Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

BACKGROUND: The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. METHODS: A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. RESULTS: Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). CONCLUSIONS: ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation.

Effects of atorvastatin on endothelial function and the expression of proinflammatory cytokines and adhesion molecules in young subjects with successfully repaired coarctation of aorta.

OBJECTIVE: To investigate the effects of atorvastatin on endothelial function and low-grade systemic inflammation in subjects with successful surgery for aortic coarctation repair (SCR). DESIGN: Open-label study. SETTING: Outpatients visiting the adult congenital heart disease department of our hospital. PATIENTS: 34 young people with SCR. INTERVENTIONS: Patients with SCR received atorvastatin 10 mg/day (n=17) or no treatment (n=17) for 4 weeks. At baseline and at 4 weeks, endothelial function was assessed by flow-mediated dilatation (FMD) of the right brachial artery, and blood samples were obtained. Serum levels of interleukin (IL) 1b, IL-6 and soluble vascular cell adhesion molecule-1 (sVCAM-1) were determined by ELISA. MAIN OUTCOME MEASURES: Effects of treatment on FMD and serum levels of IL-1b, IL-6 and sVCAM-1. RESULTS: FMD in the atorvastatin group was significantly improved after 4 weeks (from 6.46±0.95% to 11.24±1.38%, p<0.01), while remaining unchanged in the control group (from 6.74±0.58% to 6.95±0.53%, p=NS). Even though atorvastatin had no effect on serum IL-6 levels (0.62 (0.37-0.88) pg/ml to 0.53 (0.28-0.73) pg/ml, p=NS), it significantly reduced circulating levels of IL-1b (from 1.17 (0.92-1.77) pg/ml to 1.02 (0.75-1.55) pg/ml, p<0.05) and sVCAM-1 (from 883.4 (660.3-1093.1) ng/ml to 801.4 (566.7-1030.2) ng/ml, p<0.05). No changes were seen in serum levels of IL-6, IL-1b and sVCAM-1 in the control group after 4 weeks compared with baseline (p=NS for all). CONCLUSIONS: Atorvastatin treatment for 4 weeks in subjects with SCR significantly improved endothelial function and suppressed systemic inflammatory status by decreasing circulating levels of IL-1b and sVCAM-1.

Echocardiographic and signal averaged ECG indices associated with non-sustained ventricular tachycardia after repair of tetralogy of fallot.

OBJECTIVE: To identify any possible association between different readily available non-invasive indices and potential malignant ventricular arrhythmias in patients with repaired tetralogy of Fallot. DESIGN: 27 consecutive patients, mean (SD) age 27.3 (11.7) years, were studied 15.7 (6.7) years after corrective surgery for tetralogy of Fallot, using 12 lead ECG, 24 hour Holter recordings, signal averaged ECG, and echocardiography. The following variables were measured: standard QRS duration, filtered QRS duration (fltQRS), low amplitude signal duration, and root mean square voltage of the last 40 ms of the fltQRS (RMS-40), as well as right ventricular systolic pressure, right ventricular ejection fraction, and the ratio of the maximum short axis diameters of the right and left ventricles (RD:LD). RESULTS: All patients had right bundle branch block, with a mean QRS duration of 137.1 (14.9) ms. There were no patients with sustained arrhythmia. Five patients had runs of non-sustained ventricular tachycardia (group A) and the other 22 patients did not (group B). Univariate analysis showed that fltQRS and RD:LD ratio were significantly associated with non-sustained ventricular tachycardia. In addition, a fltQRS >/= 148 ms, low amplitude signal >/= 32.5 ms, RMS-40 /= 1.05 were cut off points with a high sensitivity for detecting patients with non-sustained ventricular tachycardia. CONCLUSIONS: Abnormal signal averaged ECG and echocardiographic variables are associated with potentially malignant ventricular arrhythmias on the Holter recordings in asymptomatic patients with repaired tetralogy of Fallot.

Relevance of adenosine deaminase and lysozyme measurements in the diagnosis of tuberculous pericarditis.

OBJECTIVE: To assess the value of pericardial fluid adenosine deaminase (ADA) and pericardial lysozyme (Lys) as tools in diagnosing tuberculous pericarditis. METHODS: Forty-one patients (age range 17--77 years) with significant pericardial effusion were included in the study. Diagnostic pericardiocentesis and pericardial biopsy were performed while serum and pericardial fluid ADA and Lys were measured in all patients. Grouping of patients resulted as follows: group I = 7 patients with tuberculous pericarditis; group II = patients with neoplastic pericarditis; group III = 30 patients with idiopathic pericarditis. RESULTS: Pairwise multiple comparison procedures revealed a significant difference of ADA in group I versus group III (p < 0.05) but not versus group II. Furthermore, pericardial Lys in group I was higher than in groups II and III (p < 0.05). A strong correlation between pericardial ADA and Lys was found (r = 0.733, p = 0.01) for all the patients. Receiver operating curves showed a value of 72 U/l as cutoff point of pericardium ADA, with a sensitivity of 100% and a specificity of 94% in the diagnosis of tuberculous pericarditis. Similarly for pericardial Lys, a value of 6.5 microg/dl had a sensitivity and specificity of 100 and 91.17%, respectively. CONCLUSIONS: Both measurements of pericardial ADA and Lys need to be taken into account when attempting the early diagnosis of tuberculous pericarditis.

