Match!

Cause of death in adults with congenital heart disease — An analysis of the German National Register for Congenital Heart Defects

Published on May 1, 2016in International Journal of Cardiology3.471
· DOI :10.1016/j.ijcard.2016.02.133
Claudia C. Engelings1
Estimated H-index: 1
(MHH: Hannover Medical School),
Paul C. Helm4
Estimated H-index: 4
+ 7 AuthorsOktay Tutarel15
Estimated H-index: 15
(MHH: Hannover Medical School)
Abstract
Abstract Background Due to the great advances in the care of patients with congenital heart disease (CHD), mortality has decreased significantly over the last decades. Nonetheless, mortality for adults with congenital heart disease (ACHD) is still higher than for the general population. An analysis regarding causes of death in a nationwide contemporary cohort of ACHD is lacking. Methods A well-characterized cohort of the German National Register for Congenital Heart Defects was screened for patients over the age of 18years who died between January 2001 and January 2015. Data relating to the cardiac diagnosis, symptoms, operations, interventions, comorbidities, and causes of death were analyzed. Results During a median follow-up of 3.67years (IQR 1.32–9.41), 239 (9.2%) out of 2596 patients died during the study period (110 female (46%), mean age at death 39.8±17.8years). The majority of these deaths was CHD-related (171 patients (71.5%)). Leading causes of death were heart failure (n=66, 27.6%), and sudden cardiac death (n=55, 23.0%). Deceased patients had a more complex CHD and more extracardiac comorbidities compared with living patients. Conclusions Causes of death of ACHD patients in a large contemporary cohort from a national register are in the majority still CHD-related, with heart failure being the leading cause of death. Additionally, extracardiac comorbidities gain increasing importance.
  • References (19)
  • Citations (58)
📖 Papers frequently viewed together
312 Citations
156 Citations
357 Citations
78% of Scinapse members use related papers. After signing in, all features are FREE.
References19
Newest
Authors/Task Force Members: Silvia G. Priori* (Chairperson) (Italy), Carina Blomstrom-Lundqvist* (Co-chairperson) (Sweden), Andrea Mazzanti† (Italy), Nico Blom (The Netherlands), Martin Borggrefe (Germany), John Camm (UK), Perry Mark Elliott (UK), Donna Fitzsimons (UK), Robert Hatala (Slovakia), Gerhard Hindricks (Germany), Paulus Kirchhof (UK/Germany), Keld Kjeldsen (Denmark), Karl-Heinz Kuck (Germany), Antonio Hernandez-Madrid (Spain), Nikolaos Nikolaou (Greece), Tone M. Norekval (Norway), Chr...
1,201 CitationsSource
#1Gerhard-Paul Diller (Imperial College London)H-Index: 41
#2Aleksander Kempny (Imperial College London)H-Index: 23
Last. Michael A. Gatzoulis (Imperial College London)H-Index: 76
view all 10 authors...
156 CitationsSource
#1Paul Khairy (UdeM: Université de Montréal)H-Index: 45
#2Van Hare GfH-Index: 48
Last. Carole A. Warnes (UdeM: Université de Montréal)H-Index: 56
view all 22 authors...
188 CitationsSource
The number of adults with congenital heart disease is increasing due to the great achievements in the field of paediatric cardiology, congenital heart surgery and intensive care medicine over the last decades. Mortality has shifted away from the infant and childhood period towards adulthood. As congenital heart disease patients get older, a high prevalence of cardiovascular risk factors is encountered similar to the general population. Consequently, the contribution of acquired morbidities, espe...
25 CitationsSource
#1Eric V. Krieger (UW: University of Washington)H-Index: 14
#2Anne Marie Valente (Brigham and Women's Hospital)H-Index: 33
Heart failure (HF) is the leading cause of death in adults with repaired congenital heart disease (CHD). However there is currently little evidence to guide treatment strategies in this growing group of patients. Unlike the majority of HF, which is usually caused by LV systolic or diastolic dysfunction, CHD-HF is more often a consequence of RV disease, valve dysfunction, shunting or pulmonary hypertension. It is therefore not appropriate to extrapolate from the acquired HF literature and apply i...
