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 Cardiology3.47
· 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 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 (53)
Published on Dec 1, 2015in Circulation23.05
Gerhard-Paul Diller36
Estimated H-index: 36
(Imperial College London),
Aleksander Kempny23
Estimated H-index: 23
(Imperial College London)
+ 7 AuthorsMichael A. Gatzoulis73
Estimated H-index: 73
(Imperial College London)
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 centres has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary centre. Methods and Results —We included 6,969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertai...
Published on Nov 1, 2015in European Heart Journal23.24
Silvia G. Priori125
Estimated H-index: 125
ACC : American College of Cardiology ACE : angiotensin-converting enzyme ACS : acute coronary syndrome AF : atrial fibrillation AGNES : Arrhythmia Genetics in the Netherlands AHA : American Heart Association AMIOVIRT : AMIOdarone Versus Implantable cardioverter-defibrillator:
Published on Sep 1, 2014in Heart5.08
Oktay Tutarel15
Estimated H-index: 15
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...
Published on Sep 1, 2014in Heart5.08
Eric V. Krieger13
Estimated H-index: 13
(UW: University of Washington),
Anne Marie Valente32
Estimated H-index: 32
(Brigham and Women's Hospital)
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...
Published on May 6, 2014in Circulation23.05
Darren Mylotte19
Estimated H-index: 19
(MUHC: McGill University Health Centre),
Louise Pilote61
Estimated H-index: 61
(McGill University)
+ 5 AuthorsAriane J. Marelli34
Estimated H-index: 34
(MUHC: McGill University Health Centre)
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...
Published on Dec 1, 2013in European Heart Journal23.24
Werner Budts44
Estimated H-index: 44
(Katholieke Universiteit Leuven),
Mats Börjesson34
Estimated H-index: 34
(Karolinska University Hospital)
+ 7 AuthorsMichael Papadakis6
Estimated H-index: 6
(St George's, University of London)
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...
Published on Feb 26, 2013in Circulation23.05
Rafael Alonso-Gonzalez16
Estimated H-index: 16
(Imperial College London),
Francesco Borgia14
Estimated H-index: 14
(Imperial College London)
+ 11 AuthorsMenelaos Charalambides2
Estimated H-index: 2
(Imperial College London)
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...
Published on Feb 1, 2012in Circulation-arrhythmia and Electrophysiology4.97
Zeliha Koyak10
Estimated H-index: 10
(UvA: University of Amsterdam),
Joris R. de Groot30
Estimated H-index: 30
(UvA: University of Amsterdam)
+ 9 AuthorsPetronella G. Pieper33
Estimated H-index: 33
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...
Published on Oct 1, 2011in International Journal of Cardiology3.47
A. Carla Zomer10
Estimated H-index: 10
(UU: Utrecht University),
Cuno S.P.M. Uiterwaal55
Estimated H-index: 55
(UU: Utrecht University)
+ 8 AuthorsDiederick E. Grobbee136
Estimated H-index: 136
(UU: Utrecht University)
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...
Published on Jan 1, 2011in Circulation23.05
Laurianne Le Gloan5
Estimated H-index: 5
(MHI: Montreal Heart Institute),
Lise-Andrée Mercier22
Estimated H-index: 22
(MHI: Montreal Heart Institute)
+ 7 AuthorsPaul Khairy55
Estimated H-index: 55
(MHI: Montreal Heart Institute)
As a result of major achievements in pediatric cardiac care, a growing number of patients with congenital heart disease (CHD) are flourishing well into adulthood. This heterogeneous and aging population of patients, many of whom represent the first generation of middle-age survivors, faces unique issues and challenges. As a field, adult CHD has evolved markedly during the past decade on several fronts, including imaging, arrhythmia management, percutaneous interventions, surgical techniques, res...
Cited By53
Published on Apr 8, 2019in Orphanet Journal of Rare Diseases3.69
Laura Llamosas-Falcón , Eva Bermejo-Sánchez8
Estimated H-index: 8
(ISCIII: Instituto de Salud Carlos III)
+ 3 AuthorsVerónica Alonso-Ferreira3
Estimated H-index: 3
(ISCIII: Instituto de Salud Carlos III)
Background Tetralogy of Fallot (TOF) is the most frequent cyanotic congenital heart defect. TOF mortality has fallen remarkably in recent years due to therapeutic advances. Accordingly, the aim of this study was to assess temporal and spatial variability in TOF-related mortality in Spain across the period 1981–2016, using data drawn from the nationwide population-based registry.
