Risk factors for excess mortality in adults with congenital heart diseases

Oliver, José M.; Gallego, Pastora; González, Ana; Garcia-Hamilton, Diego; Ávila, Pablo; Yotti, Raquel; Ferreira, Ignacio; Fernández‐Avilés, Francisco

doi:10.1093/eurheartj/ehw590

Cited by 32 publications

(38 citation statements)

References 28 publications

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“…However, overall survival was reduced irrespective of the level of complexity, repair status, or underlying CHD 19. Mortality among our patients with severe ACHD was 3.164‐fold higher than that among the general population.…”

Section: Discussionmentioning

confidence: 63%

Adult Congenital Heart Disease in a Nationwide Population 2000–2014: Epidemiological Trends, Arrhythmia, and Standardized Mortality Ratio

Chen

et al. 2018

JAHA

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BackgroundThe adult congenital heart disease (ACHD) population will grow with medical advances, but data are limited. We investigated the epidemiological profile of ACHD in Taiwan, a country with a congenital heart disease program since 1955, population of 23 million, and easily accessible high‐standard medical care.Methods and Results ACHD patients, born after 1954, were identified from the nationwide database 2000–2014. The ACHD prevalence in the population aged 18 to 59 was 140.53, 157.08, 182.45, and 217.00 per 100 000 in 2000, 2005, 2010, and 2014, respectively (increasing time trend, P<0.0001). Percentage of severe ACHD also increased over time (P<0.0001) and was 11.70% in 2014. The 5 leading ACHD diagnoses were ventricular septal defect, secundum atrial septal defect, patent ductus arteriosus, pulmonary stenosis, and tetralogy of Fallot. Freedom from tachyarrhythmia at age 50 years was 0.574 and 0.710 for severe and simple ACHD, respectively. Cardiac causes accounted for the majority of deaths, followed by malignancy in simple ACHD and external causes/sudden death/out‐of‐hospital death in severe ACHD patients. The proportion of unexpected death was 10%. Compared with the general population, the standardized mortality ratio was higher not only in severe ACHD (3.164; 95% confidence interval, 2.664–3.664), but also in women with simple ACHD (1.704; 95% confidence interval, 1.499–1.909), with a higher proportion of cardiac, labor, and sudden death as causes of death.ConclusionsWe demonstrated an increasing trend in ACHD prevalence and medical complexity. They are at risk of tachyarrhythmia, higher mortality, and unexpected deaths, suggesting a gap in their medical care.

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Section: Discussionmentioning

confidence: 63%

Adult Congenital Heart Disease in a Nationwide Population 2000–2014: Epidemiological Trends, Arrhythmia, and Standardized Mortality Ratio

Chen

et al. 2018

JAHA

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“…From the perspective of a cardiologist caring for adults with CHD, the study provides reassuring data for patients with none of the factors associated with higher risk, since survival of this subgroup was comparable to a reference population (3). The authors noted that late referral to an adult CHD specialist did not compromise survival.…”

mentioning

confidence: 83%

“…Nevertheless, mortality rates remain higher than the general population, with patients most commonly succumbing to heart failure and to sudden death of presumed arrhythmic etiology. In a recent issue of the European Heart Journal, Oliver et al explored factors associated with excess mortality in a cohort of 3,311 adults with CHD followed for a median of 10.5 years in a dedicated referral center (3). They confirmed the observation that overall survival of adults with heterogeneous forms of CHD of varied complexity is lower than the general population.…”

mentioning

confidence: 92%

“…Oliver et al provided helpful insights into higher risk subsets (3). For example, patients with non-reparable lesions died at a younger age than surgically palliated adults with CHD.…”

mentioning

confidence: 99%

“…This was addressed in part by Oliver et al's online supplement, which presents survival curves separately for individual types of CHD (3). Median age at death was >75 years for simple lesions and uncomplicated left-to-right shunts, 60 to 75 years for subvalvular aortic stenosis, Ebstein anomaly, aortic coarctation, tetralogy of Fallot, and complete transposition of the great arteries, and <60 years for Eisenmenger syndrome, atrioventricular discordance, pulmonary atresia, and single ventricle physiology (3).…”

mentioning

confidence: 99%

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Risk markers for excess mortality in adults with congenital heart disease: does one size fit all?

