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.

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.

Heart transplantation in adults with end-stage congenital heart disease

2012 ◽  
Vol 8 (2) ◽  
pp. 329-342 ◽  
Author(s):  
Luke J Burchill ◽  
Heather J Ross
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Congenital Heart ◽  
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 Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Christine H. Attenhofer Jost ◽  
Dörthe Schmidt ◽  
Michael Huebler ◽  
Christian Balmer ◽  
Georg Noll ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Lung Transplantation ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease ◽  
Eisenmenger’S Syndrome ◽  
Number Of Patients ◽  
Increased Risk

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger’s syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.

Heart failure

ESC CardioMed ◽  
2018 ◽  
pp. 784-788
Author(s):  
Pieter De Meester ◽  
Werner Budts ◽  
Marc Gewillig
Keyword(s):  
Heart Failure ◽  
Patient Outcome ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Congenital Heart ◽  
Common Presentation ◽  
Improve Patient ◽  
Residual Lesions

Heart failure is a common presentation of neonates and children with congenital heart disease and is the most important long-term complication in adults. It may be due to residual lesions and/or the complex circulation which are present even after repair. The mechanisms of heart failure are often different from normally built hearts and this will influence optimal treatment approaches. Several interventions (surgical, transcatheter, or pharmacological) can improve patient outcome and assist devices and heart transplantation are options in severe refractory cases.

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