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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.
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COVID-19 , Derrame Pericárdico , Disfunción Ventricular Izquierda , Masculino , Femenino , Humanos , Niño , Preescolar , Adolescente , Volumen Sistólico , Estudios Retrospectivos , Función Ventricular Izquierda , SARS-CoV-2 , Imagen por Resonancia Magnética , Disfunción Ventricular Izquierda/diagnóstico por imagenRESUMEN
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.
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Bloqueo Atrioventricular , COVID-19 , Infarto del Miocardio con Elevación del ST , Adulto , Humanos , Masculino , Niño , Adolescente , Femenino , COVID-19/complicaciones , COVID-19/diagnóstico , Bradicardia , Estudios Retrospectivos , Niño Hospitalizado , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/epidemiología , Arritmias Cardíacas/etiología , Bloqueo Atrioventricular/diagnóstico , Electrocardiografía , SíndromeRESUMEN
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.
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Artritis Juvenil , Aneurisma Coronario , Síndrome Mucocutáneo Linfonodular , Niño , Preescolar , Vasos Coronarios , Humanos , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/genéticaRESUMEN
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.
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Candidemia/diagnóstico por imagen , Endocarditis/diagnóstico por imagen , Endocarditis/microbiología , Infecciones Relacionadas con Prótesis/diagnóstico por imagen , Infecciones Relacionadas con Prótesis/microbiología , Derivación Ventriculoperitoneal/efectos adversos , Antifúngicos/uso terapéutico , Candidemia/tratamiento farmacológico , Endocarditis/tratamiento farmacológico , Femenino , Humanos , Hidrocefalia/cirugía , Lactante , Masculino , Infecciones Relacionadas con Prótesis/tratamiento farmacológicoRESUMEN
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.
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Corazón Triatrial , Adolescente , Niño , Preescolar , Corazón Triatrial/diagnóstico por imagen , Corazón Triatrial/patología , Corazón Triatrial/cirugía , Femenino , Humanos , Lactante , Masculino , Radiografía , Estudios RetrospectivosRESUMEN
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.
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Enfermedades Cardiovasculares , Osteogénesis Imperfecta , Humanos , Niño , Adolescente , Osteogénesis Imperfecta/complicaciones , Osteogénesis Imperfecta/diagnóstico por imagen , Osteogénesis Imperfecta/genética , Estudios de Casos y Controles , Volumen Sistólico , Grosor Intima-Media Carotídeo , Estudios Transversales , Función Ventricular Izquierda , Colágeno Tipo I , Enfermedades Cardiovasculares/etiología , Enfermedades Cardiovasculares/diagnóstico , Estudios de Asociación GenéticaRESUMEN
AIM: Despite a mostly self-limiting course, infantile hemangiomas can cause severe functional and/or cosmetic problems. The aim of this study was to determine the efficiency of propranolol treatment on infantile hemangiomas. METHODS: Sixty-seven infantile hemangioma patients were included in propranolol protocol in two institutions from 2009 to 2011. Participants included 36 boys and 31 girls. An associate protocol with radiology and pediatric cardiology was constructed for appropriate patient selection. Patients received a dose of 2 mg/kg/day, and all were admitted for the first 24 hours of therapy. RESULTS: Sixty-seven patients were included in the study. Mean age at the initiation of therapy was 7 months (1 to 24 months), and eleven patients were older than 12 months of age when propranolol was started. All patients showed improvement with varying responses. No side effects were detected during the treatment. CONCLUSION: Previously defined treatments for hemangiomas were efficient, yet had a limited usage because of side effects. Propranolol, with a high efficacy (not as total involution but stabilization and regression) and feasibility deserves to be the first line therapy for infantile hemangiomas even after the proliferation phase.
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Antagonistas Adrenérgicos beta/uso terapéutico , Hemangioma/tratamiento farmacológico , Propranolol/uso terapéutico , Antagonistas Adrenérgicos beta/administración & dosificación , Antagonistas Adrenérgicos beta/efectos adversos , Preescolar , Estudios de Factibilidad , Femenino , Estudios de Seguimiento , Hemangioma/patología , Humanos , Lactante , Masculino , Propranolol/administración & dosificación , Propranolol/efectos adversos , Resultado del TratamientoRESUMEN
Embolization of a catheter fragment is a very rarely seen complication, and few cases have been reported in children. Catheter fragments must be urgently extracted due to life-threatening complications. Most catheter fragments are removed very soon after being lost in the cardiovascular system, including the venous system and right side of the heart. In our report, we describe a child with catheter fragment, which was removed from the left ventricle 32 days after embolization. This catheter fragment was successfully retrieved percutaneously using a gooseneck snare catheter through the femoral artery.
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Catéteres de Permanencia/efectos adversos , Migración de Cuerpo Extraño/terapia , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos , Preescolar , Femenino , HumanosRESUMEN
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.
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Cardiomiopatías , Ataxia de Friedreich , Caminata/fisiología , Cardiomiopatías/tratamiento farmacológico , Cardiomiopatías/etiología , Femenino , Ataxia de Friedreich/complicaciones , Ataxia de Friedreich/diagnóstico , Ataxia de Friedreich/tratamiento farmacológico , Humanos , Interferón gamma/uso terapéuticoRESUMEN
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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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.
