Publications by authors named "Gerhard-Paul Diller"

175 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

Maternal and neonatal complications in women with congenital heart disease: a nationwide analysis.

Eur Heart J 2021 Oct 12. Epub 2021 Oct 12.

Department of Cardiology III, Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Münster, Germany.

Aims: The aim of this study was to provide population-based data on maternal and neonatal complications and outcome in the pregnancies of women with congenital heart disease (CHD).

Methods And Results: Based on administrative data from one of the largest German Health Insurance Companies (BARMER GEK, ∼9 million members representative for Germany), all pregnancies in women with CHD between 2005 and 2018 were analysed. In addition, an age-matched non-CHD control group was included for comparison and the association between adult CHD (ACHD) and maternal or neonatal outcomes investigated. Overall, 7512 pregnancies occurred in 4015 women with CHD. The matched non-CHD control group included 6502 women with 11 225 pregnancies. Caesarean deliveries were more common in CHD patients (40.5% vs. 31.5% in the control group; P < 0.001). There was no excess mortality. Although the maternal complication rate was low in absolute terms, women with CHD had a significantly higher rate of stroke, heart failure and cardiac arrhythmias during pregnancy (P < 0.001 for all). Neonatal mortality was low but also significantly higher in the ACHD group (0.83% vs. 0.22%; P = 0.001) and neonates to CHD mothers had low/extremely low birth weight or extreme immaturity (<0.001) or required resuscitation and mechanical ventilation more often compared to non-CHD offspring (P < 0.001 for both). On multivariate logistic regression maternal defect complexity, arterial hypertension, heart failure, prior fertility treatment, and anticoagulation with vitamin K antagonists emerged as significant predictors of adverse neonatal outcome (P < 0.05 for all). Recurrence of CHD was 6.1 times higher in infants to ACHD mothers compared to controls (P < 0.0001).

Conclusions: This population-based study illustrates a reassuringly low maternal mortality rate in a highly developed healthcare system. Nevertheless, maternal morbidity and neonatal morbidity/mortality were significantly increased in women with ACHD and their offspring compared to non-ACHD controls highlighting the need of specialized care and pre-pregnancy counselling.
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http://dx.doi.org/10.1093/eurheartj/ehab571DOI Listing
October 2021

Treatment of adults with Eisenmenger syndrome-state of the art in the 21st century: a short overview.

Cardiovasc Diagn Ther 2021 Aug;11(4):1190-1199

Toronto Adult Congenital Heart Disease Program, Division of Cardiology, Peter Munk Cardiac Centre, University Health Network and University of Toronto, Toronto, Ontario, Canada.

Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. In developed countries, cardiac defects are being operated on in a timely manner, before pulmonary vascular disease develops. However, with rising immigration from underserved countries, we increasingly see patients with shunt lesions, that are not amenable for repair as pulmonary vascular disease has already established. ES describes a symptom complex and patients present with heterogeneous problems involving many organ systems (multisystem disorder). Care in tertiary specialist cardiac centers with access to multidisciplinary subspecialities is required. Central cyanosis with secondary erythrocytosis is one of the key features of patients with ES. Clinical consequences of longstanding hypoxia can lead to other organ complications, that involve other organs than the heart alone. Although ES patients have a better prognosis compared to other patients with pulmonary arterial hypertension, ES grossly affects quality of life and morbidity is frequent. Follow-up and care at specialist congenital heart disease centers is highly recommended to prevent, to early diagnose and to timely manage complications of ES. This is necessary to maintain functional capacity, decrease morbidity and increase life expectancy for these vulnerable patients. The leading reasons for mortality are sudden cardiac death, progressive heart failure, and infectious diseases. Various factors have been shown to be associated with mortality like decreased arterial oxygen saturation, functional class, impaired exercise tolerance, syncopal events, iron deficiency, presence of pre-tricuspid shunts, arrhythmias, increased (NT-pro) brain natriuretic peptide, echocardiographic variables of right ventricular dysfunction and hospitalization for heart failure. Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES.
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http://dx.doi.org/10.21037/cdt-21-135DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8410485PMC
August 2021

Study design and rationale of the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R).

ESC Heart Fail 2021 Sep 12. Epub 2021 Sep 12.

A.O.U Cittá della Salute e della Scienza, Torino, Italy.

