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PURPOSE: Congenital aortic stenosis is a common pathology in the childhood age group and its clinical spectrum varies between asymptomatic and severe heart failure. In our study, we planned to evaluate the long-term results of patients who underwent balloon aortic valvuloplasty (BAV) due to critical aortic valve stenosis in our clinic. MATERIALS AND METHODS: Patients aged 0-18 years who underwent aortic balloon valvuloplasty due to aortic stenosis in our clinic between January 2002 and January 2022 were retrospectively evaluated. RESULTS: Among the 48 patients who underwent balloon valvuloplasty due to aortic stenosis, 13 (27%) were female, and 35 (73%) were male. The median age at the time of the procedure was 27.5 months (IQR: 4-96), the median weight was 9.9 kg (IQR: 5.40-29.50), and the median height was 79 cm (IQR: 54-133). The median follow-up duration was 93.5 months (IQR: 38-132). Angiographic assessments in all patients revealed a median left ventricular pressure of 160 mmHg (IQR: 140-200) and a median pressure gradient between the left ventricle and the aorta of 60 mmHg (IQR: 42-80). The median balloon diameter used was 10 mm (IQR: 8-12). Post-procedural measurements showed a median mean gradient of 30 mmHg (IQR: 20-35) between the left ventricle and the aorta. The procedure was successful in 45 (93.5%) patients. During follow-up, 11 patients required surgical intervention. Ross procedure was performed in five patients, homograft in five patients, and mechanical valve implantation in one patient. Risk factors for the need for surgical intervention were evaluated in detail. During the follow-up, the risk factor for intervention was determined to be aortic insufficiency. CONCLUSION: Aortic valve balloon valvuloplasty is a safe and successful treatment method for critical aortic stenosis. It should be the first choice of treatment option in suitable patients.
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PURPOSE: Transcatheter closure is the first-line treatment option for hemodynamically significant secundum atrial septal defects (ASDs). This study examines our center's experience with this procedure over the last 15 years. MATERIALS AND METHODS: Pediatric patients aged 0-18 years with secundum ASDs who were planned for transcatheter closure in our clinic between January 2007 and January 2023 were retrospectively evaluated. RESULTS: Transcatheter secundum ASD closure was planned for a total of 334 patients during the study period: 191 girls (57.2%) and 143 boys (42.8%). Their mean age was 8.08±3.9 years, and their mean weight was 30±15.6 kg. Defect diameter measured transesophageally ranged from 5 to 35 mm, with a mean of 12.56±4.02 mm. Transesophageal echocardiographic examination revealed a single secundum ASD in 319 patients (95.5%) and multiple secundum ASDs in 15 patients (4.5%). In 11 patients (3.3%), the procedure was terminated before initiating transcatheter ASD closure because of insufficient vena cava rims or a very large or multi-fenestrated defect. The 323 patients (96.7%) who underwent transcatheter ASD closure had a mean pulmonary artery pressure of 15.1±4.0 mmHg and a mean Qp/Qs ratio of 1.97±0.56. The procedure failed in four patients (1.3%) because of device embolization (n=2) or the inability to properly position the device (n=2). Major complications other than device embolization observed during or after transcatheter closure included anesthetic-induced respiratory depression (n=1) and total atelectasis of the lung (n=1). No new major complications were detected during the patients' long-term follow-up. CONCLUSION: With appropriate patient and device selection, transcatheter closure is a safe and effective treatment for secundum ASD and should be the first treatment of choice.
