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Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect (CHD). Contemporary data regarding its outcome in adults are scarce. Methods: Retrospective, single-center study of all ccTGA patients over the age of 16 years treated at our center during the time period 2006–2018. Only patients with a biventricular circulation were included. The primary endpoint was all-cause mortality. Results: Altogether, 96 patients (mean age 32.8 ± 16.0 years, female 50%) with ccTGA and a systemic right ventricle (SRV) were included in the study. An additional CHD was present in 81 patients (84.4%), most common were a ventricular septal defect (VSD) and a left ventricular outflow tract obstruction. Out of the whole cohort, 45 (46.9%) had already undergone cardiac surgery at baseline. During a median follow-up of 6.5 (IQR 2.8–12.7) years, the primary endpoint occurred in 10 patients (10.8%). Cause of death was cardiac in nine patients and suicide in one. Hospitalizations due to heart failure occurred in 48 patients (51.6%). Upon univariate Cox analysis, an NYHA class ≥III, severe tricuspid regurgitation, severe SRV systolic impairment, as well as a reduced left ventricular systolic function were predictors of the primary endpoint. Upon multivariable analysis, only NYHA class ≥ III (HR: 18.66, CI 95%: 3.01–115.80, p = 0.0017) and a reduced left ventricular systolic function (HR: 7.36, CI 95%: 1.18–45.99, p = 0.038) remained as independent predictors. Conclusions: Adults with ccTGA and an SRV are burdened with significant morbidity and mortality. Predictors for mortality are NYHA class and subpulmonary left ventricular function.

Background Heart failure is an important cause of morbidity and mortality in adults with congenital heart disease (ACHD). Sacubitril/valsartan is an established treatment for heart failure with reduced ejection fraction due to acquired cardiovascular disease. Data in adults with complex congenital heart disease (CHD) is lacking. Methods Retrospective study of ACHD patients with CHD of moderate/severe complexity and heart failure under treatment with sacubitril/valsartan. Clinical data was retrieved from medical records. Results Altogether, 23 patients (mean age 41.2 ± 11.9 years, female 17.4%) were included. A systemic right ventricle was present in 12 pat. (52.2%), a single ventricle physiology in 4 (17.4%), and a systemic left ventricle in 7 (30.4%). During a median follow-up of 221 days [IQR 79–430], systemic ventricular function (p = 0.88) and functional status according to New York Heart Association class (p = 0.38) did not improve. While NT-proBNP levels did not change significantly under treatment (2561 ± 2042 ng/l vs. 1938 ± 1524 ng/l, p = 0.20), creatinine levels increased (1.14 ± 0.52 mg/dl vs. 1.35 ± 0.74 mg/dl, p = 0.002). Systolic (110 ± 15 mm Hg vs. 103 ± 14 mm Hg, p = 0.02) and diastolic blood pressures (68 ± 10 mm Hg vs. 61 ± 12 mm Hg, p = 0.01) were reduced under therapy. Five patients discontinued therapy, four of these due to side effects. Conclusion In this small group of complex ACHD patients with heart failure, treatment with sacubitril/valsartan did not improve systemic ventricular function or functional status. Renal function needs close surveillance.

Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years. Methods: Patients with cAVSD who were ≥40 years of age at any point between 2005 until 2018 were included retrospectively. Data were retrieved from hospital records. The primary endpoint was a combination of death from any cause and unplanned hospitalizations due to cardiac reasons. Results: 43 patients (60.5% female, mean age 43.7 ± 6.0 years, genetic syndrome 58.1%) were included. At begin of follow-up, the majority of patients (n = 41, 95.3%) was in New York Heart Association (NYHA) class I or II. Out of the whole cohort 26 (60.5%) had undergone cardiac surgery. At baseline, at least one extracardiac comorbidity was present in 40 patients (93.0%). Median follow-up was 1.7 years (IQR 0.3–4.6). On univariate Cox analysis, NYHA class at begin of follow-up (hazard ratio: 1.96, CI 95%: 1.04–3.72, p &lt, 0.05) was the only predictor for the primary endpoint. Conclusions: Significant morbidity and mortality is present in cAVSD patients over the age of 40 years. NYHA class is predictive for a worse outcome.

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 &lt, 40 years of age were compared to those &ge, 40 years. Results: A total of 4673 patients (mean age 33.6 &plusmn, 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 (p &lt, 0.001). Number of comorbidities was higher in patients &ge, 40 years (2.1 &plusmn, 2.1) than in patients &lt, 40 years (1.2 &plusmn, 1.5, p &lt, 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.

Background: Single-ventricle physiology (SVP) is associated with significant morbidity and mortality at a young age. However, survival prospects have improved and risk factors for a negative outcome are well described in younger cohorts. Data regarding older adults is scarce. Methods: In this study, SVP patients under active follow-up at our center who were &ge, 40 years of age at any point between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history were retrieved from hospital records. The primary end-point was all-cause mortality. Results: Altogether, 49 patients (19 female (38.8%), mean age 49.2 &plusmn, 6.4 years) were included. Median follow-up time was 4.9 years (interquartile range (IQR): 1.8&ndash, 8.5). Of these patients, 40 (81.6%) had undergone at least one cardiac surgery. The most common extracardiac comorbidities were thyroid dysfunction (n = 27, 55.1%) and renal disease (n = 15, 30.6%). During follow-up, 10 patients (20.4%) died. On univariate analysis, renal disease and liver cirrhosis were predictors of all-cause mortality. On multivariate analysis, only renal disease (hazard ratio (HR): 12.5, 95% confidence interval (CI): 1.5&ndash, 106.3, p = 0.021) remained as an independent predictor. Conclusions: SVP patients &ge, 40 years of age are burdened with significant morbidity and mortality. Renal disease is an independent predictor of all-cause mortality.

Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking. Methods: PAH-ACHD patients &ge, 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality. Results: In total, 65 patients (67.7% female, mean age 45.19 &plusmn, 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2&ndash, 7.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2&ndash, 14.4, p = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2&ndash, 118.7, p = 0.006) remained as independent predictors of all-cause mortality. Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors.