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While many studies agree that consanguinity increases the rate of congenital heart disease (CHD), few genome analyses have been conducted with consanguineous CHD cohorts.We recruited 73 CHD probands from consanguineous families in Turkey and used whole-exome sequencing (WES) to identify genetic lesions in these patients.On average, each patient had 6.95 rare damaging homozygous variants, 0.68 of which are loss-of-function (LoF) variants. Seven patients (9.6%) carried damaging homozygous variants in five causal CHD genes. Six of those patients exhibited laterality defects (six HTX and one D-TGA). Three additional patients (4.1%) harbored other types of CHD-associated genomic alterations, which overall explained 13.7% (10/73) of the cohort. The contribution from recessive variants in our cohort is higher than 1.8% reported from a cohort of 2871 CHD subjects where 5.6% of subjects met the criteria for consanguinity.Our WES screen of a Turkish consanguineous population with structural CHD revealed its unique genetic architecture. Six of seven damaging homozygous variants in CHD causal genes occur in the setting of laterality defects implies a strong contribution from consanguinity to these defects specifically. Our study thus provided valuable information about the genetic landscape of CHD in consanguineous families in Turkey.

Objective Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients. Methods Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage-1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re-anastomosis of the superior vena cava to right atrium after an interstage period of 21-30 months (stage-2: anatomical repair). Results Any mortality was not encountered. The left ventricular mass indices increased from 18-32 to 44-74 g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow-up period was 20 months (9-32 months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function. Conclusions Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic-to-pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.

Objective:Two-stage arterial switch operation and left ventricle retraining are necessary for the patients with left ventricle dysfunction and transposition of great vessels with intact ventricular septum (TGA-IVS) who are referred late.Material and methods:Forty-seven patients with the diagnosis of TGA-IVS and left ventricle dysfunction who underwent arterial switch operation in our centre between July 2013 and August 2017 were analysed retrospectively. The inclusion criteria for left ventricle retraining were patients older than 2 months of age at presentation, having an echocardiographic left ventricle mass index of less than 35 g/m², and having an echocardiographic “banana-shaped” left ventricle geometric appearance. The patients were divided into two groups: pulmonary artery banding and Blalock Taussig shunt were performed as the initial surgical procedure for later arterial switch operation in Group I (n = 19) and pulmonary artery banding and bidirectional cava-pulmonary shunt in Group 2 (n = 28).Results:The average age was found to be 122.3 ± 45.6 days in Group I and 145.9 ± 37.2 days in Group II. There was no statistically significant difference (p = 0.232 versus p = 0.373) between the average left ventricle mass index of the two groups neither before the first stage nor the second stage (26.6 ± 4.8 g/m² versus 25.0 ± 4.9 g/m² and 70.5 ± 12 g/m² versus 673.8 ± 12.0 g/m², respectively). The average time interval for the left ventricle to retrain was 97.7 ± 42.9 days for Group I and 117.3 ± 40.3 days for Group II, significantly lower in Group I (p = 0.027). The time spent in ICU, length of the period during which inotropic support was required, and the duration of hospital stay were significantly higher in Group I (pConclusion:Pulmonary artery banding and bidirectional cava-pulmonary shunt can be performed as a safe and effective alternative to pulmonary artery banding and arterial Blalock Taussig shunt for patients with TGA-IVS in whom arterial switch operation is needed beyond the neonatal period. This approach involves a shorter hospital stay and fewer post-operative complications.

Background: Pulmonary arterial hypertension is of importance in congenital cardiac surgery as being a significant cause of morbidity and mortality. Although therapy options are limited, inhaled nitric oxide (NO) is used as a standard therapy. The present study aimed to compare inhaled NO and aerosolized iloprost in children with secondary pulmonary hypertension who underwent congenital cardiac surgery. Methods: Sixteen children included in the study were randomized into either inhaled NO or aerosolized iloprost group. For both groups, the observation period terminated at 72 h after cardiopulmonary bypass. Results: There was no significant difference between the groups in terms of mean age, weight, cross clamp time, pump time, and extubation time. No significant change was observed in the arterial tension and central venous pressure of both groups before the operation, 30 min after the pump, 45 min after the pump, and after extubation, whereas an increase was observed in the heart rate and cardiac output, and a decrease was observed in the pulmonary artery pressure. The mean values at the above-mentioned time points showed no difference between the groups. No serious adverse event and mortality was detected. Conclusions: Both inhaled NO and aerosolized iloprost were found to be effective and comparable in the management of pulmonary hypertension.

