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Frontiers in Cardiovascular Medicine 2021This study aimed to identify the clinical features of Kawasaki disease shock syndrome (KDSS) in children. The case-control studies of KDSS and KD children up until...
This study aimed to identify the clinical features of Kawasaki disease shock syndrome (KDSS) in children. The case-control studies of KDSS and KD children up until April 30, 2021 were searched in multiple databases. The qualified research were retrieved by manually reviewing the references. Review Manager 5.3 software was used for statistical analysis. The results showed that there was no significant difference in the incidence of male and female in children with KDSS. Children with KDSS compared with non-shocked KD, there were significant difference in age, duration of fever, white blood cell (WBC) count, percentage of neutrophils (NEUT%), platelet count (PLT), c-reactive protein level (CRP), alanine transaminase concentration (ALT), aspartate transaminase concentration (AST), albumin concentration (ALB), sodium concentration (Na), ejection fraction, and length of hospitalization as well as the incidence of coronary artery dilation, coronary artery aneurysm, left ventricular dysfunction, mitral regurgitation, pericardial effusion, initial diagnosis of KD, intravenous immunoglobulin (IVIG) resistance and receiving second dose of IVIG, vasoactive drugs, hormones, and albumin. In contrast, there was no difference in the hemoglobin concentration, erythrocyte sedimentation rate, and the incidence of conjunctival injection, oropharyngeal change, polymorphous rash, extremity change, and incomplete KD. Current evidence suggested that the children with KDSS had more severe indicators of inflammation and more cardiac abnormalities. These patients were resistant to immunoglobulin treatment and required extra anti-inflammatory treatment. PROSPERO registration number CRD42021241207.
PubMed: 34621802
DOI: 10.3389/fcvm.2021.736352 -
BMC Cardiovascular Disorders Feb 2018Myxomatous mitral valve prolapse is a common cardiac abnormality. Morbus Barlow is characterized by excess myxomatous leaflet tissue, bileaflet prolapse or billowing,...
BACKGROUND
Myxomatous mitral valve prolapse is a common cardiac abnormality. Morbus Barlow is characterized by excess myxomatous leaflet tissue, bileaflet prolapse or billowing, chordae elongation and annular dilatation with or without calcification. Extensive myxoid degeneration with destruction of the normal three-layered leaflet tissue architecture is observed histologically in such patients. Autosomal dominant inheritance with an age and sex-dependent expression has long been recognised. This review explores the current understanding of the genetics of bileaflet prolapse, with a focus on genetic analysis and the role for echocardiographical screening of the first degree relatives of affected patients.
METHODS
Systematic literature searches were performed using PubMed and Embase up to September 2017. In Disse et al.'s study (study one) first degree relatives of 25 patients with Morbus Barlow who underwent mitral valve repair were screened for bileaflet valve prolapse. In Nesta et al.'s study one family with three living generations of 43 individuals with 9 confirmed cases of MVP was screened. Genotyping was performed in four families for 344 microsatellite markers from Chromosome 1 to 16.
RESULTS
In study one, autosomal dominant inheritance was shown in four pedigrees. Genome-wide linkage analysis of the most informative pedigree (24 individuals, three generations) showed a significant linkage for markers mapping to chromosome 16p. Linkage to this locus was confirmed in a second family within the same study, but was excluded in the remaining two pedigrees. In study two an autosomal dominant locus was mapped to chromosome 13. 8 of the 9 individuals affected were found to suffer from bileaflet prolapse.
CONCLUSIONS
Barlow's disease is a heritable trait but the genetic causes remain largely elusive. Ch16p11.2-p12.1 is the only locus proven to be associated with bileaflet prolapse. Locus 13.q31.3-q32.1 was shown to cause bileaflet as well as posterior leaflet prolapse. This review intends to make physicians aware of genetic causes of myxomatous mitral valve prolapse, thereby emphasising the importance of cardiological examination of first-degree relatives of patients with Morbus Barlow. Integrated and more comprehensive studies are needed for identification of genes involved in this heterogenic disease. Further genomic studies may facilitate more individualised and accurate risk assessment and may help to develop possible preventive stategies for patients in the future.
