Sinus node dysfunction as the first manifestation of left ventricular noncompaction with multiple cardiac abnormalities.

Left ventricular noncompaction (LVNC) is a genetically heterogenous form of cardiomyopathy which may remain undiagnosed till adulthood due to the late presentation of typical symptoms such as dyspnea, congestion, ventricular arrhythmias and thromboembolism. Symptomatic bradycardia secondary to persistent sinus node dysfunction is very rare. Coexistent cardiac defects are common in children however in adults the disease is usually in isolated form. Here, we present a case of twenty-three year-old female LVNC patient with patent ductus arteriosus, bicuspid aortic valve and persistent sinus node dysfunction who presented with dizziness as the first manifestation of the disease.

Assessment of interleukin-1 gene cluster polymorphisms in lone atrial fibrillation: new insight into the role of inflammation in atrial fibrillation.

BACKGROUND: Systemic inflammation is accepted as one of the pathophysiological mechanisms of atrial fibrillation (AF). The role of inflammation has been shown previously. Interleukin (IL) system is the main modulator of the inflammatory responses and genetic polymorphisms of IL-1 cluster genes are associated with increased risk for inflammatory diseases. OBJECTIVES: To investigate the association between polymorphisms of IL-1 cluster genes and lone AF. SUBJECTS AND METHODS: DNA samples were collected from 70 proven lone AF patients and 70 healthy subjects. Genomic DNA was typed for the variable number of the tandem repeat (VNTR) IL-1 receptor antagonist (RN) gene polymorphism, IL-1B -511 C > T(rs16944) promoter polymorphism, and +3953 C > T(rs1143634) polymorphism in exon 5 by polymerase chain reaction. RESULTS: In lone AF group the frequency of IL-1RN2/2 and IL-1RN1/2 genotypes were higher than in the control group (7.2% vs 4.3% and 48.5% vs 22.8%, respectively; χ(2) = 14.1; P = 0.028). The frequency of allele 2 was significantly higher in the lone AF group (32.1% vs 15.7%; χ(2) = 10.7; P = 0.005). Allele and genotype distribution of IL-1B -511 C > T and +3953 C > T polymorphisms were not statistically different between the groups. C-reactive protein (CRP) levels were higher in lone AF patients compared to the control group (median = 1.25, interquartile range [IQR] = 0.85 vs median = 1.08, IQR 0.46 mg/L, respectively; P = 0.02). In multivariate regression analysis, presence of allele 2 of IL-1 VNTR polymorphism and elevated plasma high-sensitive-CRP levels were the independent predictors of lone AF. CONCLUSION: Presence of allele 2 of VNTR polymorphism of IL-1RN gene may cause increased risk for lone AF probably due to the inadequate limitation of inflammatory reactions.

[Left atrial myxoma with severe neovascularization: role of preoperative coronary angiography]. / Ciddi damarlanma ile ortaya çikan sol atriyum miksomasi: Ameliyat öncesinde koroner anjiyografinin yeri.

Cardiac myxomas are highly vascular tumors and there is no consensus on the use of coronary angiography to assess their vascularity. A 64-year-old male patient presented with complaints of exertional dyspnea, fatigue, arthralgia, weight loss, intermittent high fever, and palpitation. He had an 18-month history of stent implantation for the left anterior descending coronary artery. Echocardiography showed a mobile mass in the left atrium with regular contours. Coronary angiography was performed with the initial diagnosis of myxoma and vascular supply of the tumor by the proximal branches of the right coronary artery (RCA) was visualized. Re-evaluation of previous angiograms of the patient showed existence of the same mass, in significantly smaller size, and supply from the RCA. The mass which was 5.5x1x0.5 cm in size was removed by surgical resection and the branches of the RCA supplying the tumor were ligated. Histopathologic examination confirmed the diagnosis. During 20 months of follow-up, the patient was asymptomatic and echocardiographic examinations were normal.

Coexistent Brugada syndrome and Wolff-Parkinson-White syndrome: what is the first clinical presentation?

The coexistence of Brugada syndrome and Wolff-Parkinson-White (WPW) syndrome is a very rare phenomenon. We describe a 31-year-old patient without any previous cardiac disorder admitted to our hospital due to palpitations and concomitantly diagnosed as WPW syndrome and treated with radiofrequency catheter ablation. He was later diagnosed with Brugada syndrome and followed-up 2 years without any symptoms. We discuss other previously reported cases in literature, in which these two conditions exist simultaneously.

Longitudinal left ventricular systolic function is impaired in patients with coronary slow flow.

