Publications by authors named "Oktay Tutarel"

80 Publications

Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects.

Cardiol Young 2021 Oct 18:1-11. Epub 2021 Oct 18.

Department of Cardiology, University Hospital Aachen, Aachen, Germany.

Background: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.

Methods: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.

Results: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.

Conclusion: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.
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http://dx.doi.org/10.1017/S1047951121003954DOI Listing
October 2021

Favorable Atrial Remodeling After Percutaneous Pulmonary Valve Implantation and Its Association With Changes in Exercise Capacity and Right Ventricular Function.

J Am Heart Assoc 2021 10 6;10(20):e021416. Epub 2021 Oct 6.

Department of Congenital Heart Disease and Pediatric Cardiology German Heart Centre MunichTechnical University of Munich Munich Germany.

Background Right atrial (RA) dilatation and impaired right ventricular (RV) filling are common in patients with RV outflow tract dysfunction. We aimed to study potential correlations between atrial function with clinically relevant hemodynamic parameters and to assess the predictive impact of atrial performance on the recovery of exercise capacity and RV pump function after percutaneous pulmonary valve implantation (PPVI). Methods and Results Altogether, 105 patients with right ventricular outflow tract dysfunction (median age at PPVI, 19.2 years; range, 6.2-53.4 years) who underwent cardiac magnetic resonance imaging before and 6 months after PPVI were included. RA and left atrial maximal and minimal volumes as well as atrial passive and active emptying function were assessed from axial cine slices. RA emptying function was inversely related to invasive RV end-diastolic pressure, and RA passive emptying correlated significantly with peak oxygen uptake. After PPVI, a significant decrease in RA minimum volume was observed, whereas RA passive emptying function improved, and RA active emptying function decreased significantly. Patients with predominant right ventricular outflow tract stenosis showed more favorable changes in RA active and left atrial passive emptying than those with primary volume overload. None of the RA and left atrial emptying parameters was predictive for recovery of peak oxygen uptake or RV ejection fraction. Conclusions In patients with right ventricular outflow tract dysfunction, impaired RA emptying assessed by cardiac magnetic resonance imaging was associated with increased RV filling pressures and lower exercise capacity. PPVI leads to a reduction in RA size and improved passive RA emptying function. However, RA function was not associated with improved exercise performance and RV pump function.
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http://dx.doi.org/10.1161/JAHA.121.021416DOI Listing
October 2021

Congenitally Corrected Transposition of the Great Arteries in Adults-A Contemporary Single Center Experience.

J Cardiovasc Dev Dis 2021 Sep 15;8(9). Epub 2021 Sep 15.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, 80636 Munich, Germany.

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect (CHD). Contemporary data regarding its outcome in adults are scarce.

Methods: Retrospective, single-center study of all ccTGA patients over the age of 16 years treated at our center during the time period 2006-2018. Only patients with a biventricular circulation were included. The primary endpoint was all-cause mortality.

Results: Altogether, 96 patients (mean age 32.8 ± 16.0 years, female 50%) with ccTGA and a systemic right ventricle (SRV) were included in the study. An additional CHD was present in 81 patients (84.4%); most common were a ventricular septal defect (VSD) and a left ventricular outflow tract obstruction. Out of the whole cohort, 45 (46.9%) had already undergone cardiac surgery at baseline. During a median follow-up of 6.5 (IQR 2.8-12.7) years, the primary endpoint occurred in 10 patients (10.8%). Cause of death was cardiac in nine patients and suicide in one. Hospitalizations due to heart failure occurred in 48 patients (51.6%). Upon univariate Cox analysis, an NYHA class ≥III, severe tricuspid regurgitation, severe SRV systolic impairment, as well as a reduced left ventricular systolic function were predictors of the primary endpoint. Upon multivariable analysis, only NYHA class ≥ III (HR: 18.66, CI 95%: 3.01-115.80, = 0.0017) and a reduced left ventricular systolic function (HR: 7.36, CI 95%: 1.18-45.99, = 0.038) remained as independent predictors.

Conclusions: Adults with ccTGA and an SRV are burdened with significant morbidity and mortality. Predictors for mortality are NYHA class and subpulmonary left ventricular function.
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http://dx.doi.org/10.3390/jcdd8090113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8467717PMC
September 2021

Ventricular assist devices in paediatric cardiomyopathy and congenital heart disease: An analysis of the German National Register for Congenital Heart Defects.

