Publications by authors named "Helmut Baumgartner"

225 Publications

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

Atrial septal defect in adulthood: a new paradigm for congenital heart disease.

Eur Heart J 2021 Sep 18. Epub 2021 Sep 18.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton & Harefield Hospitals, National Heart and Lung Institute, Imperial College, Sydney Street, London SW3 6NP, UK.

Atrial septal defects (ASDs) represent the most common congenital heart defect diagnosed in adulthood. Although considered a simple defect, challenges in optimal diagnostic and treatment options still exist due to great heterogeneity in terms of anatomy and time-related complications primarily arrhythmias, thromboembolism, right heart failure and, in a subset of patients, pulmonary arterial hypertension (PAH). Atrial septal defects call for tertiary expertise where all options may be considered, namely catheter vs. surgical closure, consideration of pre-closure ablation for patients with atrial tachycardia and suitability for closure or/and targeted therapy for patients with PAH. This review serves to update the clinician on the latest evidence, the nuances of optimal diagnostics, treatment options, and long-term follow-up care for patients with an ASD.
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http://dx.doi.org/10.1093/eurheartj/ehab646DOI Listing
September 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

Secundum Type Atrial Septal Defect in Patients with Trisomy 21-Therapeutic Strategies, Outcome, and Survival: A Nationwide Study of the German National Registry for Congenital Heart Defects.

J Clin Med 2021 Aug 25;10(17). Epub 2021 Aug 25.

Klinik für Angeborene Herzfehler und Kinderkardiologie, Universitätsklinikum Schleswig-Holstein, 24105 Kiel, Germany.

(1) Secundum type atrial septal defect (ASD II) is usually considered a relatively benign cardiac lesion amenable to elective closure at preschool age. Patients with trisomy 21 (T21), however, are known to have a higher susceptibility for pulmonary vascular disease (PVD). Therefore, T21 children may present with clinical symptoms earlier than those without associated anomalies. In addition, early PVD may even preclude closure in selected T21 patients. (2) We performed a retrospective analysis of the German National Register for Congenital Heart Defects including T21 patients with associated isolated ASD II. We report incidence, demographics, therapeutic strategy, outcome, and survival of this cohort. (3) Of 46,628 patients included in the registry, 1549 (3.3%) had T21. Of these, 156 (49.4% female) had an isolated ASD II. Fifty-four patients (34.6%) underwent closure at 6.4 ± 9.9 years of age. Over a cumulative follow-up (FU) of 1148 patient-years, (median 7.4 years), only one patient developed Eisenmenger syndrome and five patients died. Survival of T21 patients without PVD was not statistically different to age- and gender-matched controls from the normal population ( = 0.62), whereas children with uncorrected T21/ASD II (including patients with severe PVD, in whom ASD-closure was considered contraindicated) showed a significantly higher mortality. (4) The outcome of T21-patients with ASD II and without PVD is excellent. However, PVD, either precluding ASD-closure or development of progressive PVD after ASD-closure, is associated with significant mortality in this cohort. Thus T21 patients with ASD II who fulfill general criteria for closure and without PVD should be offered defect closure analogous to patients without T21.
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http://dx.doi.org/10.3390/jcm10173807DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432184PMC
August 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

Against all odds-late repair of multiple shunt lesions in a patient with Down syndrome: a case report.

Eur Heart J Case Rep 2021 Jul 18;5(7):ytab234. Epub 2021 Jul 18.

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

Background: Children with congenital heart defects (CHD) usually undergo elective surgical repair of haemodynamically relevant shunt lesions within the first year of life. Due to susceptibility for pulmonary arterial hypertension (PAH) in patients with Down syndrome, repair is usually aimed for no later than 6 months of life. However, with rising immigration from developing countries to Europe, more patients with unrepaired CHD are diagnosed at a later age. Anatomical repair may be precluded, when advanced pulmonary vascular disease has been established.

Case Summary: We report a 39-month-old male patient with Down syndrome with a large non-restrictive perimembranous ventricular septal defect, a large patent ductus arteriosus, and a secundum-type atrial septal defect with a prominent left-to-right shunting. Haemodynamic assessment revealed only a mild increase of pulmonary artery pressures (mPAP) with low pulmonary vascular resistance index (PVRi). Vasodilator testing led to a further increase of the left-to-right shunt and decrease of PVRi, suggesting operability. After careful consideration, the patient underwent complete surgical repair with a good post-operative clinical outcome. Cardiac catheterization 6 months after corrective repair showed a normal mPAP. No signs of PAH have been detected in the medium-term follow-up.