Aim: Due to improved therapy in childhood, many patients with congenital heart disease reach adulthood and are termed adults with congenital heart disease (ACHD). ACHD often develop heart failure (HF) as a consequence of initial palliative surgery or complex anatomy and subsequently require advanced HF therapy. ACHD are usually excluded from trials evaluating heart failure therapies, and in this context, more data about heart failure trajectories in ACHD are needed to guide the management of ACHD suffering from HF.

Methods And Results: The pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R) will collect data from ACHD evaluated or listed for heart or heart-combined organ transplantation from 16 countries in Europe and the Asia/Pacific region. We plan retrospective collection of data from 1989-2020 and will include patients prospectively. Additional organizations and hospitals in charge of transplantation of ACHD will be asked in the future to contribute data to the register. The primary outcome is the combined endpoint of delisting due to clinical worsening or death on the waiting list. The secondary outcome is delisting due to clinical improvement while on the waiting list. All-cause mortality following transplantation will also be assessed. The data will be entered into an electronic database with access to the investigators participating in the register. All variables of the register reflect key components important for listing of the patients or assessing current HF treatment.

Conclusion: The ARTORIA-R will provide robust information on current management and outcomes of adults with congenital heart disease suffering from advanced heart failure.
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http://dx.doi.org/10.1002/ehf2.13574DOI Listing
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

Optimizing Care for Adults with Congenital Heart Disease: Results of a Conjoint Analysis Based on a Nationwide Sample of Patients Included in the German National Register.

J Clin Med 2021 Aug 6;10(16). Epub 2021 Aug 6.

Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer Campus 1, 48149 Münster, Germany.

(1) Background: Congenital heart disease (CHD) requires lifelong specialized care. Failure to follow up and gaps in care are common in this group and lead to increased morbidity/mortality. We evaluated patients' perceived needs and expectations regarding specialized care using state-of-the-art statistical and market research techniques based on a nationwide sample of CHD patients. (2) Methods: A random sample of adults with CHD registered in the German National Register for Congenital Heart Defects were invited to answer an adaptive online questionnaire based on the conjoint analysis (CA) technique. CA determines the relative importance of various aspects of health care provision and allows individuals to trade between characteristics, thus recognizing limited resources. (3) Results: 637 patients participated (mean age 33.8 ± 12.6 years; 55.6% female; disease complexity: simple defect 12.6%, moderate complexity 40.3%, complex CHD 40.2%) in the analysis. Patients assigned the highest relative importance to aspects of patient-physician communication, physician qualifications, waiting time, medical care, and medical equipment. Comfort-related aspects such as driving time or hotel aspects of care received much lower scores. We identified four well-defined clusters of patients with differing expectation patterns: (i) time sensitive patients; (ii) excellence seeking patients; (iii) continuity seekers, and (iv) support seeking patients. (4) Conclusions: Adult CHD patients rank effective patient-physician interaction and communication as the most important factors. As we identified significant heterogeneity between CHD patients, centers should cater for individual preferences and integrate individual needs into treatment plans to prevent failure to follow up and ensure patient compliance.
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http://dx.doi.org/10.3390/jcm10163483DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8396998PMC
August 2021

Impact of specialized electrophysiological care on the outcome of catheter ablation for supraventricular tachycardias in adults with congenital heart disease: Independent risk factors and gender aspects.

Heart Rhythm 2021 Jul 13. Epub 2021 Jul 13.

Department of Cardiology III, Adult Congenital and Valvular Heart Disease, University Hospital Münster, Münster, Germany.

Background: Limited data exist on the impact of gender and specialized care on the requirement of repeat treatment of supraventricular tachycardia (SVT) in adult patients with congenital heart disease (ACHDs).

Objective: The study aimed to assess independent predictors of a combined end point of re-catheter ablation (CA) or cardioversion at 3 years of follow-up, including the impact of gender and specialized ACHD care.

Methods: All ACHDs registered in a database of one of the largest German health insurers (≈9.2 million members) who underwent CA for SVT were analyzed.

Results: Of 38,892 ACHDs 16 years or older, 485 (49.5% women; median age 58.4 years; interquartile range 42.1-70.8 years) underwent CA for SVT. Over 3-year follow-up, the number of yearly CA procedures increased significantly, particularly for atrial fibrillation (+195%) and atrial flutter (+108%). Moderate to severe complexity heart disease (odds ratio [OR] 1.66; P = .01), advanced age (OR 1.85 per year; P = .02), chronic kidney disease (OR 1.70; P = .01), and atrial fibrillation (OR 2.02; P = .002) emerged as independent predictors of retreatment. Retreatment was significantly less often performed if primary CA was carried out at a specialized CHD center (P = .009) in patients with moderate to severe complexity heart disease. Women treated in specialist centers had a 1.6-fold reduced risk of undergoing retreatment (P = .01).

Conclusion: CA for SVT is increasingly performed in ACHDs, especially for atrial flutter and atrial fibrillation. Patients with moderate and severe complexity congenital heart defects and female ACHDs benefit from upfront referral to specialized CHD centers for CA. Centralization of care for ACHD arrhythmias should thus be advocated.
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http://dx.doi.org/10.1016/j.hrthm.2021.07.009DOI Listing
July 2021

Lack of specialist care is associated with increased morbidity and mortality in adult congenital heart disease: a population-based study.

Eur Heart J 2021 Jul 16. Epub 2021 Jul 16.

Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer Campus 1, Building A1, Münster 48149, Germany.

Aims: The aim of this study was to provide population-based data on the healthcare provision for adults with congenital heart disease (ACHD) and the impact of cardiology care on morbidity and mortality in this vulnerable population.

Methods And Results: Based on administrative data from one of the largest German Health Insurance Companies, all insured ACHD patients (<70 years of age) were included. Patients were stratified into those followed exclusively by primary care physicians (PCPs) and those with additional cardiology follow-up between 2014 and 2016. Associations between level of care and outcome were assessed by multivariable/propensity score Cox analyses. Overall, 24 139 patients (median age 43 years, 54.8% female) were included. Of these, only 49.7% had cardiology follow-up during the 3-year period, with 49.2% of patients only being cared for by PCPs and 1.1% having no contact with either. After comprehensive multivariable and propensity score adjustment, ACHD patients under cardiology follow-up had a significantly lower risk of death [hazard ratio (HR) 0.81, 95% confidence interval (CI) 0.67-0.98; P = 0.03) or major events (HR 0.85, 95% CI 0.78-0.92; P < 0.001) compared to those only followed by PCPs. At 3-year follow-up, the absolute risk difference for mortality was 0.9% higher in ACHD patients with moderate/severe complexity lesions cared by PCPs compared to those under cardiology follow-up.

Conclusion: Cardiology care compared with primary care is associated with superior survival and lower rates of major complications in ACHD. It is alarming that even in a high resource setting with well-established specialist ACHD care approximately 50% of contemporary ACHD patients are still not linked to regular cardiac care. Almost all patients had at least one contact with a PCP during the study period, suggesting that opportunities to refer patients to cardiac specialists were missed at PCP level. More efforts are required to alert PCPs and patients to appropriate ACHD care.
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http://dx.doi.org/10.1093/eurheartj/ehab422DOI Listing
July 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

Lifespan Perspective on Congenital Heart Disease Research: JACC State-of-the-Art Review.

J Am Coll Cardiol 2021 May;77(17):2219-2235

McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit), Department of Medicine, McGill University, Montréal, Québec, Canada. Electronic address:

More than 90% of patients with congenital heart disease (CHD) are nowadays surviving to adulthood and adults account for over two-thirds of the contemporary CHD population in Western countries. Although outcomes are improved, surgery does not cure CHD. Decades of longitudinal observational data are currently motivating a paradigm shift toward a lifespan perspective and proactive approach to CHD care. The aim of this review is to operationalize these emerging concepts by presenting new constructs in CHD research. These concepts include long-term trajectories and a life course epidemiology framework. Focusing on a precision health, we propose to integrate our current knowledge on the genome, phenome, and environome across the CHD lifespan. We also summarize the potential of technology, especially machine learning, to facilitate longitudinal research by embracing big data and multicenter lifelong data collection.
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http://dx.doi.org/10.1016/j.jacc.2021.03.012DOI Listing
May 2021

Antiarrhythmic drugs-safety and efficacy during pregnancy.

Herzschrittmacherther Elektrophysiol 2021 Jun 29;32(2):145-151. Epub 2021 Mar 29.

Electrophysiology Bremen, Heart Center Bremen, Bremen, Germany.