Int J Cardiol 2021 Nov 3;343:37-44. Epub 2021 Sep 3.

Competence Network for Congenital Heart Defects Berlin, Germany; Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, Germany; DZHK (German Center for Cardiovascular Research), partner site Munich Heart Alliance, Munich, Germany.

Background: Ventricular assist devices (VAD) are increasingly used in patients with end-stage heart failure due to acquired heart disease. Limited data exists on the use and outcome of this technology in children.

Methods: All children (<18 years of age) with VAD support included in the German National Register for Congenital Heart Defects were identified and data on demographics, underlying cardiac defect, previous surgery, associated conditions, type of procedure, complications and outcome were collected.

Results: Overall, 64 patients (median age 2.1 years; 45.3% female) receiving a VAD between 1999 and 2015 at 8 German centres were included in the analysis. The underlying diagnosis was congenital heart disease (CHD) in 25 and cardiomyopathy in 39 children. The number of reported VAD implantations increased from 13 in the time period 2000-2004 to 27 implantations in the time period 2010-2014. During a median duration of VAD support of 54 days, 28.1% of patients experienced bleeding complications (6.3% intracerebral bleeding), 14.1% thrombotic (10.9% VAD thrombosis) and 23.4% thromboembolic complications (including cerebral infarction in 18.8% of patients). Children with cardiomyopathy were more likely to receive a cardiac transplantation (79.5% vs. 28.0%) compared to CHD patients. Survival of cardiomyopathy patients was significantly better compared to the CHD cohort (p < 0.0001). Multivariate Cox-proportional analysis revealed a diagnosis of CHD (hazard ratio [HR] 4.04, p = 0.001), age at VAD implantation (HR 1.09/year, p = 0.04) and the need for pre-VAD extracorporeal membrane oxygenation (ECMO) support (HR 3.23, p = 0.03) as independent predictors of mortality.

Conclusions: The uptake of VAD therapy in children is increasing. Morbidity and mortality remain high, especially in patients with congenital heart disease and those requiring ECMO before VAD implantation.
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http://dx.doi.org/10.1016/j.ijcard.2021.08.047DOI Listing
November 2021

Complete Atrioventricular Septal Defects after the Age of 40 Years.

J Clin Med 2021 Aug 19;10(16). Epub 2021 Aug 19.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, 80636 Munich, Germany.

Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years.

Methods: Patients with cAVSD who were ≥40 years of age at any point between 2005 until 2018 were included retrospectively. Data were retrieved from hospital records. The primary endpoint was a combination of death from any cause and unplanned hospitalizations due to cardiac reasons.

Results: 43 patients (60.5% female, mean age 43.7 ± 6.0 years, genetic syndrome 58.1%) were included. At begin of follow-up, the majority of patients ( = 41, 95.3%) was in New York Heart Association (NYHA) class I or II. Out of the whole cohort 26 (60.5%) had undergone cardiac surgery. At baseline, at least one extracardiac comorbidity was present in 40 patients (93.0%). Median follow-up was 1.7 years (IQR 0.3-4.6). On univariate Cox analysis, NYHA class at begin of follow-up (hazard ratio: 1.96, CI 95%: 1.04-3.72, < 0.05) was the only predictor for the primary endpoint.

Conclusions: Significant morbidity and mortality is present in cAVSD patients over the age of 40 years. NYHA class is predictive for a worse outcome.
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http://dx.doi.org/10.3390/jcm10163665DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8396930PMC
August 2021

Transition to adulthood and transfer to adult care of adolescents with congenital heart disease: a global consensus statement of the ESC Association of Cardiovascular Nursing and Allied Professions (ACNAP), the ESC Working Group on Adult Congenital Heart Disease (WG ACHD), the Association for European Paediatric and Congenital Cardiology (AEPC), the Pan-African Society of Cardiology (PASCAR), the Asia-Pacific Pediatric Cardiac Society (APPCS), the Inter-American Society of Cardiology (IASC), the Cardiac Society of Australia and New Zealand (CSANZ), the International Society for Adult Congenital Heart Disease (ISACHD), the World Heart Federation (WHF), the European Congenital Heart Disease Organisation (ECHDO), and the Global Alliance for Rheumatic and Congenital Hearts (Global ARCH).

Eur Heart J 2021 11;42(41):4213-4223

KU Leuven Department of Public Health and Primary Care, KU Leuven, Kapucijnenvoer 35, Box 7001, B-3000 Leuven, Belgium.

