The Failing Right Ventricle in Congenital Heart Disease

Roche, S. Lucy; Redington, Andrew N.

doi:10.1016/j.cjca.2013.04.018

Cited by 56 publications

(47 citation statements)

References 123 publications

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“…While the presence of one or more of the aforementioned factors is universal in the population of adults late after TOF repair, there is a long (possibly indefinite) preclinical phase where symptoms and signs of overt HF are absent despite important underlying right heart dilation and dysfunction. This reflects the tremendous adaptive capacity of the RV, as end-diastolic volume increases to maintain stroke volume and mass augments to maintain wall stress [26]. If compensatory mechanisms of the RV fail however, endsystolic volume will increase, ejection fraction (EF) will decrease, and mass-to-volume ratio will decrease; this results in a vulnerable subset of patients at increased risk of development of overt HF [27].…”

Section: Heart Failure In the Tetralogy Of Fallot Populationmentioning

confidence: 98%

Heart failure in adult congenital heart disease: Emerging concepts with a focus on tetralogy of Fallot

Wald¹,

Valente²,

Marelli³

2015

Trends in Cardiovascular Medicine

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Section: Heart Failure In the Tetralogy Of Fallot Populationmentioning

confidence: 98%

Heart failure in adult congenital heart disease: Emerging concepts with a focus on tetralogy of Fallot

Wald¹,

Valente²,

Marelli³

2015

Trends in Cardiovascular Medicine

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“…Diseases affecting the heart, in fact, have usually a different pathophysiology in children compared to adult population [8]. Furthermore, as a result of the different pathophysiological function of the defective heart, the paediatric and adult patients are differently susceptible to stress insults, although there is still disagreement on whether the vulnerability of immature heart is less or more than for adult heart [9][10][11][12].…”

Section: Hypoxia In Cardiovascular Disease and Congenital Heart Diseasementioning

confidence: 99%

Hypoxia-Induced Molecular and Cellular Changes in the Congenitally Diseased Heart: Mechanisms and Strategies of Intervention

Iacobazzi¹,

Caputo²,

Ghorbel³

2017

Hypoxia and Human Diseases

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Tissue hypoxia plays a critical role in the pathobiology of congenital heart diseases, especially with regard to cyanotic patients. Here, we describe the cellular and molecular mechanisms induced by hypoxia in the diseased heart, with particular attention to the metabolic and functional changes that underlie the hypoxia-induced right ventricle remodelling. The role of reactive oxygen species in transcriptomic changes, DNA damage, contractile dysfunction and extracellular matrix remodelling will be addressed. Furthermore, the reoxygenation injury, which occurs when oxygen is reintroduced upon initiation of cardiopulmonary bypass, will be discussed. This allows a better understanding of the risks associated with the reoxygenation injury in children undergoing openheart surgery and helps to improve strategies of intervention for myocardial protection.

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“…As a result systemic RV dysfunction is common in these adult patients with the prevalence increasing with age. (13)(14)(15)(16) Whilst the number of patients with a previous atrial switch is decreasing due to the introduction of the arterial switch operation in the 1980s, (12) the current population is ageing and will therefore be at a higher risk of developing long term sequelae of their condition, including ventricular dysfunction, atrial arrhythmias and death.…”

Section: Spring 2013mentioning

confidence: 99%

“…To date, though, most trials in this population involve small patient numbers and frequently use surrogate end points. (12) Two groups of patients who pose specific problems in the management of ventricular failure are those with a systemic right ventricle (RV) and a univentricular circulation.…”

Section: Spring 2013mentioning

confidence: 99%

Management of advanced adult congenital heart disease

Bowater

2017

SAHJ

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As surgical techniques continue to develop, for example as seen with the Norwood procedure for hypoplastic left heart syndrome, the number of patients with the most complex forms of CHD will continue to increase along with their associated long term complications. Our ability to manage these patients appropriately, therefore, must also develop alongside. The purpose of this review is to examine the management of these patients once they have developed advanced disease, including drug therapy, devices, transplant and end of life care. In particular, the unique problems posed by patients with a systemic right ventricle (RV) and univentricular circulations are discussed. DRUG THERAPY IN HEART FAILUREIn patients with complex congenital heart disease, either palliated or unoperated, the development of ventricular dysfunction and clinical heart failure are common and as discussed above, is a common cause of morbidity and mortality in this population.

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The Failing Right Ventricle in Congenital Heart Disease

Cited by 56 publications

References 123 publications

Heart failure in adult congenital heart disease: Emerging concepts with a focus on tetralogy of Fallot

Heart failure in adult congenital heart disease: Emerging concepts with a focus on tetralogy of Fallot

Hypoxia-Induced Molecular and Cellular Changes in the Congenitally Diseased Heart: Mechanisms and Strategies of Intervention

Management of advanced adult congenital heart disease

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