When deciding on antiarrhythmic drug (AAD) treatment, a thorough knowledge of the physiological adaptation processes that occur during pregnancy and their effect on metabolism and the efficacy of AAD is mandatory. Beyond the desired effects of AAD therapy, side effects can occur in pregnant women. Furthermore, potential harm to fetal development-depending on gestational age-needs to be considered. A thorough evaluation of potential risks opposed to expected benefits for mother and fetus should be carried out before initiation of AAD treatment. Regular maternal echocardiography and fetal sonographic examination during pregnancy under AAD treatment are advisable. If possible, serum concentrations of AAD should be measured on a regular basis. Due to electrolyte and volume imbalances after delivery, maternal monitoring is recommended for approximately 48 h under AAD therapy. Current guidelines are based on almost historic analyses, where AAD were often prescribed for other indications than rhythm disorders. In clinical practice, AAD predominantly used during pregnancy are intravenous adenosine for acute treatment of atrioventricular nodal dependent tachycardias, whereas betablockers, sotalol, and flecainide can be orally administered for long-term therapy.
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http://dx.doi.org/10.1007/s00399-021-00759-2DOI Listing
June 2021

Management considerations in the adult with surgically modified d-transposition of the great arteries.

Heart 2021 Oct 19;107(20):1613-1619. Epub 2021 Mar 19.

Department of Cardiology and Angiology, Adult Congenital and Valvular Heart Disease Center, University Hospital Muenster, Muenster, Germany.

Dextro-transposition of the great arteries (D-TGA) has undergone a significant evolution in surgical repair, leading to survivors with vastly different postsurgical anatomy which in turn guides their long-term cardiovascular morbidity and mortality. Atrial switch repair survivors are limited by a right ventricle in the systemic position, arrhythmia and atrial baffles prone to obstruction or leak. Functional assessment of the systemic right ventricle is complex, requiring multimodality imaging to include specialised echocardiography and cross-sectional imaging (MRI and CT). In the current era, most neonates undergo the arterial switch operation with increasing understanding of near-term and long-term outcomes specific to their cardiac anatomy. Long-term observations of the Lecompte manoeuvre or coronary stenoses following transfer continue, with evolving understanding to improve surveillance. Ultimately, an understanding of postsurgical anatomy, specialised imaging techniques and interventional and electrophysiological procedures is essential to comprehensive care of D-TGA survivors.
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http://dx.doi.org/10.1136/heartjnl-2020-318833DOI Listing
October 2021

Peripheral microangiopathy in Eisenmenger syndrome: A nailfold video capillaroscopy study.

Int J Cardiol 2021 08 16;336:54-59. Epub 2021 Mar 16.

Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, 49 Konstantinoupoleos Street, 54642 Thessaloniki, Greece. Electronic address:

Background: Eisenmenger syndrome (ES) comprises a severe phenotype of pulmonary arterial hypertension characterized by angiopathy of the lung circulation. The aim of the present study was to demonstrate the presence of systemic microvascular abnormalities in patients with ES using nailfold video-capillaroscopy (NVC) and to identify potential correlations of nailfold capillaroscopic characteristics with non-invasive markers of systemic organ function.

Methods: Α cross-sectional NVC study was performed in 17 consecutive patients with ES and 17 healthy controls matched for age and sex. NVC quantitative (capillary density, capillary dimensions, haemorrhages, thrombi, shape abnormalities) and qualitative (normal, non-specific or scleroderma pattern) parameters were evaluated.

Results: Patients with ES [median age 40 (18-65) years, 11 women] presented reduced capillary density [8.8 (7.2-10.2) loops/mm vs. 9.9 (8.3-10.9) loops/mm, p = .004] and increased loop width [15.9 (10.3-21.7) μm vs. 12.3 (7.6-15.2) μm, p < .001], while they had significantly more abnormal capillaries than healthy controls [2.5 (0.9-5.4) abnormal loops/mm vs. 1.0 (0.0-1.7) abnormal loops/mm, p < .001]. NVC shape abnormalities in ES were positively correlated with NT-proBNP (r = 0.52, p = .03) and were negatively associated with estimated glomerular filtration rate (r = -0.60, p = .02). Additionally, capillary loop diameter was positively correlated with increased haemoglobin levels (r = 0.55, p = .03) and negatively correlated with reduced peripheral oxygen saturation (r = - 0.56, p = .02).

Conclusions: This study supports the hypothesis of peripheral microvascular involvement in ES parallel to pulmonary microangiopathy detected by NVC. Further longitudinal studies are needed to confirm our preliminary results.
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http://dx.doi.org/10.1016/j.ijcard.2021.03.033DOI Listing
August 2021

A new approach to modelling in adult congenital heart disease: artificial intelligence.