The vast majority of children with congenital heart disease (CHD) in high-income countries survive into adulthood. Further, paediatric cardiac services have expanded in middle-income countries. Both evolutions have resulted in an increasing number of CHD survivors. Expert care across the life span is necessitated. In adolescence, patients transition from being a dependent child to an independent adult. They are also advised to transfer from paediatrics to adult care. There is no universal consensus regarding how transitional care should be provided and how the transfer should be organized. This is even more challenging in countries with low resources. This consensus document describes issues and practices of transition and transfer of adolescents with CHD, accounting for different possibilities in high-, middle-, and low-income countries. Transitional care ought to be provided to all adolescents with CHD, taking into consideration the available resources. When reaching adulthood, patients ought to be transferred to adult care facilities/providers capable of managing their needs, and systems have to be in place to make sure that continuity of high-quality care is ensured after leaving paediatric cardiology.
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http://dx.doi.org/10.1093/eurheartj/ehab388DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8560210PMC
November 2021

Emergency department management of patients with adult congenital heart disease: a consensus paper from the ESC Working Group on Adult Congenital Heart Disease, the European Society for Emergency Medicine (EUSEM), the European Association for Cardio-Thoracic Surgery (EACTS), and the Association for Acute Cardiovascular Care (ACVC).

Eur Heart J 2021 07;42(26):2527-2535

DZHK (German Centre for Cardiovascular Research), partner site Munich Heart Alliance, Munich, Germany.

Adult congenital heart disease (ACHD) patients represent a growing population with increasing use of acute emergency department (ED) care. Providing comprehensive ED care necessitates an understanding of the most common clinical scenarios to improve morbidity and mortality in this population. The aim of this position document is to provide a consensus regarding the management of the most common clinical scenarios of ACHD patients presenting to the ED.
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http://dx.doi.org/10.1093/eurheartj/ehab272DOI Listing
July 2021

A National Comparative Investigation of Twins With Congenital Heart Defects for Neurodevelopmental Outcomes and Quality of Life (Same Same, but Different?): Protocol for a Prospective Observational Study.

JMIR Res Protoc 2021 May 13;10(5):e26404. Epub 2021 May 13.

Department of Congenital Heart Defects and Pediatric Cardiology, German Heart Center of Munich, Munich, Germany.

Background: Due to the increased survival rates of patients with congenital heart defects (CHD), associated disorders are an increasing focus of research. Existing studies figured out an association between CHD and its treatment, and neurodevelopmental outcomes including motor competence impairments. All these studies, however, compared their test results with reference values or results of healthy control groups. This comparison is influenced by socioeconomic and genetic aspects, which do have a known impact on neurodevelopmental outcomes.

Objective: This study protocol describes a setting that aims to find out the role of CHD and its treatments on neurodevelopmental outcomes, excluding socioeconomic and genetic aspects. Only a twin comparison provides the possibility to exclude these confounding factors.

Methods: In a German-wide prospective cohort study, 129 twin siblings registered in the National Register for Congenital Heart Defects will undergo testing on cognitive function (Wechsler Intelligence Tests age-dependent: Wechsler Adult Intelligence Scale, fourth edition; Wechsler Intelligence Scale for Children, fifth edition; and Wechsler Preschool and Primary Scale of Intelligence, fourth edition) and motor competence (Movement Assessment Battery for Children, second edition). Additionally, the self-reported health-related quality of life (KINDL-R for children, Short Form 36 for adults) and the parent-reported strength and difficulties of the children (Strength and Difficulties Questionnaire, German version) will be assessed by standardized questionnaires. CHD data on the specific diagnosis, surgeries, transcatheter procedures, and additional medical information will be received from patient records.

Results: The approval of the Medical Ethics Committee Charité Mitte was obtained in June 2018. After getting funded in April 2019, the first enrollment was in August 2019. The study is still ongoing until June 2022. Final results are expected in 2022.

Conclusions: This study protocol provides an overview of the study design's technical details, offering an option to exclude confounding factors on neurodevelopmental outcomes in patients with CHD. This will enable a specific analysis focusing on CHD and clinical treatments to differentiate in terms of neurodevelopmental outcomes of patients with CHD compared to twin siblings with healthy hearts. Finally, we aim to clearly define what is important to prevent patients with CHD in terms of neurodevelopmental impairments to be able to develop targeted prevention strategies for patients with CHD.

