Exercise Intolerance in Adult Congenital Heart Disease

Diller, Gerhard Paul; Dimopoulos, Konstantinos; Okonko, Darlington O.; Li, Wei; Babu‐Narayan, Sonya V.; Broberg, Craig S.; Johansson, Bengt; Bouzas, Beatriz; Mullen, Michael; Poole‐Wilson, Philip A.; Francis, Dárrel P.; Gatzoulis, Michael Α.

doi:10.1161/circulationaha.104.529800

Cited by 757 publications

(271 citation statements)

References 19 publications

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“…Most patients with repaired CHD may exercise safely, but medical providers, as well as parents and caregivers, often impose additional unwarranted exercise restrictions 21, 22. Further, ACHD patients frequently self‐restrict exercise because of perceived risks of underlying CHD or because of limited capacity for exercise 23, 24. Aside from predisposing to obesity and hyperlipidemia, the lack of aerobic exercise is associated with an increased risk of hospitalization and death in ACHD patients 14, 23, 25.…”

Section: Discussionmentioning

confidence: 99%

“…Further, ACHD patients frequently self‐restrict exercise because of perceived risks of underlying CHD or because of limited capacity for exercise 23, 24. Aside from predisposing to obesity and hyperlipidemia, the lack of aerobic exercise is associated with an increased risk of hospitalization and death in ACHD patients 14, 23, 25. Enhanced physical activity and aerobic exercise play an important role in decreasing cardiovascular risk 25.…”

Section: Discussionmentioning

confidence: 99%

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Metabolic Syndrome in Adults With Congenital Heart Disease

Deen

Krieger

Slee

et al. 2016

JAHA

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BackgroundMetabolic syndrome increases risk for atherosclerotic coronary artery disease, and its prevalence increases with increasing age and body mass index. Adults with congenital heart disease (ACHD) are now living longer and accruing coronary artery disease risk factors. However, the prevalence of metabolic syndrome in ACHD patients is unknown.Methods and ResultsWe conducted a retrospective cohort study of ACHD patients at our center to quantify the prevalence of metabolic syndrome in an ACHD population. Using case‐control matching, we constructed a comparable control group from a population‐based sample of 150 104 adults. International Diabetes Federation criteria were used to define metabolic syndrome. We used logistic regression to compare the risk of metabolic syndrome across the resulting cohorts, which were composed of 448 ACHD patients and 448 controls matched by age and sex. Mean age of both groups was 32.4±11.3 years, and 51.3% were female. Obesity was present in 16.1% of the ACHD patients and 16.7% of the controls. Metabolic syndrome was more common in ACHD patients than in controls (15.0% versus 7.4%; odds ratio 1.82, 95% CI 1.25–2.65).ConclusionsOur data suggest that metabolic syndrome is more common among adults with congenital heart disease than in the general population. Thus, patients with congenital heart disease should be screened for metabolic syndrome and risk factors mitigated where possible to prevent atherosclerotic coronary artery disease. Preventive cardiology should be included during routine ACHD care.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Metabolic Syndrome in Adults With Congenital Heart Disease

Deen

Krieger

Slee

et al. 2016

JAHA

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“…Some individuals are genetically predisposed to develop the vascular injury that ultimately progresses to PAH through the possession of mutations in key molecules, such as the BMPR2 gene, which encodes for a transforming growth factor (TGF)-b type II receptor [1]. This may be associated with a secondary trigger, such as the presence of underlying diseases, including connective tissue disease [2], congenital heart disease [3] or HIV infection [4]. Whatever the origins of the initial vascular insult, the end result is vascular smooth muscle cell dysfunction and endothelial cell dysfunction.…”

