Piccolo in transcatheter PDA closure multi-centre study from premature to adolescent children.

In this multi-centre study, the mid- to long-term efficacy and safety of the Amplatzer Piccolo Occluder in patent ductus arteriosus closure in premature and term infants as well as children were discussed. Methods. Between 2016 and 2021, 645 patients, 152 of whom were less than 1 month old, underwent ductus closure with the Piccolo device from five different centres in Turkey. The median age of the patients was 2.2 years, and the mean narrowest point of duct diameter was 1.8 mm. Sixty-two patients weighed ≤ 1.5 kg, 90 patients 1.5-3 kg, and the mean follow-up was 20.4 months. In 396, the duct was closed by the retrograde route. Ductal anatomy was Type A in 285, C in 72, E in 171, and F in 64 patients. Fluoroscopy duration was 6.2 min. The procedure success rate was 99.1%. Device embolisation occurred in 13 patients (2%), and 11 were retrieved with a snare. Cardiac perforation and death developed in one premature baby. The left pulmonary artery and the descending aorta stenosis were observed in 3 (0.4%) and in 5 patients (0.5%). Results. Piccolo device is safe and effective in closing ductus in all age groups. It has low profile for use in premature and newborn babies, a small embolisation risk, and a low residual shunt rate after closure. Conclusion. The Piccolo device can be considered as close an ideal occluder. The lower profile, smaller delivery catheter size, and symmetry of this device allow for a venous or arterial approach.

Safety and efficacy of Amplatzer duct occluder II and konar-MF™ VSD occluder in the closure of perimembranous ventricular septal defects in children weighing less than 10†kg.

Introduction: Device closure of perimembranous ventricular septal defects (pmVSD) is a successful off-label treatment alternative. We aim to report and compare the outcomes of pmVSD closure in children weighing less than 10 kg using Amplatzer Duct Occluder II (ADOII) and Konar-MF VSD Occluder (MFO) devices. Methods: Retrospective clinical data review of 52 children with hemodynamically significant pmVSD, and sent for transcatheter closure using ADOII and MFO, between January 2018 and January 2023. Baseline, procedural, and follow-up data were compared according to the implanted device. Results: ADOII devices were implanted in 22 children with a median age of 11 months (IQR, 4.1-14.7) and weight of 7.4 kg (IQR, 2.7-9.7). MFO devices were implanted in 30 children with a median age of 11 months (IQR, 4.8-16.6) and weight of 8 kg (IQR, 4.1-9.6). ADOII were implanted (retrograde, 68.1%) in defects with a median left ventricular diameter of 4.6 mm (IQR, 3.8-5.7) and right ventricular diameter of 3.5 mm (IQR, 3.1-4.9) while MFO were implanted (antegrade, 63.3%) in defects with a median left ventricular diameter of 7 mm (IQR, 5.2-11.3) (p > 0.05) and right ventricular diameter of 5 mm (IQR, 2.0, 3.5-6.2) (p < 0.05). The procedural and fluoroscopy times were shorter with the MFO device (p < 0.05). On a median follow-up of 41.2 months (IQR, 19.7-49.3), valvular insufficiency was not observed. One 13-month-old child (6.3 kg) with ADOII developed a complete atrioventricular heart block (CAVB) six months postoperative and required pacemaker implantation. One 11-month-old child (5.9 kg) with MFO developed a CAVB 3 days postoperative and the device was removed. At 6 months post-procedure, only one child with MFO still experiences a minor residual shunt. There was one arterio-venous fistula that resolved spontaneously. Conclusion: Both the MFO and ADOII are effective closure devices in appropriately selected pmVSDs. CAVB can occur with both devices. The MFO is inherently advantageous for defects larger than 6 mm and subaortic rims smaller than 3 mm. In the literature, our series represents the first study comparing the mid-term outcomes of MFO and ADOII devices in children weighing less than 10 kg.

The immediate haemodynamic response and right and left cardiac remodelling after percutaneous transcatheter closure of secundum atrial septal defect in children: a longitudinal cohort study.