Trial Registration: German Clinical Trials Register DRKS00021087; https://tinyurl.com/2rdw8w67.

International Registered Report Identifier (irrid): DERR1-10.2196/26404.
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http://dx.doi.org/10.2196/26404DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8160812PMC
May 2021

Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC).

Heart 2021 Apr 28. Epub 2021 Apr 28.

Department of Cardiology, Erasmus Medical Center Rotterdam, Rotterdam, The Netherlands

Objective: Cardiac disease is a major cause of maternal mortality. Data regarding pregnancy outcomes in women with a systemic right ventricle (sRV) are scarce. We studied pregnancy outcomes in women with an sRV after the atrial switch procedure for transposition of the great arteries (TGA) or congenitally corrected TGA (CCTGA).

Methods: The ESC EORP Registry of Pregnancy and Cardiac Disease is an international prospective registry of pregnant women with cardiac disease. Pregnancy outcomes (maternal/fetal) in all women with an sRV are described. The primary end point was a major adverse cardiac event (MACE) defined as maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischaemic coronary event and other thromboembolic events.

Results: Altogether, 162 women with an sRV (TGA n=121, CCTGA n=41, mean age 28.8±4.6 years) were included. No maternal mortality occurred. In 26 women, at least one MACE occurred, heart failure in 16 (9.8%), arrhythmias (atrial 5, ventricular 6) in 11 (6.7%) and others in 4 (2.5%). Prepregnancy signs of heart failure as well as an sRV ejection fraction <40% were predictors of MACE. One woman experienced fetal loss, while no neonatal mortality was observed. No significant differences were found between women with CCTGA and TGA. In the subset of women who had an echocardiogram before and after pregnancy, no clear deterioration in sRV was observed.

Conclusion: The majority of women with an sRV tolerated pregnancy well with a favourable maternal and fetal outcome. Heart failure and arrhythmias were the most common MACE.
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http://dx.doi.org/10.1136/heartjnl-2020-318685DOI Listing
April 2021

Risk stratification and management of women with cardiomyopathy/heart failure planning pregnancy or presenting during/after pregnancy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy.

Eur J Heart Fail 2021 04 17;23(4):527-540. Epub 2021 Mar 17.

Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany.

This position paper focusses on the pathophysiology, diagnosis and management of women diagnosed with a cardiomyopathy, or at risk of heart failure (HF), who are planning to conceive or present with (de novo or previously unknown) HF during or after pregnancy. This includes the heterogeneous group of heart muscle diseases such as hypertrophic, dilated, arrhythmogenic right ventricular and non-classified cardiomyopathies, left ventricular non-compaction, peripartum cardiomyopathy, Takotsubo syndrome, adult congenital heart disease with HF, and patients with right HF. Also, patients with a history of chemo-/radiotherapy for cancer or haematological malignancies need specific pre-, during and post-pregnancy assessment and counselling. We summarize the current knowledge about pathophysiological mechanisms, including gene mutations, clinical presentation, diagnosis, and medical and device management, as well as risk stratification. Women with a known diagnosis of a cardiomyopathy will often require continuation of drug therapy, which has the potential to exert negative effects on the foetus. This position paper assists in balancing benefits and detrimental effects.
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http://dx.doi.org/10.1002/ejhf.2133DOI Listing
April 2021

Adults with congenital heart disease -  we need more Europe for a better care.

Eur J Heart Fail 2021 03 3;23(3):454-455. Epub 2021 Feb 3.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, Munich, Germany.

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http://dx.doi.org/10.1002/ejhf.2107DOI Listing
March 2021

Acquired Comorbidities in Adults with Congenital Heart Disease: An Analysis of the German National Register for Congenital Heart Defects.

J Clin Med 2021 Jan 16;10(2). Epub 2021 Jan 16.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine-Technical University of Munich, 80636 Munich, Germany.

Background: As adults with congenital heart disease (ACHD) are getting older, acquired comorbidities play an important role in morbidity and mortality. Data regarding their prevalence in ACHD that are representative on a population level are not available.

Methods: The German National Register for Congenital Heart Defects was screened for ACHD. Underlying congenital heart disease (CHD), patient demographics, previous interventional/surgical interventions, and comorbidities were retrieved. Patients <40 years of age were compared to those ≥40 years.