We report the successful closure of an extracardiac Fontan fenestration with a bio-absorbable device, which may be refenestrated by a transcatheter route when needed, in a 10-year-old boy. The patient presented with cyanosis two years after an extracardiac Fontan operation. Echocardiography revealed a moderate shunt from the Fontan circulation into the systemic circulation with a mean pressure gradient of 3-4 mmHg. Treadmill testing revealed a significant decrease in oxygen saturation (down to the low 50's from a baseline level of 80-85%). Cardiac catheterization revealed normal pressure in the Fontan circuit. A temporary balloon occlusion test showed that the defect was suitable for permanent occlusion. The fenestration was then occluded by a bio-absorbable Biostar (NMT medical, Boston, USA) atrial septal occluder device. The oxygen saturation on room air increased up to 95% after closure.

Postmyocardial infarction ventricular septal defect (VSD) carries a high mortality and, even after successful surgery, residual defect is common. A 75-year-old woman was admitted with the diagnosis of hyperacute anterior myocardial infarction. Primary percutaneous intervention was performed by stenting of a totally obstructed segment in the proximal left anterior descending artery. The patient's condition deteriorated on the second postprocedural day with a 3/6 pansystolic murmur at the mesocardium. Echocardiography revealed an apical anteroseptal VSD and moderate pulmonary hypertension. She underwent surgical VSD closure with a Gore-Tex patch and coronary artery bypass grafting to the left anterior descending and circumflex arteries. The patient's condition continued to be unstable due to septicemia and hemodynamically significant residual VSD. After medical management of septicemia, the residual defect was successfully closed using a 10-mm Cardio-O-Fix septal occluder under fluoroscopic and transesophageal echocardiographic guidance. The clinical condition of the patient was then stabilized and there was no significant residual shunt on echocardiography on the third postprocedural day.

The purpose of this study was to evaluate the safety and efficacy of transcatheter atrial septal defect (ASD) closure guided by transthoracic echocardiography (TTE). Since 2004, ASD closure was performed successfully in total 337 patients. Transthoracic echocardiography guidance was used in 206 patients (61.1%) (group 1). Closure was guided by transesophageal echocardiography under general anesthesia in patients with poor transthoracic acoustic windows, defects with aneurysmatic septum and/or multiple defects in 131 patients (38.9%) (group 2). The median age (9 vs. 16 years, P < 0.001), mean defect diameter (14.9 ± 4 vs. 17.2 ± 5 mm, P < 0.001), ratio of complex atrial septal defect (14 vs. 34%, P = 0.01), the median balloon stretch dimensions (21 vs. 18.7 mm, P = 0.003) and the median device diameters (22 vs. 19 mm, P < 0.001) were significantly greater in group 2 compared to group 1. Both the median procedure time and the median fluoroscopy time was significantly shorter in group 1 (60 vs. 75; and 13 vs. 16.5 min; P < 0.0001 and P < 0.0001, respectively). The incidence of residual shunt did not differ significantly in two groups during follow up. Transthoracic echocardiography guidance during transcatheter ASD closure is safe and effective in children and in many adults. Even complex ASDs could be closed with TTE in patients with good acoustic windows. Performing the procedure under TTE guidance significantly reduces procedure time and also provides increased patient's comfort.

