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.
Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, ...pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex 'double switch' repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care.
Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. ...We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center.
We included 6969 adult patients (age 29.9 ± 15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2-14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient's age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased.
ACHD patients continue to be afflicted by increased mortality in comparison with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.
The prognostic benefit of atrial septal defect (ASD) closure in adulthood, particularly in advanced age, remains uncertain. The aim of our study was to examine the impact of ASD closure in a ...contemporary adult cohort on mid to longer term survival as compared with expected survival in the general population.
We study herewith all consecutive patients (≥16 years of age) who underwent ASD closure, catheter or surgical, at our tertiary centre between 2001 and 2012. Furthermore, we compare survival of our ASD closure cohort with expected survival in age and gender-matched general population and standardised mortality ratios (SMR) were calculated.
A total of 608 patients (mean age 45.4±16.7 years) underwent ASD closure (catheter 433(71.2%), surgical 175(28.8%)). There was no 30-day mortality and periprocedural complications were low (n=40, 6.6%). During a median follow-up of 6.7 (IQR 4.2-9.3) years 16 (2.6%) patients died; survival was similar to the general population (p=0.80) including patients >40 or >60 years of age at ASD closure (p=0.58 and p=0.64, respectively). There was no survival difference between gender (male: SMR 0.93; 95% CI 0.52 to 1.64, p=0.76; female: SMR 0.99; 95% CI 0.58 to 1.66, p=0.95) or mode of closure compared with general population (catheter: SMR 1.03; 95% CI 0.68 to 1.55, p=0.89; surgical: SMR 0.65; 95% CI 0.22 to 1.88, p=0.38).
Perioperative mortality and morbidity in a large contemporary adult cohort undergoing ASD closure, catheter or surgical, is extremely low. Mid to longer term survival is excellent irrespective of age, gender and mode of closure, and similar to matched general population.
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.
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.
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.
Abstract Purpose The epidemiology of acute kidney injury (AKI) after cardiac surgery depends on the definition used. Our aims were to evaluate the Risk/Injury/Failure/Loss/End-stage (RIFLE) criteria, ...the AKI Network (AKIN) classification, and the Kidney Disease: Improving Global Outcomes (KDIGO) classification for AKI post–cardiac surgery and to compare the outcome of patients on renal replacement therapy (RRT) with historical data. Methods Retrospective analysis of 1881 adults who had cardiac surgery between May 2006 and April 2008 and determination of the maximum AKI stage according to the AKIN, RIFLE, and KDIGO classifications. Results The incidence of AKI using the AKIN and RIFLE criteria was 25.9% and 24.9%, respectively, but individual patients were classified differently. The area under the receiver operating characteristic curve for hospital mortality was significantly higher using the AKIN compared with the RIFLE criteria (0.86 vs 0.78, P = .0009). Incidence and outcome of AKI according to the AKIN and KDIGO classification were identical. The percentage of patients who received RRT was 6.2% compared with 2.7% in 1989 to 1990. The associated hospital mortality fell from 82.9% in 1989 to 1990 to 15.6% in 2006 to 2008. Conclusions The AKIN classification correlated better with mortality than did the RIFLE criteria. Mortality of patients needing RRT after cardiac surgery has improved significantly during the last 20 years.
Abstract
Aims
The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with ...limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality.
Methods and results
This study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P < 0.0001). Although a large number of ‘asymptomatic’ (NYHA class 1) patients did not achieve a ‘normal’ peak VO2, the NYHA class was a strong predictor of mortality, with an 8.7-fold increased mortality risk in class 3 compared with class 1 (hazard ratio 8.68, 95% confidence interval: 5.26–14.35, P < 0.0001).
Conclusion
Despite underestimating the degree of limitation in some ACHD patients, NYHA classification remains a valuable clinical tool. It correlates with objective measures of exercise and the severity of underlying cardiac disease, as well as mid- to long-term mortality and should, thus, be into incorporated the routine assessment and risk stratification of these patients.
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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