The impact of fragmented QRS on clinical findings and outcomes in children with dilated cardiomyopathy with or without left ventricular non-compaction.

OBJECTIVE: The aim of this study is to investigate the frequency of fragmented QRS and its associations with clinical findings and prognosis in children diagnosed with dilated cardiomyopathy with or without left ventricular non-compaction. METHODS: This retrospective study was conducted between 2010 and 2020. Patients with dilated cardiomyopathy were classified into two groups according to the presence of left ventricular non-compaction: Dilated cardiomyopathy with left ventricular non-compaction and dilated cardiomyopathy without left ventricular non-compaction. Patients were also divided into two groups according to the presence of fragmented QRS (fragmented QRS group and non-fragmented QRS group). RESULTS: Twenty-three of 44 patients (52.3%) were male. Among left ventricular non-compaction patients, the fragmented QRS group had more complex ventricular arrhythmias (p = 0.003). Patients with fragmented QRS had a significantly higher rate of major adverse cardiac events and/or cardiac death in both cardiomyopathy groups (p = 0.003 and p = 0.005). However, the rate of major adverse cardiac events and/or cardiac death was similar between dilated cardiomyopathy patients with and without left ventricular non-compaction. Multivariate logistic regression analysis showed that the presence of fragmented QRS strongly predicts major adverse cardiac events and/or cardiac death (odds ratio, 31.186; 95% confidence interval, 2.347-414.307). Although the survival rates between cardiomyopathy groups were similar, patients with fragmented QRS had a markedly lower survival rate during the follow-up period, as mean of 15 months (p = 0.001). CONCLUSION: Our study showed that the presence of fragmented QRS may be an important ECG sign predicting an major adverse cardiac event and/or cardiac death in patients with dilated cardiomyopathy. We believe that recognising fragmented QRS could be valuable in forecasting patient prognosis and identifying high-risk patients who require additional support.

Diagnostic role of neutrophil-lymphocyte ratio in oral cavity cancers.

AIM: In this study, we aimed to evaluate the diagnostic role of neutrophil-lymphocyte ratio (NLR) and other hemogram parameters in differentiating nonmalignant oral cavity lesions from oral cavity cancers. METHODS: Ninety-five patients who were performed oral cavity biopsy between the years 2013 and 2015 were retrospectively reviewed. The control group comprised consecutive 70 patients who underwent septoplasty/septorhinoplasty procedures. Inclusion criteria were to be available with common blood count (CBC) just prior to procedure. The hemogram parameters including NLR were compared between the groups. RESULTS: Lymphocyte count and mean platelet volume (MPV) were found to be significantly decreased in oral cavity squamous cell carcinoma (SCC) and in other oral cavity cancers (OCCs) compared with benign oral cavity lesions and control group. In contrast, NLR revealed significantly higher in OCCs and in oral SCC compared with nonmalignant oral cavity lesions and control group. The receiver operating characteristics curve analysis suggested cutoff value of 2.88 for NLR in predicting malignancy [area under curve (AUC) 0.756, sensitivity 51%, specificity 88%]. CONCLUSIONS: NLR was first shown to be significantly elevated in oral cavity cancers and in oral cavity SCC in this study. In our opinion, NLR may be helpful in identifying the oral cavity lesions at high risk for harboring malignancy.

Evaluation of VIIth-XIIth cranial nerve anastomosis results by age.