Aiming to increase mixing at the atrial level, atrial septal stenting was performed in two pediatric cases with cyanotic congenital cardiac diseases. The first case was a 3-month-old male infant with transposition of the great arteries. The second case was an 18-month-old male infant with increased central venous pressure due to postoperative right ventricular outflow tract obstruction. Premounted bare stents of 8 mm in diameter were used in both cases. The length of the stent was 20 mm in the first case and 30 mm in the latter. The procedure was accomplished without any complications. In the first case, oxygen saturation increased approximately 20-25% with no significant interatrial gradient. In the latter, central venous pressure decreased from 16 to 8 mmHg immediately after the procedure. The patient was weaned from the ventilator on the second day and discharged from intensive care unit on the fifth day. Follow-up echocardiograms of both patients showed patent stents with good position relative to the atrial septum. Stenting of the atrial septum seems to be a safe and effective method to create a reliable, nonrestrictive interatrial communication.

Background: This study was conducted to evaluate left ventricle (LV) functions using conventional and tissue Doppler imaging in childhood obesity and to identify the effects of diet on LV diastolic functions. Methods: Conventional and tissue Doppler echocardiographic measurements were compared in 34 obese children and 24 age- and gender-matched lean controls. Fasting plasma glucose, insulin and homeostatic model assessment of insulin resistance levels were also obtained. Thirty-one of the obese children were subjected to a low-carbohydrate diet and their follow-up measurements were obtained after 6 months. Results: Left atrial diameter, LV mass and LV mass index were higher in obese children than in lean controls. Lateral mitral myocardial early diastolic (Em) and peak Em/myocardial late diastolic (Am) were lower, and mitral E/Em and lateral mitral myocardial isovolumetric relaxation time were higher in obese subjects than in lean controls. Insulin and homeostatic model assessment of insulin resistance levels were higher in obese patients and decreased significantly after diet. After diet therapy, lateral mitral Em and peak Em/Am, were increased, mitral E/Em and myocardial isovolumetric relaxation time were decreased. Conclusions: Obesity predisposes children to increased preload reserve, left ventricular subclinical diastolic dysfunction and deterioration in diastolic filling. Weight reduction with a low-carbohydrate diet seems to be associated with significant improvement in LV diastolic function and a decrease in diastolic filling, as well as causing reversal in insulin resistance seen in obese children.

ObjectiveOur study was undertaken to assess cardiac functions by tissue Doppler echocardiography in patient with primary episode of rheumatic carditis.MethodsWe divided 82 patients with rheumatic carditis were divided in two groups; 50 patients with mild and 32 patients with mitral regurgitation of grade two or more. A control group consisted of 30 healthy children free of any disease. All children underwent conventional and tissue Doppler echocardiography initially and at the time of the follow-up examination.ResultsMyocardial systolic wave velocity of the mitral annulus was significantly higher in patients with mitral regurgitation of grade two or more when compared to the control group, but was not different between patients with mild mitral regurgitation and healthy subjects at the time of the initial attack. Myocardial precontraction time, myocardial contraction time, and the ratio of myocardial precontraction and contraction times were significantly prolonged, and the systolic myocardial velocity of the mitral annulus was significantly decreased in patients with mitral regurgitation of grade two or more at the time of the follow-up examination. The myocardial systolic wave velocity was significantly lower, and myocardial precontraction time, myocardial contraction time, and the ratio of the precontraction and contraction times, were significantly longer or greater between patients with grade two or more mitral regurgitation and the control group at follow-up examination.ConclusionWe detected subclinical systolic dysfunction of the left ventricle in children with a primary episode of rheumatic carditis due to ongoing ventricular volume overload. Tissue Doppler imaging provides a quantifiable indicator useful for cardiac monitoring of disease during the period of follow up.

