Intermittent Levosimendan to Maintain Quality of Life in an Ambulatory Patient with End-Stage Heart Failure with Complex Congenital Heart Disease

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.

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‘End-stage’ heart failure therapy: potential lessons from congenital heart disease: from pulmonary artery banding and interatrial communication to parallel circulation

Heart ◽

10.1136/heartjnl-2015-309110 ◽

2016 ◽

Vol 103 (4) ◽

pp. 262-267 ◽

Cited By ~ 13

Author(s):

Dietmar Schranz ◽

Hakan Akintuerk ◽

Norbert F Voelkel

Keyword(s):

Heart Failure ◽

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Artery ◽

Congenital Heart ◽

Pulmonary Artery Banding ◽

Heart Failure Therapy ◽

Interatrial Communication ◽

End Stage Heart Failure ◽

End Stage

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Moderate to complex congenital heart disease

10.1093/med/9780198713333.003.0040 ◽

2016 ◽

Author(s):

Daryl P. Dob ◽

Elspeth E. Pickering ◽

Michael A. Gatzoulis

Keyword(s):

Quality Of Life ◽

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Early Death ◽

Western World ◽

Complex Congenital Heart Disease ◽

Congenital Aortic Stenosis ◽

Eisenmenger’S Syndrome

Children born with congenital heart disease no longer face the prospect of early death and a poor quality of life. In fact, most neonates with moderate to complex congenital heart disease have a survival rate to adulthood of over 80%. The ratio of adults to children with congenital heart disease is increasing, due to better surgical repairs, and longer survival with a better quality of life. In the Western world, there are more adults than children alive with congenital heart disease. This remarkable medical effort has allowed young women with congenital heart disease to mature to an age where they wish to have babies of their own. Early generations of women, palliated with Mustard or Senning repairs, have shown it is possible to face the cardiovascular challenges of pregnancy and survive. As the number of women with congenital heart disease is predicted to grow by 25% in the next decade and more women with congenital heart disease become pregnant; a better understanding of moderate to complex heart disease, different surgical repair procedures, and residual anomalies is paramount. This chapter examines the management of parturients with transposition complexes (both classical and congenitally corrected), tetralogy of Fallot, the Fontan circulation, Eisenmenger’s syndrome, and congenital aortic stenosis, taking into consideration the effect of pregnancy, labour, delivery, and anaesthesia on each circulation.

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