Abstract 13626: Human Leukocyte Antigen A*30 is Associated With Congenital Heart Disease

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Horacio G Carvajal ◽  
Fei Wan ◽  
Anoop K Brar ◽  
Chang Liu ◽  
Pirooz Eghtesady
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Large Population ◽  
Population Sample ◽  
Hla Antigens ◽  
Human Leukocyte ◽  
Hla Typing ◽  
Race And Gender ◽  
And Gender

Introduction: Congenital heart disease (CHD) arises from many etiologies, ranging from genetic to environmental exposures, such as viral infections. The human leukocyte antigens (HLA) play an important role in the immune response to pathogens. HLA has been extensively explored and linked with several diseases; no such investigations, however, have been done in context of CHD. Hypothesis: We sought to identify potential HLA associations with CHD in a large population sample with molecular-based HLA typing. Methods: Available data on race, gender, HLA-A, B, and DRB1 typing (1-field, antigen level) was collected from the 2,349 patients in the United Network for Organ Sharing (UNOS) database undergoing heart transplant due to CHD between 2005 and 2017. These were compared to all 80,893 deceased kidney donors in the same period. Nominal variables were compared with Pearson’s chi-square, and logistic regression was used to compare all HLA antigens between groups, adjusting for race and gender. The false discovery rate was used to control for multiple comparisons, with adjusted p-values <0.05 considered significant. Results: There were 14 HLA-A, 25 HLA-B, and 14 HLA-DRB1 antigens with an overall frequency of 1% or higher. The frequency of HLA-A*30, B*44, and DRB1*08 differed significantly between groups (Table 1). Unconditional regression showed significantly increased odds of CHD in patients with HLA-A*30 and HLA-DRB1*08, as well as significantly lower odds in those with HLA-B*44 (Table 1). The demographics between groups were comparable, with the exception of fewer Caucasians in the cases (CHD 60.7% vs controls 65.6%, p=0.026). When adjusted for race and gender, only HLA-A*30 remained statistically significant. Conclusions: HLA-A*30 appears to be linked with CHD independent of race. Further analyses with detailed CHD diagnoses and high-resolution HLA typing data are needed to explore potential associations between specific cardiac defects and HLA at the allele level.

scholarly journals Infective Endocarditis in Children With Congenital Heart Disease

Circulation ◽  
2013 ◽  
Vol 128 (13) ◽  
pp. 1412-1419 ◽  
Author(s):  
Dinela Rushani ◽  
Jay S. Kaufman ◽  
Raluca Ionescu-Ittu ◽  
Andrew S. Mackie ◽  
Louise Pilote ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Infective Endocarditis ◽  
Confidence Interval ◽  
Cumulative Incidence ◽  
Congenital Heart ◽  
Large Population ◽  
Heart Association ◽  
Population Based ◽  
Control Analysis

Background— The American Heart Association guidelines for prevention of infective endocarditis (IE) in 2007 reduced the groups of congenital heart disease (CHD) patients for whom antibiotic prophylaxis was indicated. The evidence base in CHD patients is limited. We sought to determine the risk of IE in children with CHD. Methods and Results— We performed a population-based analysis to determine the cumulative incidence and predictors of IE in children (0–18 years) with CHD by the use of the Quebec CHD Database from 1988 to 2010. In 47 518 children with CHD followed for 458 109 patient-years, 185 cases of IE were observed. Cumulative incidence of IE was estimated in the subset of 34 279 children with CHD followed since birth, in whom the risk of IE up to 18 years of age was 6.1/1000 children (95% confidence interval, 5.0–7.5). In a nested case-control analysis, the following CHD lesions were at highest risk of IE in comparison with atrial septal defects (adjusted rate ratio, 95% confidence interval): cyanotic CHD (6.44, 3.95–10.50), endocardial cushion defects (5.47, 2.89–10.36), and left-sided lesions (1.88, 1.01–3.49). Cardiac surgery within 6 months (5.34, 2.49–11.43) and an age of <3 years (3.53, 2.51–4.96; reference, ages 6–18) also conferred an elevated risk of IE. Conclusions— In a large population-based cohort of children with CHD, we documented the cumulative incidence of IE and associated factors. These findings help identify groups of patients who are at the highest risk of developing IE.

Persistence of anti-human leukocyte antibodies in congenital heart disease late after surgery using allografts and whole blood

2013 ◽  
Vol 32 (4) ◽  
pp. 390-397 ◽  
Author(s):  
Matthew J. O’Connor ◽  
Curt Lind ◽  
Xinyu Tang ◽  
Jeffrey Gossett ◽  
Janice Weber ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Whole Blood ◽  
Congenital Heart ◽  
Human Leukocyte

scholarly journals Prognostic factor of heart failure in children with left-to-right shunt acyanotic congenital heart disease

2019 ◽  
Vol 59 (2) ◽  
pp. 63-6
Author(s):  
Weny Inrianto ◽  
Indah K. Murni ◽  
Sri Mulatsih ◽  
Sasmito Nugroho
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Congenital Heart Disease ◽  
Logistic Regression ◽  
Heart Disease ◽  
Prognostic Factor ◽  
Congenital Heart ◽  
Defect Size ◽  
And Gender ◽  
Acyanotic Congenital Heart Disease