Background: The aim of the present study was to evaluate the role of tissue Doppler echocardiography in assessment of ventricular function in pediatric patients with bronchial asthma (BA). Patients and methods: Fifty-one pediatric patients with BA and 30 age- and sex-matched healthy subjects were studied. BA patients were divided into two groups: mild BA (n = 33) and moderate to severe BA (n = 18). All subjects were examined on conventional and tissue Doppler echocardiography, and 44 patients had pulmonary function tests on spirometry within 1 week of echocardiographic examination. Results: Conventional echocardiographic parameters were all similar in mild asthmatic patients and control subjects. Tricuspid E velocity, E/A ratio and isovolumetric relaxation time (IVRT) in moderate and severe cases differed significantly from mild cases and control subjects. E′, A′, E′/A′ ratio and IVRT of the lateral tricuspid annulus, and IVRT of the medial and lateral mitral annuli were different between mild cases and control subjects. E′ velocity and IVRT of the lateral tricuspid annulus and IVRT of the medial and lateral mitral annuli were also different between mild cases and moderate to severe cases. Pulmonary function tests correlated well with E′, E′/A′ and IVRT of lateral tricuspid annulus. Conclusion: Patients with BA have subclinical right ventricular diastolic dysfunction even in the early stages. The severity of the functional impairment is parallel with the severity of the disease. Tissue Doppler echocardiography has a greater predictive value than conventional imaging, and is useful for evaluating ventricular function in patients with BA.

Objectives: Pathoanatomic changes in cirrhosis result in impaired ventricular filling and diastolic dysfunction and were named as cirrhotic cardiomyopathy. However, cardiac functions have not been studied in patients with chronic hepatitis. We hypothesized that such patients might have subclinical ventricular dysfunction, detectable by tissue Doppler echocardiography and related to the severity of hepatic inflammation and fibrosis. Methods: We studied 63 clinically stable patients, 27 patients with mild chronic hepatitis (group 1), 22 patients with moderate chronic hepatitis (group 2) and 14 patients with severe chronic hepatitis (group 3) according to the scoring system of Knodell, and 36 age-matched healthy subjects. Results: Patients with severe chronic hepatitis had impaired right ventricular diastolic function. The early diastolic velocity of the tricuspid valve annulus was lower in patients from group 3 than in healthy subjects (P < 0.001). Patients in group 3 had a greater isovolumic relaxation time (P < 0.001), indicating right ventricular diastolic dysfunction. Comparing group 3 with the healthy subjects, the ratio of peak early myocardial tissue velocity and peak late (or atrial) myocardial tissue velocity was significantly decreased (P < 0.001), at 1.4 (0.7) and 1.9 (0.7), respectively. There was also a slightly lower peak early myocardial tissue velocity and peak late (or atrial) myocardial tissue velocity ratio and a slightly longer isovolumic relaxation time in patients from group 2 than in healthy subjects (P < 0.05). Conclusions: We detected subclinical dysfunction of the right ventricle in children with chronic hepatitis in whom hepatic inflammation and fibrosis is prominent. Tissue Doppler echocardiography provides a quantifiable indicator useful for detection and monitoring of disease progression.

Background:Disturbances of conduction are well known in the setting of acute rheumatic fever. The aim of this study is to investigate the QT dispersion as seen in the surface electrocardiogram of children with acute rheumatic fever.Methods:QT dispersion was quantitatively evaluated in 88 children with acute rheumatic fever. Patients were divided into two groups based on the absence or presence of carditis. As a control group, we studied 36 healthy children free of any disease, and matched for age with both groups. Repeat echocardiographic examinations were routinely scheduled in all patients at 3 months after the initial attack to study the evolution of valvar lesions.Results:The mean QT dispersion was significantly higher in children with rheumatic carditis. But there was no statistical difference between children without carditis and normal children. Among the children with carditis, the mean dispersion was higher in those with significant valvar regurgitation. Dispersion of greater than 55 milliseconds had a sensitivity of 85%, and specificity of 70%, in predicting rheumatic carditis, while a value of 65 milliseconds or greater had sensitivity of 81% specificity of 85% in predicting severe valvar lesions in acute rheumatic carditis. At follow-up examination, a clear reduction on the QT dispersion was the main finding, reflecting an electrophysiological improvement.Conclusions:These observations suggest that QT dispersion is increased in association with cardiac involvement in children with acute rheumatic fever.

