Sacubitril/Valsartan in Pediatric Heart Failure (PANORAMA-HF): A Randomized, Multicenter, Double-Blind Trial.

BACKGROUND: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD). METHODS: Children with HF attributable to LVSD were randomized to sacubitril/valsartan versus enalapril to assess the efficacy and safety of sacubitril/valsartan at 52 weeks of follow-up. The primary end point of the study was to determine whether sacubitril/valsartan was superior to enalapril for the treatment of pediatric patients with HF attributable to systemic LVSD, assessed using a primary global rank end point consisting of ranking patients from worst to best on the basis of clinical events such as death, listing for urgent heart transplant, mechanical life support requirement, worsening HF, New York Heart Association (NYHA)/Ross class, Patient Global Impression of Severity (PGIS), and Pediatric Quality of Life Inventory physical functioning domain. The change from baseline to 52 weeks in NT-proBNP (N-terminal pro-B-type natriuretic peptide) was an exploratory end point. RESULTS: A total of 375 children (mean age, 8.1±5.6 years; 52% female) were randomized to sacubitril/valsartan (n=187) or enalapril (n=188). At week 52, no significant difference was observed between the 2 treatment arms in the global rank end point (Mann-Whitney probability, 0.52 [95% CI, 0.47-0.58]; Mann-Whitney odds, 0.91 [95% CI, 0.72-1.14]; P=0.42). At week 52, clinically meaningful reductions were observed in both treatment arms in NYHA/Ross, PGIS, Patient Global Impression of Change, and NT-proBNP, without significant differences between groups. Adverse events were similar between treatment arms (incidence: sacubitril/valsartan, 88.8%; enalapril, 87.8%), and the safety profile of sacubitril/valsartan was acceptable in children. CONCLUSIONS: In this study, sacubitril/valsartan did not show superiority over enalapril in the treatment of children with HF attributable to systemic LVSD using the prespecified global rank end point. However, both treatment arms showed clinically meaningful improvements over 52 weeks. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02678312.

The evaluation of the burden of multisystem inflammatory syndrome in children on health economics.

Objectives: This study aimed to evaluate the diagnostic tests and treatments applied in patients with multisystem inflammatory syndrome in children (MIS-C) and to determine the effect of the disease on health costs. Patients and methods: This retrospective cohort study included 59 MIS-C patients (40 males, 19 females; mean age: 7.7±4.2 years; range, 4 months to 16.5 years) who were admitted and treated between April 1, 2020, and November 1, 2021. Demographic and clinical features with hospital costs and length of stay were retrospectively reviewed from the medical files and computerized system of the hospital. Direct medical care costs of items were calculated with the hospital perspective using a combination of microcosting technique (resource-based accounting method) and hospital list data. Cases were classified as mild, moderate, or severe, and the patients were divided into two groups: the mild group and the moderate-severe group. Classification was determined by the vasoactive inotropic score (VIS), degree of respiratory support, and evidence of organ damage. Results: The mean age of the cases in the mild group was 6.5±3.7 years, and the mean age of the cases in the moderate-severe group was 9.2±4.3 years. Of 59 patients, 19 (32.2%) were followed up in the pediatric intensive care unit. The median duration of hospitalization in the hospital was 8 (interquartile range: 7-12) days. The total cost of the patients hospitalized with the diagnosis of MIS-C during the study period was 849,242.93$, and the mean cost per patient was 14,393.94±9,631.92$. In the distribution of the total cost of hospitalization according to expenses, the highest rate was pharmacy and blood products (51.99%) and IVIG costs (43.99%). While the mean total cost per person was 13,682.87±8,799.63$ in mild cases, it was 16,433.82±9,440.02$ in moderate-severe cases, and no statistically significant relationship was found between the two groups (p>0.05). There was no difference in the mean cost per patient between the cases with and without heart, lung, kidney, or neurologic involvement and advanced respiratory support (p>0.05). There was a strong positive correlation between the total costs and age (r=0.883, n=59, p<0.0001), with increased amount of costs with increased age. Conclusion: In the study, no statistically significant correlation was found between the total cost of per person in the mild group and the moderate-severe group (p>0.05). This finding may be due to the wide use of IVIG in MIS-C treatment, in addition to low transfer rates to pediatric intensive care units due to high-flow nasal cannula usage.

