P wave dispersion in assessment of dysrhythmia risk in patients with secundum type atrial septal defect and the effect of transcatheter or surgical closure.

Atrial dysrhythmia is an important cause of mortality and morbidity in patients with atrial septal defect. Increased P wave duration can predict the risk of atrial dysrhythmia. The aim of this study is to evaluate the risk of atrial dysrhythmia by measuring P wave dispersion, and to observe the effect of surgical and transcatheter closure. Sixty-one patients and 30 controls were investigated. In patient group, before and after closure and in control group at the time of presentation, 12-lead electrocardiography records were evaluated. P wave duration and amplitude, P wave axis, PR interval, P wave dispersion, QRS axis, corrected QT interval, and QT dispersion were calculated. The measurements in patient and control groups, measurements before and after closure, and measurements of surgical and transcatheter group were compared. There were 27 patients in surgical group and 34 patients in transcatheter group. In patient group, signs of right heart volume overload were apparent but there was no significant difference in terms of P wave dispersion between two groups. We compared patient group in itself and found that while the use of medication, cardiothoracic index, ratio of right ventricular dilation, and paradoxical movement in interventricular septum were increased, mean age of closure was younger in surgical group. While P wave dispersion decreased in transcatheter group after closure, it increased in surgical group (p = 0.021). In conclusion, atrial septal defects may cause atrial repolarisation abnormalities and this effect persists even after surgical closure. Transcatheter closure in childhood may decrease dysrhythmia risk in long-term follow-up.

Aortic elasticity and the influence of valve morphology in children with bicuspid aortic valve.

AimWe investigated dimensions and elasticity of whole aorta in patients with bicuspid aortic valve and influence of valve phenotype. METHOD: The study group included 44 patients and 42 controls. Patients were divided into groups according to the type of valve opening as horizontal - fusion between right and left coronary cusps - and vertical - fusion between right-non-coronary cusps; according to age they were divided into younger (5-10 years) and older patients (11-16 years). Our study did not include valve phenotype with fusion between left and non-coronary cusps. Systolic-diastolic diameters of aortic annulus, sinus valsalva, sinutubular junction, arcus, and ascending-descending and abdominal aorta were measured and z-scores were obtained. Aortic strain, distensibility, and stiffness index were calculated. Flow-mediated dilatation of brachial artery was studied. RESULTS: z-Scores at annulus, sinus valsalva, sinutubular junction, and ascending aorta were higher in study patients (p=0.001, p=0.0001, p=0.0001, p=0.0001, respectively). z-Scores of sinus valsalva and sinotubular junction were higher in the horizontal group than in the vertical group (p=0.006, p=0.023, respectively). z-Score was over +2 in 51% of patients with horizontal morphology and 33% of patients with vertical morphology (p=0.0001). Ascending aorta was more distensible and less stiff in the study group (11.3±5.63 versus 7.91±4.5, p=0.002; 4.76±3.60 versus 6.19±3.44 cm2.dyn-1.10-6, p=0.033, respectively). Stiffness index of ascending, arcus, and abdominal aorta were higher in the horizontal group (p=0.004, p=0.038, p=0.006, respectively). Ascending aorta was more distensible and less stiff in the younger group (p=0.007, p=0.027, respectively) but did not differ in the older group compared with the control group. CONCLUSION: Aortic dimensions are enlarged in patients with bicuspid aortic valve starting from childhood, suggesting the presence of generalised aortopathy. Aortic elasticity is increased at young age and decreased with age.

Assessment of pulmonary arterial hypertension and vascular resistance by measurements of the pulmonary arterial flow velocity curve in the absence of a measurable tricuspid regurgitant velocity in childhood congenital heart disease.