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OBJECTIVES: Osteogenesis imperfecta (OI) is a disease caused by defective collagen synthesis. Collagen type 1 is found in many structures in the cardiovascular system. Endothelial dysfunction, which develops prior to the emergence of structural and clinical signs of atherosclerosis, is believed to play a key role in atherogenesis. Endothelial dysfunction may be detected presymptomatically by non-invasive radiologic methods, such as flow-mediated dilatation (FMD) and carotid intima-media thickness (CIMT). These modalities may provide early indicators of endothelial dysfunction. This cross-sectional comparative study aimed to investigate early-stage radiological markers of endothelial dysfunction and cardiovascular diseases in OI patients and healthy controls and to investigate the correlation of findings with OI genotype. METHODS: Thirty patients diagnosed with OI were paired with thirty healthy age- and gender-matched controls and echocardiogram findings were compared. RESULTS: None of the patients had known underlying cardiovascular disease. The mean age was 13.18 ± 2.91 years. According to Sillence classification, 15 patients had type 1 OI, 10 had type III, and 5 had type IV. Mean CIMT in the OI group was higher in the control group (OI group: 0.42 ± 0.06 vs. healthy controls: 0.34 ± 0.04 mm, p<0.01), and mean FMD percent was lower in the patient group (p<0.01). Left ventricular ejection fraction was 78.97 ± 10.32 vs. 77.56 ± 8.50â¯%, (OI group: 7.00 ± 3.06 vs. healthy controls: 12.14 ± 1.99, p=0.56), and fractional shortening was 42.68 ± 11.94 vs. 40.23 ± 7.99â¯%, (p=0.35), in OI patients and controls, respectively. CONCLUSIONS: Pediatric patients with OI without clinical signs of cardiovascular abnormality had significantly worse CIMT and FMD findings than healthy controls. However, no difference was determined when comparing left ventricular ejection fraction or fractional shortening. OI patients may need to be screened for cardiovascular system complications starting from an early age.
Subject(s)
Cardiovascular Diseases , Osteogenesis Imperfecta , Humans , Child , Adolescent , Osteogenesis Imperfecta/complications , Osteogenesis Imperfecta/diagnostic imaging , Osteogenesis Imperfecta/genetics , Case-Control Studies , Stroke Volume , Carotid Intima-Media Thickness , Cross-Sectional Studies , Ventricular Function, Left , Collagen Type I , Cardiovascular Diseases/etiology , Cardiovascular Diseases/diagnosis , Genetic Association StudiesABSTRACT
OBJECTIVES: Cardiac manifestations of the coronavirus disease 2019 (COVID-19) have mainly been reported in adults. Therefore, we aimed to determine the electrocardiographic abnormalities in hospitalised paediatric patients with COVID-19 and multisystemic inflammatory syndrome in children. METHODS: We retrospectively evaluated hospitalised paediatric patients <18 years of age with a diagnosis of COVID-19 (n = 168) and multisystem inflammatory syndrome in children (n = 48) between March 2021 and December 2021. A daily electrocardiography was performed for the patients who had electrocardiographic abnormalities on admission or developed electrocardiographic abnormality on the follow-up. The characteristics of these patients, underlying predisposing conditions, and clinical course were also examined. RESULTS: Two-hundred sixteen paediatric patients (55% were male) with a mean age of 10.7 ± 4.69 years were evaluated. There was an underlying disease in 84 (38.8%) patients and 51 (23.6%) required paediatric ICU admission. Electrocardiography abnormality was detected in 12 (5.5%) which were as follows: 7 (3.2%) had sinus bradycardia, 3 (1.4%) patients had transient ST elevation and concomitant T negativity, and 2 (0.9%) developed first-degree Atrioventricular (AV) block. The median time from the onset of disease symptoms to detecting electrocardiographic abnormality was 9 days. Electrocardiographic abnormalities returned to normal uneventfully 3 days later. CONCLUSIONS: The prevalence of arrhythmia in paediatric patients with COVID-19 was detected in 5.5% of the patients. While two-thirds of the electrocardiography abnormalities were sinus bradycardia, ST elevation was remarkable (1.4%). Clinicians should be aware of electrocardiographic abnormalities and consider electrocardiographic monitoring in paediatric patients with COVID-19 and multisystemic inflammatory syndrome in children.