OBJECTIVE: To evaluate VIIth-XIIth cranial nerve (hypoglossal-facial nerve) anastomosis results by age. METHOD: A total of 34 patients who attended a follow-up visit in 2016, aged 20-63 years, were enrolled. The House-Brackmann facial nerve function grading system and the Facial Clinimetric Evaluation scale were applied. RESULTS: Regarding post-anastomosis facial nerve function, in the group aged 40 years or less, 14 patients (78 per cent) had House-Brackmann grade III and 4 patients (22 per cent) had House-Brackmann grade IV facial nerve function post-anastomosis. In the group aged over 40 years, nine patients (56 per cent) had House-Brackmann grade III and seven patients (44 per cent) had House-Brackmann grade IV facial nerve function post-anastomosis. There was a statistically significant difference between the two groups in mean facial movement domain scores (p = 0.02). Analysis between age and facial movement score in all 34 patients demonstrated a moderate negative correlation (Pearson correlation coefficient: -0.38) and statistical significance (p = 0.02). CONCLUSION: Facial reanimation yielded better results in younger than in older patients.

Extracorporeal membrane oxygenation support in neonates: a single center experience in Turkey

Background/aim: Extracorporeal membrane oxygenation (ECMO) is a form of life support for patients with respiratory failure, cardiac failure, or both. The aim of this study was to evaluate neonates supported with ECMO and report our experience as a Turkish neonatal intensive care unit. Materials and methods: We retrospectively reviewed 11 newborn infants treated with ECMO at Ankara University for respiratory and cardiac failure. We reported the demographic, diagnostic, laboratory, and clinical data of the patients. Results: Eleven patients (9 male, 2 female) received ECMO support with a mean gestational age of 39.1 ± 1.6 weeks and mean birth weight of 3513 ± 506 g. Six patients received venoarterial (VA) ECMO and five patients received venovenous (VV) ECMO. Mean age at initiation and duration of ECMO was 7.2 ± 7.4 days (2­24 days) and 10.4 ± 4.9 days (5­21 days), respectively. Mean oxygenation index (OI) before ECMO was 48.5 ± 5.7. ECMO was withdrawn from one patient due to severe brain injury. The survival rate for ECMO was 73% and the survival rate to discharge was 64%, whereas the survival rate in congenital diaphragmatic hernia (CDH) cases was 40%. Conclusion: Our early results from ECMO for neonates are encouraging. Identification of patients for ECMO support and timely referral will offer a survival opportunity to complex neonatal cases.

Comparison of Carina active middle-ear implant with conventional hearing aids for mixed hearing loss.

OBJECTIVE: To compare the auditory outcomes of Carina middle-ear implants with those of conventional hearing aids in patients with moderate-to-severe mixed hearing loss. METHODS: The study comprised nine patients (six males, three females) who underwent middle-ear implantation with Carina fully implantable active middle-ear implants to treat bilateral moderate-to-severe mixed hearing loss. The patients initially used conventional hearing aids and subsequently received the Carina implants. The hearing thresholds with implants and hearing aids were compared. RESULTS: There were no significant differences between: the pre-operative and post-operative air and bone conduction thresholds (p > 0.05), the thresholds with hearing aids and Carina implants (p > 0.05), or the pre-operative (mean, 72.8 ± 19 per cent) and post-operative (mean, 69.9 ± 24 per cent) speech discrimination scores (p > 0.05). One of the patients suffered total sensorineural hearing loss three months following implantation despite an initial 38 dB functional gain. All except one patient showed clinical improvements after implantation according to quality of life questionnaire (Glasgow Benefit Inventory) scores. CONCLUSION: Acceptance of Carina implants is better than with conventional hearing aids in patients with mixed hearing loss, although both yield similar hearing amplification. Cosmetic reasons appear to be critical for patient acceptance.

Dispersion of QT and QTc interval in healthy children, and effects of sinus arrhythmia on QT dispersion.

