to normal. Of children without dysfunction at age 0-1 year, 8.9% developed new dysfunction between ages >1 and 17 years. Among 34 with echocardiograms at ages >1-17 years and >17 years, 6.5% with normal function at age >1-17 years developed dysfunction in adulthood.
CONCLUSIONS: Risk factors in fetal life can influence cardiac morbidity into adulthood.Although limited by a small number of cases, cardiac dysfunction in the first year often normalises by later childhood. New-onset dysfunction, although rare, can occur de novo after the first year.

PMID: 31672776 [PubMed - indexed for MEDLINE]

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Health Related Quality of Life of Children with Congenital Heart Disease Attending at Tertiary Level Hospital.

J Nepal Health Res Counc. 2019 Nov 13;17(3):288-292

Authors: Mishra TA, Sharma P

BACKGROUND: Congenital heart disease is one of the common congenital anomaly among the children affecting growth and development of the child and increasing susceptibility of the child to failure to thrive. The objective of the study is to find out the overall health related quality of life of children with congenital heart disease.
METHODS: A descriptive cross sectional study was carried out among 150 children with congenital heart disease attending in the pediatric out-patient department of Shahid Gangalal National Heart Centre, Bansbari, Kathmandu. The data was collected within the period of 2017/07/16 to 2017/08/16 using purposive sampling technique. Data was collected through interview technique using semi structured questionnaire. Collected data were entered in Statistical Package for Social Science 16 version and analyzed by using descriptive and inferential statistics.
RESULTS: The study findings revealed that more than half (52.7%) of the children had satisfactory overall health related quality of life. Health related quality of life is significantly associated with age group of the child (p=0.018), and education of mother (p=0.017)).
CONCLUSIONS: It can be concluded that more than half of the children tends to have satisfactory overall health related quality of life.Ventricular septal defect is the commonest congenital heart disease. The health related quality of life of children with congenital heart disease tends to be better among those with educated mother and increasing age of the children. Therefore, awareness raising of mothers related to disease condition and care of children with congenital heart disease through mass media or proper intervention program might help to promote the quality of life of children with congenital heart disease.

PMID: 31735919 [PubMed - indexed for MEDLINE]

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A Woman with Systemic Lupus Erythematosus and Odd Valvular Presentation: A Case Report.

Am J Case Rep. 2019 Nov 19;20:1705-1708

Authors: Bazyar Z, Moaref A, Amirghofran AA, Nazarinia M, Kojuri J

BACKGROUND Systemic lupus erythematosus (SLE) is a systemic disease with various cardiac and non-cardiac presentations. We present the case of a young woman with odd presentation of SLE mistakenly identified as a valve abscess that was scheduled for surgery. CASE REPORT This 35-year-old woman presented with rapid progression of aortic stenosis, and the transesophageal echocardiography report showed a misdiagnosed aortic web (congenital) and aortic wall abscess. She was scheduled for surgery as a case of subacute bacterial endocarditis (SBE) and aortic abscess, despite lack of fever. CONCLUSIONS Cardiovascular involvement should be considered in any SLE patient, especially those with high SLE scores, even with negative antiphospholipid antibody. Cardiovascular involvement may be odd and misleading in some cases, which may warrant especial attention and experienced caregivers for clinical reasoning and proper management.

PMID: 31740655 [PubMed - indexed for MEDLINE]

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Associations between maternal social support and stressful life event with ventricular septal defect in offspring: a case-control study.

BMC Pregnancy Childbirth. 2019 Nov 21;19(1):429

Authors: Lyu J, Zhao K, Xia Y, Zhao A, Yin Y, Hong H, Li S

BACKGROUND: Previous studies suggested that maternal subjective feeling of stress seemed to be involved in the incidence of congenial heart disease in offspring. To better understand the findings, our study would discuss the relationships of maternal exposure to stressful life event and social support, which are more objective and comprehensive indicators of stress, around periconceptional period with the risk of ventricular septal defect (VSD), the most popular subtype of congenital heart disease.
METHODS: A hospital-based case-control study was conducted through June, 2016 to December, 2017. We collected maternal self-reports of 8 social support questions in 3 aspects and 8 stressful life events among mothers of 202 VSD cases and 262 controls. Social support was categorized into low, medium high, and high (higher is better), and stressful life event was indexed into low, medium low, and high (higher is worse). Logistic regression models were applied to estimate adjusted odds ratios and 95% confidence intervals (95% CI).
RESULTS: The adjusted odds ratio of high stressful life event was 2.342 (95% CI: 1.348, 4.819) compared with low stressful life event. After crossover analysis, compared with low event & high support, the adjusted odds ratio of low event & low support, high event & high support, and high event & low support were 2.059 (95% CI: 1.104, 3.841), 2.699 (95% CI: 1.042, 6.988) and 2.781 (95% CI: 1.033, 7.489), respectively.
CONCLUSIONS: In summary, we observed an increased risk of VSD when pregnant women exposed to stressful life events, however, social support could, to some extent, reduce the risk of stressful life event.

PMID: 31752736 [PubMed - indexed for MEDLINE]

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Risk Factors and Outcomes of Tetralogy of Fallot: From Fetal to Neonatal Life.

Pediatr Cardiol. 2020 Jan;41(1):155-164

Authors: Silva JA, Neves AL, Flor-de-Lima F, Soares P, Guimarães H

Tetralogy of Fallot (ToF) is the most prevalent cyanotic congenital heart disease. Genetic syndromes are present in up to one quarter of patients with this condition, leading to increased morbidity and mortality. Our aim in this work is to characterize our population, evaluate ToF based on the presence of genotype anomalies, and investigate early intervention predictors and outcomes. A retrospective study was performed on neonates with ToF born between August 1, 2008, and August 31, 2018, and admitted to a level III neonatal intensive care unit (NICU). Patients were categorized based on the presence of genotype anomalies and timing of intervention. Thirty-nine neonates were included. The overall mortality during the follow-up period was 5.1% (n = 2). Threatened preterm labor/preterm labor was more prevalent in patients with associated genotype anomalies (p = 0.015). Multivariate analysis showed an association between an abnormal amount of amniotic fluid and ToF with altered genotype, adjusted for smoking, maternal age, gestational age and birth weight [OR = 29.92, 95% CI (1.35-662.44), p = 0.032]. We also found an association between cesarean delivery and neonatal procedures (p = 0.006). Mortality was significantly higher in neonates who underwent early intervention (p = 0.038). Our results indicate that an abnormal amount of amniotic fluid is an independent predictive factor for ToF with genotype alterations. This finding could ultimately have an impact on both prenatal and neonatal counseling and management.

PMID: 31768578 [PubMed - indexed for MEDLINE]