Subject(s)
Atrioventricular Block , COVID-19 , ST Elevation Myocardial Infarction , Adult , Humans , Male , Child , Adolescent , Female , COVID-19/complications , COVID-19/diagnosis , Bradycardia , Retrospective Studies , Child, Hospitalized , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/epidemiology , Arrhythmias, Cardiac/etiology , Atrioventricular Block/diagnosis , Electrocardiography , SyndromeABSTRACT
Multisystem Inflammatory Syndrome (MIS-C) is a new entity that emerges 2-4 weeks after the SARS-CoV-2 infection in children. MIS-C can affect all systems, the most severe of which is cardiac involvement. The duration of the cardiac symptoms is still uncertain and may be persistent or prolonged. The American College of Rheumatology Clinical Guidelines recommends cardiac magnetic resonance imaging (MRI) 2-6 months after the diagnosis of MIS-C in patients presenting with significant transient left ventricular (LV) dysfunction in the acute phase of illness (LV ejection fraction 50%) or persistent LV dysfunction. There are a few studies investigating cardiac MRI findings in MIS-C patients. In this study, we aimed to evaluate cardiac MRI findings, at the earliest 3 months after diagnosis, and compare these findings with the echocardiograms in children with MIS-C. A retrospective study including 34 MIS-C patients was conducted at a tertiary-level University Hospital between June 2020 and July 2021. Centers for Disease Control and Prevention criteria were used in the diagnosis of MIS-C. Cardiac MRI was performed at least 3 months after MIS-C diagnosis. The study included 17 (50%) boys and 17 (50%) girls with a mean age of 9.31 ± 4.72 years. Initial echocardiographic evaluation revealed cardiac abnormality in 13 (38.2) patients; 4 (11.8%) pericardial effusion, 4 (11.8%) left ventricular ejection fraction (LVEF) < 55%, and 5 (14.7%) coronary artery dilatation. Echocardiography showed normal LV systolic function in all patients during follow-up; coronary dilatation persisted in 2 of 5 (40%) patients at the 6th-month visit. Cardiac MRI was performed in 31 (91.2%) patients, and myocardial hyperemia was not detected in any patients (T1 relaxation time was < 1044 ms in all children). However, 9 (29%) patients' MRI showed isolated elevated T2 levels, and 19 (61.3%) revealed at least one of the following findings: pericardial effusion, right ventricular dysfunction, or LVEF abnormality. In patients with MIS-C, a high rate of cardiac involvement, particularly pericardial effusion was determined by cardiac MRI performed at the earliest 2-6 months after diagnosis. Even if echocardiography does not reveal any abnormality in the initial phase, cardiac MRI should be suggested in MIS-C patients in the late period. This is the first study reporting cardiac MRI findings in the late period of MIS-C patients.
Subject(s)
COVID-19 , Pericardial Effusion , Ventricular Dysfunction, Left , Male , Female , Humans , Child , Child, Preschool , Adolescent , Stroke Volume , Retrospective Studies , Ventricular Function, Left , SARS-CoV-2 , Magnetic Resonance Imaging , Ventricular Dysfunction, Left/diagnostic imagingABSTRACT
Friedreich's ataxia (FA) is a rare, progressive, and degenerative hereditary disorder caused by a deficiency of frataxin protein. This disease is characterised by severe neurological dysfunction and life-threatening cardiomyopathy. Various drugs are used to slow down / stop the neurodegenerative progress. However, recent clinical trials and animal experiments demonstrate that interferon-gamma (IFN-É£) treatment might improve signs of FA as well. A 9-year-old girl was admitted to our hospital with gait instability, mild dysarthria, and sensorimotor polyneuropathy. Her genetic examination was consistent with FA. IFN-É£ treatment was started 3 times a week. The treatment was evaluated by physical examination and side effects assessment. Friedreich Ataxia Rating Scale (FARS), 9-hole peg test (9HPT), and time of 25-foot walk (T25FW) were measured. Ataxia and cerebellar findings improved within 9 months. Although clinical neurological improvement was achieved, there was no improvement in cardiomyopathy. Key Words: Interferon-gamma, Friedreich ataxia, FARS, Children, Cardiomyopathy.
Subject(s)
Cardiomyopathies , Friedreich Ataxia , Walking/physiology , Cardiomyopathies/drug therapy , Cardiomyopathies/etiology , Female , Friedreich Ataxia/complications , Friedreich Ataxia/diagnosis , Friedreich Ataxia/drug therapy , Humans , Interferon-gamma/therapeutic useABSTRACT
INTRODUCTION: We searched for risk factors of cardiovascular assessment among children on dialysis. METHODS: This is a cross-sectional study of cardiovascular assessment of all patients on dialysis at Ege University Children's Hospital. Pediatric patients between the ages of 6 and 21 who were on HD and peritoneal dialysis treatment were included in the study. Cardiovascular evaluation included left ventricular mass index (LVMI), pulse wave velocity (PWV), and carotid intima-media thickness (cIMT) measurements. RESULTS: Nineteen patients were included in this study. The LDL had a correlation with the cIMT z-score, but not to PWVz-score. Binary Logistic regression analysis found that only LDL was significantly associated to increased cIMT. CONCLUSION: This study reports an association between high LDL and high BP increased cIMT on dialyzed children. Strategies to reduce LDL and BP in dialysis patients may prevent vasculopathy and long-term cardiovascular complications.
