Transcatheter Closure of Patent Ductus Arteriosus in Children with the Occlutech Duct Occluder.

The aim of this study was to evaluate the feasibility, efficacy and safety of transcatheter closure of patent ductus arteriosus (PDA) with the Occlutech duct occluder (ODO) in children. We reviewed the clinical records of 71 patients who underwent percutaneous closure of PDA with an ODO between September 2014 and August 2016. The Occlutech duct occluder was applied to 71 patients during the study period (September 2014-August 2016), and the results were analyzed in this study. Forty-two of the patients were female and 29 male. The median age was 20.5 months (range, 6-194 months) and median weight was 16 kg (range, 6-68 kg). The PDA was classified as type A in 54 patients (76.1%), type E in 14 (19.7%), type C in 2 (2.8%) and type B in 1 (1.4%) based on the Krichenko classification. A standard ODO device was used for the transcatheter closure procedure in 66 patients and the long-shank ODO device in 5. In the echocardiographic measurement of PDA, the median smallest diameter was 2.7 mm (range, 1.5-7.0 mm), and in the angiographic measurement, the median smallest diameter was 2.5 mm (range, 1.5-6.5 mm). All 71 patients underwent successful PDA closure with the ODO. Angiography following the procedure showed complete closure in 47 patients (66.2%), mild residual shunt in 13 patients (18.3%) and a trivial shunt in 11 patients (15.5%). Color flow Doppler echocardiogpaphy at 24 h post-implantation showed that complete closure was achieved in 65 patients (91.5%), and 6 patients (8.5%) had mild residual shunt. All patients (100%) had complete closure at 30 days of follow-up. The results of this study showed that the Occlutech PDA occluder device is safe and effective in the closure of PDA. As the pulmonary artery side of the device is wider than the aortic side, protrusion toward the aortic side and embolization are prevented, but there is residual shunt in the early period, although this residual shunt disappeared after a few months.

Short-Term Outcomes of Patent Ductus Arteriosus Closure With New Occlutech® Duct Occluder: A Multicenter Study.

AIM: Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO. METHODS: We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA. RESULTS: The median patient age was 2.5 years (6 months-35 years), and median PDA diameter was 2.5 mm (1.2-11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24-48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months. CONCLUSION: Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325-331).

Does surgically induced right bundle branch block really effect ventricular function in children after ventricular septal defect closure?

In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p < 0.05). When compared to controls, operated group had statistically lower [corrected] tricuspid annular plane systolic excursion (p < 0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values <0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

Increased P-wave and QT dispersions necessitate long-term follow-up evaluation of Down syndrome patients with congenitally normal hearts.

Reports state that Down syndrome (DS) patients with congenitally normal hearts might experience the development of cardiac abnormalities such as cardiac autonomic dysfunction, valvular lesions, bradycardia, and atrioventricular block. However, the presence of any difference in terms of P-wave dispersion (PWd) and QT dispersion (QTd) was not evaluated previously. This study prospectively investigated 100 DS patients with structurally normal hearts and 100 age- and sex-matched healthy control subjects. Standard 12-lead electrocardiograms were used to assess and compare P-wave and QT durations together with PWd and QTd. The median age of the DS patients and control subjects was 48 months. Heart rates and P-wave and QT dispersions were significantly greater in the DS group than in the control group (113 ± 22.9 vs 98.8 ± 16.6 bpm, p < 0.001; 31.3 ± 9.5 vs 24 ± 8.6 ms, p < 0.001; and 46.6 ± 15.9 vs 26 ± 9.1 ms, p < 0.001, respectively). A positive correlation was found between PWd and age in the DS patients (p < 0.05; r = 0.2). All children with DS should be followed up carefully with electrocardiography in terms of increased P-wave and QT dispersions even in the absence of concomitant congenital heart disease for management of susceptibility to arryhthmias.

Screening of families of patients with left-sided cardiovascular anomalies.

BACKGROUND: There is increasing evidence of clustering of certain cardiac anomalies in some families. The frequency and echocardiographic features of such anomalies among the relatives of patients with bicuspid aortic valve (BAV) or other left-sided cardiovascular anomalies (LSCA) were evaluated. METHODS: The patients with BAV or any other LSCA and their relatives were enrolled in the study. They underwent an echocardiographic examination. The probands were assessed in three groups: BAV, BAV + coarctation of aorta (CoA), and other LSCA. Their relatives were also grouped and evaluated accordingly. The echocardiographic measurements were standardized by Z-scores. RESULTS: Eighty-six probands and 261 relatives were evaluated. The numbers of the patients in the BAV, BAV + CoA, and other LSCA group were 52, 14, and 20, respectively. Any LSCA was determined in 17 (6.5%) of the relatives. Thirteen (5%) had aortic dilatation and the remainder (1.5%) had BAV. Accordingly, BAV incidence among relatives of patients with BAV was found to be 1.9%. A second individual with an LSCA was observed in 12.8% of 86 families investigated. The frequencies of aortic stenosis, aortic regurgitation, aortic stenosis + aortic regurgitation, and aortic dilatation in the patients with BAV were found to be 37.9%, 53%, 25.8% and 48.5%, respectively. In contrast to previous reports, no enlargement was observed in the pulmonary arteries of BAV patients. CONCLUSIONS: BAV and other LSCA are of clinical significance. Because the clustering of LSCA in some families is observed, we recommend echocardiographic screening of those relatives. If this is not possible, at least it should be achieved for BAV patients.

Rheumatic silent carditis: echocardiographic diagnosis and prognosis of long-term follow up.

BACKGROUND: Rheumatic fever and rheumatic heart disease continue to be an important public health problem in developing countries. Doppler echocardiography is now widely used for early detection and recurrence of clinical evident carditis (CC) and silent (subclinical) carditis (SC). The aim of this study was to determine the frequency of SC and to compare clinical and echocardiographic features of CC and SC. METHODS: A total of 156 consecutive patients diagnosed with acute rheumatic fever were included in the study. The patients without clinical evidence but with echocardiographic findings of carditis were diagnosed as having SC. RESULTS: Acute rheumatic fever was diagnosed in 156 patients, and 103 of these (66%) had carditis. The prevalence of SC was 28.2% among these 103 patients. Seventy-four of the patients with carditis were followed up for >1 year, and 20 of those had SC. Valvular regurgitation disappeared completely in 18.5% and improved in 45.5% of the CC patients. The recovery and improvement rates in the SC group were 15% and 30%, respectively. CONCLUSION: It is suggested that Doppler echocardiography be performed in all patients with suspected acute rheumatic fever for early detection of SC. Echocardiography should be used as a diagnostic criterion in order not to miss a diagnosis of SC.

Coexistence of left ventricular noncompaction and double-orifice mitral valve in a patient with congestive heart failure.

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy. It usually presents with ventricular dysfunction, thromboembolic events and arrhythmias. An asymptomatic clinical course is also possible. LVNC is frequently associated with other congenital heart diseases including heart valve abnormalities. The coexistence of LVNC with double-orifice mitral valve was observed rarely. The presence of such coexistence in a patient presented with heart failure in the newborn period is reported herein because of its rarity.

Transcatheter closure of extracardiac fontan fenestration in a cyanotic patient.

Fontan operation is the connection of the systemic venous return to the pulmonary arteries, performed in patients with single ventricle physiology. The Fontan circuit is commonly fenestrated because of early postoperative risks such as high systemic venous pressure and low cardiac output. As it causes progressive cyanosis and increased risk of paradoxical embolism in the follow-up period, occlusion of these fenestrations is generally suggested. Successful closure of extracardiac Fontan fenestration with atrial septal occluder in a 10-year-old girl is reported herein. To our knowledge, this is the first such report from Turkey.

Factors affecting mortality in children with dilated cardiomyopathy.

Sabaz MN, Akin A, Bilici M, Demir F, Türe M, Balik H. Factors affecting mortality in children with dilated cardiomyopathy. Turk J Pediatr 2019; 61: 485-492. Dilated cardiomyopathy (DCMP) is a heart disease with high mortality rates that is often seen in children. Genetic and infectious reasons are primary in the etiology. The aim of this study was to investigate the etiology of DCMP and the parameters predicting mortality. A retrospective examination was made of 37 patients diagnosed with DCMP between January 2012 and October 2016. Data were recorded from the patient files of age, gender, complaints on presentation, findings of the physical examination, laboratory test results, echocardiography and electrocardiography findings at the time of diagnosis. These parameters were then compared between the surviving and nonsurviving patients. The patients comprised 21 males with a mean age of 27.50±50 months. Diagnosis was made at the age of < 12 months in 67.6% patients. Within mean 8 months of diagnosis, 16.2% of the patients were lost to mortality and 83.8% of the patients survived. In 83.3% of the non-surviving patients and in 29% of the surviving patients, sinus tachycardia was present at the time of diagnosis (p=0.023). Corrected QT (QTc) at the time of diagnosis was longer in the non-surviving patients (p=0.007). On ECG, the rate of ST-T wave change was higher in the non-surviving patients (80% vs. 17.8%, p=0.012). In conclusion, a significant proportion of the patients were diagnosed below the age of one year. In the non-surviving patients, as sinus tachycardia and ischaemic changes on ECG were seen more often and the QTc was longer, these findings could be considered to be predictors of mortality.