OBJECTIVE: To determine the normal values of QT and QTc dispersion and the effects of sinus arrhythmia on QT dispersion in healthy children. PATIENTS AND SETTING: The study was carried out in a university hospital on 372 local schoolchildren (200 male, 172 female), aged seven to 18 years. METHODS: The QT and preceding RR intervals of at least one sinus beat were measured manually in a range of nine to 12 leads on standard 12 lead surface ECGs. The corrected QT interval was computed by the method of Bazett. Dispersion of QT and QTc were defined as (1) the difference between the maximum and minimum QT and QTc intervals occurring in any of the 12 leads (QTD and QTcD), (2) the standard deviation of the QT and QTc interval in the measurable leads (QT-SD and QTc-SD). RESULTS: There was no significant difference in QT, QTc, and RR dispersion between girls and boys. Overall 53% of children had sinus arrhythmia. Although QTD and QT-SD were not affected by sinus arrhythmia, both QTcD and QTc-SD were significantly greater in children with sinus arrhythmia than in those without (QTcD: 52.9 (17.4) v 40.9 (13.1); QTc-SD: 17.5 (5.9) v 13.2 (4.0); p < 0.001). CONCLUSIONS: As calculation of QTc dispersion is affected by sinus arrhythmia, which is common in childhood, we suggest that QT dispersion should not be corrected for heart rate in children.

Plasma endothelin-1 levels in patients with left-to-right shunt with or without pulmonary hypertension.

The aim of this study was to evaluate the role of endothelin-1 (ET-1) in pathophysiology of pulmonary hypertension (PH) secondary to congenital heart disease with left-to-right shunt. Twenty-three children (12 male, 11 female) aged 0.58-13 years were enrolled the study. Blood samples were drawn from superior vena cava, right atrium, right ventricle, pulmonary artery and pulmonary wedge or pulmonary vein during cardiac catheterization. Plasma ET-1 levels were assayed by ELISA. Patients were divided into two groups according to the presence or absence of PH. Plasma ET-1 levels of the study group were compared to the peripheral venous and arterial ET-1 levels of 11 healthy infants and children (aged 0.75-13 years). Plasma ET-1 levels in patients with left-to-right shunt were found significantly higher than those of controls. However, plasma ET-1 levels were similar between the two groups of the patients. Pulmonary venous ET-1 levels were higher than the levels of superior vena cava, this suggested an increased production of ET-1 in pulmonary vascular bed in patients with PH. No correlations were found between plasma ET-1 levels and pulmonary arterial pressure, pulmonary vascular resistance and pulmonary blood flow in the patients. Plasma ET-1 levels of the patients with left-to-right shunt were increased independently from pulmonary arterial pressure and pulmonary vascular resistance. This increase was related to the production of ET-1 in pulmonary vascular bed in patients with PH. ET-1 could not be found to be directly related to the development of PH in the patients with left-to-right shunt.

QT/corrected QT (QTc) intervals and QT/QTc dispersions in children with chronic renal failure.

UNLABELLED: We aimed to examine QT/corrected QT (QTc) intervals, QT/QTc dispersions (QTD/QTcD) and also the effect of different clinical and laboratory variables on these parameters in children with chronic renal failure. Serum biochemistry, 12-lead electrocardiogram, telecardiogram, and echocardiography were performed in 50 children with chronic renal failure (23 female and 27 male; aged 12.3+/-3.6 years, range 5 to 20 years). None of them had symptoms related to arrhythmias. When compared with a control group (372 children, aged 7 to 18 years, mean 12.4+/-2.6) patients with chronic renal failure had greater QT/QTc intervals and QT/QTc dispersion values (Patient: QT = 360.9+/-53.3; QTc = 438.5+/-33.2; QTD = 42.4+/-20.8; QTcD = 57.5+/-23.8; CONTROL: QT = 325.9+/-24.1; QTc = 398.7+/-19.7; QTD = 29.9+/-10.2; QTcD = 47.3+/-16.6; P<0.01). QT, QTc, and QTcD values were significantly greater in patients who had renal failure duration longer than 2 years. Patients who had impaired left ventricular systolic function on echocardiogram had greater QTc, QTD, and QTcD values. It was found that sex, cardiomegaly on chest X-ray, and left ventricular hypertrophy on echocardiogram were not related to these parameters. It is concluded that, impaired cardiac systolic function and longer renal failure duration are related to an increase in QT, QTc, QTD, and QTcD values and hence these variables may be risk factors for ventricular arrhythmias in uremic patients.

Echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract: possible role in the pathogenesis of discrete subaortic stenosis.

Although the clinical features and natural course of discrete subaortic stenosis (DSS) are well defined, the etiology remains speculative. The purpose of this study was to identify the echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract associated with DSS in children and to determine whether these variations have a role in the pathogenesis of DSS. The aortoseptal angle (ASA), mitral-aortic valve separation (MAS), and the size of the aortic annulus were determined in two groups of children. Group 1 comprised 11 patients with isolated DSS, who were compared with an age- and body surface area- (BSA) matched healthy children (Group 1A, n: 20). Group 2 comprised 10 patients with DSS and ventricular septal defect (VSD). Group 2 was compared with an age- and BSA-matched patients with isolated perimembranous VSD (Group 2A, n: 22). Measurements were carried out from previously recorded echocardiographic studies. The ASA was steeper (119.3 +/- 6.1 degrees vs 137.5 +/- 5.6 degrees , p < 0.001), and the MAS was wider (6.1 +/- 1.6 vs 3.2 +/- 0.7 mm, p < 0.001) in patients with isolated DSS than in healthy control subjects. Similar differences were found between patients in Group 2 and Group 2A; the ASA was steeper (122.2 +/- 6.5 degrees vs 141.3 +/- 5.0 degrees, p < 0.001), and the MAS was wider (5.8 +/- 1.5 vs 3.8 +/- 1.1 mm, p < 0.001). The size of the aortic annulus was not different among the four study groups. Although the MAS was significantly wider in patients with DSS, there was significant overlap in MAS between patients and controls. However, if an ASA < or = 130 degrees was chosen as a predictive variable, it was found to be a highly sensitive, specific, and positive predictive marker for the development of DSS. This study demonstrates that DSS is associated with a steeper ASA, and a wider MAS, in patients with or without associated VSD. These morphologic abnormalities, especially a steeper ASA, may be risk factors for the development of DSS.

Relation of mass/volume ratio to ECG abnormalities and symptoms in children with aortic stenosis/insufficiency.

In this study, to determine whether symptoms and ECG abnormalities relate to left ventricular (LV) mass, volume, and mass/volume (M/V) ratio in children with aortic stenosis (AS) and/or insufficiency (AI), the authors examined 23 patients with echocardiography. LV volumes and mass were calculated with echocardiography. Also, the peak and mean gradients across the aortic valve and left ventricular meridional wall stress (ESWS) were determined by use of echo Doppler techniques. Fourteen patients (mean age 5.84+/-3.49 years) had AS alone. Of these, 14 had symptoms and seven had abnormal-appearing ECGs. Nine patients (mean age 6.91+/-4.35 years) had AS/AI. Of these nine, four had symptoms and two had ECG abnormalities. The authors observed that the incidences of symptoms and ECG abnormalities were higher in the patients with AS than in those with AS/AI. There was no significant correlation between symptoms and ECG abnormalities with peak gradient, mean gradient, valve area, LV mass, volume, and mass/volume ratio in patients with AS/AI. However, significant correlation was found between symptoms and ECG abnormalities and ESWS in AS/AI patients. In conclusion, ECG abnormalities and symptoms do not always indicate the severity of AS and AS/AI.

Recurrent pericardial effusion due to gunshot wound of the heart in a hemodynamically stable child--a case report.

A 12-year-old girl presented with recurrent pericardial effusion due to firearm pellet injury to the left ventricle. The pellet was localized by two-dimensional echocardiography within the left ventricular apical wall. Since the patient was asymptomatic, left ventriculotomy was avoided to extract the pellet and only pericardial tube drainage was carried out. A slightly elevated blood lead level of the patient was alarming for potential subsequent lead poisoning due to retained pellets.

