Late-term cardiac magnetic resonance imaging in multisystem inflammatory syndrome in children.

BACKGROUND: Cardiac involvement in multisystem inflammatory syndrome in children may have a spectrum ranging from mild disease to severe heart failure due to fulminant myocarditis. Cardiac involvement usually resolves after clinical recovery. However, the adverse effects of myocarditis on cardiac function after recovery are not fully known. This study aims to investigate cardiac involvement by performing cardiac magnetic resonance imaging (MRI) after the acute and recovery periods. METHODS: 21 patients with clinical and laboratory signs of myocarditis, including left ventricular systolic dysfunction, mitral regurgitation, elevated troponin T, elevated N-terminal pro-B-type natriuretic peptide and electrocardiographic changes, who had given consent for cardiac MRI, underwent cardiac MRI after completion of the acute and recovery phases. RESULTS: When compared to 16 patients with normal cardiac MRI, five patients with cardiac fibrosis on MRI were older, had greater body mass indexes, lower leucocyte counts, lower neutrophil counts, higher blood urea nitrogen levels and higher creatinine levels. Cardiac fibrosis on MRI was located in the posterior right ventricle insertion point and in mid ventricular septum. CONCLUSIONS: Adolescence and obesity appear as risk factors for the development of fibrosis as a late-term sequela of myocarditis. Furthermore, future studies reporting the follow-up data of patients with fibrosis are necessary to predict and manage adverse outcomes.

Perfusion index in newborns with CHD without clinical signs of hypoperfusion and heart failure: comparison with healthy newborns.

INTRODUCTION: Peripheral perfusion index has been proposed as a possible method for detecting circulatory impairment. We aimed to determine the normal range of peripheral perfusion index in healthy newborns and compare it with that of newborns with CHD. METHODS: Right-hand saturation and right-hand peripheral perfusion index levels were recorded, and physical examination and echocardiography were performed in newborns who were 0-28 days old and whom were evaluated in our paediatric cardiology outpatient clinic. The saturation and peripheral perfusion index levels of newborns with normal heart anatomy and function were compared with those of newborns with CHD. RESULTS: Out of 358 newborns (238 mature and 75 premature) enrolled in the study, 39 had CHD (20 mild CHD, 13 moderate CHD, and 6 severe CHD), of which 29 had CHD with left-to-right shunting, 5 had obstructive CHD, and 5 had cyanotic CHD. No newborn had clinical signs of hypoperfusion or heart failure, such as prolonged capillary refill, weakened pulses, or coldness of extremities. Peripheral perfusion index level was median (interquartile range) 1.7 (0.6) in healthy newborns, 1.8 (0.7) in newborns with mild CHD, and 1.8 (0.4) in newborns with moderate and severe CHD, and there was no significant difference between the groups regarding peripheral perfusion index level. CONCLUSION: Peripheral perfusion index remains unchanged in newborns with CHD without the clinical signs of hypoperfusion or heart failure. Larger studies with repeated peripheral perfusion index measurements can determine how valuable this method will be in the follow-up of newborns with CHD.

Lymphocyte Subsets in Mild COVID-19 Pediatric Patients.

OBJECTIVE: The reasons for a high prevalence of asymptomatic or mild coronavirus disease (COVID-19) and rare severe disease in children have been explained by non-immune and immune mechanisms. This study aimed to evaluate the immune system's response to severe acute respiratory syndrome coronavirus 2 by investigating lymphocyte subsets. MATERIALS AND METHODS: This study included 33 coronavirus disease positive children, of whom 12 had mild disease and 21 had an asymptomatic infection as the patient group and 26 ageand gender-matched healthy children as the control group. The demographic information, symptoms, physical examination findings, complete blood count, C-reactive protein (CRP), procalcitonin, and lymphocyte subsets were recorded in all subjects. RESULTS: Leukocyte, lymphocyte, monocyte count, and hemoglobin levels of our pediatric coronavirus disease patients were similar to the control group. Neutrophil was lower in the coronavirus disease cases compared to the control group. CRP and procalcitonin levels of asymptomatic cases were similar to the control group. B cell count, CD8+ T cell count, and CD4/CD8 ratio (dividing the CD4 cell count by the CD8 cell count) ratio were similar in the patient and control groups. Natural killer, T cell, and CD4+ T cell counts were significantly higher in the whole patient group compared to the control group. CONCLUSION: One reason for mild severe acute respiratory syndrome coronavirus 2 infection in children may be an increase in some lymphocyte subsets such as natural killer cells, T cell, and CD4+ T cell. Understanding the answer to the question of why children develop more protective immunity to the virus could be an essential step for developing new treatments.

Aldosterone, Galectin-3, and NTproBNP Levels and their Values as Biomarkers in Infants with Ventricular Septal Defect.

OBJECTIVE: Galectin-3 is a biomarker used to detect cardiac remodelling and fibrosis. It could also potentially be a biomarker for developing new treatments. Aldosterone and galectin-3 levels and their relationship to N-terminal pro-brain natriuretic peptide (NT-proBNP) and left ventricular dilatation have not yet been studied in infants with ventricular septal defect (VSD). In this study, we aimed to investigate the biomarker feature of galectin-3 in infants with VSD. METHODS: Aldosterone, galectin-3, and NT-ProBNP levels were quantified and left ventricular diameters were measured with M mode echocardiography in infants with isolated VSD who had received heart failure treatment. The results were compared with those of healthy children of similar age and gender. RESULTS: This study included 22 infants (13 girls, nine boys) with VSD who formed the patient group and 22 healthy infants (13 girls, nine boys) who formed the control group. There was a significant difference between the two groups regarding the median left ventricular end-dia stolic diameter and the median left ventricular end-systole diameter. The patient and control groups had no significant difference with respect to aldosterone levels (median values 43.5 pg/mL vs 41.3 pg/mL, respectively) (P = .851), although there was a significant difference with regard to galectin-3 levels (median values: 4 vs 2.5 ng/mL, respectively) (P = .015) and NT-proBNP levels (median values: 204.3 vs 94.2 pg/mL, respectively) (P = .003). CONCLUSION: Galectin-3 increases independent of left ventricular dilatation and may have a biomarker value with similar strength as NT-proBNP in infants with VSD.

