Prognostic Value and Determinants of High-Sensitivity Cardiac Troponin T in Patients With a Systemic Right Ventricle: Insights From the SERVE Trial.

BACKGROUND: The determinants and prognostic value of high-sensitivity cardiac troponin T (hs-cTnT) among patients with a systemic right ventricle are largely unknown. METHODS AND RESULTS: Ninety-eight patients from the randomized controlled SERVE (Effect of Phosphodiesterase-5 Inhibition With Tadalafil on Systemic Right Ventricular Size and Function) trial were included. The correlation between baseline hs-cTnT concentrations and biventricular volumes and function quantified by cardiac magnetic resonance or cardiac multirow detector computed tomography was assessed by adjusted linear regression models. The prognostic value of hs-cTnT was assessed by adjusted Cox proportional hazards models, survival analysis, and concordance statistics. The primary outcome was time to the composite of clinically relevant arrhythmia, hospitalization for heart failure, or all-cause death. Median age was 39 (interquartile range, 32-48) years, and 32% were women. Median hs-cTnT concentration was 7 (interquartile range, 4-11) ng/L. Coefficients of determination for the relationship between hs-cTnT concentrations and right ventricular end-systolic volume index and right ventricular ejection fraction (RVEF) were +0.368 (P=0.046) and -0.381 (P=0.018), respectively. The sex- and age-adjusted hazard ratio for the primary outcome of hs-cTnT at 2 and 4 times the reference level (5 ng/L) were 2.89 (95% CI, 1.14-7.29) and 4.42 (95% CI, 1.21-16.15), respectively. The prognostic performance quantified by the concordance statistics for age- and sex-adjusted models based on hs-cTnT, right ventricular ejection fraction, and peak oxygen uptake predicted were comparable: 0.71% (95% CI, 0.61-0.82), 0.72% (95% CI, 0.59-0.84), and 0.71% (95% CI, 0.59-0.83), respectively. CONCLUSIONS: Hs-cTnT concentration was significantly correlated with right ventricular ejection fraction and right ventricular end-systolic volume index in patients with a systemic right ventricle. The prognostic accuracy of hs-cTnT was comparable to that of right ventricular ejection fraction and peak oxygen uptake predicted. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03049540.

Decreased clinical performance in TGA-ASO patients after RVOT interventions; a multicenter European collaboration.

BACKGROUND: In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions. OBJECTIVES: We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention. METHODS: TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention. The following outcome parameters were included: predicted (%) peak oxygen uptake (peak VO2), tricuspid annular plane systolic excursion (TAPSE), tricuspid Lateral Annular Systolic Velocity (TV S'), right ventricle (RV)-arterial coupling (defined as TAPSE/RV systolic pressure ratio), and N-terminal proBNP (NT-proBNP). RESULTS: 447 TGA patients with a mean age of 25.0 (interquartile range (IQR) 21-29) years were included. Patients without previous RVOT intervention (n = 338, 76%) had a significantly higher predicted peak VO2 (78.0 ± 17.4%) compared to patients with single approach catheter-based RVOT intervention (73.7 ± 12.7%), single approach surgical RVOT intervention (73.8 ± 28.1%), and patients with multiple approach RVOT intervention (66.2 ± 14.0%, p = 0.021). RV-arterial coupling was found to be significantly lower in patients with prior catheter-based and/or surgical RVOT intervention compared to patients without any RVOT intervention (p = 0.029). CONCLUSIONS: TGA patients after a successful arterial switch repair have a decreased exercise capacity. A considerable amount of TGA patients with either catheter or surgical RVOT intervention perform significantly worse compared to patients without RVOT interventions.

Epidemiology and Definition of Heart Failure in Adult Congenital Heart Disease.