Complete atrioventricular block associated with clozapine intoxication: case report.

Türe M, Bilici M, Akin A, Demir F, Balik H, Darakçi SM. Complete atrioventricular block associated with clozapine intoxication: case report. Turk J Pediatr 2019; 61: 618-621. Clozapine is one of the atypical anti-psychotic drugs used in the treatment of resistant schizophrenia. Although cardiac side-effects are rare, it has been reported that there may be development of myocarditis, dilated cardiomyopathy, postural orthostatic hypotension and prolonged QT duration. Complete atrioventricular (AV) block is characterized by the inability to transmit all of the atrial signal to the ventricles. Causes may be congenital, idiopathic or acquired which are associated with surgery, infection, or muscle disease. AV block is extremely serious and permanent pacemaker insertion is usually necessary for all patients. Complete AV block may develop due to clozapine intoxication through increase in vagal tonus, sinoatrial node (SN) and the inhibition of atrioventricular node signalling. The case presented here is of a 15-year old female patient who developed AV total cardiac block associated with the taking of clozapine in a suicide attempt.

Prolonged QT dispersion is associated with pediatric syncope.

Bilici M, Fidanci-Dedeoglu Z, Demir F, Akin A, Türe M, Balik H, Tan I, Ertugrul S. Prolonged QT dispersion is associated with pediatric syncope. Turk J Pediatr 2019; 61: 85-91. We aimed to find out whether QT dispersion can be used as a diagnostic marker in terms of syncope type, and recurrence risk. Fifty-two patients (28 male, 53.8%) admitted to the pediatric cardiology clinic with syncope were compared with 50 healthy controls (28 male, 56%; mean age: 13.8±2.3 years, range: 11-17 years) for QT dispersion (QTd) and other electrocardiographic findings. Gender, age, echocardiography, blood pressure while standing and sitting, electrolyte levels, liver and kidney function tests, and QTd and QTcd (calculated with corrected QT: QTc) in electrocardiography (ECG) of patients were all evaluated. Patients` ages varied between 7-17 years and the mean age at first syncope was 13.9±2.4 years. Mean follow-up duration of the patients was 10±5 months (range: 5-18 months). Mean number of syncope attacks was 2.8±2.2. QTd (72±46 ms vs. 34±14 ms) and QTcd (77±45 ms vs. 33±14 ms) values of the patients were significantly longer, compared to control group (p < 0.001). The age, gender, QTd, and QTcd values did not differ between the subtypes of syncope based on pathogenetic mechanism. Additionally, these parameters did not differ in terms of the number of syncope recurrence and tilt test. Patients` complaints reduced after cardiologic evaluation and advice regarding prevention of syncope. We think that in follow-up period, education and preventive precautions that can be taken in the daily life must be emphasized, and drug treatment can be started in unresponsive cases.

Percutaneous retrieval of umbilical vein catheter fragment in an infant two months after embolization.

Akin A, Bilici M, Demir F, Yilmazer MM, Ipek MS, Kara H. Percutaneous retrieval of umbilical vein catheter fragment in an infant two months after embolization. Turk J Pediatr 2018; 60: 191-193. Umbilical vein catheterization is frequently preferred and a safe route of venous access especially in newborns. However, some cases with breaking and embolization of those catheters have been rarely reported. Herein we present a two-and-a-half-month-old infant being catheterized within first postnatal week and diagnosed to have embolization of the catheter fragment to conjunction of hepatic vein and right atrium. Percutaneous withdrawal of broken catheter was achieved despite several months after the embolization took place. We suggest that transcatheter removal of catheter fragment embolizations may be safe even in late diagnosis cases.