Leukotriene B4 levels in CHD-associated paediatric pulmonary hypertension.

BACKGROUND: The aim of this study is to evaluate the role of leukotriene B4, an inflammatory mediator, in the development of pulmonary hypertension in paediatric patients with CHD with left-right shunt. METHODS: The study included forty patients with CHD with left-right shunts. Based on haemodynamic data obtained from cardiac diagnostic catheterisation, 25 patients who met the criteria for pulmonary arterial hypertension were included in the patient group. The control group comprised 15 patients who did not meet the criteria. The standard cardiac haemodynamic study was conducted. Leukotriene B4 levels were assessed in blood samples taken from both pulmonary arteries and peripheral veins. RESULTS: The median age of patients with pulmonary arterial hypertension was 10 months (range: 3-168), while the median age of the control group was 50 months (range: 3-194). In the pulmonary hypertension group, the median pulmonary artery systolic/diastolic/mean pressures were 38/18/24 mmHg, compared to 26/10/18 mmHg in the control group. Leukotriene B4 levels in pulmonary artery blood samples were significantly higher in the pulmonary arterial hypertension group compared to the controls (p < 0.05). Peripheral leukotriene B4 levels were also elevated in the pulmonary arterial hypertension group in comparison to the control group, though the difference was not statistically significant. CONCLUSION: The discovery of elevated leukotriene B4 levels in pulmonary artery samples from paediatric patients with pulmonary arterial hypertension secondary to CHD with left-to-right shunt suggests that local inflammation may have a pathological role in the development of pulmonary arterial hypertension.

Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Readmission reasons of pediatric patients diagnosed with multisystem inflammatory syndrome after discharge.

Objectives: There is no clear information in the literature about causes of reactivation of multisystem inflammatory syndrome in children (MIS-C) or indications for readmissions for MIS-C after discharge; as a result, the conditions that may develop after infection in children with MIS-C were discussed, and the reasons for hospitalization were screened. Patients and methods: This single-center retrospective study was conducted with 95 patients (65 males, 30 females; mean age: 92.8±55.5 months; range, 5 to 17 months) between November 11, 2020, and December 30, 2021. Children who were rehospitalized in the study center after their discharge with the diagnosis of MIS-C were included in the study, and the indications for readmissions were evaluated. Results: During the study period, six (6.3%) patients (4 males, 2 females; median age: 114.5 months [interquartile range: 122 months]) had to be rehospitalized. Four of these patients had an underlying disease, while the other two were previously healthy children. Fever was the most common reason for readmissions in half of the patients, while the remaining patients were readmitted with the indications of myocarditis, pneumonia, and posttraumatic pain syndrome. Conclusion: Although no evidence for the reactivation of MIS-C was detected in patients in the literature, it should also be emphasized that close follow-up of these patients is a must, considering possible cardiac complications.

Turkish validity and reliability study of Pediatric Quality of Life Inventory (PedsQL) 3.0 cardiac module for 8-18 years old children and parents.

BACKGROUND: This study aimed to evaluate the Turkish validity and reliability of the 8-12- and 13-18-years child and parent forms of the Pediatric Quality of Life Inventory (PedSQL) 3.0 Cardiac Module. METHODS: This methodological study was conducted in children (8-18 years old) with cardiac disease and their parents. The PedsQL 4.0 were also used to collect data for the parallel form method. Pearson correlation coefficients between the scale and its sub-dimensions were evaluated for construct validity. For construct validity, mean scores of children with cardiac disease and healthy children and their parents were compared. Cronbach's alpha coefficient was calculated to evaluate the internal consistency of the items. RESULTS: In this study, 136 children aged 8-12 years and 135 adolescents aged 13-18 years with cardiac diagnosis and their parents participated. The Cronbach alpha coefficients were found to be above 0.80 for all sub-dimensions and the total scale. Correlations between PedsQL 3.0 Cardiac module and PedsQL 4.0 scores were moderate to highly significant. A significant difference was found between the mean scores of the children with cardiac disease and healthy child and parent forms (p < .001). CONCLUSION: PedsQL 3.0 Cardiac Module's 8-12- and 13-18-year child and parent forms are valid and reliable for the Turkish language. APPLICATION TO PRACTICE: It is important to evaluate the PedsQL cardiac module, which is a very comprehensive scale, with accurate measurements to increase the general health level and life satisfaction of these patient groups.