Adults with congenital heart disease (ACHD) are facing lifelong complications, notably heart failure (HF). This review focuses on classifications, incidence, prevalence, and mortality of HF related to ACHD. Diagnosing HF in ACHD is intricate due to anatomic variations, necessitating comprehensive clinical evaluations. Hospitalizations and resource consumption for ACHD HF have significantly risen compared with non-ACHD HF patients. With more than 30% prevalence in complex cases, HF has become the leading cause of death in ACHD. These alarming trends underscore the insufficient understanding of ACHD-related HF manifestations and management challenges within the context of aging, complexity, and comorbidity.

[Cardiology: what's new in 2023]. / Cardiologie: ce qui a changé en 2023.

The year 2023 has been extremely rich in new publications in the various subfields of cardiology. Furthermore, the European Society of Cardiology (ESC) has issued revised guidelines focused on the management of acute coronary syndrome (ACS) and endocarditis, as well as an update on the recommendations for the management of heart failure and cardiovascular prevention. The most significant updates according to the Cardiology Department of CHUV are summarized in this review article.

L'année 2023 a été extrêmement riche en nouvelles publications dans les différents sous-domaines de la cardiologie. De plus, la Société européenne de cardiologie (ESC) a formulé des directives révisées axées sur le management du syndrome coronarien aigu (SCA) et de l'endocardite ainsi qu'une mise à jour des recommandations sur la prise en charge de l'insuffisance cardiaque et la prévention cardiovasculaire. Les nouveautés les plus importantes selon l'équipe du Service de cardiologie du CHUV sont résumées dans cet article de synthèse.

Prevalence of Pericardial Late Gadolinium Enhancement in Patients After Cardiac Surgery: Clinical and Histological Correlations.

BACKGROUND: Pericardial late gadolinium enhancement (LGE) is usually associated with active pericarditis, but it is not infrequently found in patients after cardiac surgery even a long time after the intervention. The clinical relevance of this finding and its histological correlates are unknown. We sought to determine the prevalence of chronic pericardial LGE in patients after cardiac surgery. METHODS: All consecutive patients with previous cardiac surgery, who were referred to cardiovascular magnetic resonance between January 2017 and December 2021 were enrolled in the study. Cardiovascular magnetic resonance examination protocol was adapted to clinical indication but always included standard LGE acquisitions. Two independent observers blinded to clinical data assessed the presence of pericardial enhancement on LGE sequences. Fifteen patients underwent cardiac reintervention and pericardial biopsies were obtained. The primary study end point was to assess the prevalence of pericardial enhancement after cardiac surgery and identify possible determinants. The secondary end point was to correlate pericardial enhancement with clinical symptoms and histopathology. RESULTS: Two hundred four patients were included in the study. The median time between surgery and cardiovascular magnetic resonance was 160 months (35-226 months). Pericardial LGE was observed in 90 patients (44%). All patients were asymptomatic, and no specific treatment for pericarditis was started. All patients remained asymptomatic at a 1-year clinical follow-up. Pericardial LGE was significantly correlated with the number of previous surgeries (P=0.03). Pericardial fibrosis was detected in all 15 pericardial biopsy specimens; pericardial LGE was present in 7 patients (47%) who underwent biopsy. Histological signs of low-grade inflammation were detected in 6 patients (40%) with severe, circumferential pericardial LGE but in no patient without pericardial enhancement. CONCLUSIONS: Pericardial LGE is a frequent finding even several years after cardiac surgery. Its histological correlate is a chronic subclinical post-pericardiotomy inflammation.

Effect of phosphodiesterase-5 inhibition on SystEmic Right VEntricular size and function. A multicentre, double-blind, randomized, placebo-controlled trial: SERVE.