Subject(s)
Cardiovascular Diseases , Renal Dialysis , Adolescent , Child , Humans , Young Adult , Blood Pressure/physiology , Cardiovascular Diseases/epidemiology , Carotid Intima-Media Thickness , Cross-Sectional Studies , Lipoproteins, LDL , Pulse Wave Analysis , Renal Dialysis/adverse effects , Risk FactorsABSTRACT
La enfermedad de Tay-Sachs es una enfermedad metabólica hereditaria neurodegenerativa. Existen cuatro tipos según el inicio de los síntomas clínicos: infantil, infantil de inicio tardío, juvenil y adulto. El tipo infantil tiene el peor pronóstico. Recientemente, se describieron diferentes anomalías que acompañan a los trastornos metabólicos e influyen en el pronóstico. Presentamos el caso de un lactante con enfermedad de Tay-Sachs junto con coartación aórtica y reflujo vesicoureteral bilateral (RVU) de grado V. Se realizó el seguimiento del paciente en el consultorio externo de Cardiología Pediátrica. En la ecografía abdominal, se observó ectasia pielocalicial, y se detectó reflujo vesicoureteral bilateral de grado V en la cistouretrografía miccional. No se ha informado previamente la coexistencia de estas anomalías. Este caso pone de manifiesto que no se deben subestimar las anomalías del examen neurológico en los pacientes con una cirugía cardíaca reciente, porque podría perderse la oportunidad de diagnosticar enzimopatías congénitas.
Tay-Sachs disease is a neurodegenerative inherited metabolic disease. There are four forms classified by the time of first clinical symptoms: infantile, late infantile, juvenile and adult. Infantile form has the poorest prognosis. Lately, different abnormalities which accompany metabolic disorders and affect the prognosis have been described. We present an infant with Tay-Sachs disease accompanied by coarctation of the aorta and bilateral grade V vesicoureteral reflux (VUR). The patient was followed up in the outpatient clinic of Pediatric Cardiology. The abdominal ultrasonography showed pelvicalyceal ectasia; bilateral grade V VUR in voiding cystourethrography was found. This coexistence has not been previously reported. This case emphasizes that abnormalities in the neurological examination of cardiac postsurgical patients should not be underestimated because the opportunity to diagnose inborn errors of metabolism could be missed.
Subject(s)
Humans , Male , Infant , Aortic Coarctation/complications , Aortic Coarctation/diagnosis , Tay-Sachs Disease/diagnosis , Vesico-Ureteral Reflux/complications , Vesico-Ureteral Reflux/diagnosisABSTRACT
Tay-Sachs disease is a neurodegenerative inherited metabolic disease. There are four forms classified by the time of first clinical symptoms: infantile, late infantile, juvenile and adult. Infantile , Ebru Candab, Ertürk Leventc , The infantile form has the poorest clinical prognosis. First symptoms of this form, such as muscle weakness and hypotonia, occur around form has the poorest prognosis. Lately, different abnormalities which accompany metabolic disorders and affect the prognosis have been described. We present an infant with Tay-Sachs disease accompanied by coarctation of the aorta and bilateral grade V vesicoureteral reflux (VUR). The patient was followed up in the outpatient clinic of Pediatric Cardiology. The abdominal ultrasonography showed pelvicalyceal ectasia; bilateral grade V VUR in voiding cystourethrography was found. This coexistence has not been previously reported. This case emphasizes that abnormalities in the neurological examination of cardiac postsurgical patients should not be underestimated because the opportunity to diagnose inborn errors of metabolism could be missed.
La enfermedad de Tay-Sachs es una enfermedad metabólica hereditaria neurodegenerativa. Existen cuatro tipos según el inicio de los síntomas clínicos: infantil, infantil de inicio tardío, juvenil y adulto. El tipo infantil tiene el peor pronóstico. Recientemente, se describieron diferentes anomalías que acompañan a los trastornos metabólicos e influyen en el pronóstico. Presentamos el caso de un lactante con enfermedad de Tay-Sachs junto con coartación aórtica y reflujo vesicoureteral bilateral (RVU) de grado V. Se realizó el seguimiento del paciente en el consultorio externo de Cardiología Pediátrica. En la ecografía abdominal, se observó ectasia pielocalicial, y se detectó reflujo vesicoureteral bilateral de grado V en la cistouretrografía miccional. No se ha informado previamente la coexistencia de estas anomalías. Este caso pone de manifiesto que no se deben subestimar las anomalías del examen neurológico en los pacientes con una cirugía cardíaca reciente, porque podría perderse la oportunidad de diagnosticar enzimopatías congénitas.
