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.

Multiple intra-cardiac masses: A life-threatening complication of Behçet's disease.

The incidence of multiple intra-cardiac masses is rare, and the differential diagnosis may be challenging sometimes. The most common etiologies of multiple intra-cardiac masses are thrombus, vegetation, and tumors. Intra-cardiac thrombus is more common in patients with central catheters, with a wall-motion abnormality and global hypokinesis. Certain autoimmune disorders may favor the development of intra-cardiac thrombus, and very rarely, multiple thrombi may be seen. Here, a patient with multiple intra-cardiac masses in the right ventricle and diagnosed with Behçet's disease is presented.

Echocardiographic screening for rheumatic heart disease in Turkish schoolchildren.

BACKGROUND: The aim of this study is to investigate the prevalence of subclinical rheumatic heart disease in schoolchildren aged 5-18 by using portable echocardiography in Ankara, Turkey. METHODS: The portable echocardiography screening was performed by a paediatric cardiologist for all of the cases. The mean age of 2550 healthy students was 11.09 ± 2.91 years (1339 females, 1211 males) in three private and three public schools. Echocardiographic studies were assessed according to 2012 World Heart Federation criteria for rheumatic heart disease. RESULTS: After reviewing the echocardiographic images, 73 students were reevaluated by an advanced echocardiography device in the university hospital. Evidence of definite subclinical rheumatic heart disease was found in 39 students (15/1000) and borderline rheumatic heart disease in 20 students (8/1000). No children had any clinical symptoms. The mean age of children diagnosed with rheumatic heart disease and borderline rheumatic heart disease is 12.4 and 11.4 years, respectively. The risk of rheumatic heart disease was found to be increased sevenfold in girls between 14 and 18 years. We surprisingly observed that the prevalence of definite rheumatic heart disease in private schools located in high-income areas of Ankara was higher than that in public schools. CONCLUSIONS: This is the first and largest single-centred echocardiographic screening study for subclinical rheumatic heart disease in Turkish schoolchildren. The frequency of rheumatic heart disease has been found to be 15/1000. This finding is similar to those of recent echocardiographic screening studies performed in middle and high-risk populations. We conclude that to decrease the burden of rheumatic heart disease, echocardiographic screening studies are necessary, and long-term follow-up of children with echocardiographically diagnosed subclinical rheumatic heart disease is needed.

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.

Acute kidney injury in congenital cardiac surgery: Pediatric risk-injury-failure-loss-end-stage renal disease and Acute Kidney Injury Network.

BACKGROUND: Acute kidney injury (AKI) is associated with an increased risk of mortality, especially in pediatric intensive care units. The aim of this study was to determine the risk factors of AKI in children undergoing cardiac surgery for congenital heart disease and to compare two different classification systems: pediatric risk-injury-failure-loss-end-stage renal disease (pRIFLE) and Acute Kidney Injury Network (AKIN). METHODS: We retrospectively analyzed 145 patients undergoing pediatric congenital heart surgery who were between 1 month and 18 years of years, and treated at a cardiovascular surgery department from January 2009 to October 2011. RESULTS: One hundred and thirty-seven patients (mean age, 36.6 ± 43.3 months) were enrolled: 84 (61.3%) developed AKI according to the pRIFLE criteria (25.5%, risk; 20.4%, injury; 15.3%, failure); and 65 patients (47.4%) developed AKI according to the AKIN criteria (15.3%, stage I; 18.2%, stage II; and 13.9%, stage III). Children younger than 11 months were more likely to develop AKI (P < 0.005). Longer cardiopulmonary bypass time was associated with an increased risk of AKI (P < 0.05). pRIFLE identified AKI more frequently than AKIN (P < 0.0005). pRIFLE may help in the early identification of patient at risk for AKI and seems to be more sensitive in pediatric patients (P < 0.05). Any degree of AKI in both classifications was associated with increased mortality (pRIFLE: OR, 15.1; AKIN: OR, 11.2; P = 0.007). CONCLUSION: pRIFLE identified AKI more frequently than the AKIN criteria. pRIFLE identified patients at risk for AKI earlier, and was more sensitive in pediatric patients. Any degree of AKI in both classifications was associated with increased mortality. Both sets of criteria had the same association with mortality.

Predictors of Early Extubation After Pediatric Cardiac Surgery: A Single-Center Prospective Observational Study.