Discussion: Expertise, increased physician awareness, and a thorough pre-operative multidisciplinary evaluation are paramount to determine the best treatment approach for patients, who may present late with multiple shunts, and-in our case-underlying Down syndrome. Long-term close post-surgical follow-up in an expert centre is warranted to promptly diagnose and treat a possible late presentation of PAH appropriately.
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http://dx.doi.org/10.1093/ehjcr/ytab234DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8343471PMC
July 2021

High-Density Mapping Revealing Figure-of-Eight Re-Entrant Atrial Tachycardia in Uhl's Anomaly.

JACC Clin Electrophysiol 2021 Aug 28;7(8):1074-1075. Epub 2021 Jul 28.

Department of Cardiology II-Electrophysiology, University Hospital Münster, Münster, Germany.

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http://dx.doi.org/10.1016/j.jacep.2021.03.016DOI Listing
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

Reaching consensus for unified medical language in Fontan care.

ESC Heart Fail 2021 Oct 30;8(5):3894-3905. Epub 2021 Jun 30.

Adult Congenital Heart Disease Service, Heart Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Aims: The Fontan operation has resulted in improved survival in patients with single-ventricle congenital heart disease. As a result, there is a growing population of teenagers and adults with a Fontan circulation. Many co-morbidities have been increasingly recognized in this population due to the unique features of the Fontan circulation. Standardization of how Fontan co-morbid conditions are defined will help facilitate understanding, consistency and interpretability of research and clinical experience. Unifying common language usage in Fontan is a critical precursor step for data comparison of research findings and clinical outcomes and ultimately accelerating improvements in management for this growing group of patients. This manuscript aimed to create unified definitions for morbidities seen after the Fontan palliation.

Methods: In association of many congenital heart disease organizations, this work used Delphi methodology to reach a broad consensus among recognized experts regarding commonly used terms in Fontan care and research. Each definition underwent at least three rounds of revisions to reach a final definition through surveys sent to experts in the field of single-ventricle care.

Results: The process of reaching a consensus on multiple morbidities associated with the Fontan procedure is summarized in this manuscript. The different versions that preceded reaching the consensus are also presented in the Supporting Information. Table 1 represents the final definitions according to the consensus.

Conclusions: We propose the use of these definitions for clinical care, future research studies, registry development and clinical trials.
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http://dx.doi.org/10.1002/ehf2.13294DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8497335PMC
October 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

Therapy of supraventricular and ventricular arrhythmias in adults with congenital heart disease-narrative review.

Cardiovasc Diagn Ther 2021 Apr;11(2):550-562

Department of Cardiology II - Electrophysiology, University Hospital Muenster, Cardiology, Muenster, Germany.

Arrhythmias are among the most common late complications in adults with congenital heart disease (ACHD) and a frequent reason for hospital admission. Both, supraventricular and ventricular arrhythmias, not only cause debilitating symptoms, but may be life-threatening by increasing risk of stroke, causing or worsening heart failure and being associated with sudden death. Substrate and risk for arrhythmia differs widely between congenital defects with specific arrhythmias being much more common in some patients than others. Atrial macroreentrant arrhythmias are particularly frequent in patients with atrial septal defects and repair that involves atrial incisions including patients with transposition of the great arteries (TGA) and atrial switch. Accessory pathways and related arrhythmias are often associated with Ebstein's anomaly and congenitally corrected TGA. Monomorphic ventricular arrhythmias occur in patients with ventricular incisions, namely patients with Tetralogy of Fallot. Changes in surgical repair techniques influence arrhythmia prevalence and substrate as well as anatomical access for catheter ablation procedures. In addition, epidemiologic changes associated with improved long-term survival will further increase the prevalence of atrial fibrillation in ACHD. This article summarizes current understanding of prevalence of specific arrhythmias, underlying mechanisms, medical and interventional treatment options and their outcome in ACHD.
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http://dx.doi.org/10.21037/cdt-20-634DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8102262PMC
April 2021

Narrative review of: risk stratification and implantable cardioverter-defibrillator therapy in adults with congenital heart disease.

Cardiovasc Diagn Ther 2021 Apr;11(2):538-549

Department of Cardiology II - Electrophysiology, University Hospital Muenster, Muenster, Germany.