This study aimed to determine mean pulmonary arterial pressure (PAPmean) and pulmonary vascular resistance (PVR) using transthoracic echocardiography (TTE) measurements of the pulmonary artery flow velocity curve in children with pulmonary arterial hypertension (PAH) and congenital heart disease when the tricuspid regurgitant velocity (TRV) is not sufficient. This study enrolled 29 congenital heart disease cases with pulmonary arterial hypertension and 40 healthy subjects followed at our center. The mean age was 66.9 ± 77.9 months in the patient group and 76.3 ± 62.1 months in the control group. A positive correlation was found between TRV and systolic pulmonary arterial pressure (r = 0.394, p = 0.035, 95% confidence interval [CI] = 0.032-0.665), whereas a negative correlation was found between corrected acceleration time (AcTc) and PAPmean (r = -0.559, p = 0.002, 95% CI = -0.768 to -0.242). Furthermore, a negative correlation was found between parameters TRV and AcTc (r = -0.383, p = 0.001, 95% CI = -0.657 to -0.019). Based on the cutoff criterion of 124 ms for AcTc, sensitivity was found to be 79.3% and specificity to be 77.5% in distinguishing between the PAH patients and the healthy control patients (receiver operating characteristic [ROC] area under the curve [AUC] = 0.804, 95% CI = 0.691-0.890, p < 0.0001). The sensitivity and specificity of the concomitant use of AcTc and/or TRV were found to be 90 and 73%, respectively, in distinguishing between the PAH patients and the the healthy control patients. The data obtained by TTE also can be appropriate for measuring PAPmean, PVR, and the vasoreactivity test and for determining the priority of implementing cardiac catheterization even if there is no measurable TRV value.

Quantitative evaluation of right ventricle function by transthoracic echocardiography in childhood congenital heart disease patients with pulmonary hypertension.

INTRODUCTION AND OBJECTIVE: The present study aims to quantitatively evaluate the right ventricle (RV) function by means of transthoracic echocardiography in normal children and childhood congenital heart disease patients with pulmonary hypertension. PATIENTS AND METHODS: This study was conducted in a cohort including 40 healthy children and 30 pediatric patients with pulmonary hypertension who were diagnosed under close surveillance at the study center between October 2009 and November 2010. RESULTS: Statistically significant differences were found between the patient and control groups for the right ventricle myocardial performance index (RVMPI), the left ventricle myocardial performance index (LVMPI), the tricuspid valve systolic flow velocity (Ts), the ratio of systolic pulmonary artery pressure to the right ventricle outflow tract systolic flow velocity time integral (sPAP/RVOT VTI), and the ratio of systolic pulmonary artery pressure to right ventricle outflow tract systolic flow velocity time integral × heart rate (sPAP/[RVOT VTI×HR]). When the children were divided into three groups based on their pulmonary vascular resistance significant differences emerged that predicted an increasing severity of RV dysfunction. Significant differences were also observed for the RVMPI, the LVMPI, and the Ts as well as for echocardiographic pulmonary flow (Qp) and systemic flow (Qs). DISCUSSION: The present study demonstrates that echocardiographic parameters can be used for the quantitative detection of RV dysfunction in childhood congenital heart disease patients with high pulmonary artery pressure (systolic, diastolic, and mean) or pulmonary vascular resistance.

Myocardial performance after successful intervention for native aortic coarctation.

Coarctation of the aorta is associated with increased risk for hypertension in adulthood, despite successful repair. The intrinsic mechanisms underscoring hypertension and left ventricular performance in these patients, however, remains to be determined. Our objective was to evaluate left ventricular performance by means of echocardiographic and biochemical parameters at midterm follow-up in normotensive children who have had undergone successful surgical or catheter interventional treatment of coarctation with a residual gradient of less than 20 mmHg at rest. We studied prospectively 14 patients with native aortic coarctation who underwent surgery or balloon angioplasty, the cohort made up of equal numbers of boys and girls, and having a mean age of 8.5 plus or minus 4 years. We also studied 30 age-matched healthy subjects, measuring mitral inflow pulsed wave signals, isovolumic relaxation and contraction times, myocardial performance index parameters, and levels of B-type natriuretic peptide and endothelin-1 in both groups. We found no differences in systolic blood pressure at rest between the patients and their controls. The ventricular septal diastolic dimensions, left ventricular posterior wall dimensions, mitral valve E wave, deceleration time, isovolumic relaxation time, isovolumic contraction time and myocardial performance index were all significantly increased in the patients. Levels of plasma B-type natriuretic peptide and endothelin-1 were also significantly higher in the patients when compared to the control group. We conclude that aortic coarctation is a chronic disease characterized by persistency of myocardial and vascular alterations. The elevated levels of plasma b-type natriuretic peptide and endothelin-1 may be indicative of late onset hypertension after successful treatment of native coarctation in early childhood.

Sydenham's chorea: clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens.