Mongeon¹,

Khairy²

2017

J. Thorac. Dis.

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Over the past few decades, improved survival in patients with congenital heart disease (CHD), particularly those with severe defects, has given rise to a rapidly growing and aging population of adult survivors with increasingly complex disease (1,2). Nevertheless, mortality rates remain higher than the general population, with patients most commonly succumbing to heart failure and to sudden death of presumed arrhythmic etiology. In a recent issue of the European Heart Journal, Oliver et al. explored factors associated with excess mortality in a cohort of 3,311 adults with CHD followed for a median of 10.5 years in a dedicated referral center (3). They confirmed the observation that overall survival of adults with heterogeneous forms of CHD of varied complexity is lower than the general population. An analysis of clinic registry data allowed for detailed phenotyping, whereas vital status was confirmed by means of a national database.Overall, the cohort appeared generally representative of patients followed by adult CHD referral centers, with 51% of patients having moderate or complex lesions, and 49% simple defects. As expected, the mortality rate was highest in patients with complex CHD, although rates were not significantly different among those with moderate versus simple defects.Oliver et al. provided helpful insights into higher risk subsets (3). For example, patients with non-reparable lesions died at a younger age than surgically palliated adults with CHD. Reinterventions during adulthood were not associated with reduced survival, implying that candidates for transcatheter or surgical interventions were carefully selected. In a multivariable Cox regression model, the following variables were associated with decreased survival: single ventricle physiology, clinical cyanosis, severe pulmonary outflow tract obstruction, infective endocarditis, severe pulmonary hypertension, more than moderate subaortic atrioventricular valve regurgitation, moderate or severe systemic ventricular dysfunction, moderate or severe subpulmonary ventricular dysfunction, ischemic heart disease, aortic aneurysm (including aortic dissection or rupture) and genetic syndromes. The multivariable model was not over fitted (22 variables for 336 events). Naturally, elements that emerge as independent predictors of mortality are highly dependent on variables considered in the model in the first place. In that regard, factors such as electrocardiographic metrics, Holter data, clinical or inducible ventricular arrhythmias, cardiovascular implantable electronic devices, New York Heart Association functional class, and exercise capacity were not assessed.From the perspective of a cardiologist caring for adults with CHD, the study provides reassuring data for patients with none of the factors associated with higher risk, since survival of this subgroup was comparable to a reference population (3). The authors noted that late referral to an adult CHD specialist did not compromise survival. This observation, together with a Canadian study that repo...

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Lesion‐specific mortality due to congenital heart disease in U.S. adults from 1999 to 2017

Lynn

Salemi

Meath

et al. 2022

Birth Defects Research

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Background: Adults with congenital heart disease (ACHD) comprise an increasing proportion of individuals living with CHD in the United States (US) although little is known regarding lesion or age-specific mortality in the modern era. Our study aimed to describe current era ACHD mortality rates by age and CHD lesion and provide temporal mortality rate trends for the last two decades. Methods: We conducted a 19-year analysis (1999-2017) of publicly available, de-identified Multiple Cause of Death data compiled and produced by the National Center for Health Statistics. Age and lesion-specific mortality rates were calculated using joinpoint regression.Results: ACHD mortality rates decreased by an average of 2%-4% per year for all adults. CHD lesions resulting in the highest ACHD-related mortality varied by age. Unlike the other lesions, mortality attributed to single ventricle physiology failed to improve in early adulthood (average of 1.6% increase per year).In decedents age 65 years or older, simpler forms of CHD like shunt lesions became more prominent contributors, accounting for 46% of deaths. Conclusion:Rates of mortality due to ACHD have declined significantly for adults with CHD, however, continued mortality due to single ventricle physiology remains an area requiring improved strategies to increase survival.

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Risk factors for excess mortality in adults with congenital heart diseases

Abstract: Clinical parameters, such as anatomical features, haemodynamic sequelae, or acquired complications, were independent predictors of excess mortality in adults with CHD. Survival of individuals with no risk factors did not differ from the reference population.

Cited by 32 publications

References 28 publications

Adult Congenital Heart Disease in a Nationwide Population 2000–2014: Epidemiological Trends, Arrhythmia, and Standardized Mortality Ratio

Adult Congenital Heart Disease in a Nationwide Population 2000–2014: Epidemiological Trends, Arrhythmia, and Standardized Mortality Ratio

Risk markers for excess mortality in adults with congenital heart disease: does one size fit all?

Lesion‐specific mortality due to congenital heart disease in U.S. adults from 1999 to 2017

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