Fortunately, the population of adults with congenital heart disease (ACHD) is growing due to improved operation techniques. Life expectancy is continuously rising, nevertheless, sudden cardiac death is one of the leading causes of mortality in ACHD late after initial diagnosis. Risk stratification in ACHD remains challenging as large study results are missing, congenital defects and operation methods differ considerably between individual patients and results from acquired heart diseases are often not conferrable. The purpose of this narrative review is to objectively summarize the current knowledge on arrhythmogenic risk of ACHD and to give an overview on implantable cardioverter-defibrillator (ICD) therapy in this collective. Remarkable progress has been made in electrophysiological understanding of critical areas of slow conduction especially in patients with Tetralogy of Fallot (ToF). In patients with transposition of the great arteries after atrial baffling (Mustard/Senning procedure) atrial arrhythmias play a crucial role in sudden cardiac death. ICD therapy in ACHD may pose special technical challenges due to limited access for intracardiac leads. The introduction of the totally subcutaneous ICD improved therapeutic options for ACHD especially when contraindications for transvenous leads are present. Risk stratification in ACHD has to be seen as a team approach, requires thorough understanding of congenital heart defects and the operation techniques and needs unconventional technical approaches in some cases.
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http://dx.doi.org/10.21037/cdt-20-633DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8102248PMC
April 2021

Clinical outcome of COVID-19 in patients with adult congenital heart disease.

Heart 2021 Mar 8. Epub 2021 Mar 8.

Departament of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.

Aims: Patients with adult congenital heart disease (ACHD) are a potentially vulnerable patient cohort in case of COVID-19. Some cardiac defects may be associated with a poor COVID-19 outcome. Risk estimation in ACHD is currently based on expert opinion. The aim of this study was to collect clinical outcome data and to identify risk factors for a complicated course of COVID-19 in patients with ACHD.

Methods: Twenty-five ACHD centres in nine European countries participated in the study. Consecutive patients with ACHD diagnosed with COVID-19 presenting to one of the participating centres between 27 March and 6 June 2020 were included. A complicated disease course was defined as hospitalisation for COVID-19 requiring non-invasive or invasive ventilation and/or inotropic support, or a fatal outcome.

Results: Of 105 patients with a mean age of 38±13 years (58% women), 13 had a complicated disease course, of whom 5 died. In univariable analysis, age (OR 1.3, 95% CI 1.1 to 1.7, per 5 years), ≥2 comorbidities (OR 7.1, 95% CI 2.1 to 24.5), body mass index of >25 kg/m (OR 7.2, 95% CI 1.9 to 28.3) and cyanotic heart disease (OR 13.2, 95% CI 2.5 to 68.4) were associated with a complicated disease course. In a multivariable logistic regression model, cyanotic heart disease was the most important predictor (OR 60.0, 95% CI 7.6 to 474.0).

Conclusions: Among patients with ACHD, general risk factors (age, obesity and multiple comorbidities) are associated with an increased risk of complicated COVID-19 course. Congenital cardiac defects at particularly high risk were cyanotic lesions, including unrepaired cyanotic defects or Eisenmenger syndrome.
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http://dx.doi.org/10.1136/heartjnl-2020-318467DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7944416PMC
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

The year in cardiovascular medicine 2020: valvular heart disease.

Eur Heart J 2021 02;42(6):647-656

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

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http://dx.doi.org/10.1093/eurheartj/ehaa1060DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7878012PMC
February 2021

Imaging the adult with simple shunt lesions: position paper from the EACVI and the ESC WG on ACHD. Endorsed by AEPC (Association for European Paediatric and Congenital Cardiology).

Eur Heart J Cardiovasc Imaging 2021 05;22(6):e58-e70

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

In 2018, the position paper 'Imaging the adult with congenital heart disease: a multimodality imaging approach' was published. The paper highlights, in the first part, the different imaging modalities applied in adult congenital heart disease patients. In the second part, these modalities are discussed more detailed for moderate to complex anatomical defects. Because of the length of the paper, simple lesions were not touched on. However, imaging modalities to use for simple shunt lesions are still poorly known. One is looking for structured recommendations on which they can rely when dealing with an (undiscovered) shunt lesion. This information is lacking for the initial diagnostic process, during repair and at follow-up. Therefore, this paper will focus on atrial septal defect, ventricular septal defect, and persistent arterial duct. Pre-, intra-, and post-procedural imaging techniques will be systematically discussed. This position paper will offer algorithms that might help at a glance. The document is prepared for general cardiologists, trainees, medical students, imagers/technicians to select the most appropriate imaging modality and to detect the requested information for each specific lesion. It might serve as reference to which researchers could refer when setting up a (imaging) study.
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http://dx.doi.org/10.1093/ehjci/jeaa314DOI Listing
May 2021

Comparison of Early Surgical or Transcatheter Aortic Valve Replacement Versus Conservative Management in Low-Flow, Low-Gradient Aortic Stenosis Using Inverse Probability of Treatment Weighting: Results From the TOPAS Prospective Observational Cohort Study.

