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.

Arrhythmias in congenital heart disease

2020 ◽  
pp. 259-276
Author(s):  
Alexander Van De Bruaene ◽  
Shouvik Haldar ◽  
Krishnakumar Nair
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Scientific Data ◽  
Right Atrial ◽  
Ebstein Anomaly ◽  
Adequate Knowledge ◽  
Arrhythmogenic Substrate ◽  
End Stage Heart Failure ◽  
End Stage

Patients with congenital heart disease are prone to the entire spectrum of atrial and ventricular arrhythmias. The arrhythmia can be related to the malformation itself (e.g. Wolff–Parkinson–White in Ebstein anomaly), structural remodelling due to ageing/longer life expectancy (e.g. atrial fibrillation), mode of repair (e.g. intra-atrial re-entry tachycardia after right atrial incision or monomorphic ventricular tachycardia after tetralogy of Fallot repair), and severe ventricular dysfunction in those with end-stage heart failure. Therefore, adequate knowledge of the congenital heart defect, its current haemodynamics and arrhythmogenic substrate, precise assessment, and risk stratification will lead to appropriate arrhythmia management. This chapter discusses the diagnosis, assessment, and management of arrhythmia in two adult patients with congenital heart disease. The aim is to offer practical advice for managing these patients, supported by scientific data.

scholarly journals Outcome following heart transplant assessment in adults with congenital heart disease

Heart ◽  
2019 ◽  
Vol 105 (22) ◽  
pp. 1741-1747 ◽  
Author(s):  
David Steven Crossland ◽  
Katrijn Jansen ◽  
Gareth Parry ◽  
Andrew Harper ◽  
Gianluigi Perri ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplant ◽  
Congenital Heart ◽  
Conventional Surgery ◽  
Orthotopic Heart Transplant ◽  
Ventricular Assist ◽  
Systemic Right Ventricle ◽  
End Stage Heart Failure ◽  
End Stage

ObjectivesAdults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT).MethodsCase notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV).Results196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period.ConclusionsThe number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.

Support/Transplantation

ESC CardioMed ◽  
2018 ◽  
pp. 788-790
Author(s):  
Michael Burch ◽  
Dilveer Panesar
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Congenital Heart ◽  
Therapeutic Option ◽  
Blood Transfusions ◽  
End Stage

Heart transplantation is the only realistic therapeutic option for children with end-stage heart disease. Unlike in adults, ischaemic heart failure is rare and most paediatric transplant referrals are for cardiomyopathy and the rest for congenital heart disease. Patients with congenital heart disease pose difficulties in terms of their anatomy and the chronicity of the illness, often having had multiple surgeries and blood transfusions prior to transplantation.

scholarly journals Clinical use of percutaneous mechanical circulatory assistance in a patient with end-stage right-sided heart failure and massive tricuspid insufficiency due to congenital heart disease: first-in-the-world case report

2021 ◽  
Vol 5 (8) ◽  
Author(s):  
Mario García Gómez ◽  
Aitor Uribarri ◽  
José Alberto San Román Calvar ◽  
Alexander Stepanenko
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Right Ventricle ◽  
Heart Transplantation ◽  
Right Ventricular ◽  
Congenital Heart ◽  
Ebstein’S Anomaly ◽  
Exclusion Criteria ◽  
End Stage

Abstract Background Due to improvement in the management of patients with congenital heart disease (CHD), the likelihood of their survival to adulthood is increasing. A relevant population suffers end-stage right ventricular failure (RVF) in their 3rd–4th decade of life. Hence, heart transplantation is still gold standard of treatment of end-stage heart failure, mechanical circulatory assistance has become a valuable tool in the bridging to heart transplant or definitive therapy. Use of implantable short-term or long-term devices is reported by others. However, within this clinical context, presence of significant tricuspid regurgitation (TR) or CHD is used as exclusion criteria for insertion of a percutaneous right ventricular circulatory support. Case summary We described a 36-year-old patient diagnosed with Ebstein's anomaly and severe TR who is admitted to hospital due to RVF refractory to standard medical treatment. After case presentation to the heart team, an Impella RP device insertion was scheduled, in spite of the presence of TR or CHD after evaluation of pulmonary valve competency and 3D reconstruction with virtual device implantation. During support, the patient improved clinically and haemodynamically. Due to device displacement to the right ventricle, it was bedside explanted after 30 days of support. After mechanical unloading during 30 days patients’ right ventricle recovered partially, permitting patient to improve his functional class. Discussion Although TR and CHD are exclusion criteria for the implantation of the Impella RP device, we report clinical experience in patient with Ebstein's anomaly and severe TR supported with percutaneous device as bridge to heart transplantation during 30 days.

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