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Aims: Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by catheter-based interventions and complemented by various surgical procedures. We aim to determine a long-term treatment strategy to enable patients to be surgery free, depending solely on percutaneous interventions. Methods and Results: We selected five patients from among a cohort of patients with PA/IVS treated at birth with radiofrequency perforation and dilatation of the pulmonary valve. Patients had reached a pulmonary valve annulus of 20 mm or larger on their biannual echocardiographic follow-up, with right ventricular dilatation. The findings, together with the right ventricular outflow tract and pulmonary arterial tree, were confirmed by multislice computerised tomography. Based on the angiographic size of the pulmonary valve annulus, all patients were successfully implanted with either Melody® or Edwards® pulmonary valves percutaneously, regardless of their small weights and ages. No complications were encountered. Conclusion: We managed to stretch the age and weight limitations for performing percutaneous pulmonary valve implantation (PPVI): interventions were attempted whenever a pulmonary annulus size of >20 mm was reached, which was rationalised by the prevention of progressive right ventricular outflow tract dilatation and accommodating valves between 24 and 26 mm, which is enough to sustain a normal pulmonary flow in adulthood.
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Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Atresia Pulmonar , Válvula Pulmonar , Recién Nacido , Humanos , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugía , Cardiopatías Congénitas/cirugía , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Ventrículos Cardíacos , Resultado del TratamientoRESUMEN
OBJECTIVES: To assess the long-term safety and efficacy of the Occlutech® ACCELL® Flex II device used for atrial septal defect (ASD) closure. This device differs from the regular device by having two very thin patches that are made of polyethylene terephthalate (PET). These patches enhance faster sealing of the defect. BACKGROUND: Transcatheter closure has become the method of choice to manage most patients with secundum ASDs. There are different types of devices. The regular Occlutech device used to close an ASD is called the Occlutech Figulla Flex II. A newer modification of this device (Occlutech® ACCELL® Flex II) has been designed to eliminate/reduce thrombus formation and to enhance faster sealing. METHODS: Thirty patients were followed up after occlusion of secundum ASD using the Occlutech® ACCELL® Flex II Device. The follow-up period ranged from 5.2-5.5 years with median of 5.3 years. Detailed history and full clinical examination, twelve-lead electrocardiogram (ECG), plain chest radiograph, and full 2D transthoracic echocardiography (TTE) were performed at discharge, at one month, six months, and yearly thereafter. RESULTS: The mean age of the study group at the last follow-up was 10.4 ± 4.6 years, with 63.3% (nineteen patients) females. There were no residual shunts or complications encountered immediately after the procedure and at the latest follow-up. CONCLUSION: This study confirmed the transcatheter closure (TCC) of secundum ASDs using the Occlutech® ACCELL® Flex II device to be safe and effective with no complications detected in children and adolescents.
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Foramen Oval Permeable , Defectos del Tabique Interatrial , Dispositivo Oclusor Septal , Adolescente , Cateterismo Cardíaco/efectos adversos , Niño , Preescolar , Ecocardiografía Transesofágica , Femenino , Estudios de Seguimiento , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interatrial/cirugía , Humanos , Masculino , Estudios Retrospectivos , Dispositivo Oclusor Septal/efectos adversos , Resultado del TratamientoRESUMEN
Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.
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Cardiopatías Congénitas , Atresia Pulmonar , Válvula Pulmonar , Tabique Interventricular , Humanos , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugía , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , ReologíaRESUMEN
BACKGROUND: Paediatric cardiomyopathy is a progressive, often lethal disorder and the most common cause of heart failure in children. Despite its severe outcomes, the genetic aetiology is still poorly characterised. High-throughput sequencing offers a great opportunity for a better understanding of the genetic causes of cardiomyopathy. AIM: The current study aimed to elucidate the genetic background of cardiomyopathy in Egyptian children. METHODS: This hospital-based study involved 68 patients; 58 idiopathic primary dilated cardiomyopathy and 10 left ventricular noncompaction cardiomyopathy. Cardiomyopathy-associated genes were investigated using targeted next-generation sequencing. RESULTS: Consanguinity was positive in 53 and 70% of dilated cardiomyopathy and left ventricular noncompaction cardiomyopathy patients, respectively. Positive family history of cardiomyopathy was present in 28% of dilated cardiomyopathy and 10% of the left ventricular noncompaction cardiomyopathy patients. In 25 patients, 29 rare variants were detected; 2 likely pathogenic variants in TNNI3 and TTN and 27 variants of uncertain significance explaining 2.9% of patients. CONCLUSIONS: The low genetic detection rate suggests that novel genes or variants might underlie paediatric cardiomyopathy in Egypt, especially with the high burden of consanguinity. Being the first national and regional report, our study could be a reference for future genetic testing in Egyptian cardiomyopathy children. Genome-wide tests (whole exome/genome sequencing) might be more suitable than the targeted sequencing to investigate the primary cardiomyopathy patients. Molecular characterisation of cardiomyopathies in different ethnicities will allow for global comparative studies that could result in understanding the pathophysiology and heterogeneity of cardiomyopathies.
