Prevalence and Treatment Outcomes of Arrhythmias in Patients with Single Ventricle Physiology over the Age of 40 Years.

Background: Arrhythmias are a well known complication in patients with single ventricle physiology (SVP). However, there is still a lack of data regarding arrhythmias in older patients. The aim of this study was to analyze arrhythmia type and frequency, treatment and recurrence rates in patients with SVP over the age of 40 years. Methods: Data was obtained retrospectively from clinical records. All patients > 40 years with SVP with arrhythmias between 2005 and 2018 were included in the study. Treatment was classified as medical, interventional (electrophysiological studies (EPS) in combination with catheter ablation) or direct current cardioversion (DCCV). Results: Altogether, 29 patients (11 female; mean 47.5 ± 4.6 years) with 85 arrhythmia episodes were identified. The median follow-up time was 6.3 years. Cavo-tricuspid (CTI) and non-CTI related intra-atrial reentrant tachycardia (IART) and atrial fibrillation (AF) were most common (48.2% and 37.6%, respectively). In total, 18 EPS/ablations were performed in 9 patients and 52 DCCVs in 20 patients. Acute success was 98% for DCCV and 72.2% for EPS/ablation. Recurrence rate was high (70% for DCCV and 55% for EPS). AT recurrences occurred after a median of 8 and 2.5 months, respectively. On multivariate analyses, age was the only risk factor for arrhythmia recurrence (HR 0.58, 95% C.I. 0.43−0.78, p < 0.0001). Pacemaker implantation was necessary in seven patients (AV block n = 4, sinus node dysfunction n = 3) and one patient received an ICD for secondary prophylaxis. Sudden death occurred in three patients. Conclusions: The most common arrhythmias in patients with SVP > 40 years are IART and AF. Arrhythmia recurrence following EPS or DCCV is frequent. Older age is an independent risk factor for arrhythmia recurrence.

Endangered patients with congenital heart defect during transition-Germany-wide evaluation of medical data from National Register for Congenital Heart Defects (NRCHD).

BACKGROUND: Appropriate care over the entire lifespan is essential in the population with congenital heart defect since the number of patients with congenital heart defect is increasing steadily worldwide. More than 90% survive into adulthood nowadays. The transition from pediatric to adult care in patients with congenital heart defect is a major challenge in clinical practice and often fails. Patients with congenital heart defect are generally at higher risk for different acquired secondary diagnoses. This cross-sectional retrospective study analysed data from the German National Register for Congenital Heart Defects to gain insight into the clinically relevant health-status of the transition population among congenital heart defect patients in Germany. METHODS: Adolescents and young adults with congenital heart defect between the ages of 15 to 25 years (which have been defined as the transition generation) were identified using the National Register of Congenital Heart Defects medical database. Out of 55,687 patients with congenital heart defect, 8,834 adolescents and young adults with congenital heart defect [4,063 female (46.0%); 20.3±3.1 years] were included in the statistical analyses. Statistical analyses were conducted using the student's t-test, χ2-test and Fisher's exact test. RESULTS: Severity of congenital heart defect: simple (23.4%), moderate (45.1%) and complex (31.5%). Most common congenital heart defect: atrial septal defects (14.9%) followed by ventricular septal defects (12.8%) and tetralogy of Fallot (10.5%). Most frequent acquired cardiac diagnosis: arrhythmia (25.5%) followed by secondly pulmonary hypertension (4.5%) and thirdly systemic arterial hypertension (3.6%). Almost 10% had chromosomal abnormalities and other genetic syndromes. Patients had neurological defects overall with 7.3%, followed by musculoskeletal defects with 6.9% and psychological disorders with 5.6%. CONCLUSIONS: Adolescents and young adults with congenital heart defect need to bridge the gap between pediatric and adult cardiology as they already show up to 4 cardiac and up to 7 extracardiac acquired secondary diagnoses during the transition period. Otherwise, early detection of an acquired secondary diagnosis, which affects the lives of young adults with congenital heart defect, fails with all its consequences. KEYWORDS: Transition; congenital heart defect (CHD); epidemiology; health services; National Register for Congenital Heart Defects (NRCHD).

Patients with Single-Ventricle Physiology over the Age of 40 Years.