Subject(s)
Aortic Coarctation , Tay-Sachs Disease , Vesico-Ureteral Reflux , Adult , Aortic Coarctation/complications , Aortic Coarctation/diagnosis , Child , Humans , Infant , Tay-Sachs Disease/diagnosis , Vesico-Ureteral Reflux/complications , Vesico-Ureteral Reflux/diagnosisABSTRACT
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare condition. Surgical intervention is indicated in all diagnosed patients. Successful repair is expected to correct left ventricle (LV) functions and heart failure, reduce mitral regurgitation, and resolve infarct patterns observed on electrocardiogram. For this reason, compared to idiopathic dilated cardiomyopathy, ALCAPA is considered a curable form of dilated cardiomyopathy. In this article, we present the case of 3-month-old girl who underwent the Takeuchi procedure for ALCAPA syndrome and developed hypertrophic cardiomyopathy despite expectations of improved LV function.
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Kawasaki disease, known as mucocutaneous lymph node syndrome, is a multi-system disease of unknown aetiology that occurs in young children under 5 years of age. The recurrence rate of Kawasaki disease is as rare as 1-3%. Especially in cases with coronary artery involvement, recurrent Kawasaki disease should be investigated in terms of underlying rheumatologic diseases such as periodic fever syndromes, microscopic polyangiitis, polyarteritis nodosa, and systemic-onset juvenile arthritis. In this study, we report homozygote mutations in mevalonate kinase and familial Mediterranean fever genes in a recurrent Kawasaki disease with coronary dilatation.
Subject(s)
Arthritis, Juvenile , Coronary Aneurysm , Mucocutaneous Lymph Node Syndrome , Child , Child, Preschool , Coronary Vessels , Humans , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/geneticsABSTRACT
Candida endocarditis (CE) is a rare and serious complication of candidemia. Using current diagnostic tools a confirmed diagnosis is often delayed and outcomes remain poor. The majority of new cases occur following cardiac valvular surgery, and/or in patients with the following risk factors: intravenous drug use, cancer chemotherapy, prolonged presence of central venous catheters, and prior history of bacterial endocarditis. It is not a common complication in non-neutropenic patients particularly with the absence of a prosthetic valve, and very few reports in the literature are available. Attempting to add to the limited data, 211 candidemia episodes from 172 nonneutropenic pediatric cases between January 2008 and December 2017 were evaluated. All patients were considered asymptomatic for underlying heart disease. However, 2 (0.9%) patients with a central venous catheter were determined as having endocarditis following echocardiography.
Subject(s)
Candidemia/diagnostic imaging , Endocarditis/diagnostic imaging , Endocarditis/microbiology , Prosthesis-Related Infections/diagnostic imaging , Prosthesis-Related Infections/microbiology , Ventriculoperitoneal Shunt/adverse effects , Antifungal Agents/therapeutic use , Candidemia/drug therapy , Endocarditis/drug therapy , Female , Humans , Hydrocephalus/surgery , Infant , Male , Prosthesis-Related Infections/drug therapyABSTRACT
BACKGROUND: Approximately 25% of congenital heart diseases (CHD) are estimated to be critical and require an intervention. In this study, we aimed to investigate the additional value of peripheral perfusion index (PPI) measurements to pulse oximetry screening for critical CHD (CCHD). METHODS: Infants born at Ege University Hospital between May 2013 and September 2015 were prospectively included in the study. In addition to physical examination, pre- and postductal oxygen saturations and PPI values were measured with a new generation pulse oximeter before discharge from the hospital. RESULTS: A total of 3175 newborns (33 with an antenatal diagnosis of CCHD) were included in the study. With the combination of physical examination, pulse oximetry screening and peripheral perfusion index (PPI) measurements, all newborns with CCHD were detected in our study including three infants without an antenatal diagnosis in whom pulse oximetry screening was negative. CONCLUSION: PPI measurements may be valuable for early detection of obstructive left heart lesions where pulse oximetry screening has limitations in diagnosis.