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Enfermedades Cardiovasculares , Diálisis Renal , Adolescente , Niño , Humanos , Adulto Joven , Presión Sanguínea/fisiología , Enfermedades Cardiovasculares/epidemiología , Grosor Intima-Media Carotídeo , Estudios Transversales , Lipoproteínas LDL , Análisis de la Onda del Pulso , Diálisis Renal/efectos adversos , Factores de RiesgoRESUMEN
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.
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Coartación Aórtica , Enfermedad de Tay-Sachs , Reflujo Vesicoureteral , Adulto , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico , Niño , Humanos , Lactante , Enfermedad de Tay-Sachs/diagnóstico , Reflujo Vesicoureteral/complicaciones , Reflujo Vesicoureteral/diagnósticoRESUMEN
Cardiovascular disease is a leading cause of long-term morbidity and mortality among children with chronic kidney disease (CKD). At which stage of CKD these appear in children is unknown. This study aimed to determine the prevalence of cardiovascular disease in pediatric CKD patients and to explore the relationship of these changes and treatment methods. The study enrolled pediatric patients with stages 1-5 CKD including 20 patients receiving predialysis (PreD), 8 receiving peritoneal dialysis, and 14 receiving hemodialysis. Aortic stiffness, defined as decreased aortic strain (S) and increased pressure strain normalized by diastolic pressure (Ep*), was described. Sonography of the common carotid artery and left ventricle was performed. The mean age of the children was 13.3 + or - 5.3 years. The patients had lower S values (0.35 + or - 0.23) than the control subjects (0.44 + or - 0.2) (P < 0.05) but higher Ep* (2.46 + or - 1.31 vs. 1.32 + or - 0.09; P < 0.05). Aortic stiffness was found in 13 patients. The PreD group had lower As levels than the dialysis group but higher levels than the control group. The patients (n = 32) had greater carotid intima-media thickness than the control subjects (0.58 + or - 0.14 vs. 0.35 + or - 0.12; P < 0.05). The intima-media thickness was greatest in the PreD group (P < 0.05). The patients had a higher left ventricular mass index (LVMI; 42.4 + or - 15.6) than the control subjects (28.8 + or - 8.47) (P < 0.05) and a larger left ventricle end diastolic diameter (LVEDD; 3.44 + or - 0.76 vs. 2.59 + or - 0.34; P < 0.05). Left ventricular hypertrophy was found in 32 patients. Both LVMI and LVEDD were higher in the groups receiving hemodialysis and lower in the PreD group. Increased carotid-intima media thickness and left ventricle hypertrophy appeared without hypertension in the PreD group. The indications and timing of dialysis should be reevaluated for children with CKD. In the dialysis groups, fewer cardiovascular changes were found with peritoneal dialysis than with hemodialysis. Therefore, peritoneal dialysis should be preferable to hemodialysis for children with CKD.
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Enfermedades Cardiovasculares/epidemiología , Fallo Renal Crónico/complicaciones , Adolescente , Presión Sanguínea , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/etiología , Arteria Carótida Común/diagnóstico por imagen , Niño , Preescolar , Estudios Transversales , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Fallo Renal Crónico/epidemiología , Masculino , Morbilidad/tendencias , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia/tendencias , Turquía/epidemiología , Ultrasonografía , Adulto JovenRESUMEN
Prolonged QTc interval (>440 ms) is a common abnormality in adult patients with CLD and has been reported to predict patient survival. In this study, 88 children who underwent evaluation for LT, including a 12-lead electrocardiogram and echocardiogram included to determine the frequency of QTc prolongation and related factors in children with CLD and the effect of LT on these factors. Sixty-nine healthy, age- and sex-matched children served as controls. QTc interval was prolonged in 40 CLD patients (45.4%). It was found to be related to PELD score and presence of portal hypertension. Mean QTc was higher in patients who died prior to LT than in the survivors without LT. Mortality risk was increased 3.66-fold in patients with prolonged QTc (p = 0.001, 95% CI: 2-7.2). Cox regression analysis showed that only PELD score was an independent predictor of survival (p = 0.001, beta = -0.41, 95% CI: 5.58-1.82). Five of 48 transplanted children died within three months post-transplant; QTc was not related to post-transplant survival (p = 0.27). QTc normalized in 63.8% patients after LT. After LT, LAD, LVEF, and LVPWT decreased. In conclusion, QTc prolongation is common in children with CLD and associated with high mortality. It may be useful for assessment of the severity of CLD and for the timing for transplantation.