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Cardiomiopatías , Predisposición Genética a la Enfermedad , Cardiomiopatías/diagnóstico , Cardiomiopatías/genética , Niño , Egipto/epidemiología , Pruebas Genéticas , Humanos , FenotipoRESUMEN
BACKGROUND: Early diagnosis and treatment of myocardial affection in patients with systemic lupus erythematosus (SLE) are crucial. OBJECTIVES: To evaluate the ventricular systolic function in juvenile-onset systemic lupus erythematosus (j-SLE) patients by 3-D speckle tracking echocardiography (3D-STE) and to determine the predictors of left ventricular (LV) dysfunction if present. METHODS: Twenty-six SLE patients without heart failure and 21 healthy controls were studied by standard echocardiogram and 3D-STE. Conventional parameters included LV ejection fraction (EF), fractional shortening (FS), and mitral annular plane systolic excursion (MAPSE). Global LV strain (GLS) and global area strain (GAS) were obtained by 3D-STE. Medical records, including diagnosis criteria, duration of disease, and SLE disease activity index (SLEDAI) were evaluated. RESULTS: The mean age was similar in patients and controls 11.42 vs 11.48 years p = 0.93. The mean duration of the disease was 1.87 ± 1.02 years and SLEDAI ranged from 0 to 9. By conventional and tissue Doppler imaging echocardiography, only MAPSE was significantly lower in SLE patients compared to controls (14.56 vs 18.46 mm, p < 0.001). By 3D speckle tracking echocardiography, GLS and GAS were significantly reduced in SLE patients compared to controls (-15.07 vs -19.9.4%, -34.6% vs -39.7%, respectively, p < 0.001). Multiple linear regression and ROC analyses indicated that the SLEDAI score was the only predictive factor for the left ventricular remodeling. CONCLUSIONS: These results indicate that early subclinical LV dysfunction occur in jSLE patients even with normal EF and SLE disease activity might be a potential driver for LV deformation.
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Lupus Eritematoso Sistémico , Disfunción Ventricular Izquierda , Niño , Ecocardiografía , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico por imagen , Reproducibilidad de los Resultados , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/etiología , Función Ventricular Izquierda , Remodelación VentricularRESUMEN
INTRODUCTION: Residual patent ductus arteriosus (rPDAs) can occur following surgical or transcatheter treatment, and are indicated for closure because of the risks of infective endarteritis and hemolysis in addition to the hemodynamic effect of the residual left-to-right shunt. METHODS: This retrospective descriptive study describes our experience at two Egyptian centers (Cairo University Children's Hospital & Tanta University Hospital) with transcatheter treatment of rPDAs, from January 2009 to October 2017. RESULTS: Twenty cases were treated: 17/20 postsurgical and 3/20 post-transcatheter, at a mean period of 13.4 ± 9.3 months from the initial procedure. The median rPDA size was 2 mm (range2-3.5 mm). Most common ductal anatomy was the conical shape. All rPDAs were successfully closed with either coils (13/20) or devices (6/20), except one case where the residual flow was within the device mesh material. Coils could be deployed from the antegrade or the retrograde approaches although the latter was associated with a higher incidence of late shunt occlusion. One case with a malpositioned device required simultaneous device and LPA stent deployment. CONCLUSION: Transcatheter closure of rPDAs is feasible in most cases, but may be technically challenging.