Topics: Adult; Echocardiography; Female; Genetic Markers; Genetic Predisposition to Disease; Genome-Wide Association Study; Heredity; Humans; Male; Middle Aged; Mitral Valve; Mitral Valve Prolapse; Pedigree; Prognosis; Risk Factors
PubMed: 29486707
DOI: 10.1186/s12872-018-0755-y -
Pediatric Cardiology Apr 2023There is a paucity of longitudinal data on cardiac outcomes in multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. We aimed to investigate... (Meta-Analysis)
Meta-Analysis
There is a paucity of longitudinal data on cardiac outcomes in multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. We aimed to investigate the longitudinal cardiovascular outcomes in MIS-C. PubMed and EMBASE were searched through May 2022. Observational studies were included, reporting mid-term (≥ 3 months) outcomes in children (aged < 21) with MIS-C. Data were extracted by two researchers. Longitudinal outcomes were synthesized by a one-group meta-analysis using a random-effects model. Eleven studies with a follow-up period (3 months to 1 year) were identified, including 547 MIS-C patients. The mortality was 2.5% (95% CI 1.3-4.9). The majority of left ventricular (LV) systolic dysfunction present in 46.8% (95% CI 32.7-61.3) in the acute phase resolved by 3 months, and the prevalence of LV systolic dysfunction was 1.7% (95% CI 0.5-5.7) and 2.1% (95% CI 0.8-5.4) at 3 month and 6 month follow-up, respectively. Additionally, the persistent LV systolic dysfunction in the small population was mild. However, coronary abnormalities such as coronary artery dilatation or aneurysms, seen in 23.7% (95% CI 17.7-31.1) at baseline, persisted in 4.7% (95% CI 1.5-14.3) at 3 months and 5.2% (95% CI 3.0-8.9) at 6 months. Mitral regurgitation (MR), which was observed in 56.6% (95% CI 27.7-81.6) at baseline, also persisted in 7.5% at 6 months. In conclusion, our study demonstrated largely favorable cardiac outcomes, suggesting resolution of LV systolic dysfunction in the majority of cases. However, coronary abnormalities and MR persisted in a subset of patients at mid-term follow-up.
Topics: Child; Humans; COVID-19; Heart; Systemic Inflammatory Response Syndrome; Mitral Valve Insufficiency; Ventricular Dysfunction, Left
PubMed: 36416893
DOI: 10.1007/s00246-022-03052-2 -
BMC Cardiovascular Disorders Mar 2012Maternal mortality in South Africa is rising, and heart conditions currently account for 41 per cent of indirect causes of deaths. Little is known about the burden of... (Review)
Review
BACKGROUND
Maternal mortality in South Africa is rising, and heart conditions currently account for 41 per cent of indirect causes of deaths. Little is known about the burden of heart disease in pregnant South Africans.
METHODS
We systematically reviewed the contemporary epidemiology and peripartum outcomes of heart disease in South African women attending antenatal care. Searches were performed in PubMed, ISI Web of Science, the EBSCO Africa-Wide database, the South African Union Catalogue, and the Current and Completed Research database (South Africa). References of included articles were also hand-searched. Studies reporting epidemiologic data on antenatal heart disease in South Africa were included. Data on morbidity and mortality were also collected.
RESULTS
Seven studies were included in the systematic review. The prevalence of heart disease ranged from 123 to 943 per 100,000 deliveries, with a median prevalence of 616 per 100,000. Rheumatic valvular lesions were the commonest abnormalities, although cardiomyopathies were disproportionately high in comparison with other developing countries. Peripartum case-fatality rates were as high as 9.5 per cent in areas with limited access to care. The most frequent complications were pulmonary oedema, thromboembolism, and major bleeding with warfarin use. Perinatal mortality ranged from 8.9 to 23.8 per cent, whilst mitral lesions were associated with low birth weight. Meta-analysis could not be performed due to clinical and statistical heterogeneity of the included studies.