Results: A total of 4673 patients (mean age 33.6 ± 10.7 years, female 47.7%) was included. At least one comorbidity was present in 2882 patients (61.7%) altogether, and in 56.8% of patients below vs. 77.7% of patients over 40 years of age ( < 0.001). Number of comorbidities was higher in patients ≥40 years (2.1 ± 2.1) than in patients <40 years (1.2 ± 1.5, < 0.001). On multivariable regression analysis, age and CHD complexity were significantly associated with the presence and number of comorbidities.

Conclusions: At least one acquired comorbidity is present in approximately two-thirds of ACHD. Age and complexity of the CHD are significantly associated with the presence of comorbidities. These findings highlight the importance of addressing comorbidities in ACHD care to achieve optimal long-term outcomes.
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http://dx.doi.org/10.3390/jcm10020314DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7830982PMC
January 2021

Patients with Single-Ventricle Physiology over the Age of 40 Years.

J Clin Med 2020 Dec 18;9(12). Epub 2020 Dec 18.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany.

Background: Single-ventricle physiology (SVP) is associated with significant morbidity and mortality at a young age. However, survival prospects have improved and risk factors for a negative outcome are well described in younger cohorts. Data regarding older adults is scarce.

Methods: In this study, SVP patients under active follow-up at our center who were ≥40 years of age at any point between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history were retrieved from hospital records. The primary end-point was all-cause mortality.

Results: Altogether, 49 patients (19 female (38.8%), mean age 49.2 ± 6.4 years) were included. Median follow-up time was 4.9 years (interquartile range (IQR): 1.8-8.5). Of these patients, 40 (81.6%) had undergone at least one cardiac surgery. The most common extracardiac comorbidities were thyroid dysfunction ( = 27, 55.1%) and renal disease ( = 15, 30.6%). During follow-up, 10 patients (20.4%) died. On univariate analysis, renal disease and liver cirrhosis were predictors of all-cause mortality. On multivariate analysis, only renal disease (hazard ratio (HR): 12.5, 95% confidence interval (CI): 1.5-106.3, = 0.021) remained as an independent predictor.

Conclusions: SVP patients ≥40 years of age are burdened with significant morbidity and mortality. Renal disease is an independent predictor of all-cause mortality.
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http://dx.doi.org/10.3390/jcm9124085DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7765901PMC
December 2020

Pregnancy Outcomes in Women After Arterial Switch Operation for Transposition of the Great Arteries: Results From ROPAC (Registry of Pregnancy and Cardiac Disease) of the European Society of Cardiology EURObservational Research Programme.

J Am Heart Assoc 2021 01 22;10(1):e018176. Epub 2020 Dec 22.

Department of Cardiology Erasmus University Medical Center Rotterdam the Netherlands.

Background In the past 3 decades, the arterial switch procedure has replaced the atrial switch procedure as treatment of choice for transposition of the great arteries. Although survival is superior after the arterial switch procedure, data on pregnancy outcomes are scarce and transposition of the great arteries after arterial switch is not yet included in the modified World Health Organization classification of maternal cardiovascular risk. Methods and Results The ROPAC (Registry of Pregnancy and Cardiac disease) is an international prospective registry of pregnant women with cardiac disease, part of the European Society of Cardiology EURObservational Research Programme. Pregnancy outcomes in all women after an arterial switch procedure for transposition of the great arteries are described. The primary end point was a major adverse cardiovascular event, defined as combined end point of maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischemic coronary events, and thromboembolic events. Altogether, 41 pregnant women (mean age, 26.7±3.9 years) were included, and there was no maternal mortality. A major adverse cardiovascular event occurred in 2 women (4.9%): heart failure in one (2.4%) and ventricular tachycardia in another (2.4%). One woman experienced fetal loss, whereas no neonatal mortality was observed. Conclusions Women after an arterial switch procedure for transposition of the great arteries tolerate pregnancy well, with a favorable maternal and fetal outcome. During counseling, most women should be reassured that the risk of pregnancy is low. Classification as modified World Health Organization risk class II seems appropriate.
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http://dx.doi.org/10.1161/JAHA.120.018176DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7955508PMC
January 2021

Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years.

J Clin Med 2020 Dec 17;9(12). Epub 2020 Dec 17.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany.

Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking.

Methods: PAH-ACHD patients ≥ 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality.

Results: In total, 65 patients (67.7% female, mean age 45.19 ± 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2-7.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2-14.4, = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2-118.7, = 0.006) remained as independent predictors of all-cause mortality.

Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors.
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http://dx.doi.org/10.3390/jcm9124071DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7766787PMC
December 2020

The Fontan operation: Possible anywhere?

Authors:
Oktay Tutarel

Int J Cardiol 2021 04 8;328:96. Epub 2020 Dec 8.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, Munich, Germany; DZHK (German Centre for Cardiovascular Research), partner site Munich Heart Alliance, Munich, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2020.12.001DOI Listing
April 2021

Expanding the indications for sacubitril/valsartan to unchartered territories.

Int J Cardiol 2020 12 24;321:128. Epub 2020 Jul 24.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, Munich, Germany; DZHK (German Centre for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2020.07.033DOI Listing
December 2020

Tetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect after the Age of 40 Years: A Single Center Study.

J Clin Med 2020 May 19;9(5). Epub 2020 May 19.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany.

Background The population of adults with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA/VSD) is growing and aging. Data regarding older patients are scarce. Prognostic outcome parameters in adults with TOF or PA/VSD ≥ 40 years were studied.

Methods: This was a retrospective study of patients ≥ 40 years of age during the study period (January 2005-March 2018). Major adverse cardiac events (MACE) were a combined primary endpoint including death from any cause, prevented sudden cardiac death, pacemaker implantation, arrhythmia, and new-onset heart failure. Additionally, MACE II (secondary endpoint) was a combination of death from any cause and prevented sudden cardiac death.

Results: 184 (58.7% female, mean age 45.3 ± 7.2 years) patients were included (159 (86.4%) TOF and 25 (13.6%) PA/VSD). During a median follow-up of 3.1 years (IQR: 0.6-6.5), MACE occurred in 35 and MACE II in 13 patients. On multivariable analysis, New York Heart Association class [HR: 2.1, 95% CI: 1.2-3.6, = 0.009] emerged as an independent predictor for MACE, and age at corrective surgery [HR: 13.2, 95% CI: 1.6-107.1, = 0.016] for MACE II.

Conclusions: Adults with TOF or PA/VSD ≥ 40 years are burdened with significant morbidity and mortality. New York Heart Association class and age at corrective surgery were independent predictors of outcome.
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http://dx.doi.org/10.3390/jcm9051533DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290291PMC
May 2020

Continuous, complete and comparable NT-proBNP reference ranges in healthy children.

Clin Chem Lab Med 2020 08 18;58(9):1509-1516. Epub 2020 Apr 18.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, Munich, Germany.

Background: NT-proBNP is one of the most important biomarkers for the diagnosis and risk assessment of heart failure in adults. Age- and gender-independent reference intervals (RIs) have been reported. In contrast, RIs in children are strongly age-dependent, do not exist for all ages and reveal a right-skewed distribution. Accordingly, no common Z-score can be formed and a cross-age interpretive method, so far, is missing.

Methods: Within the paper on hand, new evaluation techniques are applied to already published NT-proBNP study results and additionally to newly gained data. Upper limits (ULs), lower limits (LLs) and 50th percentiles are tested for power-like behavior as a function of age using linear regression analysis. Functions for continuous RIs are derived and reference limits are calculated on a per day basis. A corresponding Zlog formula is deduced and its usefulness is stated in two clinical examples.

Results: The power-like behavior of NT-proBNP concentration from birth to 18 years is demonstrated. With age in days t and measured NT-proBNP value x in pg/mL, an age-specific Zlog value may directly be calculated using the equation:ZlogNT-proBNP=log x+0.512⋅log t-3.4171.489+0.014⋅log t⋅3.92${\rm{Zlo}}{{\rm{g}}_{{\rm{NT - proBNP}}}} = {{\log \;x + 0.512 \cdot \log \;t - 3.417} \over {1.489 + 0.014 \cdot \log \;t}} \cdot 3.92$.

Conclusions: Using formulas for UL and LL, continuous RIs from 0 to 18 years may be obtained. Continuity corresponds to physiological changes in the body much better than discrete RIs. With the advent of an NT-proBNP-specific Zlog value, a cross-age Z-score equivalent is providing an easy interpretation aid in everyday pediatric practice. This new approach allows to identify clinical worsening much better, sooner and more clearly than previous absolute values.
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http://dx.doi.org/10.1515/cclm-2019-1185DOI Listing
August 2020

Direct oral anticoagulants in adults with congenital heart disease - Role of chronic kidney disease.