Effects of azithromycin on ventricular repolarization in children with COVID-19.

Introduction: Azithromycin is used to treat pediatric COVID-19 patients. It can also prolong the QT interval in adults. This study assessed the effects of azithromycin on ventricular repolarization in children with COVID-19. Method: The study prospectively enrolled children with COVID-19 who received azithromycin between July and August 2020. An electrocardiogram was performed before, one, three, and five days post-treatment. Using ImageJ®, the following parameters were measured: QT max, QT min, Tp-e max, and Tp-e min. The parameters QTc max, QTc min, Tp-ec max, Tp-ec min, QTcd, Tp-ecd, and the QTc/Tp-ec ratio were calculated using Bazett's formula. Results: The study included 105 pediatric patients (mean age 9.8±5.3 years). The pretreatment heart rate was higher than after treatment (before 92 [79-108]/min vs. Day 1 82 [69-108)]/min vs. Day 3 80 [68-92.2]/min vs. Day 5 81 [70-92]/min; p=0.05). Conclusion: Azithromycin does not affect the ventricular repolarization parameters on ECG in pediatric COVID-19 cases.

Introdução: A azitromicina (AZ) é utilizada no tratamento da COVID-19 em pediatria. Como este fármaco pode prolongar o intervalo QT nos adultos, este estudo avaliou os efeitos da AZ na repolarização ventricular de crianças com COVID-19. Método: Este estudo prospetivo incluiu crianças com COVID-19 que foram tratadas com AZ em julho-agosto 2020. Foi efetuado um eletrocardiograma (ECG) antes e um, 3 e 5 dias após o tratamento. Utilizando ImageJ ®, foram medidos os parâmetros seguintes: QT max, QT min, Tp-e max, e Tp-e min. Os parâmetros QTc min, Tp-ec max, Tp-ec min, QTcd, Tp-ecd e QTc/Tp-ec ratio foram calculados utilizando a fórmula Bazett. Resultados: O estudo incluiu 105 doentes pediátricos (idade média 9,8±5,3 anos). A frequência cardíaca no pré-tratamento foi mais elevada do que após o tratamento (antes 92 [79­108]/min versus dia 1 82 [69­108)]/min versus dia 3 80 [68­92,2]/min versus dia 5 81 [70­92]/min; p=0,05). Conclusão: A AZ não afeta os parâmetros de repolarização ventricular no ECG nos casos pediátricos da COVID-19.

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.

Effect of fluoxetine and psychotherapy on heart rate variability in children and adolescents diagnosed with panic disorder: 24-hour Holter monitoring study before and after treatment.

OBJECTIVE: Panic disorder (PD) is now recognized as a common and important problem in children, and particularly adolescents, and one that can negatively affect daily well-being and educational performance. The aim of this study was to investigate the relationship between heart rate variability (HRV) and the severity of symptoms before and after treatment with psychotherapy and fluoxetine. METHODS: The PD study group consisted of 23 children diagnosed with PD and the healthy control (HC) group comprised 27 healthy children. Panic-anxiety symptoms were measured using 2 assessments performed before and after treatment. HRV was evaluated with a 24-hour Holter examination. RESULTS: According to the analysis of the 24-hour, all-day Holter device recordings, the high frequency (HF) and parasympathetic (%) scores in the PD group were lower than those of the HC group (p<0.05). The low frequency (LF)/HF ratio and sympathetic (%) scores in the PD group were higher than those of the HC group (p<0.05). The analysis of daytime readings indicated that the HF values of the PD group were lower than those of the HC group (p<0.05), while the very LF/HF ratio and LF/HF ratio were higher than those of the HC group (p<0.05). Analysis of nighttime Holter results revealed that the rMSSD, pNN50, and HF readings of the PD group were lower than those of the HC group (p<0.05), while the LF/HF ratio in PD patients was higher than that seen in the HC group (p<0.05). CONCLUSION: In children and adolescents with PD, increased sympathetic activity can cause changes in some HRV parameters. Some of these changes may return to normal with treatment.

Arrhythmia in thiamine responsive megaloblastic anemia syndrome.

Argun M, Baykan A, Hatipoglu N, Akin L, Sahin Y, Narin N, Kurtoglu S. Arrhythmia in thiamine responsive megaloblastic anemia syndrome. Turk J Pediatr 2018; 60: 348-351. Thiamine responsive megaloblastic anemia syndrome (TRMAS) is a rare, autosomal recessive disorder characterized by megaloblastic anemia, diabetes mellitus, and progressive sensorineural deafness. Mutations in the SLC19A2 gene that codes for thiamine transporter 1 protein cause TRMAS, and more than 30 homozygous mutations have been identified to date. Congenital heart diseases and arrhythmias have been reported in few patients. We present cardiac features of five patients with TRMAS. Five patients had macrocytic anemia, diabetes mellitus, and sensorineural deafness. Two siblings had also optic atrophy. SLC19A2 gene mutation was shown in all patients. Two patients developed supraventricular tachycardia during an episode of diabetic ketoacidosis. Five patients had absent P waves on baseline electrocardiography, and one patient had additional low QRS voltage. None of the patients had structural heart disease. Discontinuation of thiamine treatment appears to trigger supraventricular tachycardia episodes at puberty.

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.

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.

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.

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.

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.