Published on Jul 1, 2019in Herz0.99
Rhoia Neidenbach2
Estimated H-index: 2
Stephan Achenbach91
Estimated H-index: 91
+ 13 AuthorsNicole Nagdyman24
Estimated H-index: 24
Angeborene Herzfehler (AHF) sind die haufigsten angeborenen Organanomalien. Dank medizinischer Fortschritte erreichen heutzutage fast alle Patienten das Erwachsenenalter. Trotz primar erfolgreicher Behandlungen konnen Rest- und Folgezustande sowie (nicht-)kardiale Komorbiditaten den chronischen Krankheitsverlauf beeinflussen und zu einer erhohten Morbiditat und Mortalitat fuhren. Erwachsene mit angeborenen Herzfehlern (EmaH) in Deutschland sind trotz des grosen Nachsorgebedarfs nicht an die Vers...
Published on 2019in Heart5.08
Fei Wang , Lee Harel-Sterling (U of T: University of Toronto)+ 4 AuthorsAriane J. Marelli (MUHC: McGill University Health Centre)
To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). We performed a systematic search of MEDLINE, EMBASE and Cochrane databases from January 1996 to December 2018. Studies were eligible if they developed multivariable models for risk prediction of decompensated HF in adult patients with CHD (ACHD), death in patients with ACHD-HF or both, or if they reported correspondin...
Published on Jul 1, 2019in Canadian Journal of Cardiology5.59
Paul Khairy55
Estimated H-index: 55
(MHI: Montreal Heart Institute)
Abstract The expanding population of adults with congenital heart disease (CHD) combined with the pervasiveness of arrhythmias has resulted in the rapid growth of a dedicated sector of cardiology at the intersection between two subspecialties: electrophysiology and adult CHD. Herein, practical considerations are offered regarding urgent referral for catheter ablation of atrial arrhythmias, anticoagulation, and primary prevention implantable cardioverter-defibrillators (ICDs). Patients with Ebste...
Published on Apr 1, 2019in Canadian Journal of Cardiology5.59
Fouke Ombelet (Katholieke Universiteit Leuven), Eva Goossens15
Estimated H-index: 15
(Katholieke Universiteit Leuven)
+ 3 AuthorsPhilip Moons46
Estimated H-index: 46
(Katholieke Universiteit Leuven)
Abstract Background Disease severity and functional indices are widely used for risk stratification of patients with congenital heart disease (CHD). The predictive value of these classification systems for assessing long-term mortality is unknown. We aimed to determine and compare the predictive value of disease severity and functional indices for 15-year mortality in adults with CHD. Methods Between 2000 and 2002, we categorized 629 patients with CHD (median age, 24 years; 60% were men) on 5 in...
Published on Mar 12, 2019in Heart5.08
Oktay Tutarel15
Estimated H-index: 15
Adults with congenital heart disease (ACHD) are an increasing and ageing population.1 Despite all advances in medical and surgical treatment, patients with ACHD are still afflicted by significant morbidity and mortality.2 Timely identification of those patients that are at risk can be difficult. In acquired cardiovascular disease, biomarkers play an important role for the diagnosis and even for monitoring the treatment. Brain natriuretic peptides, which are firmly established in the diagnosis an...
Published on Jun 1, 2019in International Journal of Cardiology3.47
Susanne J. Maurer (TUM: Technische Universität München), Claudia Pujol Salvador1
Estimated H-index: 1
(TUM: Technische Universität München)
+ 3 AuthorsO. Tutarel3
Estimated H-index: 3
(TUM: Technische Universität München)
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...
Published on Apr 1, 2019in Anaesthesist0.90
Christina Massoth1
Estimated H-index: 1
Alexander Zarbock41
Estimated H-index: 41
Manuel Wenk11
Estimated H-index: 11
Hintergrund Erwachsene mit angeborenen Herzfehlern (EMAH) stellen einen wachsenden Anteil der Patienten mit nichtkardialen Operationen.
Published on Mar 1, 2019in American Journal of Cardiology2.84
Jonathan Chan19
Estimated H-index: 19
Ronnie Thomas Collins (Lucile Packard Children's Hospital)+ 1 AuthorsAnitha John1
Estimated H-index: 1
ABSTRACT We sought to analyze the trends and resource utilization of adult congenital heart disease (ACHD) related heart failure admissions at children's hospitals. Heart failure admissions in patients with ACHD continue to rise at both pediatric and adult care facilities. Data from the Pediatric Health Information Systems (PHIS) database [2005-2015] were used to identify patients (≥18 years) admitted with congenital heart disease (745.xx-747.xx) and principal diagnosis of heart failure (428.xx)...
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