OBJECTIVE: We aimed to assess the immediate haemodynamic response and the timing of cardiac remodelling in paediatric secundum atrial septal defect patients who underwent percutaneous transcatheter closure. METHODS: In this longitudinal cohort study with 41 paediatric secundum ASD patients who underwent PTC with Amplatzer Occluder device were assessed for immediate post-interventional haemodynamic response measured by catheterisation and was evaluated for right and left cardiac remodelling during a follow-up period of 12 months by transthoracic echocardiography. SPSS 20.0 was used for statistical analyses of pre- and post-interventional invasive haemodynamic parameters of the patients, and pre- and post-interventional TTE data compared with the values of the control group consisted of 39 healthy children. RESULTS: The mean diameter of ASD was 13.9 ± 4.7 mm. PTC intervention in all patients completed with 100% success and 0% complication rates. All invasive haemodynamic data, except the ratio of pulmonary resistance to systemic resistance, significantly reduced after PTC (p < 0.05). TTE and PW Doppler revealed that right and left cardiac remodelling started as soon as the post-interventional 24th hour and completed in the 12th month. CONCLUSIONS: This study with a very high interventional success rate can be counted as the first example of research on the haemodynamic response and timing of cardiac remodelling after PTC of secundum ASD in children. We suggest that future multicentric studies with larger cohorts and a comprehensive methodology like ours with longer follow-up periods would better serve to further assess the cardiac remodelling in children after PTC of secundum ASD.

Should we start a nationwide screening program for critical congenital heart disease in Turkey? A pilot study on four centres with different altitudes.

BACKGROUND: To investigate the feasibility of critical congenital heart disease (CCHD) screening test by pulse oximetry in four geographical regions of Turkey with different altitudes, before implementation of a nationwide screening program. METHODS: It was a prospective multi-centre study performed in four centres, between December, 2015 and May, 2017. Pre- and post-ductal oxygen saturations and perfusion indices (PI) were measured using Masimo Radical-7 at early postnatal days. The results were evaluated according to the algorithm recommended by the American Academy of Pediatrics. Additionally, a PI value <0.7 was accepted to be significant. RESULTS: In 4888 newborns, the mean screening time was 31.5 ± 12.1 hours. At first attempt, the mean values of pre- and post-ductal measurements were: saturation 97.3 ± 1.8%, PI 2.8 ± 2.0, versus saturation 97.7 ± 1.8%, PI 2.3±1.3, respectively. Pre-ductal saturations and PI and post-ductal saturations were the lowest in Centre 4 with the highest altitude. Overall test positivity rate was 0.85% (n = 42). CCHD was detected in six babies (0.12%). Of them, right hand (91 ± 6.3) and foot saturations (92.1 ± 4.3%) were lower compared to ones with non-CCHD and normal variants (p <0.05, for all comparisons). Sensitivity, specificity, positive and negative predictive values, and likelihood ratio of the test were: 83.3%, 99.9%, 11.9%, 99.9%, and 99.2%, respectively. CONCLUSION: This study concluded that pulse oximetry screening is an effective screening tool for congenital heart disease in newborns at different altitudes. We support the implementation of a national screening program with consideration of altitude differences for our country.

Syncope due to complete atrioventricular block and treatment with a transient pacemaker in acute rheumatic fever.

Various rhythm and connection disorders can be seen in the acute phase of acute rheumatic fever. First degree atrioventricular block, one of the minor signs of acute rheumatic fever, is the most common connection disturbance in this disease. Complete atrioventricular block, which seriously affects the conduction pathways, is rare in the literature. A 15-year-old boy was admitted because of syncope caused by complete atrioventricular block and a temporary pacemaker was employed because of symptomatic complete atrioventricular block. The transient pacemaker treatment was terminated due to recovery of complete atrioventricular block on the third day of antiinflammatory treatment. Acute rheumatic fever should be kept in mind as a possible cause of acquired complete atrioventricular block. Connection disturbances in acute rheumatic fever improve with antiinflammatory treatment. Transient pacemaker treatment is indicated for patients with symptomatic transient complete atrioventricular block.

Ivabradine is an effective antiarrhythmic therapy for congenital junctional ectopic tachycardia-induced cardiomyopathy during infancy: Case studies.