Subject(s)
Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/physiopathology , Pulsatile Flow/physiology , Early Diagnosis , Female , Humans , Infant , Infant, Newborn , Male , Neonatal Screening , Oximetry , Pregnancy , Prenatal DiagnosisABSTRACT
OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of the Doty surgical approach in pediatric patients with congenital supravalvular aortic stenosis (SVAS) by examining early and mid-term outcomes. METHODS: Surgical repair using the Doty technique was performed in a total of 10 pediatric patients with SVAS between January 2005 and July 2015 at this clinic. These patients were evaluated retrospectively. Demographic characteristics, echocardiographic findings, and clinical outcomes were analyzed. RESULTS: The mean patient age was 4.8±3.9 years. Williams-Beuren syndrome was diagnosed in 4 of the patients. Early mortality was observed in 1 patient with diffuse SVAS. At the final follow-up (mean follow-up: 3.7±1.2 years; range: 6-61 months), echocardiograms revealed a mean pressure gradient of 14±4.2 mmHg. Two patients displayed minimal aortic insufficiency during the follow-up period. No reoperation or reintervention was required. CONCLUSION: The Doty technique is an anatomically and technically effective surgical approach to treating SVAS.
Subject(s)
Aortic Stenosis, Supravalvular/surgery , Aortic Stenosis, Supravalvular/congenital , Aortic Stenosis, Supravalvular/diagnostic imaging , Aortic Stenosis, Supravalvular/mortality , Cardiac Surgical Procedures/methods , Child , Child, Preschool , Coronary Angiography , Echocardiography, Doppler , Female , Humans , Infant , Male , Retrospective Studies , Severity of Illness Index , Treatment Outcome , TurkeyABSTRACT
Background Common variable immunodeficiency is a rare clinically symptomatic primary immunodeficiency disorder which manifests a wide variability of symptoms, complications. Atherosclerosis in common variable immunodeficiency patients has not been investigated yet contrary to other severe clinical complications. We aimed to investigate the chitotriosidase enzyme's role as an inflammation and atherosclerosis marker in paediatric common variable immunodeficiency patients. Methods Common variable immunodeficiency patients (n = 24) and healthy controls (n = 23) evaluated for chitotriosidase activity with other inflammation markers (hsCRP, myeloperoxidase, serum amyloid A, ferritin), lipid profile and echocardiographic findings (carotid artery intima media thickness - cIMT, brachial artery flow-mediated vazodilatation - FMD%). Results In patients, the mean chitotriosidase activity (8.98 ± 6.28) was significantly higher than the controls (5.17 ± 3.42) ( P = 0.014). Chitotriosidase showed positive relation with hs-CRP ( P = 0.011) and SAA ( P = 0.011) but had no relation with ferritin ( P = 0.155), HDL ( P = 0.152) or LDL-cholesterol ( P = 0.380). Mean cIMT increased in patients compared with the controls ( P < 0.001) but did not show any relation with chitotriosidase ( P = 0.546). FMD% decreased in patients ( P < 0.001) also showing no relation with chitotriosidase ( P = 0.298). Ventricular myocardial performance indexes had no significant difference, but RVEF% decreased in patients ( P = 0.043). Conclusions High chitotriosidase activity in common variable immunodeficiency patients demonstrated in vivo the presence of activated macrophages indicating ongoing inflammation. Echocardiographic diastolic functional deficiency, increased cIMT and decreased FMD% may be accepted as early atherosclerotic findings, but none of them showed relationship with chitotriosidase activities.