AIMS: In adults with congenital heart disease and systemic right ventricles, progressive right ventricular systolic dysfunction is common and is associated with adverse outcomes. Our aim was to assess the impact of the phosphodiesterase-5-inhibitor tadalafil on right ventricular systolic function. METHODS AND RESULTS: This was a double-blind, randomized, placebo-controlled, multicentre superiority trial (NCT03049540) involving 100 adults with systemic right ventricles (33 women, mean age: 40.7 ± 10.7 years), comparing tadalafil 20 mg once daily versus placebo (1:1 ratio). The primary endpoint was the change in right ventricular end-systolic volume after 3 years of therapy. Secondary endpoints were changes in right ventricular ejection fraction, exercise capacity and N-terminal pro-B-type natriuretic peptide concentration. Primary endpoint assessment by intention to treat analysis at 3 years of follow-up was possible in 83 patients (42 patients in the tadalafil group and 41 patients in the placebo group). No significant changes over time in right ventricular end-systolic volumes were observed in the tadalafil and the placebo group, and no significant differences between treatment groups (3.4 ml, 95% confidence interval -4.3 to 11.0, p = 0.39). No significant changes over time were observed for the pre-specified secondary endpoints for the entire study population, without differences between the tadalafil and the placebo group. CONCLUSIONS: In this trial in adults with systemic right ventricles, right ventricular systolic function, exercise capacity and neuro-hormonal activation remained stable over a 3-year follow-up period. No significant treatment effect of tadalafil was observed. Further research is needed to find effective treatment for improvement of ventricular function in adults with systemic right ventricles.

[Cardiology: what's new in 2022]. / Cardiologie : ce qui a changé en 2022.

The year of 2022 was marked by many novelties in the fields of interventional cardiology, heart failure, electrophysiology, cardiac imaging, and congenital heart disease. These advances will certainly change our daily practice, on top of improving the diagnosis and treatment of many heart conditions. In addition, the European Society of Cardiology has updated its guidelines on pulmonary hypertension, ventricular arrhythmias and sudden death, cardiovascular assessment of patients undergoing non-cardiac surgery. The members of the Cardiology division of Lausanne University Hospital (CHUV) here present the publications which they considered to be the most important of the past year.

L'année 2022 a été marquée par de nombreuses nouveautés dans les domaines de la cardiologie interventionnelle, de l'insuffisance cardiaque, de l'électrophysiologie, de l'imagerie cardiaque et des cardiopathies congénitales. Ces progrès vont certainement faire évoluer notre pratique quotidienne, en plus d'améliorer le diagnostic et le traitement de nombreuses cardiopathies. Par ailleurs, la Société européenne de cardiologie a mis à jour ses recommandations portant sur l'hypertension pulmonaire, les arythmies ventriculaires et la mort subite ainsi que le bilan cardiologique avant une chirurgie non cardiaque. Les membres du Service de cardiologie du CHUV vous présentent ici les travaux qui leur ont semblé être les plus importants de l'année écoulée.

The coronavirus disease pandemic among adult congenital heart disease patients and the lessons learnt - results of a prospective multicenter european registry.

Background: At the beginning of the COVID-19 pandemic, professionals in charge of particularly vulnerable populations, such as adult congenital heart disease (ACHD) patients, were confronted with difficult decision-making. We aimed to assess changes in risk stratification and outcomes of ACHD patients suffering from COVID-19 between March 2020 and April 2021. Methods and results: Risk stratification among ACHD experts (before and after the first outcome data were available) was assessed by means of questionnaires. In addition, COVID-19 cases and the corresponding patient characteristics were recorded among participating centres. Predictors for the outcome of interest (complicated disease course) were assessed by means of multivariable logistic regression models calculated with cluster-robust standard errors. When assessing the importance of general and ACHD specific risk factors for a complicated disease course, their overall importance and the corresponding risk perception among ACHD experts decreased over time. Overall, 638 patients (n = 168 during the first wave and n = 470 during the subsequent waves) were included (median age 34 years, 52% women). Main independent predictors for a complicated disease course were male sex, increasing age, a BMI >25 kg/m2, having ≥2 comorbidities, suffering from a cyanotic heart disease or having suffered COVID-19 in the first wave vs. subsequent waves. Conclusions: Apart from cyanotic heart disease, general risk factors for poor outcome in case of COVID-19 reported in the general population are equally important among ACHD patients. Risk perception among ACHD experts decreased during the course of the pandemic.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease.