This prospective, observational, single-center study aimed to determine the perioperative predictors of early extubation (<24 h after cardiac surgery) in a cohort of children undergoing cardiac surgery. Children aged between 1 month and 18 years who were consecutively admitted to pediatric intensive care unit after cardiac surgery for congenital heart disease between January 2012 and June 2014. Ninety-nine patients were qualified for inclusion during the study period. The median duration of mechanical ventilation was 20 h (range 1-480), and 64 patients were extubated within 24 h. Four of them failed the initial attempt at extubation, and the success rate of early extubation was 60.6 %. Older patient age (p = .009), greater body weight (p = .009), absence of preoperative pulmonary hypertension (p = .044), lower RACHS-1 category (OR, 3.8; 95 % CI 1.35-10.7; p < .05), shorter cardiopulmonary bypass (p = .008) and cross-clamp (p = .022) times, lower PRISM III-24 (p < .05) and PELOD (p < .05) scores, lower inotropic score (p < .05) and vasoactive-inotropic score (p < .05), and lower number of organ failures (OR, 2.26; 95 % CI 1.30-3.92; p < .05) were associated with early extubation. Our study establishes that early extubation can be accomplished within the first 24 h after surgery in low- to medium-risk pediatric cardiac surgery patients, especially in older ones undergoing low-complexity procedures. A large prospective multiple institution trial is necessary to identify the predictors and benefits of early extubation and to facilitate defined guidelines for early extubation.

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.

Mutations in OTOGL, encoding the inner ear protein otogelin-like, cause moderate sensorineural hearing loss.

Hereditary hearing loss is characterized by a high degree of genetic heterogeneity. Here we present OTOGL mutations, a homozygous one base pair deletion (c.1430 delT) causing a frameshift (p.Val477Glufs(∗)25) in a large consanguineous family and two compound heterozygous mutations, c.547C>T (p.Arg183(∗)) and c.5238+5G>A, in a nonconsanguineous family with moderate nonsyndromic sensorineural hearing loss. OTOGL maps to the DFNB84 locus at 12q21.31 and encodes otogelin-like, which has structural similarities to the epithelial-secreted mucin protein family. We demonstrate that Otogl is expressed in the inner ear of vertebrates with a transcription level that is high in embryonic, lower in neonatal, and much lower in adult stages. Otogelin-like is localized to the acellular membranes of the cochlea and the vestibular system and to a variety of inner ear cells located underneath these membranes. Knocking down of otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the inner ear, supporting that otogelin-like is essential for normal inner ear function. We propose that OTOGL mutations affect the production and/or function of acellular structures of the inner ear, which ultimately leads to sensorineural hearing loss.

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.

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.

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.

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.

Heart rate variability during asymptomatic periods in children with recurrent neurocardiogenic syncope.

Neurocardiogenic syncope (NS) is a common clinical problem during childhood. In order to characterize the autonomic profile in syncopal children, we evaluated heart rate variability (HRV) of 27 patients, ages 8-17 years, in an asymptomatic period. HRV was calculated over a 24-hour (h) period for the time-domain indices and frequency-domain indices. The obtained results were compared to values in 27 healthy children, ages 10-17 years. Patients with syncope did not have significantly different HRV values in comparison to healthy children. After behavior modification and education, HRV assessment was repeated six months later in the symptomatic group under follow-up, and we found no significantly different values. We conclude that there was no significant difference in baseline autonomic values between children with NS and the healthy control group. After behavior modification and education, none of the patients with NS under follow-up experienced syncope or presyncope during the follow-up period. The major complaint had disappeared but did not reflect the HRV values. There was no significant difference in baseline HRV values of the patients in follow-up. We conclude that patients are predisposed to faint by a triggering autonomic stress, without having constant autonomic instability.

Cardiovascular findings in a boy with arterial tortuosity syndrome: case report and review of the literature.

Arterial tortuosity syndrome (ATS) is a rare hereditary, autosomal recessive, connective tissue disorder. Herein, we describe a five-year-old boy. He had hyperextensible skin, atypical facial features and inguinal hernia. We present his vascular imaging studies of kinking and tortuosity of the aorta and bilateral multiple peripheral pulmonary artery stenoses determined by conventional and magnetic resonance angiographic (MRA) examination. ATS must be considered in patients with connective tissue condition associated with diffuse arterial changes and involvement of the skin, joints and other organs. Vascular imaging studies, especially MRA, are useful in the screening of these vascular abnormalities.