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 ≥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 ± 6.4 years) were included. Median follow-up time was 4.9 years (interquartile range (IQR): 1.8-8.5). 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): 1.5-106.3, p = 0.021) remained as an independent predictor. CONCLUSIONS: SVP patients ≥40 years of age are burdened with significant morbidity and mortality. Renal disease is an independent predictor of all-cause mortality.

Direct oral anticoagulants in adults with congenital heart disease - a single centre study.

BACKGROUND: Direct oral anticoagulants (DOACs) have been proven to be safe and effective in patients with acquired heart disease. However, data regarding their use in adults with congenital heart disease (ACHD) is scarce. METHODS: All ACHD under DOAC were retrospectively identified. Bleeding and thromboembolic events were registered. CHADS2, CHA2DS2-VASc and HASBLED scores were calculated. Risk factors for bleeding and thromboembolic events were identified. RESULTS: 215 ACHD patients (111 female, 48.4 ±â€¯15.5 years) were included. CHD was severe in 44.2%, moderate in 23.7%, and simple in 32.1%. Indications for anticoagulation were: atrial arrhythmias (66.8%), cerebrovascular accidents (32.7%), pulmonary thromboembolism (3.7%), deep vein thrombosis (11.2%), atrial thrombi (6.5%), and Fontan circulation/TCPC (5.6%). Mean follow-up was 15.8 ±â€¯15.8 months. Nine patients suffered a major and eight a minor bleeding. Thromboembolic events occurred in two patients. The annual risk for bleeding was 3.1%/patient/year and for thromboembolic events 0.7%/patient/year. A CHADS2-Score >2, HASBLED >3, and renal disease were identified as risk factors for bleeding (minor and major bleeding) in a univariate analysis. In the multivariate analysis, renal disease remained as an independent predictor for bleeding (HR 6.13, CI [1.04-36.27], p < 0.05). It was also the only risk factor for major bleedings (HR 13.75, CI [2.60-72.54], p = 0.002). Risk factors for thromboembolic events were not identified. CONCLUSIONS: A low rate of thromboembolic events was observed in ACHD patients under DOAC therapy. Bleeding complications were not negligible. Special attention has to be paid to those patients with advanced renal failure.

Sacubitril/valsartan for heart failure in adults with complex congenital heart disease.

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 ±â€¯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 ±â€¯2042 ng/l vs. 1938 ±â€¯1524 ng/l, p = 0.20), creatinine levels increased (1.14 ±â€¯0.52 mg/dl vs. 1.35 ±â€¯0.74 mg/dl, p = 0.002). Systolic (110 ±â€¯15 mm Hg vs. 103 ±â€¯14 mm Hg, p = 0.02) and diastolic blood pressures (68 ±â€¯10 mm Hg vs. 61 ±â€¯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.

Gene Expression Profiling in Abdominal Aortic Aneurysms After Finite Element Rupture Risk Assessment.

PURPOSE: To investigate the association between local biomechanical rupture risk calculations from finite element analysis (FEA) and whole-genome profiling of the abdominal aortic aneurysm (AAA) wall to determine if AAA wall regions with highest and lowest estimated rupture risk show different gene expression patterns. METHODS: Six patients (mean age 74 years; all men) scheduled for open surgery to treat asymptomatic AAAs (mean diameter 55.2±3.5 mm) were recruited for the study. Rupture risk profiles were estimated by FEA from preoperative computed tomography angiography data. During surgery, AAA wall samples of ~10 mm2 were extracted from the lowest and highest rupture risk locations identified by the FEA. Twelve samples were processed for RNA extraction and subsequent whole genome expression profiling. Expression of single genes and of predefined gene groups were compared between vessel wall areas with highest and lowest predicted rupture risk. RESULTS: Normalized datasets comprised 15,079 gene transcripts with expression above background. In biopsies with high rupture risk, upregulation of 18 and downregulation of 18 genes was detected when compared to the low-risk counterpart. Global analysis of predefined gene groups revealed expression differences in genes associated with extracellular matrix (ECM) degradation (p<0.001), matrix metalloproteinase activity (p<0.001), and chemokine signaling (p<0.001). CONCLUSION: Increased expression of genes involved in degrading ECM components was present in AAA wall regions with highest biomechanical stress, supporting the thesis of mechanotransduction. More experimental studies with cooperation of multicenter vascular biobanks are necessary to understand AAA etiologies and identify further parameters of FEA model complementation.