BACKGROUND Children with cyanotic heart disease undergoing cardiac surgery in which cardiopulmonary bypass is used are at increased risk of postoperative bleeding. In this study, the authors investigated the possibility of reducing postoperative blood loss by using aprotinin and tranexamic acid alone or a combination of these two agents. METHODS In a prospective, randomized, blind study, 100 children undergoing cardiac surgery were investigated. In group 1 (n = 25) patients acted as the control and did not receive either study drugs. In group 2 (n = 25) patients received aprotinin (30.000 KIU.kg(-1) after induction of anesthesia, 30.000 KIU.kg(-1) in the pump prime and 30.000 KIU.kg(-1) after weaning from bypass). In group 3 (n = 25) patients received tranexamic acid (100 mg.kg(-1) after induction of anesthesia, 100 mg.kg(-1) in the pump prime and 100 mg.kg(-1) after weaning from bypass). In group 4 (n = 25) patients received a combination of the two agents in the same manner. Total blood loss and transfusion requirements during the period from protamine administration until 24 h after admission to the intensive care unit were recorded. In addition, hemoglobin, platelet counts and coagulation studies were recorded. RESULTS Postoperative blood loss was significantly higher in the control group (group 1) compared with children in other groups who were treated with aprotinin, tranexamic acid or a combination of the two agents (groups 2, 3 and 4) during the first 24 h after admission to cardiac intensive care unit (40 +/- 18 ml.kg(-1).24 h(-1), aprotinin; 35 +/- 16 ml.kg(-1).24 h(-1), tranexamic acid; 34 +/- 19 ml.kg(-1).24 h(-1), combination; 35 +/- 15 ml.kg(-1).24 h(-1)). The total transfusion requirements were also significantly less in the all treatment groups. Time taken for sternal closure was longer in the control group (68 +/- 11 min) compared with treatment groups 2, 3 and 4, respectively (40 +/- 18, 42 +/- 11, 42 +/- 13 min, P < 0.05). The coagulation parameters were not found to be significantly different between the three groups. CONCLUSIONS Our results suggested that both agents were effective to reduce postoperative blood loss and transfusion requirements in patients with cyanotic congenital heart disease. However, the combination of aprotinin and tranexamic acid did not seem more effective than either of the two drugs alone.

Crossed pulmonary arteries are an uncommon anomaly in which the left pulmonary artery originates superiorly and to the right of the right pulmonary artery, and the two pulmonary arteries cross each other. This anomaly may accompany other cardiac anomalies. We encountered this anomaly during echocardiographic examination of a 21-month-old boy. He had mesocardia, tetralogy of Fallot, right aortic arch, and malposition of the branch pulmonary arteries. Cardiac angiography confirmed intracardiac anomalies and showed the ostium of the left pulmonary artery lying superiorly and to the right of the right pulmonary artery and their crisscross.

OBJECTIVE To present our institutional experience of endovascular Cheatham-Platinum stent implantation in children and adults with native and recurrent aortic coarctation. METHODS Between August 2007 and November 2009, 45 patients had aortic coarctation treated with 47 stents implantation. We preferred primarily stent implantation in adult patient with coarctation, in children more than five years-old it is preferred in cases of aneurysm, subatretic or blind coarctation and coarctation with patent ductus arteriosus or in restenosis. Files of stent-implanted patients were retrospectively analyzed in terms of patients' demographic features, echocardiographic and angiographic findings both before and after procedure. Patients were grouped as Group 1: native coarctation and Group 2: recoarctation developed after either surgery or balloon angioplasty. Findings of the cases' were compared using paired and unpaired Student's t, Mann-Whitney U and Chi-square tests. RESULTS Sixteen covered and 31 bare totally 47 balloon expandable stents were implanted in 45 patients. The mean follow up duration was 12.1 ± 7.1, median 11 months (2-29 months). There was no procedure related death. In two patients two stents were implanted in tandem. While the coarctation of the aorta was native in 26 patients (functionally interrupted aortic arch in one), recoarctation was detected in 7 patients after surgery, in 8 patients after balloon angioplasty, in 4 patients both after surgery and balloon angioplasty. One patient had functionally interrupted aortic arch perforated with guide wire and then covered stent implanted. The mean age 12.2 ± 5.9 years (5-33 years) and mean body mass index was 21 ± 3.7 kg/m2 (14.8-31 kg/m2). Considering all cases, a statistically significant decrease in both the invasive and echocardiographic gradients (p

