scholarly journals Pulmonary hypertension in the changing landscape of congenital heart disease: Global differences and a possible driver of end-stage heart failure

2016 ◽  
Vol 24 (6) ◽  
pp. 372-373 ◽  
Author(s):  
A. Van De Bruaene ◽  
W. Budts
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
End Stage Heart Failure ◽  
End Stage

Polymorphisms in cytokine genes do not predict progression to end-stage heart failure in children

2002 ◽  
Vol 12 (5) ◽  
pp. 461-464 ◽  
Author(s):  
Steven A. Webber ◽  
Gerard J. Boyle ◽  
Steven Gribar ◽  
Yuk Law ◽  
Pamela Bowman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Inflammatory Cytokines ◽  
Congenital Heart ◽  
Healthy Children ◽  
Ventricular Failure ◽  
Cytokine Genes ◽  
End Stage Heart Failure ◽  
End Stage

A number of cytokines have been implicated in the pathophysiology of congestive heart failure. Genetic polymorphisms of several cytokine genes are known to result in altered gene expression, enabling us to characterize patients as "high" or "low" producers of specific cytokines. We speculate that the cytokine genotypes for a population of children who underwent heart transplantation for end-stage ventricular failure due to cardiomyopathy or congenital heart disease would be enriched for "high producers" of pro-inflammatory cytokines and "low producers" of anti-inflammatory cytokines. Methods: Cytokine genotyping was performed for the following cytokines on 94 transplanted children using polymerase chain reaction-sequence specific technique: tumor necrosis factor-α (−308), interleukin 10 (−1082, −819, −592), interleukin 6 (−174), transforming growth factor-β1(codons 10 & 25), and interferon-γ (+874). Patients with ventricular failure after transplantation for dilated cardiomyopathy, numbering 37, or for congenital heart disease, numbering 34, were compared to 15 children transplanted for structural disease, such as hypoplastic left heart syndrome, without ventricular failure, and to data from healthy children. An additional 8 children with restrictive or hypertrophic cardiomyopathy were also studied. Results: No differences in genotypic distribution were seen between the groups, and all patients were comparable to genotypic distributions as assessed from published normal data. Conclusion: No evidence is found to support the hypothesis that these polymorphisms for cytokine genes influence progression to end-stage heart failure in children undergoing transplantation because of cardiomyopathy or congenital heart disease.

scholarly journals Is it possible to operate four heart valves in a patient with heart failure, congenital heart disease and pulmonary hypertension?

Cor et Vasa ◽  
2012 ◽  
Vol 54 (9-10) ◽  
pp. e295-e299 ◽  
Author(s):  
Marie Zvěřinová ◽  
Jana Popelová ◽  
Petr Pavel ◽  
Roman Gebauer ◽  
Pavel Jehlička ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Valves

scholarly journals Predictor factors of pulmonary hypertension in children with left-to-right shunting in acyanotic congenital heart disease

2021 ◽  
Vol 61 (3) ◽  
pp. 119-24
Author(s):  
Weny Inrianto ◽  
Indah Kartika Murni ◽  
Ida Safitri
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Iron Deficiency ◽  
Congenital Heart ◽  
Septal Defect ◽  
Deficiency Anemia ◽  
Acyanotic Congenital Heart Disease ◽  
Hypertension In Children

Background Left-to-right shunting in acyanotic congenital heart disease (CHD) is the most common type of defect in childhood heart disease. Limited access to specialist health services causes delays in CHD management. In limited resource settings, identification of factors that influence the occurrence of pulmonary hypertension is important in order to decide which patients should be prioritized for defect closure to prevent further complications. Objective To determine predictive factors of pulmonary hypertension after a left-to-right shunt CHD diagnosis. Methods This retrospective cohort study included children aged 1 month to 17 years with isolated atrial septal defect, or ventricular septal defect, or patent ductus arteriosus. Potential predictors studied were iron deficiency anemia, mitral regurgitation, pneumonia, and heart failure. Bivariate analysis was done with Chi-square test and multivariate analysis was done with Cox regression to determine the hazard ratio. Results Pulmonary hypertension occurred in 68 of 176 subjects. Iron deficiency anemia, mitral regurgitation, and pneumonia were not predictives of pulmonary hypertension. However, heart failure was a significant predictive factor for pulmonary hypertension, with a hazard ratio of 4.1 (95%CI 2.2 to 7.5; P=0.001). Conclusions Heart failure is a predictive factor of pulmonary hypertension in children with left-to-right shunting in acyanotic CHD.

scholarly journals Adult congenital heart disease and the COVID-19 pandemic

Heart ◽  
2020 ◽  
Vol 106 (17) ◽  
pp. 1302-1309 ◽  
Author(s):  
Robert M Radke ◽  
Tim Frenzel ◽  
Helmut Baumgartner ◽  
Gerhard-Paul Diller
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
High Risk ◽  
Congenital Heart ◽  
Pragmatic Approach ◽  
Right Heart ◽  
High Risk Patients ◽  
Risk Patients

Adults with congenital heart disease (ACHD) may be at high risk in the case of COVID-19. Due to the heterogeneity of ACHD and secondary complications, risk profiles are, however, not uniform. This document aims to give an overview of relevant data and outline our pragmatic approach to disease prevention and management. Based on anatomy and additional physiological factors including symptoms, exercise capacity, heart failure, pulmonary hypertension and cyanosis, we propose a pragmatic approach to categorising patients into low-risk, intermediate-risk and high-risk groups. We regard especially patients with complex cyanotic conditions, those with palliated univentricular hearts, heart failure, severe valvular disease or pulmonary hypertension as high-risk patients. To avoid infection, we recommend self-isolation and exemption from work for these cohorts. Infected ACHD patients with low or moderate risk and without signs of deterioration may be remotely followed and cared for at home while in self isolation. High-risk patients or those with signs of respiratory or cardiovascular impairment require admission ideally at a tertiary ACHD centre. Especially patients with complex, cyanotic disease, heart failure and arrhythmias require particular attention. Treatment in patients with cyanotic heart disease should be guided by the relative degree of desaturation compared with baseline and lactate levels rather than absolute oxygen saturation levels. Patients with right heart dilatation or dysfunction are potentially at increased risk of right heart failure as mechanical ventilation and acute respiratory distress syndrome can lead to increase in pulmonary arterial pressures.

Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease

Heart ◽  
2018 ◽  
Vol 105 (6) ◽  
pp. 465-469 ◽  
Author(s):  
Despoina Ntiloudi ◽  
Sotiria Apostolopoulou ◽  
Konstantinos Vasiliadis ◽  
Alexandra Frogoudaki ◽  
Aphrodite Tzifa ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cox Regression ◽  
Strong Predictor ◽  
Adult Congenital Heart Disease ◽  
Significant Proportion ◽  
Cox Regression Analysis

ObjectiveDespite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.MethodsData were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.ResultsOverall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1–6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class.ConclusionsLong-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.

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