Rev Esp Cardiol (Engl Ed) 2021 07 18;74(7):573-575. Epub 2021 Jan 18.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany.

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http://dx.doi.org/10.1016/j.rec.2020.12.008DOI Listing
July 2021

Coronavirus disease 2019 in adults with congenital heart disease: a position paper from the ESC working group of adult congenital heart disease, and the International Society for Adult Congenital Heart Disease.

Eur Heart J 2021 05;42(19):1858-1865

ACHD Unit, Erasmus Medical Center, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands.

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)-by definition-have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care.
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http://dx.doi.org/10.1093/eurheartj/ehaa960DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7799120PMC
May 2021

Congenital heart defects as an intrinsic additional risk factor for the occurrence and outcome of myocardial infarction.

Eur Heart J 2021 07;42(26):2587-2589

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany.

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http://dx.doi.org/10.1093/eurheartj/ehaa892DOI Listing
July 2021

Current use and safety of novel oral anticoagulants in adults with congenital heart disease: results of a nationwide analysis including more than 44 000 patients.

Eur Heart J 2020 11;41(43):4168-4177

Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert Schweitzer Campus 1, A1, 48149 Muenster, Germany.

Aims: To evaluate the use of novel oral anticoagulants (NOACs) compared with vitamin K antagonists (VKAs) in adult congenital heart disease (ACHD) and assess outcome in a nationwide analysis.

Methods And Results: Using data from one of Germany's largest Health Insurers, all ACHD patients treated with VKAs or NOACs were identified and changes in prescription patterns were assessed. Furthermore, the association between anticoagulation regimen and complications including mortality was studied. Between 2005 and 2018, the use of oral anticoagulants in ACHD increased from 6.3% to 12.4%. Since NOACs became available their utilization increased constantly, accounting for 45% of prescribed anticoagulants in ACHD in 2018. Adult congenital heart disease patients on NOACs had higher thromboembolic (3.8% vs. 2.8%), MACE (7.8% vs. 6.0%), bleeding rates (11.7% vs. 9.0%), and all-cause mortality (4.0% vs. 2.8%; all P < 0.05) after 1 year of therapy compared with VKAs. After comprehensive adjustment for patient characteristics, NOACs were still associated with increased risk of MACE (hazard rate-HR 1.22; 95% CI 1.09-1.36) and increased all-cause mortality (HR 1.43; 95% CI 1.24-1.65; both P < 0.001), but also bleeding (HR 1.16; 95% CI 1.04-1.29; P = 0.007) during long-term follow-up.

Conclusion: Despite the lack of prospective studies in ACHD, NOACs are increasingly replacing VKAs and now account for almost half of all oral anticoagulant prescriptions. Particularly, NOACs were associated with excess long-term risk of MACE, and mortality in this nationwide analysis, emphasizing the need for prospective studies before solid recommendations for their use in ACHD can be provided.
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http://dx.doi.org/10.1093/eurheartj/ehaa844DOI Listing
November 2020

Heart Failure Results in Inspiratory Muscle Dysfunction Irrespective of Left Ventricular Ejection Fraction.

Respiration 2021;100(2):96-108. Epub 2020 Nov 10.

Department of Cardiology I, University Hospital Muenster, Muenster, Germany.

Background: Exercise intolerance in heart failure with reduced ejection fraction (HFrEF) or heart failure with preserved ejection fraction (HFpEF) results from both cardiac dysfunction and skeletal muscle weakness. Respiratory muscle dysfunction with restrictive ventilation disorder may be present irrespective of left ventricular ejection fraction and might be mediated by circulating pro-inflammatory cytokines.

Objective: To determine lung and respiratory muscle function in patients with HFrEF/HFpEF and to determine its associations with exercise intolerance and markers of systemic inflammation.

Methods: Adult patients with HFrEF (n = 22, 19 male, 61 ± 14 years) and HFpEF (n = 8, 7 male, 68 ± 8 years) and 19 matched healthy control subjects underwent spirometry, measurement of maximum mouth occlusion pressures, diaphragm ultrasound, and recording of transdiaphragmatic and gastric pressures following magnetic stimulation of the phrenic nerves and the lower thoracic nerve roots. New York Heart Association (NYHA) class and 6-min walking distance (6MWD) were used to quantify exercise intolerance. Levels of circulating interleukin 6 (IL-6) and tumor necrosis factor-α (TNF-α) were measured using ELISAs.

