Many adult survivors of repaired congenital heart disease (CHD) are at premature risk of death. Sudden cardiac arrest (SCA) is 1 of the leading causes of death but little is known about determinants ...for SCA in adults with repaired lesions. We sought to determine incidence and risk factors for SCA in a study population of 936 adults with previously repaired CHD who had completed follow-up at a single tertiary center during a mean period of 9 ± 7 years. Mean age at first examination in our institution was 21 ± 7 years. Diagnostic categories included tetralogy of Fallot (216), coarctation of the aorta (157), transposition complexes (99), single ventricle (55), and other CHD (409). During a total follow-up of 8,387 person-years, 22 patients (2.6 per 1,000 person-years) presented with SCA. Incidence of SCA varied widely between specific lesions; the highest incidence was observed in transposition complexes (10 per 1,000 person-years). Independent predictors of SCA were retrospectively identified using multivariate Cox proportional hazard modeling. Age at initial examination and severely impaired subaortic ventricular systolic function were independent risk factors for SCA (severe subaortic ventricular systolic dysfunction, adjusted hazard ratio 29, 95% confidence interval 11 to 72, p <0.001). SCA occurred in 23% of patients with severe subaortic ventricular systolic dysfunction versus 0.7% of patients with nonsevere decreased subaortic ventricular function (p <0.001). In conclusion, severe subaortic ventricular systolic dysfunction is a dominant multivariate predictor of SCA in an unselected population of adult survivors after surgery for CHD. Our data support the consideration of primary prevention strategies in these patients.
Abstract Background Pulmonary arterial hypertension (PAH) is common in patients with congenital heart disease (CHD). Although Eisenmenger syndrome may be on decline, other types of PAH–CHD are ...increasing and little is known on long-term outcome of this population. We report the PAH–CHD population of Spain via a national registry with focus on long-term survival. Methods and results A total of 240 consecutive patients (age 37.7 ± 14.1 years, 67.9% females) with PAH–CHD included in the REHAP registry were analysed. Patients were classified into 3 groups: 1) Eisenmenger syndrome, 2) postoperative-PAH and 3) PAH associated with small defects. Over a median follow-up time of 4.5 1.6–7.1 years, 50 patients (20.8%) died or underwent lung/heart-lung transplantation. Patients with Eisenmenger syndrome had better survival than postoperative-PAH (HR 0.1 95% CI: 0.2–0.9, p = 0.048) but no advantage compared to small defects (HR 4.4, 95% CI 0.6–31.4, p = 0.15). In the overall PAH–CHD population, patients in NYHA functional class III–IV had a 3-fold increased risk of death (HR 3.0, 95% CI: 1.5–5.9, p = 0.001). Amongst patients with Eisenmenger syndrome, a pre-tricuspid shunt had a 2.6-fold increase risk of death (HR 2.6, 95% CI: 1.2–5.6, p = 0.03). There was no significant difference in survival between patients with postoperative-PAH and patients with iPAH (HR 0.99 95% CI: 0.6–1.7, p = 0.97). Conclusion PAH–CHD is associated with mid to long-term mortality. Outcome relates closely to functional class, type of PAH–CHD and within the Eisenmenger cohort, with location of the shunt. Adults with postoperative-PAH have the worse prognosis in the PAH–CHD cohort, reinforcing the need for lifelong close follow-up of such patients.
A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with ...transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model.
A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline interquartile range, 18-30; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty-five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell C index of 0.73 95% CI, 0.65-0.81) but tended to overestimate this risk (calibration slope of 0.20 95% CI, 0.03-0.36). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function.
We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.
The actual incidence of ascending aorta complications (AACs) in adults with bicuspid aortic valve (BAV) and the role of associated coarctation of the aorta (COA) as an independent risk factor for ...AACs remain unknown. From the Adult Congenital Heart Disease database at La Paz Hospital, 631 patients in whom a BAV was diagnosed by echocardiography or surgical inspection since December 1989 were identified. These patients were then further subdivided into 2 groups according to the presence of an associated COA. AACs included aortic aneurysms (ascending aorta ≥55 mm) and aortic dissection, rupture, or perforation. Patients with a BAV and COA had a greater prevalence of AACs (8.0%) than those with an isolated BAV (3.7%; p = 0.037). The coexistence of COA was the only significant predictor of AACs (odds ratio 4.7, 95% confidence interval 1.5 to 15; p = 0.01). From the total patient group with a BAV, the clinical and echocardiographic data were reviewed for 341 patients without an AAC at baseline (97 with and 244 without COA) who had undergone serial examinations >1 year apart. The median follow-up was 7 years (interquartile range 3.5 to 10.2; total 2,436 patient-years). A new AAC occurred in 13 patients (0.5/100 patient-years). The incidence of AACs was 1.3/100 patient-years in the COA group versus 0.2/100 patient-years in the non-COA group (hazard ratio 7.5, 95% confidence interval 2.0 to 28, p = 0.002). All acute aortic events (dissection or rupture) at follow-up occurred in patients with a BAV and COA. In conclusion, the long-term incidence of AACs in patients with isolated BAV is low, but patients with BAV and associated COA are at increased risk.