20 CitationsSource
#1Darren Mylotte (MUHC: McGill University Health Centre)H-Index: 19
#2Louise Pilote (McGill University)H-Index: 61
Last. Ariane J. Marelli (MUHC: McGill University Health Centre)H-Index: 10
view all 8 authors...
Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality. We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines e...
108 CitationsSource
#1Werner Budts (Katholieke Universiteit Leuven)H-Index: 42
#2Mats Börjesson (Karolinska University Hospital)H-Index: 32
Last. Michael Papadakis (St George's, University of London)H-Index: 31
view all 10 authors...
Studies in patients with congenital heart disease (CHD) indicate that the majority of individuals participating in such programs achieve significant improvement of their exercise capacity and psychological state.1 The challenge is to ensure safe participation in regular physical activity (PA) in order to avoid the detrimental effects associated with sedentary life style. ### Why are physical activity recommendations for adolescents and adults with congenital heart disease needed? The improved su...
72 CitationsSource
#1Rafael Alonso-Gonzalez (Imperial College London)H-Index: 17
#2Francesco Borgia (Imperial College London)H-Index: 15
Last. Michael A. Gatzoulis (Imperial College London)H-Index: 76
view all 14 authors...
Background—Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease. Methods and Results—A total of 1188 patients with adult congenital heart disease (age, 33.1±13.1 years) undergoing lung function testing between 2000 and 2009 wer...
98 CitationsSource
#1Zeliha Koyak (UvA: University of Amsterdam)H-Index: 10
#2Joris R. de Groot (UvA: University of Amsterdam)H-Index: 31
Last. Barbara J.M. Mulder (UvA: University of Amsterdam)H-Index: 62
view all 12 authors...
Background-The value of implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease (CHD) is unknown. We investigated the long-term outcome after ICD implantation and developed a simple risk stratification score for ICD therapy. Methods and Results-A total of 136 adults with CHD and ICD (mean age +/- SD, 41 +/- 13 years; 67% male) were identified from 10 tertiary referral centers in the Netherlands and Belgium. The indication for ICD implantation was primary prevention...
51 CitationsSource
#1A. Carla Zomer (UU: Utrecht University)H-Index: 10
#2Cuno S.P.M. Uiterwaal (UU: Utrecht University)H-Index: 57
Last. Barbara J.M. MulderH-Index: 62
view all 11 authors...
Abstract Background Statistics on cause-specific mortality are important for prognostic research. The aim of this study was to assess the utility of the national mortality registry in research on causes of death in adult patients with congenital heart disease (CHD). Methods The CONCOR registry of over 10,000 adults with CHD was used to verify the causes of death provided by the WHO guidelines based national mortality registry, by linkage. Results Of 7277 patients linked to the national mortality...
31 CitationsSource
Cited By58
Newest
#1Spencer Van Mil (CAMH: Centre for Addiction and Mental Health)H-Index: 1
#2Tracy Heung (CAMH: Centre for Addiction and Mental Health)H-Index: 3
Last. Anne S. Bassett (CAMH: Centre for Addiction and Mental Health)H-Index: 49
view all 10 authors...
BACKGROUND: Given the importance of identifying factors that affect late outcomes in the increasing population of those with tetralogy of Fallot (TOF), we aimed to determine the impact of a 22q11.2 microdeletion on adult mortality, while accounting for pulmonary atresia, known to be enriched in 22q11.2 deletion syndrome (22q11.2DS). METHODS: We studied 612 individuals with TOF recruited as adults at a single centre, 80 (13.1%) with molecularly confirmed 22q11.2 deletions and 532 without 22q11.2D...
Source
#1M. Honicky (UFSC: Universidade Federal de Santa Catarina)
#2S. M. Cardoso (UFSC: Universidade Federal de Santa Catarina)
Last. Yara Maria Franco Moreno (UFSC: Universidade Federal de Santa Catarina)H-Index: 7
view all 7 authors...
BACKGROUND: Over the past three decades, the prevalence rate of overweight and obesity has increased in survivors with congenital heart disease, and little is known about the body composition and its association with clinical characteristics and lifestyle factors. OBJECTIVES: To evaluate excess total-body adiposity and central adiposity and, to describe associated factors. METHODS: Cross-sectional study with children and adolescents who underwent procedure to treat congenital heart disease, from...