Echocardiographic screening for mitral valve prolapse in Turkish school children.

We determined the frequency of mitral valve prolapse (MVP) in healthy Turkish school children using the current echocardiographic diagnostic criteria. This epidemiological survey was performed on 2550 school children. All children were screened with echocardiography and the family of children with MVP were also screened. The prolapse of mitral leaflets into left atrium ≥ 2 mm in parasternal long-axis view was used as diagnostic criteria. MVP was classified as classical or non-classical according to anterior mitral leaflet thickness. The thickness of anterior mitral leaflet, the extent of prolapse, and the presence of mitral regurgitation were evaluated. The children were also questioned about the associated symptoms. The prevalence of MVP was 1.25% in children with a mean age of 11.1 ± 2.9 years. The prevalence was 0.9%, 1.2%, and 1.6% in 5-9 years, 10-13 years, and 14-18 years of age, respectively. 43.7% of the cases were classical MVP. The frequency of auscultation findings was 34.3%. 11/34 children had mitral regurgitation. There was no statistically significant difference between classical MVP and non-classical MVP in terms of mitral regurgitation, physical examination findings, and symptoms. Anxiety (37.5%) was the most common symptom. The frequency of MVP in the first-degree relatives of children with MVP was 11/84 (13.1%). Most patients with MVP don't have auscultation findings and symptoms, therefore echocardiography is an important tool in the diagnosis of MVP. It is also reasonable to screen first degree relatives of MVP patients with echocardiography.

Impact of peritoneal transport characteristics on cardiac function in paediatric peritoneal dialysis patients: a Turkish Pediatric Peritoneal Dialysis Study Group (TUPEPD) report.

BACKGROUND: The peritoneal equilibration test (PET) is recommended in paediatric peritoneal dialysis (PD) patients to assist prescription management. Despite contradictory reports, high transporter status is associated with reduced survival rate in adults. Since cardiac disease is one of the main causes of mortality in paediatric PD patients, we aimed to evaluate whether transport features have any effect on biochemical data and cardiac function in this group. METHODS: One hundred and ten PD patients (13 +/- 5 years, PD vintage: 31 +/- 27 months) were enrolled into the study. Four-hour dialysate/plasma creatinine ratio was used for differentiating PET groups. Thirty-eight patients were high transporters, 29 were high-average transporters and 43 were low-average/low transporters. Echocardiography was performed in all subjects. RESULTS: Age, PD vintage, dialysate glucose concentration, ultrafiltration volume, urine volume and blood pressure levels were similar in all PET groups. No biochemical or echocardiographic data (ejection fraction, fractional shortening, left ventricular mass index, myocardial performance index, power Doppler E/tissue Doppler E ratio reflecting diastolic function) were different among PET groups except lower albumin (P = 0.025) levels in high transporters and higher high-sensitivity C-reactive protein (P = 0.026) levels in high and high-average transporters compared to other transport groups. CONCLUSIONS: Cardiac structural and functional abnormalities are highly prevalent among paediatric PD patients. Transport rates did not have a significant effect on biochemical parameters or cardiac structural/functional parameters. It might be suggested that being a high transporter does not provide a disadvantage in terms of atherogenic tendency and cardiac disease in paediatric PD patients. Oligoanuria, anaemia and hypertension were independent predictors of cardiac disease.

Amiodarone-induced thyrotoxicosis in children and adolescents is a possible outcome in patients with low iodine intake.

BACKGROUND/OBJECTIVE: The identification of the different subtypes of amiodarone-induced thyrotoxicosis (AIT) may provide a rational basis for the choice of the appropriate medical treatment. The aim of this study was to evaluate differential diagnosis and treatment regimens of AIT in children and adolescent. PATIENTS: We reported 3 patients: A 6.7 years old boy with type I AIT; a 17.9 years old girl with type II AIT and a 14.6 years old girl with mixed type AIT. CONCLUSIONS: AIT is not an uncommon complication in countries with low iodine intake. AIT can be asymptomatic and can occur at any time in patients receiving amiodarone therapy. It is also very important to distinguish the type of AIT when planning therapy. Steroid therapy should be started when findings indicate type II or mixed-type AIT. Beta blockers may prevent heart thyrotoxicosis and recurrence of primary arrhythmia if amiodarone is discontinued.