CONCLUSION
Approximately 0.6 per cent of pregnant South Africans have pre-existing cardiac abnormalities, with rheumatic lesions being the commonest. Maternal and perinatal morbidity and mortality continue to be very high. We conclude this review by summarising limitations of the current literature and recommending standard reporting criteria for future reports.
Topics: Female; Fetal Death; Heart Diseases; Humans; Maternal Health Services; Maternal Mortality; Perinatal Mortality; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Prenatal Care; Prenatal Diagnosis; Prevalence; South Africa
PubMed: 22463484
DOI: 10.1186/1471-2261-12-23 -
PloS One 2024Echogenic Intracardiac Foci (EIF) are non-structural markers identified during the routine 18-20-week foetal anomaly ultrasound scan yet their clinical significance on...
BACKGROUND
Echogenic Intracardiac Foci (EIF) are non-structural markers identified during the routine 18-20-week foetal anomaly ultrasound scan yet their clinical significance on future outcomes for the infant is unclear.
OBJECTIVE
To examine the association between EIF and risk of preterm birth, chromosomal abnormalities, and cardiac abnormalities.
DESIGN
A review across four databases to identify English language journal articles of EIF using a cohort study design. All studies were reviewed for quality using the Critical Appraisal Skills Programme (CASP) checklist and data extracted for comparison and analysis.
RESULTS
19 papers from 9 different countries were included. Combining these studies showed 4.6% (95% CI = 4.55-4.65%) of all pregnancies had EIF which was on the left in 86% of cases, on the right in 3% of cases and bilaterally in 10%. There was no evidence that EIF was associated with higher rates of preterm birth. However, it is possible that infants with EIF were more likely to be terminated rather than be born preterm as there was a 2.1% (range 0.3-4.2%) rate of termination or death of the foetus after week 20 among those with EIF. There was no evidence that EIF alone is highly predictive of chromosomal abnormalities. There was evidence that EIF is associated with higher rates of minor cardiac abnormalities (e.g. ventricular septal defect, tricuspid regurgitation or mitral regurgitation)) with 5.1% (224 of 4385) of those with EIF showing cardiac abnormalities (3.08% in retrospective studies and 17.85% in prospective studies). However, the risk of cardiac defects was only higher with right-sided EIF and where the EIF persisted into the third trimester. However, this is a rare event and would be seen in an estimated 4 per 10,000 pregnancies.
CONCLUSION
EIF alone was not associated with adverse outcomes for the infant. Only persistent EIF on the right side showed evidence of carrying a higher risk of cardiac abnormality and would warrant further follow-up.
Topics: Female; Humans; Pregnancy; Chromosome Aberrations; Heart Defects, Congenital; Pregnancy Outcome; Pregnancy Trimester, Second; Premature Birth; Ultrasonography, Prenatal
PubMed: 38648215
DOI: 10.1371/journal.pone.0298365 -
Fetal Diagnosis and Therapy 2022Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This systematic review and meta-analysis aimed to synthesize the evidence for the role of fetal echocardiography in predicting the postnatal treatment pathway.
METHODS
PubMed/MEDLINE, CINHAL, Cochrane Library, Academic Search Complete, Web of Science, and Trip Pro were searched for observational studies published before July 2021. Random-effects meta-analysis was performed to identify factors associated with biventricular repair.
RESULTS
Eleven individual studies published between 2006 and 2021, including a total of 285 participants (159 biventricular repair; 126 univentricular palliation), met our eligibility criteria. The pooled estimated prevalence of biventricular repair among patients with PSAIVS was 55.6% (95% confidence interval 48.5-62.5%). Those who underwent biventricular repair had greater right to left ventricle and tricuspid to mitral valve dimension ratios, greater TV z score, and longer TV inflow duration/cardiac cycle length by fetal echocardiography. They were also more likely to have significant tricuspid regurgitation and less likely to have ventriculo-coronary connections (VCCs).
CONCLUSIONS
Commonly obtained fetal echocardiographic measurements have strong associations with treatment pathway choice for patients with PSAIVS. Greater RV growth appears to favor biventricular repair, whereas patients with VCC almost invariably undergo univentricular palliation. Future studies should aim to establish how these fetal echocardiographic parameters might predict outcomes for the two treatment pathways.