Junctional ectopic tachycardia (JET) is a rare form of arrhythmia that is most commonly seen during infancy. JET is continuous and incessant, characterized by persistently high heart rates that may result in impaired cardiac function and tachycardia-induced cardiomyopathy. Despite the availability of multiple antiarrhythmic treatments, including flecainide and amiodarone, management of JET is generally very difficult. Catheter ablation has a high risk of atrioventricular block and it may require the placement of a pacemaker. Ivabradine, also known as a cardiac pacemaker cell inhibitor, is a new-generation antiarrhythmic used to treat sinus tachycardia and angina pectoris in adult patients. In this article, we present three cases of subjects with infantile congenital JET who were admitted to our clinic with a tachycardia-induced cardiomyopathy. The age of the subjects ranged from 52 days to 10 months. Although the cases of tachycardia could not be controlled by multiple antiarrhythmics, including a combination of amiodarone and flecainide combined with either propranolol or digoxin, they were rapidly converted into sinus rhythm with an ivabradine treatment of 0.1-0.2 mg/kg/day. No cardiac or other side effects were observed during ivabradine treatment, and left ventricular functions and rhythms improved within 24 hours. These three cases therefore provide hope that ivabradine may be a suitable standard initial treatment for congenital JET. However, additional research is needed to confirm the validity of these results in other circumstances.

Patent Ductus Arteriosus closure in preterms less than 2kg: Surgery versus transcatheter.

BACKGROUND: As new devices come into the market, percutaneous techniques improve and interventionalists become more experienced; percutaneous closure gets more common in preterms. In this study we aimed to compare efficacy and safety of Patent Ductus Arteriosus closure surgically versus transcatheter method in preterms <2kg. Best of our knowledge this study is the first one that compares outcomes of surgery and percutaneous Patent Ductus Arteriosus closure in preterms. METHODS & RESULTS: Between the dates July 1997 to October 2014 in our center Patent Ductus Arteriosus of 26 patients <2kg were closed percutaneously (Group A) and 31 less than 2kg operated (Group B). Weight of patients in percutaneous Patent Ductus Arteriosus closure group was significantly more than the surgery group. Mean gestational age of the patients in Group A was 30±1.8weeks, in group B was 28.6±3.5weeks. In group A; all cases were closed successfully except 4 cases: device embolization in 2, cardiac tamponade and iatrogenic aortic coarctation were seen. Pneumomediastinum and chylothorax were the major complications of the surgery group. There was no statistically significance between complication and success rates between two groups. CONCLUSION: Percutaneous Patent Ductus Arteriosus closure is the candidate for taking the place of surgery in preterms. However, it is not applied routinely; can only be done in fully equipped large centers by experienced interventionalists.

Can whole-blood parameters be used in follow-up of children with rheumatic valvular heart disease?

OBJECTIVES: The aim of the present study was to investigate the relationships between red blood cell distribution width, platelet distribution width, and mean platelet volume and the presence and severity of valvular involvement in patients with rheumatic heart disease. METHODS: Between April, 2012 and December, 2015, 151 patients who were admitted to the Pediatric Cardiology Unit with diagnosis of rheumatic heart disease and 148 healthy children were included to our study. Transthoracic echocardiography for all children was performed, and the values of red blood cell distribution width, platelet distribution width, and mean platelet volume, besides other blood count parameters, erythrocyte sedimentation rate, and C-reactive protein levels were recorded. RESULTS: Red blood cell distribution width, platelet distribution width, mean platelet volume, and C-reactive protein levels were significantly higher in patients with rheumatic heart disease when compared with healthy controls (p0.05). CONCLUSION: This is the first study in children with rheumatic heart disease that demonstrated significantly increased red blood cell distribution width, platelet distribution width, and mean platelet volume levels, as well as evaluated all three parameters together. Furthermore, red blood cell distribution width values in the chronical period of acute rheumatic fever, due to the positive correlation with the other chronic inflammatory markers, may help make the diagnosis in children.

Transcatheter closure of PDA in premature babies less than 2 kg.

OBJECTIVE: Our hypothesis was that percutaneous PDA closure in babies less than 2 kg was a safe and effective method. The aim of this study is to share our experience in transcatheter PDA closure in infants whose body weight is less than 2 kg in order to support our hypothesis. METHODS: Between July 1997 and October 2014, 382 percutaneous PDA closures were done in our center. Nineteen patients who weighed less than 2 kg were included in this retrospectively study. The other inclusion criteria were 1) being symptomatic and PDA was thought as a possible contributor of medical state and 2) persistence of PDA after medical closure treatment. Patients who had sepsis and bleeding diathesis were excluded. According to size and shape of PDA, different types of devices were used such as detachable coils and Amplatzer duct occluders. Data was expressed as mean (SD) or median (minimum-maximum). Comparisons of means and medians were performed with Student's t-test and with Mann-Whitney U test, respectively. RESULTS: The median patient age and weight were 32 days and 1603 g (range 910-2000 g) respectively. Mean PDA diameter was 3.2±1.3 mm. Morphology of PDA was type A in 7 patients, type C in 10, type E in 1, and type B in 1 patient. There were no reported major complications. Stenosis of left pulmonary artery was detected in four patients, all of which resolved in 6 months follow-up. CONCLUSION: Percutaneous PDA closure in babies less than 2 kg is a safe and effective method that can be an alternative to surgery. Main distinguishing feature of this study is that it includes the largest cohort of patients less than 2 kg whose PDA closed percutaneously.

Effects of Acute Carbon Monoxide Poisoning on ECG and Echocardiographic Parameters in Children.

The aim of the current study is to investigate the effects of CO (carbon monoxide) on the cardiovascular system via electrocardiographic, echocardiographic and biochemical findings in children. This prospective study included 22 children with CO poisoning and 24 healthy children as a control group. The CO-intoxicated children were evaluated via electrocardiography and echocardiography 1 h after admission to the emergency department and daily until their discharge from the hospital. Blood gasses, complete blood account, troponin I and creatinine kinase-MB(CK-MB) were assessed daily. Tpeak-end (p:0.001), QTc durations (p:0.02), Tpeak-end dispersion (p:0.001) and Tpeak-end/QT ratio (p:0.001) of CO-intoxicated patients were significantly higher than those in the control group. Mitral E duration (p:0.001), mitral E/A ratio (p:0.001) and left ventricle contractile fraction (p:0.023) at admission were significantly lower, and left ventricle myocardial performance index was higher (p:0.001) in the CO poisoning group. Troponin I and CK-MB levels were higher at admission in 6 (27 %) and 4 (18 %) patients, respectively. The heart is the most critical organ in pediatric CO poisoning. These children present subclinical systolic and diastolic left ventricle dysfunction even in mild cases. Although, in children with acute CO-intoxication ventricular repolarization is impaired, it seems to be reversible like other findings.

Silent cerebral emboli following percutaneous closure of atrial septal defect in pediatric patients: a diffusion-weighted MRI study.

PURPOSE: The aim of this prospective study was to investigate the incidence of silent cerebrovascular embolic events associated with percutaneous closure of atrial septal defect (ASD) in pediatric patients. METHODS: A total of 23 consecutive pediatric patients (mean age, 10.4±3.8 years; range, 4-17 years) admitted for transcatheter closure of ASD were recruited in the study. The patients were scanned with a 1.5 Tesla clinical scanner. Two cranial magnetic resonance imaging (MRI) examinations were acquired before the procedure and within 24 hours following the catheterization. MRI included turbo spin-echo fluid-attenuated inversion recovery (FLAIR) sequence and diffusion-weighted imaging technique with single-shot echo-planar spin-echo sequence. The transcatheter closure of ASD was performed by three expert interventional cardiologists. Amplatzer septal occluder device was implemented for the closure of the defect. No contrast medium was administered in the course of the procedure. RESULTS: None of the patients had diffusion restricted cerebral lesions resembling microembolic infarctions on postprocedural MRI. Preprocedural MRI of two patients revealed nonspecific hyperintense white matter lesions on FLAIR images with increased diffusion, which were considered to be older ischemic lesions associated with previously occurred paradoxical embolism. CONCLUSION: The current study suggests that percutaneous closure of the ASD, when performed by experienced hands, may be free of cerebral microembolization in pediatric patients. However, due to the relatively small sample size, further studies with larger patient groups are needed for the validation of our preliminary results.

Cardioprotective effect of metformin against doxorubicin cardiotoxicity in rats.