Background Anemia is highly prevalent and affects morbidity and mortality in adults with acquired heart disease. However, its role in children with acyanotic congenital heart disease (CHD) is unclear. Objective To assess anemia and other potential prognostic factors of congestive failure in children with left-to-right shunt acyanotic CHD. Methods We conducted a case-control study in the Pediatric Cardiology Clinic, Dr. Sardjito Hospital from January to December 2017 in children with left-to-right shunt acyanotic CHD. The case and control groups consisted of subjects with and without heart failure, respectively. Anemia was defined as hemoglobin concentration <11 g/dL. Measured outcome was the prevalence of congestive heart failure, as determined by the Ross criteria. Anemia, defect type, defect size, age at diagnosis, and gender were analyzed by logistic regression analysis as potential predictive factors of heart failure. Results Of 100 children with left-to-right shunt acyanotic CHD, 50 had heart failure (the case group) and 50 did not (the control group). The prevalence of anemia was 45%. Multivariable logistic regression revealed that defect size was the most significant factor for predicting heart failure, with adjusted OR 7.6 (95%CI 2.5 to 22.8) for moderate shunts and 21.1 (95%CI 6.8 to 65.4) for large shunts. Anemia, type of defect, age of diagnosis, and gender were not statistically significant factors for predicting outcomes. Conclusion Anemia is not a significant, prognostic factor for heart failure in children with left-to-right shunt acyanotic CHD. However, moderate and large shunts in children with left-to-right shunt acyanotic CHD are predictive of the occurrence of congestive heart failure.

Validation of the grown-ups with congenital heart disease score

Heart ◽  
2017 ◽  
Vol 104 (12) ◽  
pp. 1019-1025 ◽  
Author(s):  
Jürgen Hörer ◽  
Régine Roussin ◽  
Emanuel LeBret ◽  
Mohamed Ly ◽  
Jarrah Abdullah ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Hospital Mortality ◽  
Congenital Heart ◽  
Predictive Power ◽  
Large Population ◽  
Postoperative Mortality ◽  
Operating Characteristics ◽  
Mortality And Morbidity ◽  
Additional Procedure

ObjectivesAdults with congenital heart disease in need of heart surgery frequently present with significant comorbidity. Furthermore, additional technical difficulties often related to redo operations increase the risk for postoperative mortality and morbidity. Hence, next to the type of the procedure, additional procedure-dependent and procedure-independent factors have to be considered for risk evaluation. The recently proposed grown-ups with congenital heart disease (GUCH) mortality and morbidity scores account for these additional risk factors. We sought to validate their predictive power in a large population operated in a single centre.MethodsData of all consecutive patients aged 18 years or more, who underwent surgery for congenital heart disease between 2005 and 2016, were collected. Mortality was defined as hospital mortality or mortality within 30 days following surgery. Morbidity was defined as occurrence of one or more of the following complications: renal failure requiring dialysis, neurologic deficit persisting at discharge, atrioventricular block requiring permanent pacemaker implantation, mechanical circulatory support, phrenic nerve injury and unplanned reoperation. The discriminatory power of the GUCH scores was assessed using the area under the receiver operating characteristics curve (c-index, including 95% CI).ResultsEight hundred and twenty-four operations were evaluated. Additional procedure-dependent and procedure-independent factors, as defined in the GUCH scores, were present in 165 patients (20.0%) and 544 patients (66.0%), respectively. Hospital mortality and morbidity was 3.4% and 10.0%, respectively. C-index for GUCH mortality score was 0.809 (0.742–0.877). C-index for GUCH morbidity score was 0.676 (0.619–0.734).ConclusionsWe could confirm the good predictive power of the GUCH mortality score for postoperative mortality in a large population of adults with congenital heart disease.

Impact of socioeconomic status and gender on access to healthcare and outcome in adults with congenital heart disease

2013 ◽  
Vol 34 (suppl 1) ◽  
pp. P2105-P2105
Author(s):  
A. Kempny ◽  
K. Dimopoulos ◽  
O. Tutarel ◽  
A. Tutaj ◽  
D. Marchewka ◽  
...  
Keyword(s):  
Socioeconomic Status ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Access To Healthcare ◽  
And Gender

scholarly journals Status of Pediatric Cardiac Care in Developing Countries

Children ◽  
2019 ◽  
Vol 6 (2) ◽  
pp. 34 ◽  
Author(s):  
Anita Saxena
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Low Income ◽  
Congenital Heart ◽  
Large Population ◽  
Developed Countries ◽  
Optimal Care ◽  
Middle Income ◽  
Daunting Task ◽  
Challenges And Opportunities

About 1.35 million babies are born with congenital heart disease each year globally. Most of these are expected to lead normal, productive lives if they are treated in time. However, 90% of babies born with congenital heart disease live in regions where medical care is inadequate or unavailable. The privilege of early diagnosis and timely intervention is restricted to only those born in developed countries. Added to the burden of congenital heart disease is rheumatic heart disease, which remains a global health problem in many low-income and middle-income countries. Providing optimal care for all these children is a daunting task, and requires funds and proper planning at various levels of the health care system. This article describes the burden of pediatric heart disease, including lacunae in the current state, as well as challenges and opportunities for providing optimal care to this large population of children.

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