Evaluation of Arrhythmia Prevalence, Management, and Risk Factors in Patients with Transcatheter and Surgically Closed Secundum Atrial Septal Defects.

OBJECTIVE: Atrial septal defect (ASD) accounts for 6-10% of all congenital heart disorders. Secundum ASD closure can be performed surgically or percutaneously. We aimed to identify the various arrhythmias that occur before, during, and after the procedure and evaluate their management. METHODS: The study included a total of 427 patients aged 0-18 years who underwent transcatheter or surgical closure of isolated secundum ASD between January 2008 and January 2020. Postoperative electrocardiogram (ECG) traces, intraoperative arrhythmias, and treatments were recorded for both groups. Echocardiography and ECG were evaluated at postoperative 1 week, 1, 3, and 6 months, and annually thereafter. RESULTS: After transcatheter closure, follow-up basal ECG showed incomplete right bundle branch block pattern in 21 patients and sinus rhythm in 229 patients. After surgical closure, incomplete right bundle branch block pattern was detected in 23 patients, complete right bundle branch block in 3 patients, and complete AV-block (Atrioventricular Block) pattern in 1 patient. The other 150 patients showed sinus rhythm. At least one postoperative follow-up Holter ECG record could be obtained for 104 patients in the transcatheter group and 96 patients in the surgical group. Of 104 patients who underwent transcatheter closure, 97 (93.3%) had normal Holter ECG findings and 7 (6.7%) had arrhythmia. Of the 96 patients who underwent surgical closure, 85 (88.5%) had normal Holter ECG traces and 11 (11.5%) had arrhythmia. There was no statistically significant difference in the frequency of arrhythmia (P = 0.164). CONCLUSION: The higher frequency of arrhythmia in adult studies compared to the pediatric age group once again demonstrates the importance of early diagnosis and treatment of ASD in childhood. The similar incidence of arrhythmia in both groups supports the safety and effectiveness of both closure methods in eligible patients.

The Evaluation of Troponin I Levels and Myocarditis in Children with COVID-19: A Pediatric Single-Center Experience.

Children with COVID-19 usually show milder symptoms than adults; however, a minority of them may have cardiac involvement. We aimed to identify the role of troponin I levels that may predict early cardiac involvement in children with COVID-19. A single-center retrospective study was conducted to evaluate hospitalized children diagnosed with COVID-19 between March 11, 2020, and December 31, 2021. Patients with available troponin I levels and with no known cardiac disease were included. During the study period, 412 children with COVID-19 who had troponin I levels on admission were identified. Troponin levels were elevated in 7 (1.7%) patients and were normal in 395 (98.3%) patients. The median age of patients with elevated troponin levels was 4 (min. 2-max. 144) months, which was statistically lower than the age of patients with normal troponin levels (P = 0.035). All the patients with elevated troponin levels had tachycardia. Out of 7 patients with high troponin levels, 3 (42.9%) of them were admitted to the pediatric intensive care unit (PICU), 2 (28.6%) required oxygen support, and 1 (14.3%) required a mechanical ventilator. Patients with elevated troponin levels had a statistically longer hospital stay (P < 0.001). Neutropenia, tachycardia, PICU admission, oxygen support, and mechanical ventilation were statistically more common in patients with elevated troponin levels (P values were 0.033, 0.020, < 0.001, 0.050, and < 0.001, respectively). Electrocardiography (ECG) and echocardiography (ECHO) were performed on all patients with elevated troponin levels, and 6 (85.8%) patients were diagnosed with myocarditis. The ECG and ECHO have been performed in 58 (14.3%) out of 405 patients with normal troponin levels. Two (3.5%) patients had negative T waves on ECG, and all ECHOs were normal. Our results suggest that elevated troponin I levels in children with COVID-19 can be used to evaluate cardiac involvement and decide the need for further pediatric cardiologist evaluation.

A retrospective comparative analysis of factors affecting the decision and outcome of initial intravenous immunoglobulin alone or intravenous immunoglobulin plus methylprednisolone use in children with the multisystem inflammatory syndrome.

BACKGROUND: For children with the multisystem inflammatory syndrome(MIS-C), intravenous immunoglobulins (IVIG) with or without methylprednisolone are the most effective treatment. In this study, IVIG combined with methylprednisolone was compared to IVIG used alone in children with MIS-C. METHODS: This retrospective cohort study was carried out between April 1, 2020, and November 1, 2021. This study covered all children with MIS-C. According to whether they received IVIG alone or IVIG with methylprednisolone as an initial treatment for MIS-C, the patients were split into two groups. The IVIG dosage for the patients in group I was 2 gr/kg, whereas the IVIG dosage for the patients in group II was 2 gr/kg + 2 mg/kg/day of methylprednisolone. These two groups were contrasted in terms of the frequency of fever, length of hospital stay, and admission to the pediatric intensive care unit. RESULTS: The study comprised 91 patients who were diagnosed with MIS-C and were under the age of 18. 42 (46.2%) of these patients were in the IVIG alone group (group I), and 49 (53.8%) were in the IVIG + methylprednisolone group (group II). Patients in group II had a severe MIS-C ratio of 36.7%, which was substantially greater than the rate of severe MIS-C patients in group I (9.5%) (p 0.01). When compared to group I (9.5%), the rate of hypotension was considerably higher in group II (30.6%) (p = 0.014). Additionally, patients in group II had considerably higher mean serum levels of C-reactive protein. The incidence of fever recurrence was 26.5% in group II and 33.3% in group I, however the difference was not statistically significant (p > 0.05). CONCLUSIONS: The choice of treatment for patients with MIS-C should be based on an individual evaluation. In MIS-C children with hypotension and/or with an indication for a pediatric intensive care unit, a combination of IVIG and methylprednisolone may be administered. For the treatment modalities of children with MIS-C, however, randomized double-blind studies are necessary.

Validation of the Turkish Version of the Catheterization Risk Score for Pediatrics.

BACKGROUND: The aim of this study was to perform a validity analysis of the Turkish version of the Catheterization Risk Score for Pediatrics. METHODS: The study sample consisted of 419 pediatric patients who underwent cardiac catheterization. Patient risk factors and outcomes were collected using the revised (r) Catheterization Risk Score for Pediatric score (21 points) and Catheterization Risk Score for Pediatric score-20 point (Nykanen score). The serious adverse events and nonserious adverse event complications that occurred during and after the procedure were recorded. The revised Catheterization Risk Score for Pediatrics and Catheterization Risk Score for Pediatrics score-20 points were administered by pediatric cardiologists. The content validity index was calculated based on expert opinions. Chi-square, correlation, and regression analyses were used. RESULTS: The mean age of the pediatric patients was 4.5 ± 4.8 years. Of the patients, 50.1% were male (n=210) and 85% (n=356) had acyanotic heart disease. The patients' Catheterization Risk Score for Pediatrics score-20 point and revised Catheterization Risk Score for Pediatrics score were 5.9 ± 2.5 (range, 3-16) and 4.0 ± 2.5 (range, 0-16), respectively. Serious adverse events developed in 10.7% (n=45) of the patients and were found to be related with patient status/timing of catheterization, age, weight, respiratory status, and American Society of Anesthesiologist scores (P < .05). Significant positive correlations were found between the incidence of serious adverse events and total revised Catheterization Risk Score for Pediatrics score (21 points), total Catheterization Risk Score for Pediatrics score-20 point, and American Society of Anesthesiologist score (P < .01). CONCLUSION: The revised Catheterization Risk Score for Pediatrics score (21 points) and Catheterization Risk Score for Pediatrics score-20 point are valid tools for predicting preprocedural risk in the Turkish population.

Effectiveness of music during cardiac catheterization on children's pain, fear, anxiety and vital signs: A randomized, blind controlled trial.