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Cateterismo Cardíaco , Conducto Arterioso Permeable/terapia , Adolescente , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Niño , Preescolar , Conducto Arterioso Permeable/diagnóstico por imagen , Conducto Arterioso Permeable/fisiopatología , Egipto , Femenino , Hemodinámica , Humanos , Masculino , Retratamiento , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Paediatric cardiomyopathy is a progressive and often lethal disorder and the most common cause of heart failure in children. Despite their severe outcomes, their genetic etiology is still poorly characterised. The current study aimed at uncovering the genetic background of idiopathic primary hypertrophic cardiomyopathy in a cohort of Egyptian children using targeted next-generation sequencing. The study included 24 patients (15 males and 9 females) presented to the cardiomyopathy clinic of Cairo University Children's Hospital with a median age of 2.75 (0.5-14) years. Consanguinity was positive in 62.5% of patients. A family history of hypertrophic cardiomyopathy was present in 20.8% of patients. Ten rare variants were detected in eight patients; two pathogenic variants (8.3%) in MBPC3 and MYH7, and eight variants of uncertain significance in MYBPC3, TTN, VCL, MYL2, CSRP3, and RBM20.Here, we report on the first national study in Egypt that analysed sarcomeric and non-sarcomeric variants in a cohort of idiopathic paediatric hypertrophic cardiomyopathy patients using next-generation sequencing. The current pilot study suggests that paediatric hypertrophic cardiomyopathy in Egypt might have a particular genetic background, especially with the high burden of consanguinity. Including the genetic testing in the routine diagnostic service is important for a better understanding of the pathophysiology of the disease, proper patient management, and at-risk detection. Genome-wide tests (whole exome/genome sequencing) might be better than the targeted sequencing approach to test primary hypertrophic cardiomyopathy patients in addition to its ability for the identification of novel genetic causes.
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Cardiomiopatía Hipertrófica , Adolescente , Cardiomiopatía Hipertrófica/epidemiología , Cardiomiopatía Hipertrófica/genética , Niño , Preescolar , Egipto/epidemiología , Femenino , Pruebas Genéticas , Secuenciación de Nucleótidos de Alto Rendimiento , Humanos , Masculino , Mutación , Proyectos PilotoRESUMEN
BACKGROUND: Obesity increases the risk for various cardiovascular problems. Increase in body mass index is often an independent risk factor for the development of elevated blood pressure and clustering of various cardiovascular risk factors. OBJECTIVE: To determine early markers of left ventricular affection in obese patients before the appearance of left ventricular hypertrophy. METHODS: In this cross-sectional study, we evaluated 42 obese patients and 30 healthy controls. Their ages ranged from 6 to 19 years. Studied children were subjected to anthropometric, lipid profile, and serum Troponin I level measurements. Echocardiographic evaluation performed to assess the left ventricle included left ventricular dimension measurement using motion-mode echocardiography, based on which patients with left ventricular hypertrophy (10 patients) were eliminated, as well as conventional and tissue Doppler imaging. RESULTS: Tissue Doppler findings in the study groups showed that the ratio of transmitral early diastolic filling velocity to septal peak early diastolic myocardial velocity (E/e') was significantly higher in cases compared with controls [6.9±1.4 versus 9.0±1.6, p (Pearson's coefficient)=0.001, respectively]. The level of cardiac troponin I was significantly higher in cases compared with controls [0.14±0.39 ng/ml versus 0.01±0.01 ng/ml, p (Pearson's coefficient)=0.047, respectively] and there was a significant correlation between troponin I and transmitral early diastolic filling velocity to septal peak early diastolic myocardial velocity ratio (E/e') [R (correlation coefficient)=0.6]. CONCLUSION: Tissue Doppler Imaging and Troponin I evaluation proved useful tools to detect early affection of the left ventricle in obese patients even in the absence of left ventricular hypertrophy.
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Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Obesidad Infantil/complicaciones , Troponina I/sangre , Disfunción Ventricular Izquierda/diagnóstico por imagen , Adolescente , Índice de Masa Corporal , Estudios de Casos y Controles , Niño , Estudios Transversales , Diástole , Ecocardiografía Doppler , Femenino , Humanos , Masculino , Análisis Multivariante , Disfunción Ventricular Izquierda/etiología , Función Ventricular IzquierdaRESUMEN
Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI). All demographic and functional parameters as well as pressure gradient across left ventricular outflow tract (LVOT) were correlated with the percentage of myocardial enhancement. We studied 13 female and 27 male patients from 45 days up to 18 years. The mean percentage of DE-MRI was 9.7 ± 9%. We found significant correlation between the NYHA classification and the pressure gradient across the LVOT (P = < 0.001) as well as the percentage of DE-MRI (P = 0.004). The percentage of DE-MRI showed positive correlation with LV myocardial mass index (P = 0.042). It didn't correlate with any other demographic or LV functional cardiac parameters. A good positive correlation was detected between the percentage of DE-MRI and the severity of pressure gradient across LVOT measured by echocardiography (r = 0.69 and P = <0.001). We found a significant correlation between the percentage of DE-MRI in children with HCM and the pressure gradient across LVOT, NYHA classification, and LV myocardial mass. This may help in the further management of those patients, planning for follow-up, and prognosis of the disease.