Aortic elastic properties in patients with repaired coarctation of aorta.

Patients with successfully repaired coarctation of the aorta have impaired distensibility. Age at surgery as well as left ventricular mass index are factors that are related to the distensibility index.

The effect of iron overload in the hearts of patients with beta-thalassemia.

BACKGROUND AND HYPOTHESIS: An important complication of beta-thalassemia is iron deposition in cardiac tissues resulting in fibrosis and dysfunction. Our aim was the investigation of the possible clinical effect of iron loading in the heart of patients with beta-thalassemia prior to the appearance of symptoms of depressed systolic function. METHODS: Thirty-five patients with beta-thalassemia, of whom 24 had the major type (Group 1) and 11 had the intermedia type (Group 2) were studied. Eleven age- and gender-matched controls were also studied (Group 3). All patients were evaluated echocardiographically and were shown to have normal left ventricular systolic function and dimensions. Serum ferritin, atrial natriuretic peptide (ANP), left atrial diameter (LAD), peak early mitral inflow velocity (E), peak late mitral inflow velocity (A), E/A ratio, deceleration time of the mitral inflow E wave (DT), and isovolumic relaxation time (IVRT) were measured. RESULTS: Univariate analysis showed that both groups of patients had similarly increased LAD and ANP plasma levels. Group 1 had a higher E/A ratio (2.27 +/- 0.88) SS than Group 2 (1.69 +/- 0.47, p = 0.05) and Group 3 (1.50 +/- 0.38, p = 0.01). Serum ferritin was significantly higher in Group 1 (3.526 +/- 0.352) than in Group 2 (2.808 +/- 0.288, p < 10(-5) and Group 3 (2.139 +/- 0.124, p < 10(-5). Multivariate analysis showed that ANP is a factor that is affected by the LAD and E/A ratio and that serum ferritin levels affect the LAD and E/A ratio. CONCLUSIONS: Although LAD and ANP levels are increased in patients with beta-thalassemia, the increased serum ferritin levels of patients seem to affect left atrial size and E/A ratio. ANP secretion is consecutively affected by these factors.

Juxtaposition of the morphologically right atrial appendage in solitus and inversus atria: a study of 35 postmortem cases.

Juxtaposition of the atrial appendages has previously been classified positionally, left sided being more frequent than right sided. In our study of 35 postmortem cases, juxtaposition was analyzed morphologically for the first time. Juxtaposition of the morphologically right atrial appendage (JRAA) was always left sided with solitus atria (34 cases) but was right sided with inversus atria (1 case), the latter patient being the first documented case of JRAA with atrial inversion. Thus in patients with JRAA, the sidedness of the juxtaposition depends on the type of atrial situs that coexists. Frequent associated malformations included tricuspid valve anomalies (21 [60%] of 30), hypoplasia of the right ventricular sinus (26 [74%] of 35), and an abnormal conus (subaortic or bilateral) in 100%. Hypoplasia of the right ventricular sinus, plus malformation of the conus, appears to be important in the morphogenesis of JRAA.

Juxtaposition of the morphologically left atrial appendage in solitus and inversus atria: a study of 18 postmortem cases.

Juxtaposition of the morphologically left atrial appendage (JLAA) was analyzed for the first time primarily morphologically, rather than primarily positionally. In a series of 18 postmortem cases, JLAA with solitus atria occurred in 16 (89%) cases, and JLAA with inversus atria was found in 2 (11%) cases. JLAA with solitus atria was always right-sided, whereas JLAA with inversus atria was left-sided. Thus the sidedness of the malposed (juxtaposed) LAA depended on the atrial situs, not on the type of ventricular loop (contrary to what was formerly thought). The anatomic features associated with JLAA are essentially the opposite of those with JRAA. JLAA was characterized by left atrial outlet obstruction (69%), left ventricular hypoplasia (67%), and aortic outflow tract obstruction (39%). JLAA usually has a hypoplastic left ventricle and normal conus, whereas JRAA typically has a hypoplastic right ventricle and abnormal conus.

Significance of an atrial septal aneurysm in the presence of an ostium secundum atrial septal defect. A transesophageal echocardiographic study.

The aim of this study was to elucidate the mechanism by which an atrial septal aneurysm is formed in the presence of an atrial septal defect by characterizing the distinguishing echocardiographic features of atrial septal defects between patients with and without an atrial septal aneurysm. The transesophageal echocardiograms of 30 consecutive patients who underwent surgical closure of a secundum atrial septal defect were compared with those of 8 normal controls. In patients with secundum atrial septal defect, the maximal diameter (mean +/- SD) of the fossa ovalis was 21.73 +/- 3.43 compared to 11.43 +/- 1.00 mm in the control group (p < 0.01). In the 7 (23%) patients with atrial septal aneurysm, the mean maximal diameter of the fossa ovalis was 25.28 +/- 3.03 compared to 20.65 +/- 2.78 mm in those without an atrial septal aneurysm (p < 0.01). The atrial septal defect was smaller in patients with than in those without an atrial septal aneurysm. In 4 patients with atrial septal aneurysm who had a history of a cerebrovascular event, the interatrial communication was only detected by contrast echocardiography. In conclusion, in patients with atrial septal aneurysm, atrial septal defects tend to be smaller but the incidence of cerebrovascular events is greater.