Int J Cardiol 2020 03 28;302:45. Epub 2019 Dec 28.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, Munich, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2019.12.056DOI Listing
March 2020

Direct oral anticoagulants in adults with congenital heart disease - a single centre study.

Int J Cardiol 2020 02 11;300:127-131. Epub 2019 Oct 11.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, Munich, Germany. Electronic address:

Background: Direct oral anticoagulants (DOACs) have been proven to be safe and effective in patients with acquired heart disease. However, data regarding their use in adults with congenital heart disease (ACHD) is scarce.

Methods: All ACHD under DOAC were retrospectively identified. Bleeding and thromboembolic events were registered. CHADS, CHADS-VASc and HASBLED scores were calculated. Risk factors for bleeding and thromboembolic events were identified.

Results: 215 ACHD patients (111 female, 48.4 ± 15.5 years) were included. CHD was severe in 44.2%, moderate in 23.7%, and simple in 32.1%. Indications for anticoagulation were: atrial arrhythmias (66.8%), cerebrovascular accidents (32.7%), pulmonary thromboembolism (3.7%), deep vein thrombosis (11.2%), atrial thrombi (6.5%), and Fontan circulation/TCPC (5.6%). Mean follow-up was 15.8 ± 15.8 months. Nine patients suffered a major and eight a minor bleeding. Thromboembolic events occurred in two patients. The annual risk for bleeding was 3.1%/patient/year and for thromboembolic events 0.7%/patient/year. A CHADS-Score >2, HASBLED >3, and renal disease were identified as risk factors for bleeding (minor and major bleeding) in a univariate analysis. In the multivariate analysis, renal disease remained as an independent predictor for bleeding (HR 6.13, CI [1.04-36.27], p < 0.05). It was also the only risk factor for major bleedings (HR 13.75, CI [2.60-72.54], p = 0.002). Risk factors for thromboembolic events were not identified.

Conclusions: A low rate of thromboembolic events was observed in ACHD patients under DOAC therapy. Bleeding complications were not negligible. Special attention has to be paid to those patients with advanced renal failure.
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http://dx.doi.org/10.1016/j.ijcard.2019.09.077DOI Listing
February 2020

Sarcopenia: An unrecognized, but important factor for adults with congenital heart disease.

Int J Cardiol 2019 12 14;296:63-64. Epub 2019 Aug 14.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, Munich, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2019.08.030DOI Listing
December 2019

Sacubitril/valsartan for heart failure in adults with complex congenital heart disease.

Int J Cardiol 2020 02 13;300:137-140. Epub 2019 Jun 13.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, Munich, Germany. Electronic address:

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 was retrieved from medical records.

Results: Altogether, 23 patients (mean age 41.2 ± 11.9 years, female 17.4%) were included. A systemic right ventricle was present in 12 pat. (52.2%), a single ventricle physiology in 4 (17.4%), and a systemic left ventricle in 7 (30.4%). During a median follow-up of 221 days [IQR 79-430], systemic ventricular function (p = 0.88) and functional status according to New York Heart Association class (p = 0.38) did not improve. While NT-proBNP levels did not change significantly under treatment (2561 ± 2042 ng/l vs. 1938 ± 1524 ng/l, p = 0.20), creatinine levels increased (1.14 ± 0.52 mg/dl vs. 1.35 ± 0.74 mg/dl, p = 0.002). Systolic (110 ± 15 mm Hg vs. 103 ± 14 mm Hg, p = 0.02) and diastolic blood pressures (68 ± 10 mm Hg vs. 61 ± 12 mm Hg, p = 0.01) were reduced under therapy. Five patients discontinued therapy, four of these due to side effects.

Conclusion: In this small group of complex ACHD patients with heart failure, treatment with sacubitril/valsartan did not improve systemic ventricular function or functional status. Renal function needs close surveillance.
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http://dx.doi.org/10.1016/j.ijcard.2019.06.031DOI Listing
February 2020

sST2: a new kid on the block for patients with ACHD.

Authors:
Oktay Tutarel

Heart 2019 07 12;105(13):972. Epub 2019 Mar 12.

Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Munich, Germany.

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http://dx.doi.org/10.1136/heartjnl-2019-314699DOI Listing
July 2019

Left Atrial Myxoma.

Circ Cardiovasc Imaging 2019 03;12(3):e008820

Department of Paediatric Cardiology and Congenital Heart Disease (S.S., S.J.M., C.P.S., C.M., P.E., O.T.), Technical University of Munich.