PURPOSE: This study aims to examine the effectiveness of music during pediatric cardiac catheterization procedure on children's pain, fear, anxiety and vital signs. DESIGN AND METHODS: This randomized, blind controlled study was conducted with the guidelines of Consolidated Standards of Reporting Trials (CONSORT). The sample of children was allocated to the Control Group, the Classic Music Group, and the Self-Selected Group. Pain, fear, and anxiety levels were measured before and after the pediatric cardiac catheterization procedure. Vital signs were measured before, during, and after the pediatric cardiac catheterization procedure. RESULTS: There was no significant difference between the groups in terms of demographic status, pain, fear, or anxiety levels and vital signs before the procedure. In post pediatric cardiac catheterization measurement in the recovery phase, pain, fear levels, anxiety levels, heart rate, systolic and diastolic blood pressure of children in the musical intervention groups were lower than the control group. No statistical difference was found in terms of pain, fear and anxiety levels of the children in groups Classic and Self-Selected. CONCLUSION AND PRACTICE IMPLICATIONS: The result of this research reveals that musical intervention applied during the pediatric cardiac catheterization procedure in children affects the reduction of pain, fear and anxiety levels and stabilization of vital signs. This intervention could be an effective nonpharmacological method to improve outcomes.Nurses can improve children's comfort by using musical interventions. CLINICAL REGISTRATION: This study was registered at the U.S. National Library of Medicine Clinical Trials (code: NCT04316754).

Evaluation for Metastatic Candida Focus and Mortality at Candida-associated Catheter-related Bloodstream Infections at the Pediatric Hematology-oncology Patients.

BACKGROUND: Candidemia and Candida-associated catheter-related bloodstream infections (CRBSIs) are the significant cause of mortality and morbidity in patients with malignancy. METHODS: A retrospective analysis including all pediatric hematologic/oncologic malignancies patients with CRBSIs treated in Dr. Behçet Uz Children Diseases and Surgery Training and Research Hospital between the period of 2009 and 2020. RESULTS: During the study period, 53 children with CRBSIs associated with Candida species were included. The most common malignancy was acute lymphoblastic leukemia (45.3%) and acute myeloid leukemia (15.1%). A total of 56 Candida isolates were present including non-albicans Candida species (80.4%) and Candida albicans (19.6%). The most common isolated Candida species was Candida parapsilosis (42.9%) and followed by C. albicans (19.6%). The ratio of azole prophylaxis was significantly higher in patients with the non-albicans Candida group (P=0.031). Candida-related endocarditis (vegetation) was present in 2 (3.8%) patients, and the overall rate of hepatosplenic candidiasis was 3.8%. Seven days Candida attributable mortality was 7.5% (4 patients) and 30 days Candida attributable mortality was 11.3% (6 patients). The Candida species responsible for the Candida-related deaths were as following: Candida tropicalis (n=3), C. parapsilosis (n=2), and C. lusitanae (n=1). CONCLUSION: In pediatric cancer patients with Candida-associated CRBSIs, evaluation of the patient for organ involvement including liver and spleen ultrasonography and cardiac involvement with echocardiography are essential regardless of the patients' clinical picture.

Endovascular treatment of aortic coarctation with a novel BeGraft aortic stent in children and young adults: a single-centre experience with short-term follow-up results.

OBJECTIVES: We present our experience and outcomes with the BeGraft in the treatment of aortic coarctation in a predominantly paediatric population. METHODS: This study includes a retrospective analysis of patients who had Begraft aortic stent implantation between 2018 and 2020 from a single centre. RESULTS: The BeGraft aortic stent was used in 11 patients (7 males, 4 females) with a median age of 14 (13-21) years and a median weight of 65 (46-103) kg. Coarctation was native in five patients and recurrent in six patients. Median stent diameter and length were 16 mm and 38 mm, respectively. The median peak-to-peak pressure was 30 (12-55) mmHg before the procedure and 5 (0-17) mmHg after the procedure. The stenting procedure was successful in 10 of the 11 patients. Stent migration to the abdominal aorta occurred on post-procedure day 1 in the 21-year-old patient, who had previously undergone surgical closure of the ventricular septal defect and balloon angioplasty for coarctation. After repositioning failed, the stent was safely fixed in the abdominal aorta. Strut distortion also occurred during balloon retrieval in one patient, but no aneurysm or in-stent restenosis was observed at 1-year follow-up. The patients were followed for a median of 14 (4-25) months and none required redilation. CONCLUSIONS: Our initial results demonstrated that the BeGraft aortic stent effectively reduced the pressure gradient in selected native and recurrent cases. Despite advantages such as a smaller sheath and low profile, more experience and medium- to long-term results are needed.

Assessment of clinical characteristics and treatment outcomes of pediatric patients with intracardiac thrombosis: a single-center experience.