OBJECTIVE The objective of this study was to assess medium-term results of aortic balloon valvuloplasty (ABV) in congenital aortic stenosis and to determine factors affecting development of aortic regurgitation (AR). METHODS Between September 2003 and January 2010, 114 ABV procedures performed on 97 patients analyzed retrospectively. Patients were evaluated in terms of transvalvular gradients before and after procedure, development of new aortic insufficiency or increase in the current insufficiency and factors affecting the procedural success and development of aortic insufficiency. In addition, follow up results were also evaluated in terms of restenosis and increase or decrease of aortic regurgitation. Student's t-test, ANOVA for repeated measurements, linear discriminant and Kaplan-Meier survival analyses were used for statistical analysis. RESULTS The mean age was 6.63±6.33 year (2 days-21 years). Mean follow-up was 32.6±25.1 months (1 to 75 months). Thirty-seven patients were infant (

Concomitant idiopathic hypertrophic subaortic stenosis and disseminated myocardial bridging is an uncommon clinical entity with poor prognosis. We describe a symptomatic 19-year-old girl who had myocardial debridging and transaortic and transmitral extended septal myectomy in the same surgical session. An early and simultaneous surgical approach may prevent sudden cardiac death in these high-risk patients.

ObjectiveOur aim is to compare the haemodynamic and adverse effects of propofol versus the mixture of midazolam and ketamine as used in sedation for cardiac catheterization in children.MethodsIn a prospective randomized trial, we divided patients needing sedation into 72 receiving a mixture of midazolam and ketamine and 42 receiving propofol. Their ages ranged from 6 months to 12 years, and 1 year to 16 years, respectively. We collected data relative to heart rate, mean arterial pressure, respiratory rate, peripheral saturations of oxygen, and adverse effects. We assessed cyanotic patients to establish any relationship between the haemodynamic data and peripheral arterial saturations of oxygen.ResultsDemographic data, including age, gender, and weight, was not statistically different between the groups. In those receiving midazolam and ketamine, mean systemic arterial pressures before, and 30 minutes after, sedation were 64.3, with standard deviation of 9.8, and 62.5, with standard deviation of 10.2, millimetre of mercury (p equals to 0.237). Heart rates were 131.3, with standard deviation of 13.5, and 109.2, with standard deviation of 17.3 beats per minute, (p less than 0.001) whereas in those given propofol the comparable values were 71.2, with standard deviation of 14.4, and 53.6 with standard deviation of 9.7 millimetres of mercury (p less than 0.001), and 115.2, with standard deviation of 13.6, and 100.5 with standard deviation of 20.1 beats per minute (p less than 0.01), respectively. Mean systemic arterial pressures in the subgroups of cyanotic patients before and 30 minutes after sedation were 74.8, with standard deviation of 14.6, and 72.7, with standard deviation of 12.4 millimetres of mercury for those receiving midazolam and ketamine (p equals to 0.544), and heart rates were 119.3, with standard deviation of 12.2, and 104.6 with standard deviation of 16.1 beats per minute (p equals to 0.001). In those given propofol, the comparable values were 71.1 with deviation of 15.5 and 53.9 with deviation of 9.2 millimetres of mercury (p equals to 0.001), and 126.7 with deviation of 20.8 and 107.2 with deviation of 13.5 beats per minute (p equals to 0.001), respectively.ConclusionsIn cyanotic children, propofol used as a sedative agent during cardiac catheterization causes a decrease in mean arterial pressure and arterial desaturation. Ketamine produces more stable haemodynamic data in children with congenitally malformed hearts.