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Cardiomiopatía Hipertrófica/diagnóstico por imagen , Ventrículos Cardíacos/diagnóstico por imagen , Miocardio/patología , Adolescente , Cardiomiopatía Hipertrófica/patología , Cardiomiopatía Hipertrófica/fisiopatología , Niño , Preescolar , Ecocardiografía , Femenino , Fibrosis/diagnóstico por imagen , Fibrosis/patología , Fibrosis/fisiopatología , Corazón/diagnóstico por imagen , Corazón/fisiopatología , Ventrículos Cardíacos/patología , Ventrículos Cardíacos/fisiopatología , Humanos , Lactante , Imagen por Resonancia Magnética , MasculinoRESUMEN
Published data showing the intermediate effect of transcatheter device closure of atrial septal defect (ASD) in the pediatric age-group are scarce. The objective of the study was to assess the effects of transcatheter ASD closure on right and left ventricular functions by tissue Doppler imaging (TDI). The study included 37 consecutive patients diagnosed as ASD secundum by transthoracic echocardiography and TEE and referred for transcatheter closure at Cairo University Specialized Pediatric Hospital, Egypt, from October 2010 to July 2013. Thirty-seven age- and sex-matched controls were selected. TDI was obtained using the pulsed Doppler mode, interrogating the right cardiac border (the tricuspid annulus) and lateral mitral annulus, and myocardial performance index (MPI) was calculated at 1-, 3-, 6- and 12-month post-device closure. Transcatheter closure of ASD and echocardiographic examinations were successfully performed in all patients. There were no significant differences between two groups as regards the age, gender, weight or BSA. TDI showed that patients with ASD had significantly prolonged isovolumetric contraction, relaxation time and MPI compared with control group. Decreased tissue Doppler velocities of RV and LV began at one-month post-closure compared with the controls. Improvement in RVMPI and LVMPI began at 1-month post-closure, but they are still prolonged till 1 year. Reverse remodeling of right and left ventricles began 1 month after transcatheter ASD closure, but did not completely normalize even after 1 year of follow-up by tissue Doppler imaging.
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Cateterismo Cardíaco/efectos adversos , Defectos del Tabique Interatrial/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Remodelación Ventricular , Niño , Preescolar , Estudios Transversales , Ecocardiografía Doppler , Egipto , Femenino , Humanos , Masculino , Estudios Prospectivos , Función Ventricular IzquierdaRESUMEN
BACKGROUND: Hypertrophic cardiomyopathy is an important cause of disability and death in patients of all ages. Egyptian children may differ from Western and Asian patients in the pattern of hypertrophy distribution, clinical manifestations, and risk factors. OBJECTIVES: The aim of our study was to report the clinical characteristics and outcomes of Egyptian children with hypertrophic cardiomyopathy studied over a 7-year duration and to determine whether the reported adult risk factors for sudden cardiac death are predictive of the outcome in these affected children. STUDY DESIGN AND METHODS: This retrospective study included 128 hypertrophic cardiomyopathy children. The data included personal history, family history, physical examination, baseline laboratory measurements, electrocardiogram, and Holter and echocardiographic results. Logistic regression analysis was used for the detection of risk factors of death. RESULTS: Fifty-one out of 128 patients died during the period of the study. Of the 51 deaths, 36 (70.5%) occurred in patients presenting before 1 year of age. Only eight patients had surgical intervention. Extreme left ventricular hypertrophy, that is, interventricular septal wall thickness or posterior wall thickness Z-score >6, sinus tachycardia, and supraventricular tachycardia were found to be independent risk factors for prediction of death in patients with hypertrophic cardiomyopathy. CONCLUSIONS: At our Egyptian tertiary care centre, hypertrophic cardiomyopathy has a relatively worse prognosis when compared with reports from Western and Asian series. Infants have a worse outcome than children presenting after the age of 1 year. A poorer prognosis in childhood hypertrophic cardiomyopathy is predicted by an extreme left ventricular hypertrophy, the presence of sinus tachycardia, and supraventricular tachycardia.