BACKGROUND: Sydenham's chorea is still the most frequently seen form of acquired chorea in childhood in developing world despite the use of antibiotics. It is a debilitating illness lasting for weeks or months and requires drug therapy. OBJECTIVE: To evaluate and compare the efficacies of sodium valproate and carbamazepine in the treatment of the choreiform movements in Sydenham's chorea. DESIGN: A prospective trial carried out with 24 children with Sydenham's chorea. PATIENTS: Twenty-four patients were divided into two groups having similar demographic and clinical properties. One group (n = 17) was given carbamazepine (15 mg/kg per day) and the other (n = 7) was given sodium valproate (20-25 mg/kg per day). As soon as the symptoms were taken under control, doses of the drugs were tapered slowly. The duration of the drug use was recorded. The time of response to therapy was compared between the groups and the patients were monitored for the adverse effects. RESULTS: There was no significant difference between the groups with respect to the time of clinical improvement and time of complete remission, duration of the therapy and the recurrence rates. Clinical improvement began by 8.0 +/- 4.0 days in sodium valproate and 7.4 +/- 8.2 days in carbamazepine group (P = 0.88). In the whole group no adverse effect was seen due to the drugs. CONCLUSION: Carbamazepine and valproic acid are equally effective and safe drugs in the treatment of choreiform movements in Sydenham chorea.

Left-to-Right Shunt with Congenital Heart Disease: Single Center Experience.

Objective. The objective of this study was to determine the frequency of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) with an isolated, large left-to-right shunt and to indicate the factors in the development of PAH. Methods. The pressure measurements in the cardiac chambers and the calculations based on the Fick's principle were compared among 3 separate groups of patients, respectively, with PAH, with hyperkinetic pulmonary hypertension (HPH), and with neither PAH nor HPH. Results. PAH was diagnosed in 30 (12.3%) patients, HPH in 35 (14.4%), while 177 (73.1%) were free of either. The highest risk for the development of PAH was found in the presence of perimembranous ventricular septal defect. A statistically significant difference was seen among these groups as to their left atrial pressure (p = 0.005) and the mean pulmonary arterial pressure (PAPmean; p < 0.001). While a correlation was present between RpI on one hand and age on the other (p = 0.014), a multiple linear regression could not evidence any correlation among age (p = 0.321), gender (p = 0.929). Conclusion. Our findings do not allow establishing a correlation between the duration of the high pulmonary flow and pulmonary vascular resistance increase or PAH development in isolated left-to-right shunts with congenital heart diseases.

Doppler tissue imaging provides an estimate of pulmonary arterial pressure in children with pulmonary hypertension due to congenital intracardiac shunts.

OBJECTIVE: The aim of this study is to determine the relationship between the cardiac catheterization findings and pulsed-wave (PW) Doppler and Doppler tissue imaging (DTI) in pulmonary arterial hypertension patients with congenital heart disease with intracardiac shunts. DESIGN AND PATIENTS: The present study aims to determine the relationship between the cardiac catheterization findings and PW Doppler and Doppler tissue imaging (DTI) in patients who have pulmonary arterial hypertension patients due to congenital heart disease with intracardiac shunts. Echocardiographic measurements were performed at the catheter angiography laboratory with concurrent catheterization. Left and right ventricle inflow velocities were recorded with PW Doppler and DTI studies. Maximum tricuspid regurgitation velocity (TS) was recorded in cases with measurable levels by continuous-wave Doppler. Moreover, the correlations among the echocardiographic values and invasive hemodynamic measures such as systolic pulmonary arterial pressure (PAPsystolic), mean pulmonary arterial pressure (PAPmean), diastolic pulmonary arterial pressure (PAPdiastolic) and pulmonary vascular resistance index (PVRI) were evaluated. RESULTS: A negative correlation was found between TE'/TA' and PAPsystolic, PAPdiastolic and PAPmean (P = 0.008, r = -0.480; P = 0.001, r = -0.584; P = 0.001, r = -0.567, respectively). ME/ME' was also found to be negatively correlated with PAPdiastolic, PAPmean and PVRI (P = 0.002, r = -0.556; P = 0.005, r = -0.502; P = 0.027, r = -0.411, respectively). The concurrent use of TE'/TA' (cut-off value <2.6) and TS had a sensitivity of 79% and a specificity of 93% for distinguishing between patients with healthy controls. CONCLUSION: When used in conjunction with conventional methods, TE'/TA' has the highest sensitivity and specificity in distinguishing between patients and healthy controls.

Relationship between ventricular function assessed by tissue Doppler imaging and exercise capacity in patients after repair of tetralogy of Fallot: an observational study.