J Am Heart Assoc 2020 12 8;9(24):e017870. Epub 2020 Dec 8.

Institut Universitaire de Cardiologie et de Pneumologie de Québec-Université Laval Québec Canada.

Background No randomized comparison of early (ie, ≤3 months) aortic valve replacement (AVR) versus conservative management or of transcatheter AVR (TAVR) versus surgical AVR has been conducted in patients with low-flow, low-gradient (LFLG) aortic stenosis (AS). Methods and Results A total of 481 consecutive patients (75±10 years; 71% men) with LFLG AS (aortic valve area ≤0.6 cm/m and mean gradient <40 mm Hg), 72% with classic LFLG and 28% with paradoxical LFLG, were prospectively recruited in the multicenter TOPAS (True or Pseudo Severe Aortic Stenosis) study. True-severe AS or pseudo-severe AS was adjudicated by flow-independent criteria. During follow-up (median [IQR] 36 [11-60] months), 220 patients died. Using inverse probability of treatment weighting to address the bias of nonrandom treatment assignment, early AVR (n=272) was associated with a major overall survival benefit (hazard ratio [HR], 0.34 [95% CI, 0.24-0.50]; <0.001). This benefit was observed in patients with true-severe AS but also with pseudo-severe AS (HR, 0.38 [95% CI, 0.18-0.81]; =0.01), and in classic (HR, 0.33 [95% CI, 0.22-0.49]; <0.001) and paradoxical LFLG AS (HR, 0.42 [95% CI, 0.20-0.92]; =0.03). Compared with conservative management in the conventional multivariate model, trans femoral TAVR was associated with the best survival (HR, 0.23 [95% CI, 0.12-0.43]; <0.001), followed by surgical AVR (HR, 0.36 [95% CI, 0.23-0.56]; <0.001) and alternative-access TAVR (HR, 0.51 [95% CI, 0.31-0.82]; =0.007). In the inverse probability of treatment weighting model, trans femoral TAVR appeared to be superior to surgical AVR (HR [95% CI] 0.28 [0.11-0.72]; =0.008) with regard to survival. Conclusions In this large prospective observational study of LFLG AS, early AVR appeared to confer a major survival benefit in both classic and paradoxical LFLG AS. This benefit seems to extend to the subgroup with pseudo-severe AS. Our findings suggest that TAVR using femoral access might be the best strategy in these patients. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT01835028.
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http://dx.doi.org/10.1161/JAHA.120.017870DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7955363PMC
December 2020

Bleeding and thrombotic risk in pregnant women with Fontan physiology.

Heart 2021 Sep 24;107(17):1390-1397. Epub 2020 Nov 24.

Adult Congenital Heart Disease, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

Background/objectives: Pregnancy may potentiate the inherent hypercoagulability of the Fontan circulation, thereby amplifying adverse events. This study sought to evaluate thrombosis and bleeding risk in pregnant women with a Fontan.

Methods: We performed a retrospective observational cohort study across 13 international centres and recorded data on thrombotic and bleeding events, antithrombotic therapies and pre-pregnancy thrombotic risk factors.

Results: We analysed 84 women with Fontan physiology undergoing 108 pregnancies, average gestation 33±5 weeks. The most common antithrombotic therapy in pregnancy was aspirin (ASA, 47 pregnancies (43.5%)). Heparin (unfractionated (UFH) or low molecular weight (LMWH)) was prescribed in 32 pregnancies (30%) and vitamin K antagonist (VKA) in 10 pregnancies (9%). Three pregnancies were complicated by thrombotic events (2.8%). Thirty-eight pregnancies (35%) were complicated by bleeding, of which 5 (13%) were severe. Most bleeds were obstetric, occurring antepartum (45%) and postpartum (42%). The use of therapeutic heparin (OR 15.6, 95% CI 1.88 to 129, p=0.006), VKA (OR 11.7, 95% CI 1.06 to 130, p=0.032) or any combination of anticoagulation medication (OR 13.0, 95% CI 1.13 to 150, p=0.032) were significantly associated with bleeding events, while ASA (OR 5.41, 95% CI 0.73 to 40.4, p=0.067) and prophylactic heparin were not (OR 4.68, 95% CI 0.488 to 44.9, p=0.096).