OBJECTIVE: The clinical use of doxorubicin, which is a strong antineoplastic agent, is limited due to its cardiotoxic side effects. Metformin is a drug with antihyperglycemic effects, and it has been shown to have a cardioprotective effect on left ventricular function in experimental animal models of myocardial ischemia. The present study investigated the cardioprotective effect of metformin in rats with doxorubicin cardiotoxicity. METHODS: Wistar albino rats were used in the study. Forty male, 10-week-old Wistar albino rats were randomly divided four groups. The control group rats were intraperitoneally administered saline solution twice a week, four doses in total. The doxorubicin group rats received doxorubicin (4 mg/kg, twice a week, cumulative dose: 16 mg/kg) intraperitoneally. The metformin group rats received metformin (250 mg/kg/day, every day for 14 days) via gavage. The doxorubicin + metformin group rats received doxorubicin and metformin at the same dose. Left ventricular functions were evaluated by using M-mode echocardiography one day after the last dose of doxorubicin. Heart tissue samples were histopathologically examined. Cardiomyocyte apoptosis was detected using in situ terminal deoxynucleotide transferase assay (TUNEL). Serum brain natriuretic peptide and C-type natriuretic peptide levels were measured. Catalase, superoxide dismutase, glutathione peroxidase, and tumor necrosis factor alpha levels were analyzed in the heart tissue. The assumptions of equality of variances and normal distribution were checked for all variables (Shapiro-Wilk test and Q-Q graphics).To identify intergroup differences, one-way variant analysis or the Kruskal-Wallis test was used. A p<0.05 value was accepted as statistically significant. RESULTS: Our results showed that doxorubicin treatment caused significant deterioration in left ventricular functions by echocardiography, histological heart tissue damage, and increase in cardiomyocyte apoptosis. Doxorubicin + metformin group showed protection in left ventricular function, elimination of histopathologic change, and reduced of cardiomyocyte apoptosis. CONCLUSION: The present study provided evidence that metformin has cardioprotective effects against doxorubicin cardiotoxicity.

Anti-inflammatory role of obestatin in autoimmune myocarditis.

Obestatin is a popular endogeneous peptide, known to have an autoimmune regulatory effect on energy metabolism and the gastrointestinal system. Studies regarding the anti-inflammatory effects of obestatin are scarce. The aim of this study was to show the anti-inflammatory effect of obestatin in an experimental model of autoimmune myocarditis in rats. Experimental autoimmune myocarditis was induced in Lewis rats by immunization with subcutaneous administration of porcine cardiac myosin, twice at 7-day intervals. Intraperitoneal pretreatment with obestatin (50 µg/kg) was started before the induction of myocarditis and continued for 3 weeks. The severity of myocarditis was evidenced by clinical, echocardiographic and histological findings. In addition, by-products of neutrophil activation, lipid peroxidation, inflammatory and anti-inflammatory cytokines were measured in serum. Obestatin significantly ameliorated the clinical and histopathological severity of autoimmune myocarditis. Therapeutic effects of obestatin in myocarditis were associated with reduced lipid peroxidation, suppression of polymorphonuclear leukocyte infiltration and enhancement of glutathione synthesis, inhibition of serum inflammatory and activation of anti-inflammatory cytokines. Histopathologically, the left ventricle was significantly dilated, and its wall thickened, along with widespread lymphocytic and histocytic infiltration. The myocardium was severely infiltrated with relatively large mononuclear cells. These histopathological changes were observed in lesser degrees in obestatin-treated rats. This study demonstrated a novel anti-inflammatory effect of obestatin in an experimental model of autoimmune myocarditis. Consequently, obestatin administration may represent a promising therapeutic approach for myocarditis and dilated cardiomyopathy in the future.

Does N-terminal pro-brain natriuretic peptide correlate with measured shunt fraction in children with septal defects?

BACKGROUND: The aim of this study was to investigate the potential role of N-terminal pro-brain natriuretic peptide in the assessment of shunt severity and invasive haemodynamic parameters in children with atrial septal defects and ventricular septal defects. METHODS: This is a prospective, controlled (n:62), observational study. Correlation analysis was performed between N-terminal pro-brain natriuretic peptide levels and various invasive haemodynamic measurements in 127 children (ventricular septal defect: 64; atrial septal defect: 63). A ratio of pulmonary to systemic blood flow (Qp/Qs⩾1.5) was considered to indicate a significant shunt. RESULTS: Statistically significant relationship was found between the mean N-terminal pro-brain natriuretic peptide values of the patients, with Qp/Qs⩾1.5 in both defect types and control group. For ventricular septal defect, N-terminal pro-brain natriuretic peptide level⩾113.5 pg/ml was associated with high specificity and sensitivity for determining the significant shunt. In addition, the cut-off point for determining the significant shunt for atrial septal defect was 57.9 pg/ml. Significant positive correlation was found between all invasive haemodynamic parameters and N-terminal pro-brain natriuretic peptide levels in patients with ventricular septal defects. Whereas significant positive correlation was found only between mean pulmonary artery pressure, right ventricular end-diastolic pressure, and systemic pressure to pulmonary pressure ratio and N-terminal pro-brain natriuretic peptide levels in patients with atrial septal defects. CONCLUSION: Our study demonstrated that the N-terminal pro-brain natriuretic peptide measurements could be used as a supporting parameter in determining significance of the shunt.