BACKGROUND: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II. METHODS/DESIGN: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments. DISCUSSION: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD.

A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle.

AIMS: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV). METHODS AND RESULTS: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9-34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72-0.83] and a calibration slope of 0.93 (95% CI 0.64-1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE. CONCLUSION: Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation.

[Prevention of thromboembolic complications in adult congenital heart disease]. / Prévention des complications thromboemboliques des patients adultes porteurs de cardiopathie congénitale.

Thromboembolic complications in adults with congenital heart disease are frequent and responsible for significant morbidity and mortality. Their prevention frequently requires long-term thromboprophylaxis. In this article, we discuss the specificities that must be considered when stratifying the thromboembolic risk in these patients, the different therapeutic strategies, particularly the place of direct oral anticoagulants.

Les complications thromboemboliques des adultes porteurs d'une cardiopathie congénitale sont fréquentes et responsables d'une importante morbi-mortalité. Leur prévention passe fréquemment par la mise en place d'une thromboprophylaxie au long cours. Dans cet article, nous discutons des spécificités qui doivent être prises en compte lors de la stratification du risque thromboembolique de ces patients, des différentes stratégies thérapeutiques et notamment de la place des anticoagulants oraux directs.

Young adults after arterial switch operation for transposition of the great arteries in Switzerland: a growing population.

BACKGROUND: Adults with transposition of the great arteries (d-TGA) after the arterial switch operation (ASO) are an evolving cohort in adult cardiology. We aimed to analyze cardiac function and cardiac events after transition to the adult clinic in Switzerland. MATERIALS AND METHODS: Adults with prior ASO enrolled in the Swiss Adult Congenital HEart disease Registry (SACHER) were included. We analyzed initial cardiac anatomy, surgical history, residual lesions and cardiac function at the time of inclusion, as well as cardiac events during follow-up. Patients were classified as complex (with ventricular septal defect) or simple (with intact interventricular septum) d-TGA. RESULTS: The cohort included 149 patients (99 simple d-TGA, 50 complex d-TGA; age 21±3 years; 71% male, follow-up 27 [15-46] months). Prior to inclusion, patients with complex d-TGA had undergone more interventions related to the left ventricular outflow tract (16% vs. 3%, p = 0.01). Functional and cardiovascular status were similar between the groups. Eleven patients (7%) had a total of 19 cardiac events (5 complications and 14 re-interventions) during follow-up. Patients with complex d-TGA had more cardiac-related complications compared to those with simple d-TGA (8% vs. 1%, p = 0.03). The frequency of re-interventions was not statistically different between the two groups (12% vs. 4%, p = 0.07). During follow-up, an increase in QRS duration was observed. Other parameters of cardiac function remained unchanged. CONCLUSION: The majority of adult ASO patients have normal functional class and cardiac function. Complex anatomy and residual lesions play a key role when regarding the occurrence of cardiac-related complications during follow-up. The role of QRS prolongation over time needs to be investigated further.

[Cardiology]. / Cardiologie.

Significant advances have been made in 2021 in the areas of interventional cardiology, heart failure, cardiac imaging, electrophysiology and congenital heart disease. In addition to improving the screening, diagnosis and management of many heart diseases, these advances will change our daily practice. Moreover, the European Society of Cardiology has updated its guidelines on heart failure, valve disease, cardiac pacing and cardiovascular disease prevention. As in previous years, members of the Cardiology division of Lausanne University Hospital (CHUV) came together to select and present to you the papers that they considered to be the most important of the past year.