Poststreptococcal reactive arthritis: clinical course and outcome in 15 patients.

Patients with Group A beta-hemolytic streptococcal infection and articular disease, who do not fulfill the modified Jones criteria for diagnosis of acute rheumatic fever (ARF), have been classified as having poststreptococcal reactive arthritis (PSRA). We reviewed the clinical characteristics, laboratory findings and outcome of 15 patients with PSRA. None of these patients had clinical evidence of carditis. The pattern of joint involvement was variable and included arthritis in five patients and arthralgia in the remaining ten patients. Nine patients were treated with salicylates for one to 16 weeks; the others recovered spontaneously. Usually, the patients with arthralgia responded promptly to salicylates, while the response was poor in patients with arthritis. One patient with monoarthritis developed carditis nine months after his first arthritis attack. Another patient presenting with monoarthritis later had two additional episodes of poststreptococcal reactive arthralgia. It seems there is a wide spectrum of poststreptococcal rheumatic diseases, and patients with PSRA are also at risk for cardiac disease; therefore, prophylactic antibiotic therapy should be considered in these patients.

Transmastoid labyrinthectomy for disabling vertigo after cochlear implantation.

OBJECTIVE: To document the use of transmastoid labyrinthectomy in the treatment of disabling vertigo after unilateral cochlear implantation. METHODS: A 58-year-old man with severe-to-profound bilateral sensorineural hearing loss secondary to chronic otitis media underwent cochlear implantation in his right ear with a Pulsar Med-El device. The surgery was uneventful and the electrode was positioned correctly. He had episodic vertigo three months after implant surgery, and medical treatment and aggressive vestibular rehabilitation did not relieve the vertigo attacks. RESULTS: Right transmastoid labyrinthectomy was performed one year after cochlear implantation. The patient's symptoms were immediately relieved, and cochlear implant function was not adversely affected at follow up after three years. CONCLUSION: Transmastoid labyrinthectomy seems to be an effective, safe method for ablating the vestibular end organ after unilateral cochlear implantation.

Premature thelarche in Kabuki make-up syndrome.

A 2 year old girl who has Kabuki make-up syndrome with isolated premature thelarche is presented. She has unique ocular abnormalities which have not been reported previously. In the literature at least 85 patients, most of them from Japan, have been reported. This is the first reported non-Japanese Asian case.

QRS axis in isolated perimembranous ventricular septal defect and influences of morphological factors on QRS axis.

To detect the frequency of left axis deviation (LAD) in isolated perimembranous ventricular septal defects (VSD) we retrospectively analyzed electrocardiograms of 59 patients, aged 8 months to 15 years. Patients were grouped into those having ventricular septal aneurysm (VSA) formation (n:20) and those who did not have VSA (n:39). Patients with VSA were then stratified into 2 groups according to the presence of left ventricular-to-right atrial (LV-RA) shunt. Four hundred healthy children served as the control group. We found that 12 (20.3%) of 59 patients with isolated perimembranous VSD had a LAD. Five of 6 patients with perimembranous outlet VSD and 6 with perimembranous inlet VSD had abnormal LAD with a qR pattern in I and aVL and rS in aVF. Abnormal LAD was more prevalent in patients with VSA (40%) than without VSA (7.7%) (P <.01). We also found that mild right ventricular hypertrophy (RVH) with the rsR' or rSR' pattern in V1 was more frequent in patients with VSA, especially those who had LV-RA shunt. However, we could not find significant difference between patients with or without LV-RA shunt for the incidence of abnormal LAD and mild RVH. Localization of perimembranous VSD was not found to have an effect on frequency of abnormal LAD and mild RVH in this patient group. In patients with clinical findings of VSD, the existence of abnormal LAD especially if it is associated with mild RVH, should raise the possibility of perimembranous VSD with VSA formation.

Factor V 1691 G-A mutation in children with intracardiac thrombosis: a prospective study.