Cerebrovascular complication of infective endocarditis complicated with abdominal trauma.

A 9-year-old boy presented to the emergency department with blunt abdominal trauma. Initial assessment was normal except for abdominal tenderness. On day 3, patient was transferred to the pediatric intensive care unit (PICU) for hemodynamic instability, and persistent fever despite antibiotic therapy. On PICU admission, his body temperature was 40 0C, heart rate was 160/min, respiratory rate was 36/min, blood pressure was 85/40 mmHg, and impaired consciousness was noticed. Complete blood count revealed hemoglobin of 11.5 g/dl, white blood cell count of 22,500/mm3 and platelet count of 145,000/mm3. Serum C-reactive protein and procalcitonin were 139 mg/dl and 8.80 ng/ml, respectively. Renal and liver function test results were normal. Cranial magnetic resonance imaging (MRI) was planned because of impaired consciousness and fever. On cranial MRI, multiple infarct areas were detected in both hemispheres and minimal hemorrhagic focus was found in the left temporal region. Because of the cranial MRI findings and fever echocardiographic examination was planned to exclude infective endocarditis. Transthoracic echocardiography successfully visualized mitral valve prolapse, 14x8 mm mobile vegetation on the atrial side of the posterior leaflet of the mitral valve, and severe mitral regurgitation. The left chambers were dilated. There was no evidence of a perivalvular abscess. On control transthoracic echocardiography, after 6 weeks of parenteral antibiotic treatment, there was no significant reduction of the visible vegetation therefore surgery was planned. Infective endocarditis should be considered in the differential diagnosis of fever of unknown origin. Especially during the early stage of disease, cranial MRI may be more useful to prevent fatal complications for patients with neurologic examination findings.

ADO II in Percutaneous VSD Closure in Pediatric Patients.

OBJECTIVES: Main aim of our study to show that ADO II device can be used for the small ventricular septal defects successfully and safely with low complication rates in pediatric population. BACKGROUND: It is hard to find an ideal device to use for every VSD successfully. If inappropriate device was chosen; complication rate increases, procedure time gets longer that prolongs exposure to ionizing radiation. Therefore interventionalists are in the search for new ideal devices. MATERIAL: Between the dates April 2011-October 2014, 21 VSD closures with ADO-II device. were performed. Twenty patients were included, age ranged between 4 months 18 years. Weight of the patients was between 5-76 kg. RESULTS: VSD diameter ranges between 2-6 mm (3.75 ± 1.25). VSD types were muscular in 2 patients, rest of them were perimembranous type. Most of the perimembranous defects (19/21) were aneursymatic and tunnel shaped. All the cases were successfully closed, no major complications were reported. There was no incidence of left bundle branch block, P-R prolongation, or complete heart block. CONCLUSION: Considering perimembraneous ventricular septal defects as difficult and risky for percutaneous closure because of its proximity to aortic, atrioventricular valves and conduction tissue, we suggest that ADO II device can be safely and effectively used for such defects in particular if an aneurysm formation is present which is also compatible with the literature.

Pulmonary arterial wall thickness in Eisenmenger Syndrome: Prospective, cross-sectional, controlled clinical trial.