De nombreux progrès ont été réalisés en 2021 dans les domaines de la cardiologie interventionnelle, de l'insuffisance cardiaque, de l'imagerie cardiaque, de l'électrophysiologie et des cardiopathies congénitales. En plus d'améliorer le dépistage, le diagnostic et la prise en charge de nombreuses cardiopathies, ces avancées vont faire évoluer notre pratique quotidienne. Par ailleurs, la Société européenne de cardiologie a mis à jour ses recommandations portant sur l'insuffisance cardiaque, les valvulopathies, la stimulation cardiaque et la prévention des maladies cardiovasculaires. Comme les années précédentes, les membres du Service de cardiologie du CHUV se sont réunis pour sélectionner et vous présenter les travaux qui leur ont semblé être les plus importants de l'année écoulée.

Arrhythmic Burden of Adult Survivors With Repaired Total Anomalous Pulmonary Venous Connection.

Background: The long-term outcome of adults with repaired total anomalous pulmonary venous connection (TAPVC) is poorly documented. Therefore, the present study aims to provide current clinical data on adult survivors with repaired TAPVC focusing on arrhythmia. Methods: Clinical and imaging data (prevalence and type of arrhythmias, symptoms, surgical and medical treatment, echocardiographic and cardiac magnetic resonance haemodynamic parameters) were retrospectively collected from 8 European centres and compared between patients with and without arrhythmias. Results: Fifty-seven patients were included (age 20 [16-67] years [female 28, 49%]). At the last follow-up, that is, 21 (8-51) years after surgery, 79% and 93% of patients were free of symptoms and cardiac medication, respectively. The prevalence of late arrhythmias was 21%; 9 (16%) patients showed intra-atrial re-entrant tachycardia (IART) and 2 (4%) ventricular arrhythmias. Patients with IART were older (P = 0.018) and 4 (7%) required antiarrhythmic medication. Three patients (5%) underwent an electrophysiological study, and another 3 (5%) underwent pacemaker implantation within 36 months after surgical correction, which were removed in 2 patients after 7 years. Early postoperative arrhythmias (P = 0.005), right ventricular dilatation (P = 0.003), and valvulopathy (P = 0.009) were more often present in patients with late IART. Conclusions: Adult survivors after isolated-TAPVC repair presented a high prevalence of arrhythmias. Age, right ventricular dilatation, early arrhythmias, and valvular lesions are risk factors for IART. Long-term follow-up is important as some of these currently asymptomatic patients will probably develop arrhythmias in the future.

Contexte: Les résultats de santé à long terme chez les adultes ayant subi la correction d'un retour veineux pulmonaire anormal total (RVPAT) sont mal connus. Notre étude vise donc à recueillir des données cli-niques au sujet des patients ayant subi cette intervention et ayant survécu jusqu'à l'âge adulte, en particulier pour ce qui est des arythmies. Méthodologie: Les données d'observation clinique et d'imagerie (la prévalence et le type d'arythmies, les symptômes, les traitements chirurgicaux et médicaux, et les paramètres hémodynamiques obtenus par échographie et par résonance magnétique cardiaque) ont été recueillies de façon rétrospective dans huit centres européens et comparées selon que les patients présentaient ou non une arythmie. Résultats: Cinquante-sept patients ont été retenus (âge médian : 20 [16-67] ans; 28 [49 %] femmes). Au dernier suivi, soit 21 (8-51) ans après l'intervention chirurgicale, 79 % des patients ne présentaient pas de symptômes et 93 % des patients ne prenaient pas de médicaments pour des troubles cardiaques. La prévalence d'arythmies tardives s'élevait à 21 %; neuf patients (16 %) présentaient une tachycardie par réentrée intra-atriale (TRIA) et deux patients (4 %) présentaient des arythmies ventriculaires. Les patients qui présentaient une TRIA étaient plus âgés (P = 0,018) et quatre d'entre eux (7 %) devaient prendre des médicaments antiarythmiques. Trois patients (5 %) avaient subi des études électrophysiologiques et trois autres patients (5 %) avaient subi l'implantation d'un stimulateur cardiaque au cours des 36 mois suivant la correction chirurgicale; le stimulateur cardiaque a été retiré sept ans plus tard dans deux de ces cas. Les arythmies postopératoires précoces (P = 0,005), la dilation du ventricule droit (P = 0,003) et la valvulopathie (P = 0,009) étaient plus fréquentes chez les patients qui présentaient une TRIA tardive. Conclusions: La prévalence d'arythmies chez les patients survivant jusqu'à l'âge adulte après la correction isolée d'un RVPAT était élevée. L'âge, la dilation du ventricule droit, les arythmies précoces et les lésions valvulaires sont des facteurs de risque de TRIA. Il est important d'effectuer un suivi à long terme des patients ayant subi une RVPAT puisque certains d'entre eux, asymptomatiques pour le moment, présenteront sans doute des arythmies dans les années à venir.