Prediction of premature atherosclerosis by endothelial dysfunction and increased intima-media thickness in glycogen storage disease types Ia and III.

The aim of this study was to investigate the endothelial dysfunction (ED) and carotid intima-media thickness (IMT) in patients with glycogen storage disease (GSD) types Ia and III. In 22 patients with GSD (13, type Ia; 9, type III) and 18 healthy subjects, endothelial functions of the brachial artery and carotid IMT were evaluated by high-resolution ultrasound. Endothelial-dependent dilatation (EDD) was assessed by establishing reactive hyperemia. EDD and carotid IMTs were compared between the three groups. Mean cholesterol level was slightly higher in GSD type III patients but the difference was not significant. Triglyceride levels and cholesterol to high density lipoprotein (HDL) ratio were significantly higher in GSD type Ia patients. EDD was significantly impaired in GSD type Ia (13% +/- 8%, P = .001) and type III (15% +/- 6%, P = .005) patients when compared with the healthy subjects (22% +/- 4%). The carotid IMT was significantly higher in both GSD type Ia (0.23 +/- 0.03 mm, P =.005) and type III (0.26 +/- 0.05 mm, P = .001) patients when compared with the healthy subjects (0.20 +/- 0.02 mm). Both GSD type Ia and type III patients show significant ED and increased IMT, which are predictors of atherosclerosis.

CagA positivity and its association with gastroduodenal disease in Turkish children undergoing endoscopic investigation.

BACKGROUND: Cytotoxin-associated gene A (CagA) product is a bacterial virulence factor contributing to the pathogenicity of Helicobacter pylori (HP) infection in humans. Host factors, which vary in different countries, interact with bacterial factors to determine the disease state. Our objective was to investigate the frequency of CagA-positive HP strains and evaluate the contribution of CagA positivity to symptoms and development of mucosal lesions in HP-infected Turkish children. METHODS: We conducted a prospective clinical trial in 240 consecutive Turkish children undergoing endoscopy (110 girls, 130 boys; mean age, 8.7 +/- 4.3 years). HP infection was diagnosed on the basis of a positive rapid urease test and histology of the mucosal specimens. HP IgG and CagA IgG antibodies were measured by enzyme-linked immunosorbent assay in HP-positive children. RESULTS: The HP positivity rate was 50.4% in our study group (51 girls, 70 boys; mean age, 9.9 +/- 3.9 years). CagA was positive in 74.4%. HP infection was less common in children with vomiting (25.9%, P < 0.05). CagA positivity was not associated with any clinical symptom. HP positivity was higher in children with duodenal ulcer (80% vs. 49.1%, P = 0.05); while CagA positivity was similar. Antral nodularity was strongly associated with HP positivity and CagA positivity (30.6% vs. 3.4% and 36.7% vs. 12.9%, respectively, P < 0.05). A negative association between CagA positivity and esophagitis was observed (20% vs. 76.7%, P < 0.05). CONCLUSIONS: CagA positivity is common in HP-infected Turkish children. Esophageal lesions are less common in children infected with CagA-positive strains. Although HP is associated with duodenal ulcer disease, CagA positivity does not seem to contribute to development of ulcers in children in our series.

Brucella Infection Associated with Complete Atrioventricular Block.

BACKGROUND: The clinical spectrum of Brucella infection is quite diverse and characterized by multi-system involvement. Patients present with myocarditis, endocarditis, or pericarditis. Infective endocarditis is the most common cardiovascular complication in patients with brucellosis. Although conduction abnormalities are seen in cases with endocarditis, they are reported very rarely in the setting of cardiac Brucella infection. CASE REPORT: An eight and a half-year-old male patient was referred to our clinic due to inadequate response to cotrimaxazole plus streptomycin treatment at the 15th day of admission. Although local hospital records on the patient showed a heart rate of 80 bpm, we determined a heart rate of 46 bpm. The electrocardiogram showed complete atrioventricular (AV) block. The average heart rate was determined as 48 bpm with 24-hour Holter electrocardiogram (ECG) monitoring. The echocardiographic examination showed normal-sized heart chambers and the absence of valvular involvement. An agglutination test for brucellosis was found to be positive with a titer of 1/320. High fever, arthralgia, and splenomegaly regressed following doxycycline plus rifampicin therapy, but there was no improvement in the AV block. A permanent pacemaker was implanted because of the detection of an average heart rate of 48 bpm. CONCLUSION: Because cardiac failure and rhythm abnormalities are reported in the course of Brucella infection and may be associated with significant outcomes, cases with brucellosis should be evaluated carefully in terms of cardiac involvement. This report aims to draw attention to complete AV block as an extremely rare complication of Brucella infection.