Objective: We investigated the use of the bispectral index for monitoring sedation during cardiac catheterization. The scores for the bispectral index may not reflect correct values in children, but may be helpful during titration of sedatives such as midazolam and ketamine. Methods: We conducted a prospective randomized clinical trial in 126 patients scheduled for cardiac catheterization in a teaching hospital. They ranged in age from 4 months to 15 years. In 66 patients, sedation was performed without use of the bispectral index, while the index was used in the other 60 patients. The data collected included heart rate, mean arterial pressure, respiratory rate, saturation of oxygen, amount of sedatives, awakening time and adverse effects. We subdivided the patients into age-related groups for each parameter. Results: The demographic data were not statistically different. Monitoring with the bispectral index in those aged from 1 to 3 years revealed use of lower doses of midazolam, at 2.09mg per kg per hr, with standard deviation of 0.36, and similarly lowers doses of ketamine, at 2.07mg per kg per hr, with standard deviation of 0.22, the values in those not monitored being 2.93, with standard deviation of 0.45, and 2.96 with standard deviation of 0.51 respectively, these difference being statistically significant (p 50.001 and p 50.04, respectively). In those aged from 3 to 6 years of age, dosage of midazolam was 2.09, with deviation of 0.36, and of ketamine 1.78, with deviation of 0.27, following use of the bispectral index, compared to 2.89 with deviation of 0.28, and 2.62 with deviation of 0.69 respectively, when the bispectral index was not used, these again being significant differences (p 50.033 and p 50.04). The requirements for respiratory support and adverse effects were also significantly lower when using the bispectral index (p less than 0.05). No significant difference was found regarding dosages at the ages of 4 months to 1 year, and 6 to 15 years. The awakening time, however, was shorter with use of the index in those aged from 1 to 6 years. Conclusions: When using the bispectral index for monitoring sedation during catheterization in children, we noted decreased need for doses of midazolam and ketamine, a lower need for respiratory support and less adverse effects.

The identification of a coeliac disease in patients with idiopathic dilated cardiomyopathy raises critical questions on the relationship between the two entities. But cardiac functions have not been studied in patients with coeliac disease. The present study was undertaken to assess cardiac functions by Tissue Doppler Echocardiography in patient with coeliac disease.We studied 45 clinically stable patients; twenty-five patients with positive serum IgA Antiendomysial Antibody levels (Group 1), twenty patients with negative serum IgA Antiendomysial Antibody levels (Group 2) at the time of echocardiographic study. Control group consisted of 30 healthy children free of any disease.Myocardial systolic wave velocity of the mitral annulus was significantly lower (p0.001), myocardial precontraction and contraction time were slightly longer in Group 2 when compared control group (p=0.015, p=0.044, respectively). There was a negative correlation between the serum IgA Antiendomysial Antibody levels titers and myocardial systolic wave levels of all patients included in the study (r = -0.633; p0.001). A myocardial systolic wave velocity of8.9 cm/s had a 92% sensitivity and 80% specificity in predicting serum IgA Antiendomysial Antibody levels positive patients.We detected subclinical systolic dysfunction of the left ventricle in children with coeliac disease in whom serum IgA Antiendomysial Antibody reactivity is prominent. Tissue Doppler echocardiography provides a quantifiable indicator useful for cardiac monitoring of disease during follow up.

The Ross procedure has become the first choice for aortic valve replacement in children and young adults at many institutions. Since 1997, a lack of availability of homograft valves in Turkey has prompted the use of alternative substitutes for right ventricular outflow tract (RVOT) reconstruction during the Ross procedure.Before April 2005, among 20 patients (age range: 14 months to 45 years) at the present authors' institution, the Ross procedure was performed in 14 and a Ross-Konno procedure in six. Sixteen patients underwent RVOT repair using alternative methods for homograft valve replacement. Fourteen patients received a Medtronic Freestyle valve and one patient a Medtronic Contegra bovine jugular vein conduit. An autologous RVOT repair was used in one patient. Ten of the Medtronic Freestyle valve patients were aged16 years. In all patients who received a Medtronic Freestyle valve echocardiographic evaluations were conducted shortly after surgery and during follow up.There was no early mortality. One patient died from pneumonia after six months, and another (asymptomatic) patient died suddenly at 34 months after surgery. Before hospital discharge the mean peak pressure gradient across the Freestyle valve was 12.1 +/- 11.0 mmHg, and this increased to 24.1 +/- 20.0 mmHg after a mean follow up of 51.2 +/- 6.9 months (range: 6 to 101 months) (p0.002). Mild pulmonary regurgitation was seen in two patients. One asymptomatic adult patient was reoperated on at another center because of a 60-mmHg echocardiographic peak gradient at four years postoperatively.Although long-term follow up is required to explain the durability of the Medtronic Freestyle valve, the present results show that the valve can be used with intermediate-term success in the Ross procedure - and even in children as an alternative - if homograft valves are not available.