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http://dx.doi.org/10.1161/CIRCIMAGING.118.008820DOI Listing
March 2019

Out-of-hospital cardiac arrest in adults with congenital heart disease: More questions than answers?

Authors:
Oktay Tutarel

Int J Cardiol 2019 03 20;278:88. Epub 2018 Dec 20.

Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich, Munich, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2018.12.055DOI Listing
March 2019

Psychosocial situation in adults with congenital heart defects today and 20 years ago: Any changes?

Int J Cardiol 2019 Jan 12;275:70-76. Epub 2018 Oct 12.

Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich (TUM), Munich, Germany. Electronic address:

Background: While diagnosis and treatment of congenital heart diseases have improved over the last two decades, data regarding the course of psychosocial parameters is missing.

Methods: In a cross-sectional study, 283 adults with congenital heart disease completed a slightly modified questionnaire that was applied in a comparable study twenty years ago. Significant differences between the two populations as well as possible predictors of psychosocial burden for the recent population were sought.

Results: Despite the presence of more complex heart defects in the current cohort (p < 0.001), both populations exhibited similar values in the Ability Index. Furthermore, the current cohort reported significantly improved outcomes regarding school performance, employment, and sports. Regarding psychosocial functioning, the current cohort showed better outcomes in the domains of sadness (p < 0.01), independence (p < 0.01), understanding (p < 0.001), and acceptance (p < 0.01) of heart disease. Predictors for a worse psychosocial situation in a multiple regression analysis were anxiety, lack of curiosity, and age over 33. In the current study women, as opposed to men, reported significantly more dissatisfaction with too little information provided about their illness (p < 0.05), higher anxiety levels (p < 0.01), and heightened illness-connected burden (p < 0.05). However, women showed higher levels of independence (p < 0.01) and lower alcohol consumption (p < 0.001).

Conclusion: The psychosocial situation of adults with congenital heart disease has improved over the span of 20 years. However, particular needs and concerns should be addressed individually via doctor-patient communication. The findings here suggest that especially female patients appear to have a higher demand for counselling information, e.g. reproduction issues.
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http://dx.doi.org/10.1016/j.ijcard.2018.10.030DOI Listing
January 2019

Cardiovascular risk factors in adults with congenital heart defects - Recognised but not treated? An analysis of the German National Register for Congenital Heart Defects.

Int J Cardiol 2019 Feb 4;277:79-84. Epub 2018 Aug 4.

Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich, Munich, Germany. Electronic address:

Background: As adult congenital heart disease (ACHD) patients are aging, a high prevalence of cardiovascular risk factors is encountered similar to the general population. Currently, data regarding the primary and secondary prevention of acquired cardiovascular disease in ACHD is lacking.

Methods: The German National Register of Congenital Heart Defects was systematically screened for ACHD patients with established cardiovascular risk factors or documented acquired cardiovascular conditions. Data were analyzed with regard to the according medical treatment.

Results: Overall, 539 patients were included (mean age 38.4 ± 17.7 years, 49.2% female). Diabetes was present in 57 pts. (10.6%), arterial hypertension in 113 pts. (21.0%), hyperlipidaemia in 81 pts. (15.0%) and obesity in 271 pts. (50.2%). 31 pts. (5.8%) were smokers. Coronary artery disease was established in 16 pts. (3.0%), peripheral vascular disease in 9 pts. (1.7%), and cerebrovascular accidents in 141 pts. (26.2%). Out of the patients with coronary artery disease only 81.3% received antithrombotic treatment. Only 18.8% were prescribed a statin. Of the pts. with peripheral arterial disease, 44.4% received an antiplatelet drug, and only 22.2% were on a statin. Patients with arterial hypertension received antihypertensive drugs in 66.4%.

Conclusions: Primary and secondary prevention of acquired cardiovascular disease in ACHD is underutilized. This highlights the importance of educating primary physicians as well as ACHD physicians about the need of primary and secondary prevention for acquired cardiovascular disease.
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http://dx.doi.org/10.1016/j.ijcard.2018.08.009DOI Listing
February 2019

Medical Management of the Systemic Right Ventricle.

Heart 2018 07;104(14):1226-1227

Division of Cardiology, University of Nebraska College of Medicine and Children's Hospital and Medical Center, Omaha, Nebraska, USA.

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http://dx.doi.org/10.1136/heartjnl-2018-313349DOI Listing
July 2018
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