Echocardiographic diagnosis of double-chambered left ventricle.

Double-chambered left ventricle (DCLV) is a rare congenital abnormality in which the left ventricle is divided into two separate chambers by a septum or anomalous muscular structure. The chambers are observed mostly parallel to each other without stenosis, and less frequently in a superior-inferior arrangement. An asymptomatic girl is presented here who was diagnosed with DCLV on echocardiographic examination that was performed for the evaluation of cardiac murmur detected by a pediatrician. She has been followed up without treatment.

Cardiac thrombus developing after an accidental high-voltage electric shock in a child.

Electric shock is a condition that may affect various organ systems and potentially cause death. Cardiac findings vary from asymptomatic mild injury to fatal myocardial involvement. Herein we present a five-year-old boy with a cardiac thrombus developing after an accidental electrical shock. Cardiac arrhythmias and evidence of ischemia have been reported after electric shock; we were, however, unable to identify an earlier case report of intracardiac thrombosis related to electric shock. Findings such as elevated cardiac enzymes and systolic dysfunction, which indicate myocardial damage following electric shock, were present in our patient. We think that the cardiac thrombus might have resulted from the myocardial damage and the slowed intracardiac blood flow related to systolic dysfunction. As the thrombus was thought to have been formed through known mechanisms, it was treated traditionally. However, further data regarding the etiology and management of such thrombi is needed.

ST-segment elevation following lightning strike: case report and review of the literature.

Lightning strikes may cause injury to the heart, ranging from slight electrocardiographic changes to fatal damage. As heart injury is the most important cause of mortality in these patients, cardiac monitoring is crucial. Even though various ECG changes have been reported, published data on pathologic ST-segment changes is scarce. Herein, we present a seven-year old patient with ST-segment elevation following a lightning strike. There is not sufficient data regarding lightning-related myocardial ischemia. However, because of the similar effects of lightning strikes and high-voltage electric shocks, we believe myocardial injury related to lightning may be managed in the same manner as is cardiac involvement associated with electric shock.

Epidemiologic Features of Type 1 Diabetic Patients between 0 and 18 Years of Age in Istanbul City.

OBJECTIVE: To evaluate the epidemiologic, clinical and laboratory characteristics of a group of children with type 1 diabetes mellitus (T1DM) living in a Turkish city. METHODS: The records of 395 (boys/girls: 199/196) children with newly diagnosed T1DM hospitalized in the years 1985-2004 were evaluated retrospectively. The data were assessed by gender and age subgroups (≤5, 6-10 and ≥11 years). RESULTS: Mean age of children at diagnosis was 8.1±4.1 years. At T1DM onset, the number of children ≤5, between 6-10 and ≥11 years old was 110 (27.9%), 147 (37.2%) and 138 (34.9%), respectively. The patients were mostly diagnosed at ages 6-8 years (24.1%), followed by cases aged 3-5 years (22.0%). Polyuria and polydipsia were the most common symptoms (94.7%). Mean duration of symptoms was 21.5±18.6 days. Although the patients mostly presented in autumn (30.7%), no season-related significant differences were found. The frequency of ketoacidosis was relatively high (48.5%). When compared to boys, the girls experienced higher rates of ketoacidosis (55.1% vs. 41.7%, p=0.042); had a higher frequency of anti-thyroid peroxidase antibodies (11.7% vs. 4.2%, p=0.049) and higher insulin requirement (0.89±0.41 vs. 0.77±0.36 IU/kg, p=0.005). Cases with a family history of T1DM were more likely to have anti-endomysial antibodies (42.9% vs. 8.1%, p=0.027) and higher initial blood glucose levels (510.5±145.0 vs. 436.1±156.5 mg/dL, p=0.005). CONCLUSION: The findings possibly indicate a decreasing age of T1DM onset. The high frequency of ketoacidosis at presentation is noteworthy. Girls had higher rates of ketoacidosis, higher frequency of anti-thyroid antibodies and higher insulin requirements as compared to boys. Patients with a family history of T1DM had higher initial glucose levels and higher frequency of anti-endomysial antibodies.