Conclusions: Current antithrombotic strategies appear effective at attenuating thrombotic risk in pregnant women with a Fontan. However, this comes with high (>30%) bleeding risk, of which 13% are life threatening. Achieving haemostatic balance is challenging in pregnant women with a Fontan, necessitating individualised risk-adjusted counselling and therapeutic approaches that are monitored during the course of pregnancy.
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http://dx.doi.org/10.1136/heartjnl-2020-317397DOI Listing
September 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

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

The 'Ten Commandments' in Adult Congenital Heart Disease Guidelines.

Eur Heart J 2020 11;41(43):4155

Chair of ESC Taskforce.

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http://dx.doi.org/10.1093/eurheartj/ehaa702DOI Listing
November 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

Timing of intervention in asymptomatic patients with valvular heart disease.

Eur Heart J 2020 12;41(45):4349-4356

Cardiology, University of Washington Medical Center, 1959 NE Pacific St, Seattle, WA 98195, USA.

Current management of valvular heart disease (VHD) seeks to optimize long-term outcome by timely intervention. Recommendations for treatment of patients with symptoms due to severe valvular disease are based on a foundation of solid evidence. However, when to intervene in asymptomatic patients remains controversial and decision requires careful individual weighing of the potential benefits against the risk of intervention and its long-term consequences. The primary rationale for earlier intervention is prevention of irreversible left ventricular (LV) myocardial changes that might result in later clinical symptoms and adverse cardiac events. A number of outcome predictors have been identified that facilitate decision-making. This review summarizes current recommendations and discusses recently published data that challenge them suggesting even earlier intervention. In adults with asymptomatic aortic stenosis (AS), emerging risk markers include very severe valve obstruction, elevated serum natriuretic peptide levels, and imaging evidence of myocardial fibrosis or increased extracellular myocardial volume. Currently, transcatheter aortic valve implantation (TAVI) is not recommended for treatment of asymptomatic severe AS although this may change in the future. In patients with aortic regurgitation (AR), the potential benefit of early intervention in preventing LV dilation and dysfunction must be balanced against the long-term risk of a prosthetic valve, a particular concern because severe AR often occurs in younger patients with a congenital bicuspid valve. In patients with mitral stenosis, the option of transcatheter mitral balloon valvotomy tilts the balance towards earlier intervention to prevent atrial fibrillation, embolic events, and pulmonary hypertension. When chronic severe mitral regurgitation is due to mitral valve prolapse, anatomic features consistent with a high likelihood of a successful and durable valve repair favour early intervention. The optimal timing of intervention in adults with VHD is a constantly changing threshold that depends not only on the severity of valve disease but also on the safety, efficacy, and long-term durability of our treatment options.
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http://dx.doi.org/10.1093/eurheartj/ehaa485DOI Listing
December 2020

Four-year mortality in women and men after transfemoral transcatheter aortic valve implantation using the SAPIEN 3.

Catheter Cardiovasc Interv 2021 04 4;97(5):876-884. Epub 2020 Sep 4.

Department of Cardiothoracic Surgery, King's College Hospital, London, UK.

Objectives: To investigate 4-year, post-transcatheter aortic valve implantation (TAVI) survival and predictors of survival by sex, in a real-world cohort that underwent transfemoral TAVI with SAPIEN 3 transcatheter heart valve.

Background: Previous TAVI investigations of first-generation devices demonstrated an early- to mid-term survival advantage in women compared with men.

Methods: SOURCE 3 (SAPIEN 3 Aortic Bioprosthesis European Outcome) is a post-approval, multicentre, observational registry. Patients (N = 1,694, 49.2% women, age 81.7 ± 6.7 years) with severe aortic stenosis and high surgical risk (logistic EuroSCORE 17.8%) underwent TAVI between 2014 and 2015. Kaplan-Meier event estimates were used to determine mortality by sex. Predictors of overall mortality were identified using a cox multivariate proportional hazard model.

Results: At 4 years, women had lower all-cause mortality than men (36.0 vs 39.7%; p = .0911; HR: 0.87 [95% CI: 0.75-1.02]). No difference was observed for cardiac mortality between women 24.2% and men 24.7% (p = .76; HR: 0.97 [95% CI: 0.79-1.19]). When adjusted for baseline characteristics (age, height, weight, NYHA functional class, renal insufficiency, EuroScore, and tricuspid regurgitation), sex had no impact on mortality.

Conclusions: In this large, real-world cohort, all-cause mortality trended lower in women than men at 4 years post TAVI; however, several baseline factors, but not sex, were predictors of mortality. No difference between sexes was observed for cardiovascular mortality.
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http://dx.doi.org/10.1002/ccd.29257DOI Listing
April 2021
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