Scimitar syndrome is a rare congenital disorder. It is characterized by partial or total abnormal venous drainage of the right lung into the inferior vena cava, which is often associated with anomalous systemic arterial supply to the right lung, congenital cardiac anomalies, hypoplasia of the right lung and bronchial anomalies. Symptoms depend on the degree of the shunt and severity of the associated anomalies, which determine the treatment. We present a 6-year-old boy who was diagnosed as having the adult form of scimitar syndrome during evaluation for recurrent pulmonary infections, and underwent embolization with a detachable silicon balloon of the anomalous systemic arterial supply from the abdominal aorta to the right lower lung lobe. Successful elective surgery was performed 6 months later, in which right pulmonary veins were directed to the left atrium using a Gore-Tex patch by creating an intra-atrial tunnel. The patient has been symptom-free period during 6 months of follow-up, which supports the idea that recurrent pulmonary infections can be eliminated by embolization of the anomalous arterial supply.

Objective: To evaluate whether the deleterious effect of cardiopulmonary bypass (CPB) can be prevented by controlling PaO2 in cyanotic children. Design: Prospective randomized clinical study. Setting: Single university hospital. Participants: Pediatric patients undergoing cardiac surgery for repair of congenital heart disease (n = 24). Interventions: Patients were randomly allocated into 3 groups. Patients in the acyanotic group (group I n = 10) had CPB initiated at a fraction of inspired oxygen (F1O2) of 1.0 (PO2 300 to 350 mmHg). Cyanotic patients were subdivided as follows: Group 11 (n = 7) had CPB initiated at an F1O2 of 1.0 and group III (n = 7) had CPB initiated at an F1O2 of 0.21 (PO2 90 to 110 mmHg). A biopsy specimen of right atrial tissue was removed during venous cannulation and another sample was removed after CPB before aortic cross-clamping. The tissue was incubated in 4 mmol/L of t-butylhydroperoxide and the malondialdehyde (MDA) level was measured to determine the antioxidant reserve capacity. Blood samples for cytokine levels tumor necrosis factor (TNF)-alpha and interleukin (IL)-6 response to CPB were collected after induction of anesthesia and at the end of CPB before prolamine administration. Measurements and Main Results: After initiation of CPB MDA level rose markedly in the cyanotic groups compared with the acyanotic group (210 +/- 118% v 52 +/- 34% p < 0.05) which indicated the depletion of antioxidants. After initiation of CPB TNF-&alpha and IL-6 levels of the cyanotic groups were higher than for the acyanotic group (168 +/- 77 v 85 +/- 57 p < 0.001 249 +/- 131 v 52 +/- 40 p < 0.001). When a comparison between the cyanotic groups was performed group II (initiating CPB at an F1O2 of 1.0) had significantly increased MDA production compared with group III (initiating CPB at an F1O2 of 0.21) (302 +/- 134% v 133 +/- 74% p < 0.05). Group If had higher TNF-alpha and IL-6 levels than group III (204 +/- 81 v 131 +/- 52 p < 0.001 308 +/- 147 v 191 +/- 81 p < 0.01). Conclusion: Conventional clinical methods of initiating CPB at a hyperoxemic PO2 may increase the possibility of myocardial reoxygenation injury in cyanotic children. This deleterious effect of reoxygenation can be modified by initiating CPB at a lower level of oxygen concentration. Subsequent long-term studies are needed to determine the best method of decreasing the oxygen concentration of the CPB circuit. Copyright 2002 Elsevier Science (USA). All rights reserved.