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

Abstract

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...

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease–Related Heart Failure

“…Observing 274 patients with ACHD after HF hospitalization, the Dutch CONCOR (Congenital Corvitia) registry reported 1‐ and 3‐year mortality rates of 24% and 35%, respectively. Outcomes in tertiary ACHD centers in Canada and the United Kingdom demonstrate comparable mortality, with HF accounting for 21% and 42% of deaths, respectively 3, 23. Therefore, the in‐hospital mortality reported in this study likely underestimates mortality reported after ACHD HF hospitalization.…”

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

“…66,67 Finally, pneumonia has been shown to be one of leading causes of death in adults with CHD. 16 Recurrent pneumonia in adults with CHD is discussed further in the section on infectious disease.…”

Trends in Congenital Heart Disease

“…In comparison with the general population, mortality is significantly higher in ACHD patients ( Figure 6). [67][68][69][70] Common comorbidities include heart failure, which may result from residual structural problems (eg, valvar insufficiency), the sequelae of cardiac repair (eg, ventriculotomy), or the underlying congenital lesion itself (eg, abnormal loading of systemic right ventricle), 69,[71][72][73] and progression to heart transplantation is increasingly common. 74 Arrhythmias are prevalent, resulting from congenital abnormalities, perioperative insult, and hemodynamic pathophysiology.…”