OBJECTIVE: The present study aims to study the relationship between tissue Doppler echocardiography (TDE) indices of right ventricle and exercise capacity in patients after total correction for tetralogy of Fallot (ToF). METHODS: This cross-sectional observational study included 20 patients, after undergoing total correction procedure for ToF diagnosed with mild/moderate pulmonary regurgitation and 30 age-matched healthy children. In the postoperative period, patients were invited to hospital for evaluation of the ventricular functions by 2D, M-mode, Doppler (DE) echocardiography and TDE and exercise testing to evaluate the effort capacity. Statistical analysis was performed using Mann-Whitney U and Chi-square tests, and Pearson correlation analysis. RESULTS: Compared with the controls; the mitral annular peak systolic flow velocity (Sm) value was significantly lower, while isovolumic contraction time (IVCT), isovolumic relaxation time (IVRT) and myocardial performance index (MPI) values obtained at the tricuspid and mitral (MV) valves were significantly higher (p<0.05 for all) in patients after ToF repair. There was a negative correlation between the exercise period and the total correction age (r=-0.20, p=0.015) and the same negative correlation existed between the exercise period and METS (r=-0.25, p=0.010). MV IVCT with DE and TDE was found to be correlated with METS (r=-0.45, p=0.04). Left ventricular MPI was found to be correlated with maximum heart rate (r=-0.20, p=0.03). By DE, tricuspid valve deceleration time and Sm peak flow velocity with TDE were significantly correlated with METS (r=-0.30, p=0.04; r=-025, p=0.005, respectively). MPI calculated with TDE was correlated with maximum heart rate (r=-0.15, <0.01). CONCLUSION: Even if patients, undergone total correction surgery for ToF were asymptomatic or had minimal clinical symptoms, MPI index assessed by pulse wave TDE and exercise testing may allow early diagnosis of right ventricle dysfunction.

Subclinical cardiac dysfunction in children with coeliac disease: is the gluten-free diet effective?

Objectives. The aim of this study is to investigate the effects of coeliac disease on cardiac function in children using conventional transthoracic echocardiography (TTE) and tissue Doppler echocardiography (TDE). Methods. Coeliac disease patients were evaluated in two different groups based on serum endomysial antibody (EmA) titers (EmA (+) and EmA (-)), and the data obtained by conventional and TDE studies were compared between the patient groups and healthy controls. Results. There was no significant difference between EmA (+) and EmA (-) groups in terms of the conventional TTE parameters, including ejection fraction (EF), fractional shortening (FS), and left ventricle end diastolic diameter (LVEDD), that show the left ventricular systolic function (P = 0.727, P = 0.317, P = 0.118). TDE showed a significant difference in left ventricle (LV) isovolumic relaxation time (LV IVRT) and LV myocardial performance index (LV MPI) parameters between EmA (+) and EmA (-) patient groups (P < 0.0001). Conclusion. The measurement of LV MPI and LV IVRT parameters by TDE would be beneficial in early determination of the cardiac involvement and establishing appropriate treatment and followup of patients with coeliac disease as well as in making distinction between EmA (+) and EmA (-) patients.

Assessment of myocardial involvement using cardiac troponin-I and echocardiography in rheumatic carditis in Izmir, Turkey.

BACKGROUND: Acute rheumatic carditis is still a major problem in developing countries. Cardiac troponin-I (cTnI) has been identified as a sensitive and specific marker in the diagnosis of myocarditis in children and adults. METHODS: A prospective study was performed using Doppler echocardiography and cTnI in order to detect myocardial involvement in 26 consecutive patients with acute rheumatic valvular disease. Patients were divided into two groups: group 1, rheumatic fever with carditis (n > 16); group 2, rheumatic fever without carditis (n > 10). RESULTS: Clinically age, gender, body temperature, heart rate and white blood count did not differ significantly between the groups and the age-matched control group. C-reactive protein, erythrocyte sedimentation rate, anti-streptolysin-O were significantly different. Left ventricular fractional shortening was normal in all patients (group 1, 37 +/- 10%; group 2, 34 +/- 5%; NS). Left ventricular dimensions were larger in group 1, in which all patients except two had moderate to severe mitral and/or aortic valvular regurgitation (5.05 +/- 0.75 cm/m(2)) compared to group 2, in which none had valvular regurgitation (3.27 +/- 0.26 cm/m(2), P < 0.05). None of the patients in either group presented with or developed pericarditis. Mean cTnI was 0.12 +/- 0.034 ng/mL in group 2 and 0.077 +/- 0.02 in group 1, the difference of which was not statistically significant. Neither significant cTnI elevations nor echocardiographic systolic function abnormalities were found in the present patients with rheumatic carditis. CONCLUSIONS: The present results indicate the absence of myocardial involvement in acute rheumatic carditis without congestive heart failure.