2D cine vs. 3D self-navigated free-breathing high-resolution whole heart cardiovascular magnetic resonance for aortic root measurements in congenital heart disease.

BACKGROUND: Cardiovascular magnetic resonance (CMR) is considered the method of choice for evaluation of aortic root dilatation in congenital heart disease. Usually, a cross-sectional 2D cine stack is acquired perpendicular to the vessel's axis. However, this method requires a considerable patient collaboration and precise planning of image planes. The present study compares a recently introduced 3D self-navigated free-breathing high-resolution whole heart CMR sequence (3D self nav) allowing a multiplanar retrospective reconstruction of the aortic root as an alternative to the 2D cine technique for determination of aortic root diameters. METHODS: A total of 6 cusp-commissure (CuCo) and cusp-cusp (CuCu) enddiastolic diameters were measured by two observers on 2D cine and 3D self nav cross-sectional planes of the aortic root acquired on a 1.5 T CMR scanner. Asymmetry of the aortic root was evaluated by the ratio of the minimal to the maximum 3D self nav CuCu diameter. CuCu diameters were compared to standard transthoracic echocardiographic (TTE) aortic root diameters. RESULTS: Sixty-five exams in 58 patients (32 ± 15 years) were included. Typically, 2D cine and 3D self nav spatial resolution was 1.1-1.52 × 4.5-7 mm and 0.9-1.153 mm, respectively. 3D self nav yielded larger maximum diameters than 2D cine: CuCo 37.2 ± 6.4 vs. 36.2 ± 7.0 mm (p = 0.006), CuCu 39.7 ± 6.3 vs. 38.5 ± 6.5 mm (p < 0.001). CuCu diameters were significantly larger (2.3-3.9 mm, p < 0.001) than CuCo and TTE diameters on both 2D cine and 3D self nav. Intra- and interobserver variabilities were excellent for both techniques with bias of -0.5 to 1.0 mm. Intra-observer variability of the more experienced observer was better for 3D self nav (F-test p < 0.05). Aortic root asymmetry was more pronounced in patients with bicuspid aortic valve (BAV: 0.73 (interquartile (IQ) 0.69; 0.78) vs. 0.93 (IQ 0.9; 0.96), p < 0.001), which was associated to a larger difference of maximum CuCu to TTE diameters: 5.5 ± 3.3 vs. 3.3 ± 3.8 mm, p = 0.033. CONCLUSION: Both, the 3D self nav and 2D cine CMR techniques allow reliable determination of aortic root diameters. However, we propose to privilege the 3D self nav technique and measurement of CuCu diameters to avoid underestimation of the maximum diameter, particularly in patients with asymmetric aortic roots and/or BAV.

Risk stratification of adults with congenital heart disease during the COVID-19 pandemic: insights from a multinational survey among European experts.

OBJECTIVE: Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies. METHODS: This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios. RESULTS: 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios. CONCLUSIONS: Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.

COVID-19 in Adults With Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. OBJECTIVES: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. METHODS: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.

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

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/m2 (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.