Subject(s)
Atherosclerosis/complications , Common Variable Immunodeficiency/complications , Common Variable Immunodeficiency/enzymology , Hexosaminidases/metabolism , Biomarkers/metabolism , Case-Control Studies , Child , Chronic Disease , Female , Humans , Inflammation/enzymology , MaleABSTRACT
Background Inflammation and hypercholesterolaemia contribute to atherosclerotic changes which can start in childhood. Children with hyperlipidaemias are at high risk for early coronary atherosclerosis. This study evaluates the relationship between lipoprotein-associated phospholipase A2 (Lp-PLA2), carotid intima-media thickness (CIMT) and flow-mediated dilatation in hypercholesterolaemic dyslipidaemic children. Methods We performed a case-control study consisting of 43 cases, aged 2 to 17 years, and 24 age-matched controls. Fasting blood samples were obtained from both groups for the measurement of a lipid profile (total cholesterol, LDL-C, HDL-C and triglycerides) and Lp-PLA2 in mass units. The latter was determined with a turbidimetric immunoassay method (PlacTest, DiaDexus Inc.) applied to an automated analyser. CIMT and flow-mediated dilatation measurements were undertaken by a paediatric cardiologist, using high-resolution B-mode ultrasonography. Results Total cholesterol, LDL-C and Lp-PLA2 concentrations were significantly higher in the cases than in the controls ( p < 0.001 for all three parameters). While CIMT values were also significantly higher in the patients compared to the controls ( P = 0.001), flow-mediated dilatation values were significantly lower ( P = 0.001). We found positive correlations between Lp-PLA2 and total cholesterol ( r = 0.41, P = 0.001), Lp-PLA2 and LDL-C ( r = 0.36, P = 0.004), Lp-PLA2 and CIMT ( r = 0.44, P = 0.019) and LDL-C and CIMT ( r = 0.41, P = 0.032); there were negative correlations between Lp-PLA2 and flow-mediated dilatation ( r = -0.15, P = 0.045), total cholesterol and flow-mediated dilatation ( r = -0.45, P = 0.017), LDL-C and flow-mediated dilatation ( r = -0.51, P = 0.006) and CIMT and flow-mediated dilatation ( r = -0.45, P = 0.016). Conclusion Lp-PLA2 concentrations are significantly elevated in hypercholesterolaemic dyslipidaemic children. Given the association of Lp-PLA2 with markers of atherosclerosis (total cholesterol, LDL-C, CIMT and flow-mediated dilatation), the finding of increased concentrations of Lp-PLA2 could be used to identify early atherosclerotic changes in hypercholesterolaemic dyslipidaemic children and may inform their clinical management.
Subject(s)
1-Alkyl-2-acetylglycerophosphocholine Esterase/metabolism , Cardiovascular Diseases/complications , Dyslipidemias/complications , Dyslipidemias/enzymology , Hypercholesterolemia/complications , Adolescent , Biomarkers/metabolism , Cardiovascular Diseases/epidemiology , Carotid Intima-Media Thickness , Child , Child, Preschool , Dyslipidemias/blood , Dyslipidemias/diagnosis , Female , Humans , Male , Risk AssessmentABSTRACT
OBJECTIVE: As a highly rare congenital defect, cor triatriatum sinister represents only 0.1% of congenital cardiac anomalies. Depending on the degree of obstruction and the accompanying symptoms, cor triatriatum can be diagnosed at any age. This case series described 5 patients with cor triatriatum sinister who underwent operation. METHODS: Five patients with cor triatriatum sinister were seen at our institution between 2007 and 2013. The demographic characteristics and surgical results of these patients are outlined in this retrospective review. RESULTS: The surgical approach consists of left or right atriotomy, excision of the obstructing membrane, and repair of the associated intracardiac anomalies. After an uneventful postoperative hospital stay, all patients were discharged 5-10 days postoperatively. There were no instances of recurrent constriction after surgical treatment of the cor triatriatum membrane. Patients were followed up for a median of 4 years and were symptom free. CONCLUSION: In the surgical management of this easily and fully treatable congenital cardiac anomaly, it is difficult to determine which atriotomy approach is comparatively more advantageous. However, in the management of cor triatriatum sinister, priority should be given to confirmation of the diagnosis and full resection of the membrane. Thus, the surgeon should not hesitate to perform additional incisions if deemed necessary.
Subject(s)
Cor Triatriatum , Adolescent , Child , Child, Preschool , Cor Triatriatum/diagnostic imaging , Cor Triatriatum/pathology , Cor Triatriatum/surgery , Female , Humans , Infant , Male , Radiography , Retrospective StudiesABSTRACT
A correlation has been clearly shown between inflammation markers and subclinical atherosclerosis markers in the early stages of atherogenesis in subjects with familial hypercholesterolemia (FH). The aim of this study was to investigate potential inflammation markers in the diagnosis of atherosclerosis in children with FH. A total of 48 dyslipidemic children and 24 healthy age-matched control subjects were taken into study. Inflammation and macrophage activation markers (hsCRP, myeloperoxidase, chitotriosidase, YKL-40, TNF-α, IL-6, IL-18, MMP-1 and MMP-9) and lipid parameters of all patients were measured. Carotid intima-media thickness (cIMT) and flow-mediated dilation (FMD) levels were determined. Our data suggested that clinically evidenced (by cIMT and FMD levels) atherosclerosis starts in the early ages in hypercholesterolemic children. Higher cholesterol levels strongly correlated with macrophage activation markers (ChT, YKL-40 and myeloperoxidase). ChT and YKL-40 seem to be the more predictable markers of atherosclerosis even in early ages (<6 years old) than other classical inflammation markers such as hs-CRP, IL-6 and TNF-α.