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Arritmias Cardíacas/fisiopatología , Hepatopatías/fisiopatología , Hepatopatías/cirugía , Trasplante de Hígado , Adolescente , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiología , Arritmias Cardíacas/mortalidad , Niño , Preescolar , Enfermedad Crónica , Electrocardiografía , Femenino , Humanos , Lactante , Hepatopatías/complicaciones , Hepatopatías/mortalidad , Estudios Longitudinales , Masculino , Estudios RetrospectivosRESUMEN
BACKGROUND: Much epidemiological evidence has linked low birthweight with late cardiovascular risk. Intrauterine growth retardation (IUGR) is associated with the increased risk of cardiovascular disease in adult life; it is unclear whether the relationship is present at younger ages. We evaluated whether abdominal aortic stiffness was altered in patients with IUGR (born at term with birthweight small for gestational age) in younger ages. METHODS: Thirty-two (24 girls and eight boys) IUGR children aged 8.77 +/- 2.05 years were enrolled in the study. The birthweight was traced from the medical records. Their gestational ages were 38.9 +/- 0.85 weeks and birthweights 2130 +/- 198 g, respectively. Thirty-one healthy subjects who had normal gestational age and birthweight, matched for age and sex were recruited as a control group. Aortic strain, pressure strain elastic modulus (Ep), and normalized Ep and aortic distensibility were measured by a sphygmomanometer and transthoracic echocardiography in all subjects from the abdominal aorta. RESULTS: There was no statistically significant difference between the study and the control groups in sex, mean age, body mass index, lipid profile, leptin, insulin-like growth factor-1 or insulin-like growth factor binding protein 3. In IUGR children, aortic strain (0.201 +/- 0.027 vs 0.254 +/- 0.031, P < 0.001) and aortic distensibility (1.08 +/- 0.19 vs 1.42 +/- 0.24, P < 0.001) were significantly lower compared with the control group. However Ep (188 +/- 36.2 vs 146 +/- 27.1, P < 0.001) and normalized Ep (2.97 +/- 0.40 vs 2.1 +/- 0.39, P < 0.001) were significantly higher in IUGR patients. CONCLUSIONS: This study demonstrates that abdominal aortic stiffness is increased in IUGR patients. These data suggest that prenatal events could be related to cardiovascular risk in later life.
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Retardo del Crecimiento Fetal/fisiopatología , Recién Nacido Pequeño para la Edad Gestacional , Músculo Liso Vascular/fisiopatología , Resistencia Vascular/fisiología , Aorta Abdominal/fisiopatología , Enfermedades Cardiovasculares/fisiopatología , Niño , Ecocardiografía , Femenino , Retardo del Crecimiento Fetal/diagnóstico , Humanos , Recién Nacido , Masculino , Valores de Referencia , Estudios Retrospectivos , Factores de RiesgoRESUMEN
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.
Asunto(s)
Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/fisiopatología , Flujo Pulsátil/fisiología , Diagnóstico Precoz , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Tamizaje Neonatal , Oximetría , Embarazo , Diagnóstico PrenatalRESUMEN
OBJECTIVES: The hepatopulmonary syndrome (HPS) is characterized as a triad: liver disease, intrapulmonary vascular dilatation, and arterial hypoxemia. The aim of this study is to analyze outcome of children with HPS in liver transplant era. METHODS: Between September 1996 and November 2006, 172 cirrhotic patients (median age 5 years; range 0.2-22 years, M/F; 97/75) were followed at Ege University Pediatric Gastroenterology, Hepatology and Nutrition Unit. All patients were evaluated by chest radiography, arterial blood gas analysis, and alveolar-arterial oxygen tension difference, contrast echocardiography (CEE) after and before the liver transplantation. RESULTS: HPS was diagnosed in 33 patients (19%) by CEE. None of them had pulmonary hypertension. HPS was not found related to etiology of the liver disease. Portal hypertension was found related to the development of HPS (75.7% in patients with HPS and 54.6% in others, p = 0.02). 17 of 33 patients with HPS underwent liver transplantation. Preoperative and postoperative period of these patients was uneventful. Patients were extubated in the operating room except for two. Median follow up of transplanted children was 1.9 year (range; 0.75-10 years). Arterial blood gas analysis and CEE positivity regressed in all of them by postoperative 6th month. CONCLUSIONS: HPS is a serious and important complication of cirrhotic children that leads to tissue hypoxia and central cyanosis. HPS seems reversible after liver transplantation in all patients.
Asunto(s)
Síndrome Hepatopulmonar/complicaciones , Síndrome Hepatopulmonar/terapia , Cirrosis Hepática/complicaciones , Cirrosis Hepática/terapia , Trasplante de Hígado/métodos , Adolescente , Adulto , Análisis de los Gases de la Sangre , Niño , Preescolar , Ecocardiografía , Femenino , Estudios de Seguimiento , Síndrome Hepatopulmonar/cirugía , Humanos , Lactante , Cirrosis Hepática/cirugía , Masculino , Resultado del TratamientoRESUMEN
The purpose of this study was to evaluate late cardiac toxicity by comprehensive echocardiographic study, and to determine whether plasma atrial natriuretic peptide and brain natriuretic peptide levels might be indicators of neurohumoral activation. The study included 49 long-term survivors and 21 controls. A wide variety of echocardiographic parameters were measured or calculated. Plasma peptide levels were determined. Patients had significant changes in different echocardiographic parameters that are suggestive of LV systolic and diastolic dysfunction. Plasma peptide levels were not increased. The authors have found significant subclinic cardiotoxicity by echocardiography. Survivors seem to have normal plasma natriuretic peptide levels in long-term period.