BACKGROUND: The aim of current study is to investigate echocardiographic pulmonary artery wall thickness (PAWT) association with angiocardiography, echocardiography, and biochemical findings and to demonstrate its predictive role in morbidity of disease. METHOD: Nineteen patients with Eisenmenger Syndrome (ES) (13 females; a mean age of 12.0 ± 4.1 [min-max 4-17] years) and 24 (16 females; a mean age of 12.1 ± 4.3 [min-max 3-18 years]) healthy subjects as a control group were included in this prospective, cross-sectional, controlled clinical study between December, 2012 and December, 2013. PAWT were measured at the end of systole at the distal site of pulmonary valves at the parasternal short-axis. PAWT was compared with morbidity criteria of the disease such as functional class, pulmonary vascular resistance. RESULTS: PAWT was higher in the patient group (P = 0.005) together with pulmonary arterial diameter (Z-score, P < 0.001), vena cava inferior diameter (P = 0.002), and right ventricular wall thickness (RVWT), while TAPSE was significantly lower (P = 0.002). PAWT was strongly positively correlated to RVWT (r = 0.893, P < 0.001) and moderate negatively correlated to TAPSE (r = 0.597; P < 0.011). CONCLUSION: PAWT can be used as an additional parameter with other echocardiographic parameters in the follow-up of Eisenmenger Syndrome in children.

Is it safe to close ASD with the guidance of transthoraxic echocardiography in pediatric population "ten years' experience of a single center".

OBJECTIVE: Main purpose of this study is to emphasize the usage and safety of transthoracic echocardiography (TTE) in percutaneous atrial septal defect (ASD) closure in appropriate pediatric cases. BACKGROUND: Nowadays, percutaneous closure is preferred as treatment modality for ASD in pediatric age group. METHODS: Between the dates December 2003-August 2013; 340 patients whose ASD were closed included in this study. Physical examination, electrocardiogram, TTE were done before the procedure, at the 24th hour, 1st and 6th month after the procedure. After the 6th month, routine control was done annually. Transesophageal echocardiography (TEE) was performed in 184 cases with large, multifenestrated ASD and when TTE views were poor in quality. We selected the 201 patients whose ASD diameter between 10-20 mm and formed 2 homogeneous groups according to the type of echocardiography used (TEE or TTE) in order to compare the role of echocardiography. RESULTS: The demographic features of patients of 2 groups were similar. There was not any statistically difference between ASD, balloon sizing diameters between the groups. No statistically significant difference in the success, complication, and residual shunt rates was found between the groups. Procedure, fluoroscopy time, and amount were significantly higher in TEE group. When hemodynamic variables except pulmonary blood flow to systemic blood flow (Qp/Qs; right-left atrium mean pressure, pulmonary vascular resistance, and peak-mean pulmonary arterial pressure) were compared, there was not statistically difference. CONCLUSION: TEE is an invasive procedure and requires general anesthesia, therefore, it should not be done routinely in ASD but only in selected cases. If the size and the anatomy of ASD is appropriate, TTE should be preferred primarily in percutaneous ASD closure.

Different clinical presentations of Naxos disease and Carvajal syndrome: Case series from a single tertiary center and review of the literature.

OBJECTIVE: Naxos disease is an autosomal recessive, inherited, cardiocutaneous disorder, characterized by arrhythmogenic right ventricular cardiomyopathy, woolly hair, and palmoplantar keratoderma. Carvajal syndrome is characterized by palmoplantar keratoderma, curly hair, dilated cardiomyopathy, especially on the left ventricle side, and early morbidity. The aim of this study was to evaluate the cutaneous and cardiac findings and genotype-phenotype relationship of six patients diagnosed with Naxos/Carvajal syndrome. METHODS: A retrospective review of six cases diagnosed with Naxos/Carvajal syndrome at our institution from 2002 to 2012 was performed. Demographic data; presenting complaints; cutaneous and cardiac findings; electrocardiography, echocardiography, and genetic analysis results; and treatment data were obtained from patient files. RESULTS: The patient group was composed of 4 males and 2 females, ranging from 1.5 to 13 years, with a mean age 6.4 years. Typical cutaneous and hair findings were present in all patients. Two cases presented with ventricular tachycardia attack, and 2 cases presented with severe heart failure. Two cases had only cutaneous findings without cardiac involvement at diagnosis. An implantable cardioverter-defibrillator was implanted in one case due to ongoing recurrent ventricular tachycardia attacks despite various antiarrhythmic treatments. Three of the 6 patients died during the follow-up. CONCLUSION: For cases with woolly hair and palmoplantar keratoderma, the physician should provide a cardiac assessment, considering Naxos/Carvajal disease associated with cardiomyopathy. When an early diagnosis is made, the life expectancy may be increased by treatment of heart failure and arrhythmias; also, genetic counseling should be performed.