At rest, a good Fontan circulation can provide a normal cardiac output (CO). However, as a consequence of its unique hemodynamic nature, the limitations of the Fontan circuit are exposed during ...exercise. We aimed to provide a comprehensive assessment of the pathophysiology of exercise in adult Fontan patients (FPs) and identify factors limiting their functional capacity (FC). In a single-center study conducted in 37 FPs aged ≥16 years and 19 healthy-controls (HCs) who underwent CPET on a cycle ergometer in February and March 2022, the mean peakVO
was 21 ± 5.4 mL/kg/min, which was 55% of the predicted value. Morphologically, the left single ventricle showed a higher peakVO
% predicted value (57.4 ± 14.4% vs. 43.4 ± 8.1%,
= 0.045). The factors associated with low peakVO
values were an early flattened or descending O
pulse at maximal exertion (52 ± 14% vs. 62 ± 12.5,
= 0.04 and 47.6 ± 9% vs. 60 ± 14,
= 0.018, respectively) and chronotropic insufficiency (53 ± 12% vs. 69.8 ± 20%,
= 0.008). The OUES was found to be a useful parameter to assess the FC in FPs in maximal and submaximal exercise testing. A strong positive correlation was observed between the %OUES and peakVO
%predicted (r = 0.726,
> 0.001). The lung function was impaired in the FPs, mostly with a mild restrictive pattern (56.8%). The FPs showed lower inspiratory muscle strength compared to the HCs but it was not statistically associated with either the peakVO
or VE/VCO
slope. Regular intense physical activity improves one's FC. Although FPs have inspiratory muscle weakness, its impact on their FC is unclear. The peakVO
% predicted grew progressively higher as the level of physical activity increased (low level 49.5 ± 14%, moderate level 55 ± 12%, intense level 69 ± 20%).
The long-term outcome of patients with congenitally corrected transposition of the great arteries is mainly determined by progressive morphologically tricuspid valve regurgitation, heart block, ...atrial arrhythmias, and/or systemic ventricular dysfunction. Situs abnormalities have been reported in ≤34% of cases, but whether clinical differences exist between a situs inversus and situs solitus arrangement has not yet been studied. The clinical records of 38 adults with congenitally corrected transposition of the great arteries (mean age 40 ± 15 years) followed for a mean period of 7.4 years were reviewed. Of these 38 patients, 8 presented with situs inversus and 30 with situs solitus. No significant differences were found between the 2 groups in age, gender, ventricular septal defect, pulmonary tract stenosis, previous surgical repair, or duration of follow-up. However, none of the patients with situs inversus presented with an Ebstein-like anomaly of the morphologically tricuspid valve and none developed nonoperative-related complete atrioventricular block compared to 15 (50%; p = 0.013) and 11 (42%; p = 0.032) of the patients with situs solitus, respectively. At follow-up, 2 patients with situs inversus (25%) presented with sustained atrial arrhythmia, severe tricuspid regurgitation, or severe systemic right ventricular systolic dysfunction compared to 22 (73%) of 30 those with situs solitus (p = 0.034). No patient with situs inversus presented with cardiac death or severe heart failure compared to 12 (40%) of 30 with situs solitus (p = 0.038). In conclusion, Ebstein-like anomaly or spontaneous complete atrioventricular block are rare in patients with congenitally corrected transposition of the great arteries with situs inversus, and late complications are uncommon. The long-term outcome of patients with situs inversus was significantly better than that for patients with situs solitus.
ObjectiveAdults 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.MethodsThis 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.Results82% 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.ConclusionsPulmonary 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.
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.
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.
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.
•Overall risk perception among ACHD experts decreased over time.•Apart from cyanotic heart diseases, general risk factors for poor outcome in case of COVID-19 in the general population are also important in ACHD.•Our registry helped improving risk-stratification of ACHD patients during the pandemic.
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