Slow coronary flow (SCF) is a well recognized clinical entity, characterized by delayed opacification of coronary arteries in the presence of normal coronary angiogram. There is currently no data evaluating myocardial systolic function in SCF phenomenon. This study was performed to evaluate regional and global systolic function using tissue Doppler imaging (TDI), strain (S) and strain rate imaging (SRI) in patients with slow coronary flow. A total of 35 patients with slow coronary flow and otherwise normal coronary arteries (mean age 48 +/- 7 years) (SCF group) and 21 patients with normal coronary angiograms (mean age 50 +/- 12 years) (control group) were included in the study. These patients were prospectively assessed for evaluation of regional and global left ventricular function by conventional echocardiography, systolic TDI, peak S, and peak systolic strain rates (SRs) There was a significant difference in peak SRs (-1.1 +/- 0.2 vs. -1.8 +/- 0.2 1/s, P < or = 0.0001) but similar in systolic TDI (42 +/- 20 vs. 44 +/- 21 mm/s, P = 0.77) and S (20.7 +/- 7.7 vs. 23.7 +/- 8.8, P = 0.14) between groups. SRs showed a good correlation with mean TIMI frame count (r = -0.80, P < or = 0.0001). As the number of coronary artery with SCF increased global strain rate decreased further. In case of one or two or three coronary artery with SCF global strain rates were 1.4 +/- 0.2; 1.1 +/- 0.3; 0.9 +/- 0.2 1/s, respectively, P < or = 0.0001. Although ejection fraction was preserved, global and regional strain rate were decreased in SCF. In brief, there is an impairment in longitudinal left ventricular systolic function in patients with SCF.

Effect of glucometabolic disorders on the formation of coronary collaterals in occlusive coronary artery disease.

OBJECTIVE: The primary aim of this study was to assess the effect of glucometabolic disorders on coronary collateral vessels in patients with occlusive coronary artery disease. METHODS AND RESULTS: Hundred and ninety-five consecutive patients with at least single-vessel occlusion were enrolled in this study prospectively. The standard oral glucose tolerance test was performed according to the criteria of the World Health Organization. Collateral circulation was graded according to the Rentrop classification. The mean Rentrop scores in normal glucose tolerance (NGT), impaired glucose tolerance (IGT), and diabetes were 1.40 +/- 1.02, 1.05 +/- 0.84, 1.00 +/- 0.98, respectively (P = 0.043). The percentage of patients without collateral circulation (Rentrop-0) was greatest in the diabetic group (44.4%), while the percentage was 21.8% in the IGT group and 22.0% in the NGT group. Ninety-five patients with at least one totally occluded coronary artery were analysed as a subgroup. In the totally occluded artery subgroup postprandial glycaemia was the only parameter that was associated with the Rentrop score in the univariate analysis (r = -0.34, P = 0.002) CONCLUSIONS: In conclusion, our study results, which are in agreement with previous results, indicate that not only diabetic glucose tolerance but also impaired glucose tolerance has an adverse impact on the development of coronary collaterals.

The relation between insulin resistance determined by homeostatic [corrected from haemostatic] modelling and slow coronary flow.

INTRODUCTION: In this study, we sought to determine whether insulin resistance, which is investigated by homeostatic modelling, is related to slow coronary flow (SCF). MATERIALS AND METHODS: A total of 24 patients with SCF (4 females/20 males, mean age 47 +/- 12 years) and 32 patients with normal coronary artery (10 females/22 males, mean age 52 +/- 12 years) were included in the study. Baseline glucose, insulin and plasma lipid levels were measured. A standard oral glucose tolerance test (OGTT) was performed and post-challenge insulin levels were also measured. The index of insulin resistance was calculated with the homeostatic modelling [homeostatic model assessment for insulin resistance index (HOMA-IR)]. RESULTS: There were no differences between the 2 groups with regard to age, lipid levels, blood pressure levels, history of smoking, fasting and post-challenge plasma glucose. Baseline insulin levels were augmented in the SCF group (9.64 +/- 5.93 vs 7.04 +/- 3.26, P = 0.041). HOMA-IR levels were not different between the study groups (2.20 +/- 1.44 vs 1.69 +/- 0.86, P = 0.129). Manifest insulin resistance was significantly higher in the CSF group as compared with the control group (25% vs 3%, P = 0.01). CONCLUSION: Manifest insulin resistance is seen more frequently in patients with SCF.

Endothelial nitric oxide synthase gene (T-786C) polymorphism in patients with slow coronary flow.

OBJECTIVES: In this study, we aimed to investigate the relationship between T-786C polymorphism of the endothelial nitric oxide synthase (eNOS) gene and slow coronary flow (SCF). STUDY DESIGN: A total of 56 patients with SCF but otherwise normal coronary arteries (mean age 48+/-9 years) and 37 controls with normal coronary angiograms (mean age 50+/-12 years) were enrolled in the study. Screening for the eNOS T-786C polymorphism was performed by restriction fragment length polymorphism methodology. RESULTS: In normal coronary artery and SCF groups, TT genotype frequency was 23 (62.2%) versus 22 (39.3%), TC heterozygote genotype frequency was 11 (29.7%) versus 30 (53.6%), and CC homozygote genotype frequency was 3 (8.1%) versus 4 (7.1%), respectively (P=0.07). In dominant model statistical analysis, total CC and CT genotype frequency in control and study groups was found to be 14 (37.3%) versus 34 (60.7%), respectively (P=0.025). A positive correlation was found between the mean thrombolysis in myocardial infarction frame count and C allele in patients with SCF (r=0.21, P=0.043). CONCLUSION: We concluded that the T-786C polymorphism of eNOS gene might be a risk factor for the SCF.