Topics: Echocardiography; Female; Heart Defects, Congenital; Heart Ventricles; Humans; Pregnancy; Pulmonary Atresia; Pulmonary Valve Stenosis; Treatment Outcome; Ultrasonography, Prenatal; Ventricular Septum
PubMed: 35793649
DOI: 10.1159/000525718 -
Case Reports in Genetics 2020Shprintzen-Goldberg craniosynostosis syndrome (SGS) is a rare autosomal dominant condition that was first documented in literature in 1982. The disorder is caused by...
Shprintzen-Goldberg craniosynostosis syndrome (SGS) is a rare autosomal dominant condition that was first documented in literature in 1982. The disorder is caused by pathogenic variants in the proto-oncogene gene, a known suppressor of TGF- activity, located on chromosome 1p36. There is considerable phenotypic overlap with Marfan and Loeys-Dietz syndromes. Common clinical features of SGS include craniosynostosis, marfanoid habitus, hypotonia, dysmorphic facies, cardiovascular anomalies, and other skeletal and connective tissue abnormalities. Ocular manifestations may include hypertelorism, downslanting palpebral fissures, proptosis, myopia, and ectopia lentis. We describe a 25-year-old male with the syndrome. Genetic analysis revealed a novel c.350G>A (p.Arg117His) variant, which was predicted to be pathogenic by the CTGT laboratory. The patient presented with dysmorphic features, marfanoid habitus, severe joint contractures, mitral valve insufficiency, aortic root dilatation, and a history of seizures. His ocular manifestations included hypertelorism, downslanting palpebral fissures, bilateral ptosis, and high myopia. Ophthalmic manifestations are an integral component of the syndrome; however, they have not been well characterized in the literature. From a systematic review of previously published cases to date, we summarize the eye and ocular adnexa manifestations reported.
PubMed: 33628537
DOI: 10.1155/2020/7353452 -
Journal of Cardiopulmonary... Nov 2018Type 2 diabetes (T2D) is associated with subclinical abnormalities in left ventricular function and an increased downstream risk for heart failure. Exercise training has... (Meta-Analysis)
Meta-Analysis
PURPOSE
Type 2 diabetes (T2D) is associated with subclinical abnormalities in left ventricular function and an increased downstream risk for heart failure. Exercise training has been associated with significant improvement in cardiorespiratory fitness among these patients. However, its impact on cardiac function is not well established.
METHODS
We conducted a meta-analysis including all randomized and nonrandomized trials that evaluated effects of exercise training on cardiac function among patients with T2D. Primary outcomes were measures of left ventricular systolic (global longitudinal strain) and diastolic (early diastolic velocity [é]) function. The effects of exercise training on peak oxygen uptake; other markers of diastolic dysfunction: mitral peak early-to-late diastolic filling velocity (E/A), mitral inflow to annular ratio (E/é), and deceleration time (DT); and systolic velocity were also assessed.
RESULTS
Our study included 441 patients enrolled in 6 trials. Exercise training significantly improved early diastolic velocity (standardized mean difference [SMD], 0.58; 95% CI, 0.09-1.07), global longitudinal strain (SMD, 0.62; 95% CI, 0.04-1.21), and peak oxygen uptake (SMD, 1.43; 95% CI, 0.51-2.35) as compared with control group. However, no significant changes were observed in other markers of diastolic function (E/A, E/é and DT) and systolic velocity.
CONCLUSION
Exercise training in patients with T2D is associated with a significant improvement in some echocardiographic indicators of systolic and diastolic function and cardiorespiratory fitness. These findings suggest that exercise training may improve subclinical systolic and diastolic dysfunction in patients at risk for clinical heart failure.