UNLABELLED: The objective of this study was to determine the association between intracardiac thrombosis and hereditary causes of thrombophilia, including factor V 1691 G-A (factor V Leiden, FVL) and prothrombin 20210 G-A mutations. Over a period of 3 y, genetic risk factors were evaluated in 13 consecutive children (mean age 6.27 +/- 5.44 y) with intracardiac thrombosis, diagnosed by cross-sectional echocardiography. Thrombi were localized in the left heart in four patients and the right heart in nine patients. All children had predisposing factors for thrombus formation: ventriculoatrial shunt for hydrocephalus (n = 3), indwelling catheter for chemotherapy (n = 5), cardiomyopathy (n = 2), sepsis (n = 1), homocystinuria (n = 1) and tetralogy of Fallot (n = 1). Six of the 13 children with intracardiac thrombosis were heterozygotes for FVL mutation. Three of these six children with FVL mutation had ventriculoatrial shunt for hydrocephalus, two children had cardiomyopathy and one had sepsis. None of the patients carried the prothrombin 20210 G-A mutation. CONCLUSION: It is recommended that FVL mutations should be investigated in all cases of intracardiac thrombosis irrespective of whether or not a predisposing factor is identified. When a predisposing factor is found antithrombotic prophylaxis may be considered in patients carrying the FVL mutation.

Prevalence of idiopathic long QT syndrome in children with congenital deafness.

Long QT syndrome (LQTS) is characterized by prolongation of the QT interval associated with a high risk for syncope and sudden death. Jervell and Lange-Nielsen initially described LQTS in association with congenital sensorineural deafness. We have investigated the prevalence of this syndrome in a school for deaf children, evaluating by ECG 350 congenitally deaf children with an age range of 6-19 years. The corrected QT interval (QTc) was calculated by Bazett's formula. Eight children with a QTc interval >440 ms were further studied by cardiac examination, repeat ECGs (three times), Holter monitoring, echocardiography, and exercise testing. The families were assessed for a history of syncope and deafness and underwent ECG evaluations regarding lengthened QTc interval. Among these eight children only two girls aged 14 and 15 years were diagnosed as having LQTS according to Schwartz's criteria (0.57% of the 350 deaf children; 95% confidence intervals 0,

Systolic and diastolic function in children with chronic renal failure.

BACKGROUND: Cardiovascular complications are the most frequent cause of death in patients with end-stage renal failure (ESRF). We aimed to investigate systolic and diastolic functions in children with ESRF. METHODS: Thirty-nine children with ESRF (17 on continuous ambulatory peritoneal dialysis (CAPD), eight on hemodialysis and 14 on predialysis) were examined to assess systolic and diastolic functions by echocardiography and ultrasound Doppler. Left ventricular systolic and diastolic functions were measured both in patients and age-matched healthy controls (n = 20) and the indices of cardiac performance were compared. RESULTS: Increased left ventricular mass index (LVMI) and decreased volume/mass ratio with normal systolic left ventricular function was found in patients, as compared with controls. Left ventricular diastolic dysfunction was observed in dialysis patients. In most of these patients, left ventricular isovolumic relaxation time was prolonged, except in CAPD patients. The peak of late diastolic flow (A) velocities were increased with a reduction of the early diastolic flow velocity (E)--the E/A ratio. The E velocities were unchanged in all patients as compared with controls. Our data indicated an abnormality of myocardial relaxation in patients with ESRF. We found no relationship between E/A ratio and LVMI. Among three groups of patients, the LVMI and diastolic abnormalities were highest in the hemodialysis group indicative of poor control of hypervolemia and hypertension. CONCLUSIONS: The technique of CAPD has some advantages as a renal replacement therapy for preserving cardiac functions as compared with hemodialysis. However, it must be remembered that patients with hemodialysis have features that effects cardiac status, such as higher volume load and higher afterload (hypertension).