Results: Compared with controls, both patient groups showed lower forced vital capacity (FVC) (p < 0.05), maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) (p < 0.05), diaphragm thickening ratio (p = 0.01), and diaphragm strength (twitch transdiaphragmatic pressure in response to supramaximal cervical magnetic phrenic nerve stimulation) (p = 0.01). In patients with HFrEF, NYHA class and 6MWD were both inversely correlated with FVC, PImax, and PEmax. In those with HFpEF, there was an inverse correlation between amino terminal pro B-type natriuretic peptide levels and FVC (r = -0.77, p = 0.04). In all HF patients, IL-6 and TNF-α were statistically related to FVC.

Conclusions: Irrespective of left ventricular ejection fraction, HF is associated with respiratory muscle dysfunction, which is associated with increased levels of circulating IL-6 and TNF-α.
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http://dx.doi.org/10.1159/000509940DOI Listing
November 2020

European Society of Cardiology Working Group on Adult Congenital Heart Disease and Study Group for Adult Congenital Heart Care in Central and South Eastern European Countries consensus paper: current status, provision gaps and investment required.

Eur J Heart Fail 2021 03 19;23(3):445-453. Epub 2020 Nov 19.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

Aims: To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region.

Methods And Results: We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for 2017 and/or 2018. Thirteen countries reported at least one tertiary ACHD centre with a median year of centre establishment in 2007 (interquartile range 2002-2013). ACHD centres reported a median of 2114 patients under active follow-up with an annual cardiac catheter and surgical intervention volume of 49 and 40, respectively. The majority (90%) of catheter or surgical interventions were funded by government reimbursement schemes. However, all 19 countries had financial caps on a hospital level, leading to patient waiting lists and restrictions in the number of procedures that can be performed. The median number of ACHD specialists per country was 3. The majority of centres (75%) did not have ACHD specialist nurses. The six countries with no dedicated ACHD centres had lower Gross Domestic Product per capita compared to the remainder (P = 0.005).

Conclusion: The majority of countries in CESEE now have established ACHD services with adequate infrastructure and a patient workload comparable to the rest of Europe, but important gaps still exist. ACHD care is challenged or compromised by limited financial resources, insufficient staffing levels, and reimbursement caps on essential procedures compared to Western Europe. Active advocacy and increased resources are required to address the inequalities of care across the continent.
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http://dx.doi.org/10.1002/ejhf.2040DOI Listing
March 2021

The impact of Philanthropy on Cardiovascular Medicine.

Eur Heart J 2020 11;41(43):4158-4161

Adult Congenital Heart Centre & National Centre for Pulmonary Arterial Hypertension, Royal Brompton and Harefield NHS Trust, National Heart & Lung Institute, Imperial College, London, UK.

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http://dx.doi.org/10.1093/eurheartj/ehaa894DOI Listing
November 2020

Mortality and morbidity in patients with congenital heart disease hospitalised for viral pneumonia.

Heart 2020 Oct 27. Epub 2020 Oct 27.

Adult Congenital and Valvular Heart Disease Center, Department of Cardiology and Angiology, University Hospital Muenster, Muenster, Germany.

Objectives: Data on the clinical outcome of patients with congenital heart disease (CHD) affected by severe viral pneumonia are limited. We analysed morbidity and mortality of viral pneumonia and evaluated the association between medical conditions, medication, vaccination and outcome specifically in patients with CHD requiring hospitalisation for viral pneumonia.

Methods: Based on data from one of Germany's largest health insurers, all cases of viral pneumonia requiring hospital admission (2005-2018) were studied. Mortality, and composites of death, transplantation, mechanical circulatory support, ventilation or extracorporeal lung support served as endpoints.

Results: Overall, 26 262 viral pneumonia cases occurred in 24 980 patients. Of these, 1180 cases occurred in patients with CHD. Compared with patients without CHD, mortality rate was elevated in patients with CHD. As a group, patients with CHD aged 20-59 years even exceeded mortality rates in patients without CHD aged >60 years. No mortality was observed in patients with CHD with simple defects <60 years of age without associated cardiovascular risk factors. On multivariable logistic regression analysis, age, CHD complexity, chromosomal anomalies, cardiac medication, use of immunosuppressants and absence of vaccination for influenza emerged as risk factors of adverse outcome.