Mean platelet volume in patients with slow coronary flow and its relationship with clinical presentation.

OBJECTIVES: We investigated mean platelet volume (MPV) in patients with slow coronary flow (SCF) and its possible relationship with clinical presentation. STUDY DESIGN: The study included 50 patients with SCF and otherwise normal coronary arteries and 22 patients (control group) with normal coronary arteries. In the SCF group, there were 26 patients with stable angina pectoris (SAP), and 24 patients with unstable angina pectoris (USAP). Coronary blood flow was measured using the TIMI frame count. To determine MPV, blood samples with K3 EDTA were processed after one hour of venipuncture. The relationship between MPV and SCF was sought. RESULTS: The mean TIMI frame count was markedly increased in patients with SCF compared to controls (p<0.0001). No significant differences existed between the groups with regard to white blood cell and platelet counts. Patients with SCF had significantly higher MPV values compared to controls (9.4+/-2.3 fl vs 8.1+/-2.0 fl, p=0.014). In subgroup analysis, MPV was significantly increased only in patients presenting with USAP, compared to patients with SAP (p=0.044) and controls (p=0.002). There was a positive correlation between the mean TIMI frame count and MPV in patients with SCF (r=0.32, p=0.01). In multivariate analysis, MPV was the only independent predictor of SCF (p=0.006, odds ratio=1.305, 95% CI=0.985-1.730). CONCLUSION: Our findings show that MPV is increased in patients with SCF, and SCF patients presenting with USAP exhibit significantly increased MPV values, suggesting an altered platelet reactivity and aggregation which may require effective anti-platelet therapy in this patient subgroup.

Long-term effects of cardiac resynchronization therapy on heart rate and heart rate variability.

Congestive heart failure is characterized by significant autonomic dysfunction. Development of left bundle branch block in congestive heart failure is a predictor of worse outcome. There are several lines of evidence that cardiac resynchronization therapy (CRT), by biventricular stimulation in patients with severe heart failure and left bundle branch block, improves autonomic functions which can be quantified by measuring heart rate variability. The aim of the present study was to assess the effect of CRT on autonomic functions quantified by heart rate variability and mean heart rate (HR) in patients with advanced heart failure and left bundle branch block in short and long-term follow-up. A total of 35 patients with systolic heart failure and left bundle branch block (mean-age 60 +/- 11 years; 24 male and 11 female; mean left ventricular ejection fraction [EF]: 22.3 +/- 3%) were enrolled. Clinical assessment and echocardiographic examination were performed at baseline and every three months. Continuous electrocardiographic monitorization by 24-hour Holter recordings was performed pre-implantation, 3 months and 2 years after implantation. Mean HR and one of the time-domain parameters of heart rate variability, standard deviation of the R-R intervals (SDNN) were measured. CRT was associated with a decrease in the mean duration of QRS, and an increase in diastolic filling time, the rate with which the left ventricular pressure rises (dP/dt), and left ventricular ejection fraction. Decrease in mean heart rate and increase in SDNN were statistically significant in the third month and second year recordings when compared to baseline recording (p values were < 0.001 for both). In conclusion, CRT with biventricular pacing provides sustained improvement in autonomic function in patients with advanced heart failure and left bundle branch block.

Left ventricular diastolic function and endothelial function in patients with erectile dysfunction.

The aim of this study was to assess left ventricular diastolic function and forearm endothelial function in patients with erectile dysfunction (ED) without overt cardiovascular disease. Forearm endothelial function and diastolic Doppler parameters, including tissue Doppler imaging, were studied in 32 men with ED and 27 age-matched, healthy, male control subjects. Left ventricular diastolic function in patients with ED and the relation between endothelium-dependent vasodilation and the Doppler parameters of left ventricular diastolic function, including tissue Doppler imaging, were assessed. Endothelium-dependent vasodilation (4.1+/-3.3% vs 9.7+/-4.2%, p<0.001) as well as the mitral inflow E velocity (0.66+/-0.17 vs 0.80+/-0.16 m/s, p=0.01), the E/A ratio (the ratio of mitral inflow E velocity to mitral inflow A velocity; 0.91+/-0.3% vs 1.22+/-0.26%, p<0.001), and the E/Em ratio (the ratio of mitral A-wave velocity to early diastolic velocity in the annulus derived by tissue Doppler imaging; 7.4+/-2.7% vs 6.6+/-1.6%, p=0.03) were smaller in the ED group than in the control group. Deceleration time (228.6+/-61.6 vs 192.9+/-44.6 ms, p=0.03) and isovolumetric relaxation time (112.8+/-18 vs 94+/-15.9 ms, p<0.001) were also prolonged in the ED group compared with the control group. The mitral E-wave velocity (r=0.40, p=0.022), the E/A ratio (r=0.40, p=0.027), and the E/Em ratio (r=-0.52, p= 0.003) were related to endothelium-dependent vasodilation by nivariate analysis. Only the E/Em ratio was correlated with endothelium-dependent vasodilation by multivariate analysis. In conclusion, this study indicates that endothelial function and left ventricular diastolic function are impaired in patients with ED without overt cardiovascular disease.