Subject(s)
Adipokines/blood , Atherosclerosis/diagnosis , Hexosaminidases/blood , Hyperlipoproteinemia Type II/diagnosis , Lectins/blood , Macrophage Activation/physiology , Adolescent , Biomarkers , Blood Flow Velocity , C-Reactive Protein , Carotid Intima-Media Thickness , Chemokine CCL3/blood , Chemokine CCL4/blood , Child , Child, Preschool , Chitinase-3-Like Protein 1 , Endothelium, Vascular , Humans , Hyperlipoproteinemia Type II/blood , Inflammation , VasodilationABSTRACT
BACKGROUND: A limited number of studies have reported various short-term cardiovascular changes in bronchopulmonary dysplasia (BPD) patients in the postsurfactant era. Little is known about the course of these changes in children with BPD. OBJECTIVES: It was the aim of this study to investigate cardiovascular consequences of BPD at preschool ages and to find out possible risk factors related to cardiovascular sequelae. METHODS: Prematurely born children with (n = 21) and without BPD (n = 20) were evaluated with conventional and myocardial tissue Doppler echocardiography at the age of 2-4 years. RESULTS: BPD patients had a decreased pulmonary artery acceleration time and higher left and right ventricular myocardial performance indexes, consistent with higher pulmonary pressures and impaired biventricular systolic and diastolic functions at preschool ages. Low birth weight, disease severity and postnatal cumulative steroid dose were related to these changes. CONCLUSION: Negative effects of BPD on global cardiac performances of both ventricles and pulmonary arterial pressure persist up to preschool ages.
Subject(s)
Bronchopulmonary Dysplasia/complications , Hypertension, Pulmonary/epidemiology , Infant, Low Birth Weight , Premature Birth , Ventricular Dysfunction/epidemiology , Child, Preschool , Echocardiography, Doppler , Familial Primary Pulmonary Hypertension , Female , Follow-Up Studies , Humans , Longitudinal Studies , Male , Postnatal Care , Retrospective Studies , Risk Factors , Severity of Illness Index , Steroids/therapeutic useABSTRACT
OBJECTIVE: Adiponectin and high-sensitivity C-reactive protein (hsCRP) can be used as early biochemical markers of cardiovascular diseases (CVDs). Radiologically, non-invasive flow-mediated dilation (FMD) of the brachial artery and carotid intima-media thickness (CIMT) measurements may be used as indicators in the early diagnosis of CVDs. To compare the biochemical markers of atherosclerosis with radiological markers of CVDs (CIMT, FMD, ventricular systolic and diastolic functions) and to assess the relationship of these parameters with metabolic control in diabetic children and adolescents. METHODS: A total of 55 patients with type 1 diabetes mellitus (T1DM) of at least 5-year duration and 30 healthy subjects were included in the study. Serum adiponectin, hsCRP, hemoglobin A1c (HbA1c), and lipid levels were evaluated in the patients and in the controls. CIMT, FMD, ventricular systolic and diastolic functions were assessed by echocardiography. RESULTS: Mean age of the patients with diabetes was 17.6 years; mean diabetes duration was 10.4 years. Mean serum hsCRP was elevated in children with diabetes (0.21±0.31 vs. 0.10±0.16 µg/mL, p=0.00), while no significant difference from the controls was found in adiponectin levels. Mean CIMT was significantly higher in diabetic children compared to the control group (0.53±0.11 vs. 0.34±0.46 mm, p=0.00). Mean FMD of the diabetic children was significantly lower than that of the controls (6.86±2.85% vs. 12.13±1.99%, p=0.00). Diabetes duration was positively correlated with CIMT and negatively correlated with FMD. Right ventricular (RV) and left ventricular (LV) myocardial performance index (MPI) were higher in the patient group (p=0.00). CONCLUSIONS: Our data suggest that in addition to standard echocardiography, tissue Doppler echocardiography, FMD, and CIMT can be used as early-stage radiological markers and hsCRP as an early-stage biochemical marker of atherosclerosis in the routine follow-up of T1DM patients.