The prevalence of intracardiac thrombus (ICT) is gradually increasing, though it is rare among children. Data related to the occurrence of ICT among children are limited, and treatment recommendations have been made utilizing adult guidelines. The primary objective of this study is to determine associated factors, management, and outcomes of intracardiac thrombosis in children. Between January 2013 and January 2020, patients diagnosed with ICT at the Pediatric Hematology-Oncology and Pediatric Cardiology departments in our hospital were included in the study. Demographic characteristics, clinical and laboratory findings, treatment protocols, and outcomes were analyzed retrospectively. The median age at diagnosis was 10.5 months (2 days to 14.5 years), and the median follow-up period was 6.5 months (1 month to 3.1 years). The most common primary diagnoses of the patients, in order of frequency, were heart disease (n: 8), metabolic disease (n: 3), prematurity and RDS (n: 3), burns (n: 2), pneumonia (n: 2), and asphyxia (n: 2). CVC was present in 19/23 of the patients. The reasons for CVC insertion were the need for plasmapheresis in one patient with a diagnosis of HUS and the need for well tolerated vascular access because of long-term hospitalization in others. LMWH was administered to all patients as first-line therapy. Complete response was achieved in 19 (79%) of 24 patients and 4 patients (16.6%) were unresponsive to medical treatment. It was found out that the thrombus location, type, sepsis, and hemoculture positivity, as well as the presence of CVC, had no impact on treatment response (chi-square P = 0.16, 0.12, 0.3, 0.49, 0.56). Moreover, no correlation was determined between thrombus size and treatment response (Mann Whitney U test P = 0.47). The mortality rate was determined to be 12.5% (3/24). Spontaneous occurrence of ICT is rare in childhood, without any underlying primary disease or associated factor. The presence of CVC, sepsis, and heart disease are factors associated with ICT. The success rate is increased with medical treatment. There was no significant difference in treatment response between the newborn and 1 month to 18-year-old patient group. It has been demonstrated that thrombus size, type, localization; sepsis, and hemoculture positivity had no impact on the treatment response.

Right ventricular regional deformation analysis in patients operated for tetralogy of Fallot.

PURPOSE: We aimed to compare the findings of a segmental speckle-tracking strain of right ventricle with those of cardiac magnetic resonance imaging in this setting. METHODS: In 26 patients with operated tetralogy of Fallot (mean age, 15.35 ± 2.3 years; range 11-18 years), right ventricular segmental speckle-straining (the basal, mid, apical segments of right ventricular septum and lateral free wall, and right ventricular apex) were determined using two-dimensional echocardiography. The echocardiographic findings were compared to right ventricular ejection fraction, right ventricular indexed end-diastolic volume, indexed end-systolic volume and pulmonary regurgitation fraction at cardiac magnetic resonance. RESULTS: Right ventricular global speckle strain was -18.6 ± 3.7and lateral free wall strain was -17.8 ± 4.9. Indexed right ventricular end diastolic volume was 171.7 ± 23.3 ml/m2, indexed right ventricular end systolic volume was 95.1 ± 35.0 ml/m2 and right ventricular ejection fraction was 44.76 ± 9.39%. Basal inferior septum is correlated with indexed right ventricular end-diastolic volume (RVEDV; r = -0.521, p = 0.015) and pulmonary regurgitation fraction (r = -0.584, p = 0.015). Cardiac magnetic resonance (CMR)-derived RVEDV is correlated with lateral free wall (r = -0.465, p = 0.034) and Global RV (r = 0.442, p = 0.045). CONCLUSION: Right ventricular basal inferior septal and apical longitudinal strain correlates with the measures of right ventricular performance on CMR. These parameters would be useful to monitor right ventricular performance in patients after tetralogy of Fallot repair.

Orodental, Facial and Clinical Features of Mutation-Positive Noonan Syndrome: A Monocentric Study.

