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.

Cardiovascular manifestations and cardiac magnetic resonance follow-up of multisystem inflammatory syndrome in children (MIS-C).

OBJECTIVE: This study aimed to evaluate the cardiovascular manifestations and surveillance of multisystem inflammatory syndrome in children (MIS-C) and to determine the correlation of echocardiographic findings with cardiac magnetic resonance imaging findings. METHODS: Forty-four children diagnosed as MIS-C with cardiac involvement were enrolled in this observational descriptive study. The diagnosis of MIS-C was made according to the criteria of Centers for Disease Control and Prevention. Clinical findings, laboratory parameters, and electrocardiographic and echocardiographic findings at the time of diagnosis and during follow-up were evaluated. Cardiac magnetic resonance was performed on 28 (64%) cases. The 1-year follow-up imaging was performed in all cases with abnormal initial cardiac magnetic resonance findings. RESULTS: Forty-four patients (56.8% male) with a mean age of 8.5 ± 4.8 years were enrolled in this study. There was a significant positive correlation between high-sensitivity cardiac troponin T (mean: 162 ± 444.4 pg/ml) and N-terminal pro b-type natriuretic peptide (mean: 10,054 ± 11,604 pg/ml) (p < 0.01). Number of cases with an electrocardiographic and echocardiographic abnormality was 34 (77%) and 31 (70%), respectively. Twelve cases (45%) had left ventricular systolic dysfunction and 14 (32%) cases had pericardial effusion on admission. Three cases (11%) had cardiac magnetic resonance findings that may be attributed to the presence of myocardial inflammation, and pericardial effusion was present in seven (25%) cases. Follow-up cardiac magnetic resonances of all cases were normal. Cardiac abnormalities were completely resolved in all except two cases. CONCLUSIONS: Myocardial involvement can be seen during acute disease, but MIS-C generally does not lead to prominent damage during a year of surveillance. Cardiac magnetic resonance is a valuable tool to evaluate the degree of myocardial involvement in cases with MIS-C.

The role and efficacy of routine high-sensitivity troponin T screening in paediatric COVID-19.

OBJECTIVE: We aimed to evaluate the efficacy and role of high-sensitivity troponin T in children with a confirmed SARS-CoV-2 infection and also the correlation of troponin T levels with symptoms, and echocardiographic findings were analysed. METHODS: Two hundred and fourteen patients with a confirmed SARS-CoV-2 infection between the dates of 28 March and 15 August 12020 were enrolled in this retrospective single-centre study. Patients with comorbidities and diagnosed as multisystem inflammatory syndrome in children were excluded. Demographic data, clinical and laboratory parameters were evaluated. The patients were classified and compared according to the troponin positivity. The correlation of troponin T with symptoms and echocardiographic findings was analysed. RESULTS: The most common symptoms in the whole study group were fever (53.3%) and cough (24.8%). Troponin T levels were elevated in 15 (7%) patients. The most common symptom in patients with troponin positivity was also fever (73.3%). Troponin T positivity was significantly higher in patients under the age of 12 months and troponin T levels were negatively correlated with age. C-reactive protein levels were elevated in 77 (36%) of the patients in the whole group and 7 (46.7%) of 15 patients with troponin positivity. C-reactive protein levels were similar between groups. CONCLUSION: Routine troponin screening does not yield much information in previously healthy paediatric COVID-19 patients without any sign of myocardial dysfunction. Elevated troponin levels may be observed but it is mostly a sign of myocardial injury without detectable myocardial dysfunction in this group of patients.

Long-term follow-up of subvalvular aortic stenosis in children: a single-centre experience.

OBJECTIVE: The aim of this study is to evaluate clinical and surgical outcomes of children with subaortic stenosis, to determine the risk factors for surgery and reoperation and to compare isolated subaortic stenosis and those concomitant with CHDs. METHODS: The study involved 80 children with subaortic stenosis. The patients were first classified as isolated and CHD group, and the isolated group was further classified as membranous/fibromuscular group. The initial, pre-operative, post-operative and the most recent echocardiographic data, demographic properties and follow-up results of the groups were analysed and compared. The correlation of echocardiographic parameters with surgery and reoperation was evaluated. RESULTS: There was a significant male predominance in all groups. The frequency of the membranous type was higher than the fibromuscular type in the whole and the CHD group. The median time to the first operation was 4.6 years. Thirty-five (43.7%) patients underwent surgery, 5 of 35 (14%) patients required reoperation. The rate of surgery was similar between groups, but reoperation was significantly higher in the isolated group. The gradient was the most important factor for surgery and reoperation in both groups. In the isolated group besides gradient, mitral-aortic separation was the only echocardiographic parameter correlated with surgery and reoperation. CONCLUSION: Reoperation is higher in isolated subaortic stenosis but similar in membranous and fibromuscular types. Early surgery may be beneficial in preventing aortic insufficiency but does not affect the rate of reoperation. Higher initial gradients are associated with adverse outcomes, recurrence and reoperation.