Conclusions: We present timely data on morbidity and mortality of severe viral pneumonia requiring hospital admission in patients with CHD. Need for mechanical ventilation and risk of death in CHD increase early in life, reaching a level equivalent to non-CHD individuals >60 years of age. Our data suggest that except for patients with isolated simple defects, patients with CHD should be considered higher-risk individuals when faced with severe viral pneumonia.
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http://dx.doi.org/10.1136/heartjnl-2020-317706DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8223651PMC
October 2020

Utility of deep learning networks for the generation of artificial cardiac magnetic resonance images in congenital heart disease.

BMC Med Imaging 2020 10 8;20(1):113. Epub 2020 Oct 8.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer Campus 1, Muenster, Germany.

Background: Deep learning algorithms are increasingly used for automatic medical imaging analysis and cardiac chamber segmentation. Especially in congenital heart disease, obtaining a sufficient number of training images and data anonymity issues remain of concern.

Methods: Progressive generative adversarial networks (PG-GAN) were trained on cardiac magnetic resonance imaging (MRI) frames from a nationwide prospective study to generate synthetic MRI frames. These synthetic frames were subsequently used to train segmentation networks (U-Net) and the quality of the synthetic training images, as well as the performance of the segmentation network was compared to U-Net-based solutions trained entirely on patient data.

Results: Cardiac MRI data from 303 patients with Tetralogy of Fallot were used for PG-GAN training. Using this model, we generated 100,000 synthetic images with a resolution of 256 × 256 pixels in 4-chamber and 2-chamber views. All synthetic samples were classified as anatomically plausible by human observers. The segmentation performance of the U-Net trained on data from 42 separate patients was statistically significantly better compared to the PG-GAN based training in an external dataset of 50 patients, however, the actual difference in segmentation quality was negligible (< 1% in absolute terms for all models).

Conclusion: We demonstrate the utility of PG-GANs for generating large amounts of realistically looking cardiac MRI images even in rare cardiac conditions. The generated images are not subject to data anonymity and privacy concerns and can be shared freely between institutions. Training supervised deep learning segmentation networks on this synthetic data yielded similar results compared to direct training on original patient data.
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http://dx.doi.org/10.1186/s12880-020-00511-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7542728PMC
October 2020

Neurological complications in aortic coarctation: Results of a Nationwide analysis based on 11,907 patients.

Int J Cardiol 2021 01 14;322:114-120. Epub 2020 Aug 14.

Adult Congenital Heart Disease Programme, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, UK.

Background: Patients with coarctation of the aorta (CoA) are at increased risk of periprocedural and late neurological complications. Based on a nationwide inpatient dataset, we assessed the prevalence and risk factors for neurological complications in this cohort.

Method And Results: We included all CoA patients hospitalized in England between 1997 and 2015. The risk for procedural complications, subarachnoid bleeding and ischemic stroke were quantified, and risk factors investigated. Overall, 11,907 patients (60% male) with CoA were identified. The median age at first presentation was 0.6 years with a median follow-up period of 14.4 years. Throughout the study period, 8456 surgical or interventional procedures were performed in 6796 patients. The neurological complication rate for primary surgical or interventional repair in patients born after 1997 was 0.05% and 0.2%, respectively. During a total follow-up period of 146,295 patient-years, late neurological complications such as subarachnoid bleeding and cerebral infarction occurred in 225 patients (0.15%/patient-year). The median age for subarachnoid bleeding was 29 years and 57 years for ischemic stroke. While, arterial hypertension (p = .0014), current smoking (p = .02), dyslipidaemia (p = .007) and diabetes (p = .037) were significantly related to the risk of ischemic stroke, only arterial hypertension emerged as significant risk factor for subarachnoid bleeding.

Conclusions: Neurological complication during primary CoA repair are extremely rare in the current era. In contrast, many CoA patients are afflicted by late complications such as subarachnoid bleeding and ischemic stroke at a relatively young age, emphasizing the need for meticulous and pro-active blood pressure control and reduction of cardiovascular risk factors.
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http://dx.doi.org/10.1016/j.ijcard.2020.08.041DOI Listing
January 2021

Inspiratory muscle dysfunction and restrictive lung function impairment in congenital heart disease: Association with immune inflammatory response and exercise intolerance.