OBJECTIVE: To evaluate orodental, facial, clinical and molecular characteristics of the patients with Noonan Syndrome (NS). STUDY DESIGN: The orodental, clinical and molecular characteristics of 29 mutation-positive patients with NS were recorded. Orodental examination was performed in 17 patients. All exons and exonintron boundries of PTPN11 and SOS1 genes were analyzed by Sanger sequencing. RESULTS: A total of 29 patients with NS from 27 unrelated families were included in the study. Seventeen patients were examined by a specialist in oral medicine. The most common orodental findings were high-arched palate (n=13), gingivitis (n=6) and severe caries (n=6). Anterior open bite, posterior cross bite, Class II malocclusion, hypodontia, prognathism (maxillary or mandibular), macroglossia and gingival hyperplasia were also detected. Thirteen different mutations were observed in PTPN11 gene and exon 3 was the hotspot region. Hypodontia was detected in two patients who had the same mutation in PTPN11 gene, c.181G>A, p.D61N. CONCLUSION: This study indicated a high prevalance of orodental problems including high-arched palate, severe dental caries and gingivitis in patients with mutation-positive NS. The mutation in PTPN11 gene, c.181G>A, p.D61N, may be associated with hypodontia in patients with NS.

The effect of the duration of the procedure on the risk of complications during pediatric cardiac catheterization.

BACKGROUND: This study aims to evaluate the frequency of and associated risk factors for adverse events caused by cardiac catheterization procedures in pediatric patients. METHODS: Between January 2009 and January 2012, a total of 599 pediatric patients (320 males, 279 females; mean age 5.4±4.7 years; range, 1 day to 21 years) who underwent cardiac catheterization in our cardiac catheterization laboratory were retrospectively analyzed. Demographic and clinical data of the patients including the duration of the procedure, management of anesthesia, the American Society of Anesthesiologists class, and Catheterization Risk Score for Pediatrics, and procedure-related serious adverse events were recorded. RESULTS: The incidence of procedure-related serious adverse events was 9.18%. Potential risk factors associated with serious adverse events were identified as interventional heart catheterization, high scores obtained from the Catheterization Risk Score for Pediatrics, the use of endotracheal tube in airway control, and prolonged procedural duration. CONCLUSION: Our study results suggest that prolonged duration of catheterization is a potential risk factor for procedure-related adverse events and the duration of the procedure needs to be included as a variable in the Catheterization Risk Score for Pediatrics scoring system for predicting procedure-related adverse events.

Infective endocarditis developing early after percutaneous closure of a patent ductus arteriosus in a child using the Amplatzer Duct Occluder II.

A 10-month-old girl underwent patent ductus arteriosus closure with an Amplatzer Duct Occluder II. After 1 week, she was admitted to our emergency room with tachypnoea, fatigue, and fever. Consecutive blood cultures yielded vancomycin-sensitive Staphylococcus aureus. The patient was already receiving vancomycin, but the fever did not respond to this treatment. The device was successfully removed via left lateral thoracotomy.

Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms.

Yilmazer MM, Özdemir R, Mese T, Küçük M, Öner T, Devrim I, Bayram N, Güven B, Tavli V. Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms. Turk J Pediatr 2019; 61: 648-656. Prompt diagnosis and the administration of intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery abnormalities (CAA) in Kawasaki Disease (KD). The resistance to treatment and development of the coronary sequelae remain the most important problems in KD. We aimed to determine the predicting factors of nonresponse to initial IVIG therapy and to analyze the cases who had giant coronary aneurysms. A total of 120 KD cases, including 61 children fulfilling the criteria for KD and 59 with incomplete KD were enrolled into this study. Demographic, laboratory, clinical, echocardiographic characteristics, and treatment regimens were reviewed, retrospectively. The median age of the patients was 33.5 months (range: 3-168 months). Coronary artery aneurysms were detected in 35 patients (29%) at the time of diagnosis. Twenty-eight patients had coronary aneurysms small or medium in size, one had a large, and seven had giant coronary aneurysms. CAA persisted in 8 cases in the follow-up, all of which were large or giant aneurysms. A ten month-old girl with a giant coronary aneurysm was referred to coronary bypass surgery in the subacute phase of follow-up, due to myocardial ischemia. Eighteen patients were unresponsive to the initial IVIG therapy (%15), of whom 10 were diagnosed as cKD and 8 were iKD. Patients who did not respond to initial IVIG therapy, had higher white blood cell (WBC) count, higher C-reactive protein (CRP) and lower albumin levels than those who did (P < 0.05). In univarite analysis; CRP, WBC and albumin were found to be significant predictors of nonresponse to initial IVIG therapy, while a stepwise multiple linear regression analysis showed that WBC count and albumin levels were significantly correlated with nonresponse to initial treatment with IVIG. Our study showed that WBC count and albumin levels might be used as predictors of nonresponse to the IVIG therapy in Turkish children with KD.