Circumstances of death in adult congenital heart disease

Zomer, Anoek; Vaartjes, Ilonca; Uiterwaal, Cuno S.P.M.; Velde, Enno T. van der; Merkhof, Lambert F.M. van den; Baur, L.H.B.; Ansink, Tieneke J.M.; Cozijnsen, Luc; Pieper, Petronella G.; Meijboom, Folkert J.; Grobbee, Diederick E.; Mulder, Barbara J.M.

doi:10.1016/j.ijcard.2010.09.015

Cited by 114 publications

(41 citation statements)

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“…In contrast, our data suggest that the focus of ACHD mortality has nowadays shifted to long-term cardiac and noncardiac complications of the disease. Moreover, the perioperative ACHD mortality reported here is even lower compared with results from a recent Dutch national registry (7.1% perioperative deaths between 2002 and 2008), 6 supporting the role of concentrating care at tertiary ACHD centres.14 The proportion of patients dying from heart failure in our study is, however, similar to that seen in previous studies. 4,5,13 It is likely that frequency of heart failure is increasing in ACHD patients, 15 and-given the increasing complexity of disease as well as the growing incidence of comorbid conditions-more patients present with advanced forms of heart failure.…”

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confidence: 86%

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Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre

et al. 2015

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in this population. [4][5][6] Because of ongoing improvement of care, survival prospects of adults with congenital heart disease are likely to have changed over recent decades. Khairy et al 7 demonstrated that mortality in patients with congenital heart disease has shifted away from infants and toward adults, with a steady increase in age at death. The current study was designed to evaluate a contemporary ACHD cohort from a single tertiary center and attempt comparison with data from Background-Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival.Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center. Methods and Results-We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age-and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2-14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient's age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased. Conclusions-ACHD patients continue to be afflicted by increased mortality in comparision with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients. Key words: heart defects, congenital ◼ heart failure ◼ mortality ◼ sudden cardiac death ◼ survival © 2015 American Heart Association, Inc.Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.115.017202Received April 23, 2015; accepted September 8, 2015. From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom (G. previous studies. In addition, we provide herewith mortality data in relationship to the general population, adjusted for age and sex, and propose a novel approach for presenting these data to health professionals, health policy makers, and patients alike.- Patients and MethodsWe retrospectively reviewed data on all adult patients with congenital heart disease under active foll...

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supporting

confidence: 86%

“…[4][5][6] Because of ongoing improvement of care, survival prospects of adults with congenital heart disease are likely to have changed over recent decades. Khairy et al 7 demonstrated that mortality in patients with congenital heart disease has shifted away from infants and toward adults, with a steady increase in age at death.…”

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confidence: 99%

Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre

et al. 2015

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“…Downloaded from http://ahajournals.org by on April 27, 2019 previously. 8,25,26 The majority of the sudden unexplained deaths in our population were likely to have an underlying, probably arrhythmic cardiac cause. However, information allowing a more detailed assessment of the specific cause was not available for our patient group.…”

Section: Discussionmentioning

confidence: 92%

Long-Term Nationwide Follow-Up Study of Simple Congenital Heart Disease Diagnosed in Otherwise Healthy Children

et al. 2016

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The study was conducted in Denmark, which had a population of approximately 5 million in 1970, of which <3% represented nonCaucasian citizens. The Danish National Health Service provides, then and now, tax-supported health care for all Danish citizens; free medical care is guaranteed for emergency and general hospital Background-Systematic follow-up is currently not recommended for patients with simple congenital heart disease; however, only a few data exist on the long-term prognosis of simple congenital heart disease. Methods and Results-We undertook a nationwide follow-up study of a cohort of 1241 simple congenital heart disease patients, diagnosed from 1963 through 1973, in otherwise healthy children and alive at 15 years of age. We identified 10 age-and sex-matched general population controls per patient. We followed the study population through Danish public registries from the age of 15 years up to January 1, 2013 with respect to mortality, cause of death, morbidity, and medical follow-up. The patients were followed for a total of 58 422 patient-years and had a median age at the end of follow-up of 47.4 years (interquartile range, 43.5-50.9). Mortality was increased compared with the general population, both overall (adjusted hazard ratio [aHR],1.9; 95% confidence interval [CI], 1.5-2.4)] and for patients (79%) without medical follow-up (aHR, 1.7; 95% CI, 1.3-2.2). The most common cause of death (40%) was sudden unexpected death (aHR, 4.3; 95% CI, 2.9-6.5). The incidence of critical cardiac morbidity was 3.9 per 1000 patient-years with the most frequent events being an adult (re)operation and hospitalization for heart failure or ventricular tachyarrhythmia. This corresponded to an aHR of 5.7 (95% CI, 4.6-6.9) when compared with the general population. Key Words: congenital heart disease ◼ death, sudden ◼ follow-up studies ◼ heart defects, congenital ◼ morbidity ◼ mortality © 2015 American Heart Association, Inc. Conclusions-Patients

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“…The authors extrapolated a prevalence of 4.09 per 1,000 to a Canadian and US population corresponds to 96,000 patients in Canada and 856,000 patients in the United States. A recent study in the Netherlands studied 8,595 adults with CHD, and found the most common defects in the distribution of CHD were ASD (17%), VSD (16%), AS/BAV (14%), TOF (10%) and Coarc (10%) but the highest mortality was found in patients with TA (14.7%) and patients with UV and double inlet left ventricle (11.4%) (Zomer et al, 2010). Two main reasons can explain this situation.…”

Section: Birth Prevalencementioning

confidence: 99%

“…For CHD adults, arrhythmias are more common, cardiac chambers often enlarge, and ventricles tend to develop systolic dysfunction. The main causes of death were progressive heart failure 26% and sudden cardiac arrest 22% (Zomer et al, 2010). Multidisciplinary care may also be required.…”

Section: Birth Prevalencementioning

confidence: 99%