Does obesity influence ventricular repolarisation in children?

OBJECTIVE: Ventricular repolarisation changes may lead to sudden cardiac death in obese individuals. We aimed to investigate the relationship between ventricular repolarisation changes, echocardiographic parameters, anthropometric measures, and metabolic syndrome laboratory parameters in obese children. METHODS: The study involved 81 obese and 82 normal-weight healthy children with a mean age of 12.3 ± 2.7 years. Anthropometric measurements of participants were evaluated according to nomograms. Obese patients were subdivided into two groups; metabolic syndrome and non-metabolic syndrome obese. Fasting plasma glucose, fasting insulin, and lipid profile were measured. QT/QTc interval, QT/QTc dispersions were measured, and left ventricular systolic and diastolic measurements were performed. RESULTS: Body weight, body mass index, relative body mass index, waist/hip circumference ratio, and systolic and diastolic blood pressures were significantly higher in obese children. QT and QTc dispersions were significantly higher in obese children and also obese children with metabolic syndrome had significantly higher QT and QTc dispersions compared to non-metabolic syndrome obese children (p < 0.001) and normal-weight healthy children (p < 0.001). Waist/hip circumference ratio, body mass index, and relative body mass index were the most important determinant of QT and QTc dispersions. Left ventricular wall thickness (left ventricular posterior wall thickness at end-diastole, left ventricular posterior wall thickness at end-systole, interventricular septal thickness at end-diastole) and left ventricular mass index were significantly higher and ejection fraction was lower in obese children. Left ventricular mass index and interventricular septal thickness at end-diastole were positively correlated with QT and QTc dispersions. CONCLUSIONS: Our study demonstrated that QT/ QTc interval prolongation and increase in QT and QTc dispersion on electrocardiogram may be found at an early age in obese children.

Aborted cardiac arrest during sport activity in a teenager diagnosed with short QT syndrome.

Short QT syndrome is a malignant repolarisation disorder characterised by short QT intervals. We present a previously asymptomatic 14-year-old male patient with negative family history, who suffered a sudden cardiac arrest while playing basketball and diagnosed with short QT syndrome to make emphasis on the fact that although very rare patients with this syndrome may experience cardiac arrest during exercise.

Giant intracardiac blood cyst of the mitral valve in a pediatric patient: A case report.

Intracardiac blood cysts are rare congenital malformations most commonly located in the endocardium of semilunar or atrioventricular valves. They are predominantly seen in infants, especially below 2 months of age, and disappear spontaneously in the first 6 months of life making the detection of blood cysts a rare finding in the adult population. Although most patients are asymptomatic, they may occasionally present with obstruction, valvular dysfunction, or embolism. Herein, we present a 2.5-year-old female patient, who was successfully treated with surgical excision of an intracardiac blood cyst causing mild-to-moderate mitral regurgitation and obstruction.

Transcatheter closure of aortopulmonary window with Amplatzer duct occluder II: additional size.

Aortopulmonary window is a rare cardiac defect, and early management with surgery or transcatheter closure is lifesaving. Here, a 9-month-old patient, who underwent a successful device closure with additional size-Amplatzer duct occlude, is presented to make emphasis that it may be considered as the device of choice for defects in close proximity to aortic valve and/or coronary ostium.

Acute rheumatic fever presenting with severe endocarditis involving four valves, and ventricular tachycardia.

Acute rheumatic fever is the most commonly acquired heart disease in developing countries. The most common cardiac presentation is valvular disease. Although some rhythm disturbances may occur during the acute stages of the disease, ventricular tachycardia is extremely rare. Here, a case of acute rheumatic fever with severe endocarditis involving four valves and ventricular tachycardia is presented.

Isolated cardiac hydatid cyst mimicking myocardial ischaemia in a female patient.

Hydatid disease is a zoonotic parasitic infection endemic in livestock-raising countries. Isolated cardiac hydatid cyst is a very rare disease, and chest pain, palpitations, cough, and dyspnoea are the most common presenting symptoms. Here a case of isolated cardiac hydatid cyst in a female patient with chest pain and electrocardiographic changes mimicking myocardial ischaemia is presented.

Hypertension and improved left ventricular mass index in children after renal transplantation.