Source
#1Susanne J. Maurer (TUM: Technische Universität München)H-Index: 1
#2Claudia Pujol Salvador (TUM: Technische Universität München)H-Index: 1
Last. Oktay Tutarel (TUM: Technische Universität München)H-Index: 15
view all 6 authors...
Abstract Background Heart failure is an important cause of morbidity and mortality in adults with congenital heart disease (ACHD). Sacubitril/valsartan is an established treatment for heart failure with reduced ejection fraction due to acquired cardiovascular disease. Data in adults with complex congenital heart disease (CHD) is lacking. Methods Retrospective study of ACHD patients with CHD of moderate/severe complexity and heart failure under treatment with sacubitril/valsartan. Clinical data w...
1 CitationsSource
#1Anna Björk (University of Gothenburg)H-Index: 1
#2Zacharias Mandalenakis (University of Gothenburg)H-Index: 7
Last. Mikael Dellborg (University of Gothenburg)H-Index: 36
view all 6 authors...
Abstract Background 1% of all live born children are born with a congenital heart defect (CHD) and currently 95% reach adulthood. Type 1 diabetes mellitus (T1DM) is an autoimmune disease that can develop due to i.e. heredity, exposure to infections and stress-strain. The incidence of T1DM in patients with CHD is unknown and we analysed the risk of developing T1DM for patients with CHD, and how this influences mortality. Methods By combining registries, the incidence of T1DM and the mortality was...
Source
#1Anna S. Mueller (NewYork–Presbyterian Hospital)H-Index: 1
#2Daniel M. McDonald (NewYork–Presbyterian Hospital)H-Index: 1
Last. Jonathan Ginns (Cornell University)H-Index: 7
view all 4 authors...
The number of rTOF patients who survive into adulthood is steadily rising, with currently more than 90% reaching the third decade of life. However, rTOF patients are not cured, but rather have a lifelong increased risk for cardiac and non-cardiac complications. Heart failure is recognized as a significant complication. Its occurrence is strongly associated with adverse outcome. Unfortunately, conventional concepts of heart failure may not be directly applicable in this patient group. This articl...
1 CitationsSource
Atrial septal defect (ASD) is a non-physiologic communication between the two atria, allowing the shunt between systemic and pulmonary circulation. Data about ASD prevalence among congenital heart disease patients (CHD) in Vietnam are still scarce. We aim to assess the trends in the prevalence of ASD patients and associated factors among CHD patients. This was a cross-sectional study, with data collected from medical records from 1220 CHD patients in Da Nang hospital from 1 January 2010 to 31 De...
Source
#1Rose Tompkins (Cedars-Sinai Medical Center)H-Index: 1
#2Anitra W. Romfh (Stanford University)H-Index: 3
The population of adults with congenital heart disease is now an estimated 1.4 million in the United States alone and growing. Unfortunately, survival to adulthood does not equate to a normal life expectancy, and heart failure (HF) has now emerged as the leading cause of death for the adult congenital heart disease (ACHD) patient. As this population continues to grow in number and advance in age, the prevalence of heart failure in the adult with congenital heart disease (ACHD-HF) will undoubtedl...
1 CitationsSource
#1Stefan Elde (Stanford University)
#2Woo Y. Joseph (Stanford University)H-Index: 46
Source
#1Ryo Konno (Tohoku University)H-Index: 12
#2Shunsuke Tatebe (Tohoku University)H-Index: 17
Last. Hiroaki Shimokawa (Tohoku University)H-Index: 89
view all 15 authors...
Patients with adult congenital heart disease (ACHD) are at increased risk of developing late cardiovascular complication. However, little is known about the predictive factors for long-term outcome. The Model for End-Stage Liver Disease eXcluding INR (MELD-XI) score was originally developed to assess cirrhotic patients and has the prognostic value for heart failure (HF) patients. In the present study, we examined whether the score also has the prognostic value in this population. We retrospectiv...
Source
#1Ari M. Cedars (UTSW: University of Texas Southwestern Medical Center)H-Index: 5
#2Luke J. Burchill (University of Melbourne)H-Index: 15
Last. Kristen M. Tecson (Baylor University Medical Center)H-Index: 4
view all 6 authors...
Source