Topics: Diabetes Mellitus, Type 2; Exercise; Exercise Therapy; Humans; Oxygen Consumption; Randomized Controlled Trials as Topic; Ventricular Dysfunction, Left
PubMed: 30142130
DOI: 10.1097/HCR.0000000000000353 -
Current Problems in Cardiology Mar 2021Abnormal cardiovascular changes especially hypertrophic cardiomyopathy is potentially expected in the fetuses of the diabetic pregnancy women. However, there is still... (Meta-Analysis)
Meta-Analysis
Abnormal cardiovascular changes especially hypertrophic cardiomyopathy is potentially expected in the fetuses of the diabetic pregnancy women. However, there is still little consensus on quantitative cardiac abnormalities in infants with diabetic mothers. The present study comprehensively analyzed the studies on functional changes in heart in infants of diabetic mothers with a greater focus on occurrence of hypertrophic cardiomyopathy. All comparative studies evaluating and comparing quantitatively the changes in cardiac parameters using echocardiography in fetuses with and without diabetic mothers were eligible for assessment. The included studies were identified through electronically reviewing the manuscripts databases of MEDLINE, EMBASE, Web of knowledge, and Google Scholar from inception to May 2020. The meta-analysis included 11 comparative with overall 849 fetuses for gestational diabetic mothers and 1247 for healthy mothers. Assessing cardiac diameters by fetal echocardiography showed significantly lower mitral E/A ratio, lower tricuspid E/A ratio, higher interventricular septal thickness, higher myocardial performance index, higher isovolumic relaxation time, and higher isovolumic contraction time in fetuses of gestational diabetes mellitus group as compared to healthy group adjusting for gestational diabetes mellitus. The presence of gestational diabetes mellitus can potentially affect the fetal cardiac parameters especially as hypertrophic cardiomyopathy leading both cardiac systolic and diastolic dysfunction.
Topics: Diabetes, Gestational; Echocardiography; Female; Fetal Heart; Gestational Age; Humans; Pregnancy; Ultrasonography, Prenatal
PubMed: 32861465
DOI: 10.1016/j.cpcardiol.2020.100658 -
Journal of the American College of... Feb 2010The purpose of this study was to perform a systematic review and meta-analysis of comparative studies to compare outcomes of septal ablation (SA) with septal myectomy... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
The purpose of this study was to perform a systematic review and meta-analysis of comparative studies to compare outcomes of septal ablation (SA) with septal myectomy (SM) for treatment of hypertrophic obstructive cardiomyopathy (HOCM).
BACKGROUND
SM is considered the gold standard for treatment of HOCM. However, SA has emerged as an attractive therapeutic alternative.
METHODS
A Medline search using standard terms was conducted to determine eligible studies. Due to a lack of randomized control trials, we included observational studies for review.
RESULTS
Twelve studies were found eligible for review. No significant differences between short-term (risk difference [RD]: 0.01; 95% confidence interval [CI]: -0.01 to 0.03) and long-term mortality (RD: 0.02; 95% CI: -0.05 to 0.09) were found between the SA and SM groups. In addition, no significant differences could be found in terms of post-intervention functional status as well as improvement in New York Heart Association functional class, ventricular arrhythmia occurrence, re-interventions performed, and post-procedure mitral regurgitation. However, SA was found to increase the risk of right bundle branch block (RBBB) (pooled odds ratio [OR]: 56.3; 95% CI: 11.6 to 273.9) along with need for permanent pacemaker implantation post-procedure (pooled OR: 2.6; 95% CI: 1.7 to 3.9). Although the efficacy of both SA and SM in left ventricular outflow tract gradient (LVOTG) reduction seems comparable, there is a small yet significantly higher residual LVOTG amongst the SA group patients as compared with the SM group patients.
CONCLUSION
SA does seem to show promise in treatment of HOCM owing to similar mortality rates as well as functional status compared with SM; however, the caveat is increased conduction abnormalities and a higher post-intervention LVOTG. The choice of treatment strategy should be made after a thorough discussion of the procedures with the individual patient.
Topics: Cardiomyopathy, Hypertrophic; Catheter Ablation; Ethanol; Female; Heart Septum; Humans; Male; Treatment Outcome
PubMed: 20170823
DOI: 10.1016/j.jacc.2009.09.047