This study was conducted to evaluate the changes in BP and LVH after the transplantation and to evaluate the effect of BP changes in LVH. Forty-three pediatric renal transplant patients, with a mean age of 16.99 ± 3.88 years, were enrolled in this study. Twenty-three (53.5%) of the patients were male. Medical records for pretransplantation period (closest to the time of transplantation) and for post-transplantation period (9-12 months after transplantation) were reviewed. All the patients had BP measurements and echocardiographic evaluation in pre- and post-transplantation period. Hypertension was defined as an average systolic and/or diastolic BP that is ≥95th percentile for sex, age, and height. Although the number of patients with hypertension increased from 30 (69.76%) to 35 (81.4%), the number of patients with LVH decreased from 19 (44.1%) to 9 (20.9%) after the transplantation. Although the only significant difference in BP measurements was between the mean Z scores of 24 hour and nighttime mean DBP before and after the transplantation; the mean LVMI, and the prevalence of LVH was significantly lower after the transplantation. There was no significant correlation between the LVMI and the BP measurements. Even though hypertension may persist, there is significant improvement in LVH after renal transplantation.

Necrotizing Pneumonia Caused by H1N1 Virus in a Child with Total Anomalous Pulmonary Venous Connection after Cardiac Surgery.

Total anomalous pulmonary venous connection is a rare form of congenital heart disease, occurring in only 1.5% of children with congenital heart disease. Although the mortality and morbidity of total anomalous pulmonary venous connection have decreased dramatically due to improvements in surgery, postoperative pulmonary venous obstruction is still a cause of late mortality in patients with corrected total anomalous pulmonary venous connection. Influenza A H1N1, the most common cause of human influenza in 2009, may cause pneumonia presenting with increased disease severity. Herein we have presented a well-documented case of necrotizing H1N1 pneumonia mimicking postoperative pulmonary venous obstruction in a 4-month-old patient with surgically corrected total anomalous pulmonary venous connection.

School Performance and Neurodevelopment of Very Low Birth Weight Preterm Infants: First Report From Turkey.

Very low birth weight preterm infants are under significant risk of neurologic, developmental, and somatic problems. In this study, 90 infants born with a birth weight <1500 g and/or with a gestational age <32 weeks were evaluated after the first year of elementary school to assess neurodevelopment. The Wechsler Intelligence Scale for Children-Revised (WISC-R) test, Pediatric Symptom Checklist, and Parent Evaluation of Developmental Status were performed. Mental retardation, cerebral palsy, blindness, epilepsy, and posthemorrhagic hydrocephaly incidences were 14%, 7%, 2%, 5%, and 2%, respectively. The WISC-R score of 32 patients (35.5%) were below 85. Perinatal asphyxia, abnormal neurologic examination, and delayed or impaired speech correlated significantly with low WISC-R scores. Education and income of the father had positive impact on WISC-R scores (P = .042 and P = .026). Parents' concern and presence of cognitive problems were correlated (P = .026). Environmental factors, as well as the prevention of morbidity, affected school performance positively.

Early malfunction of polyvinyl alcohol membrane of septal occluder.

Percutaneous device closure of atrial septal defect (ASD) is now considered the choice of treatment. Numerous devices with advantages/disadvantages are currently available and under development. Cardia Ultrasept II ASD occluder has a nitinol frame covered with polyvinyl alcohol (PVA) membrane. Here, a well-documented case of early malfunction of PVA membrane detected in the first week of implantation in a 4-year-old male patient, who underwent an uneventful device closure with 20 mm Cardia Ultrasept II ASD Occluder, is presented. One week after implantation left-to-right shunt through the device was detected and the explantation of device revealed PVA membrane with multiple perforations.

Recurrent Kawasaki disease in a child with retropharyngeal involvement: a case report and literature review.

Kawasaki disease (KD) is a multisystemic vasculitic disease. Recurrent KD is rare and generally presents in a similar clinical picture as the first episode, and early diagnosis with prompt treatment is the key point in preventing associated cardiovascular morbidities. A 9-year-old boy, who was diagnosed with KD when he was 1.5 years' old, was referred to our hospital for surgical drainage of retropharyngeal abscess. He had a 7-day history of high fever, sore throat, left-sided neck swelling, and restricted neck movements. Subsequently, he was diagnosed with recurrent KD and retropharyngeal involvement. He was successfully treated with a single dose of intravenous immunoglobulin (IVIG) and acetyl salicylic acid. Recurrence is rare and occurs most commonly in children. Atypical presentation, incomplete disease, short duration of fever, and reduced response to IVIG treatment were found to be the risk factors for recurrence. KD can occasionally present with clinical and radiographic findings of deep neck bacterial infection. Unusual presentations in KD may cause delay in diagnosis and increase the risk of life-threatening complications. We describe a case of recurrent KD presenting with a clinical picture resembling retropharyngeal infection who fully recovered after 1 dose of IVIG instead of surgical drainage and antibiotic use.