Int J Cardiol 2020 11 4;318:45-51. Epub 2020 Jul 4.

Department of Cardiology III, University Hospital Muenster, Muenster, Germany.

Background: In adult patients with congenital heart disease (ACHD), both underlying disease and lung restriction contribute to exercise intolerance. In ACHD the yet incompletely understood mechanism underlying restricted ventilation may be inspiratory muscle weakness. Therefore, this study comprehensively evaluated inspiratory muscle function in ACHD and associations with systemic inflammation and the clinical severity of exercise intolerance.

Methods: 30 ACHD patients (21 men, 35 ± 12 years) and 30 healthy controls matched for age, gender and body mass index underwent spirometry, measurement of mouth occlusion pressures, and diaphragm ultrasound. Six-minute walking distance (6MWD) and New York Heart Association functional class were used to quantify exercise intolerance. Interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) levels were measured using enzyme-linked immunosorbent assays.

Results: ACHD patients showed lower forced vital capacity (FVC), and maximum inspiratory (PImax) and expiratory (PEmax) pressures compared with controls (all p < 0.05). On ultrasound, ACHD patients showed a lower diaphragm thickening ratio (2.3 ± 0.5 vs. 2.8 ± 0.9, p < 0.01) and lower diaphragm excursion velocity during a voluntary sniff maneuver (5.7 ± 2.2 vs. 7.6 ± 2.0 cm/s, p < 0.01). Respiratory parameters, such as FVC (r = 0.53; p < 0.01) and PImax (r = 0.43; p = 0.02), correlated with 6MWD. Furthermore, amino terminal pro B-type natriuretic peptide levels were inversely correlated with FVC (r = -0.54; p < 0.01). Circulating pro-inflammatory cytokines were markedly increased, and IL-6 was correlated with 6MWD, dyspnea, and biomarkers of heart, lung and inspiratory muscle function (all p < 0.05).

Conclusions: Our findings show that diaphragm dysfunction is present in ACHD and relates to restrictive ventilation disorder and exercise intolerance, possibly mediated by increased IL-6 levels.
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http://dx.doi.org/10.1016/j.ijcard.2020.06.055DOI Listing
November 2020

A case of 'tiger heart': a distinct variant of left ventricular non-compaction.

Eur Heart J Cardiovasc Imaging 2020 12;21(12):1434

Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.

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http://dx.doi.org/10.1093/ehjci/jeaa138DOI Listing
December 2020

Adult congenital heart disease and the COVID-19 pandemic.

Heart 2020 09 10;106(17):1302-1309. Epub 2020 Jun 10.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany.

Adults with congenital heart disease (ACHD) may be at high risk in the case of COVID-19. Due to the heterogeneity of ACHD and secondary complications, risk profiles are, however, not uniform. This document aims to give an overview of relevant data and outline our pragmatic approach to disease prevention and management. Based on anatomy and additional physiological factors including symptoms, exercise capacity, heart failure, pulmonary hypertension and cyanosis, we propose a pragmatic approach to categorising patients into low-risk, intermediate-risk and high-risk groups. We regard especially patients with complex cyanotic conditions, those with palliated univentricular hearts, heart failure, severe valvular disease or pulmonary hypertension as high-risk patients. To avoid infection, we recommend self-isolation and exemption from work for these cohorts. Infected ACHD patients with low or moderate risk and without signs of deterioration may be remotely followed and cared for at home while in self isolation. High-risk patients or those with signs of respiratory or cardiovascular impairment require admission ideally at a tertiary ACHD centre. Especially patients with complex, cyanotic disease, heart failure and arrhythmias require particular attention. Treatment in patients with cyanotic heart disease should be guided by the relative degree of desaturation compared with baseline and lactate levels rather than absolute oxygen saturation levels. Patients with right heart dilatation or dysfunction are potentially at increased risk of right heart failure as mechanical ventilation and acute respiratory distress syndrome can lead to increase in pulmonary arterial pressures.
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http://dx.doi.org/10.1136/heartjnl-2020-317258DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7299644PMC
September 2020

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry.

J Clin Med 2020 05 13;9(5). Epub 2020 May 13.

Missionsärztliche Klinik gGmbH, Abteilung für Innere Medizin, 97074 Würzburg, Germany.

Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension.

Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data.

Methods And Results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients´ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy ( = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status.

Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.
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http://dx.doi.org/10.3390/jcm9051456DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290703PMC
May 2020
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