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confidence: 99%

Endothelin: setting the scene in PAH

Dupuis¹

2007

Eur Respir Rev

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Endothelin (ET), a 21-amino acid peptide secreted by the vascular endothelial cells, is frequently reported to be one of the most potent vasoconstrictors. ET induces endothelial cell, smooth muscle cell and fibroblast dysfunction and is now well recognised as a contributor to the complex pathogenesis of pulmonary arterial hypertension, a devastating chronic disease characterised by progressive vascular remodelling and occlusion of the pulmonary arterioles.ET produces its effects through the stimulation of two receptor subtypes, ETA and ETB, both of which are expressed on smooth muscle cells of pulmonary arterioles. Only the ETB receptor is expressed on vascular endothelial cells. In pre-clinical studies, dual blockade of both receptor subtypes was shown to produce greater inhibition of ET-induced contraction compared with blockade of either of the two receptor subtypes alone. There is also evidence that both the ETA and ETB receptors contribute to pulmonary artery smooth muscle cell proliferation. Pre-clinical research in various models of pulmonary arterial hypertension suggests that simultaneous blockade of the two receptor subtypes would be an effective therapeutic approach in the management of patients with pulmonary arterial hypertension. This has been shown to be the case in a number of randomised controlled clinical trials in pulmonary arterial hypertension due to a number of aetiologies.

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“…Nearly half (44-47%) of ACHD patients have restrictive lung disease (RLD), including 89% following Fontan repair and 76% with TOF [31,32]. Predictors of RLD include multiple prior thoracotomies (possibly due to associated chest wall deformity or weakness), diaphragmatic nerve palsy, cardiomegaly, atrial arrhythmias (perhaps related to amiodarone use), scoliosis, complex CHD, and increased BMI [32,33].…”

Section: Pulmonary Function Limitationsmentioning

confidence: 99%

Extra-cardiac manifestations of adult congenital heart disease

Gaeta

Ward

Krasuski

2016

Trends in Cardiovascular Medicine

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a b s t r a c tAdvancement in correction or palliation of congenital cardiac lesions has greatly improved the lifespan of congenital heart disease patients, resulting in a rapidly growing adult congenital heart disease (ACHD) population. As this group has increased in number and age, emerging science has highlighted the systemic nature of ACHD. Providers caring for these patients are tasked with long-term management of multiple neurologic, pulmonary, hepatic, renal, and endocrine manifestations that arise as syndromic associations with congenital heart defects or as sequelae of primary structural or hemodynamic abnormalities. In this review, we outline the current understanding and recent research into these extra-cardiac manifestations.Key words: Cardiovascular disease, Adult congenital heart disease, Pulmonary hypertension. Advances in the diagnosis and management of congenital heart disease (CHD) over the past several decades have greatly improved longevity and quality of life in the CHD population. Palliative or corrective interventions are now available for nearly every type of congenital cardiac lesion, and approximately 90% of affected children now survive to adulthood [1]. Adults with CHD (ACHD) already outnumber pediatric CHD patients, and this population is expected to grow by 5% per year [1,2]. Strikingly, the median age of severe CHD patients has increased from 11 years in 1985 to 25 years in 2010 [3]. Continued improvement in CHD interventions means that new (and more complex) populations will likely reach adulthood in the coming years.Practitioners who take care of adults with CHD are often charged with the delivery or coordination of comprehensive care for these complex patients, including the management of manifestations and complications that arise later in life. ACHD has many extra-cardiac effects, both as syndromic associations with congenital heart defects, or more commonly, as sequelae of primary structural or hemodynamic abnormalities. The role of genetic predilection remains undefined for nearly all extra-cardiac complications, though their incidence has clearly risen over the past decades. Indeed, more than a third of hospital admissions for ACHD patients are now for noncardiovascular reasons [4]. Optimal care of ACHD patients, therefore, mandates a thorough understanding of its multiorgan system manifestations. Herein, we will review current understanding of the most common manifestations of ACHD organized by the affected organ systems. Neurologic/neuropsychiatric Cerebrovascular complicationsA recent study quantified the strikingly high rates of stroke in ACHD [5]. Among nearly 30,000 ACHD patients, 8.9% of men http://dx

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Exercise Intolerance in Adult Congenital Heart Disease

Cited by 757 publications

References 19 publications

Metabolic Syndrome in Adults With Congenital Heart Disease

Metabolic Syndrome in Adults With Congenital Heart Disease

Endothelin: setting the scene in PAH

Extra-cardiac manifestations of adult congenital heart disease

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