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Cardiomiopatía Hipertrófica/mortalidad , Muerte Súbita Cardíaca/epidemiología , Hipertrofia Ventricular Izquierda/epidemiología , Taquicardia Sinusal/epidemiología , Taquicardia Supraventricular/epidemiología , Adolescente , Cardiomiopatía Hipertrófica/epidemiología , Niño , Preescolar , Egipto/epidemiología , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Pronóstico , Índice de Severidad de la Enfermedad , Estadística como AsuntoRESUMEN
The long-term treatment of congestive heart failure (CHF) in children includes digoxin, diuretics and afterload reduction with angiotensin-converting enzyme (ACE) inhibitors. In spite of the wide use of these drugs being the standard, yet, pediatric heart failure (PHF) continued to be an important cause of morbidity and mortality in childhood. Introduction of new drugs has elevated the level of tolerance of these patients and played a role in delaying their urgent need to have heart transplant or Mechanical circulatory support (MCS). Together with a patient by patient tailored combination of different diuretics. We aim to present and discuss these new drugs and the combinations of regular drugs to reach the best outcome, as well as the consensus of our pediatric heart failure working group in Egypt.
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Insuficiencia Cardíaca , Humanos , Niño , Consenso , Universidades , Insuficiencia Cardíaca/tratamiento farmacológico , Diuréticos/uso terapéutico , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , HospitalesRESUMEN
Introduction & aim of work: Transcatheter treatment for critical pulmonary stenosis and membranous pulmonary atresia has become the gold standard of care in many centers. We aimed at evaluating the predictors of outcome in interventions for treatment of duct-dependent right ventricular outflow tract obstruction with intact interventricular septum. Subjects & methods: 68 cases with pulmonary atresia with intact interventricular septum (PA/IVS) and 50 cases with critical pulmonary stenosis (CPS), all younger than 3 months of age, were operated during the period of 10 years; excluding patients with tricuspid valve annulus Z-score smaller than -4, evidence of right ventricular-dependent coronary circulation or additional malformations. Results: Age, weight, body surface area as well as tricuspid & pulmonary valve Z-scores were significantly less in PA/IVS; right ventricular pressure was similar in both groups however procedural success and survival to hospital discharge was higher in the CPS group. Lower age, weight and body surface area were associated with procedural failure. Weight was the only predictor of procedural success; while weight and lower post-procedural right ventricular pressure were independent predictors for survival to hospital discharge. Post-procedural right ventricular pressure and length of stay were less in the CPS group. tricuspid and pulmonary valve annulus Z-scores were the only independent predictors of the post-procedural milrinone duration in PA/IVS. Conclusion: We advocate for the use of larger balloon/pulmonary annulus ratio, to achieve a lower right ventricular pressure not fearing excessive pulmonary regurgitation that might be beneficial for right ventricular growth; and for the combination with ductal stenting in borderline or bipartite right ventricles.
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BACKGROUND: Childhood dilated cardiomyopathy (CDCM) is the most common cardiomyopathy in children and it is risk factor to heart failure and sudden death. Most of the different etiologic factors which have been postulated to DCM are idiopathic, and its pathogenesis remains uncertain. So it was worth investigating the potential DCM pathogenicity models to establish early noninvasive diagnosis parameters especially in CDCM patients. Beside that miRNAs in the circulatory blood are genetically considered the best option for noninvasive diagnosis; also, implementation of miRNAs as early diagnostic markers for children with DCM is urgent because those children have high risk to sudden heart death. We aimed to identify discriminator diagnostic circulatory miRNA expression levels in CDCM patients. RESULTS: The expression levels of miR-454-3p and miR-194-5p were found significant upregulated (p value = 0.001 and 0.018; CI 95%, respectively), while miR-875-3p was found significant downregulated (p value = 0.040; CI 95%). A receiver operating characteristic (ROC) curve analysis showed significant AUC = 1.000 and 0.798 for miR-454-3p and miR-194-5p, respectively, and the optimal discriminated diagnostic cut-points were computed by index of union (IU) method. Enrichment analysis for the potential targeted mature mRNAs by miR-454-3p and miR-194-5p pointed that Ca, Na and K ions homeostasis in cardiac sarcolemma consider potential CDCM pathogenicity model. CONCLUSIONS: miR-454-3p and miR-194-5p are highly influencing noninvasive biomarkers for CDCM, and further circulatory miRNAs-implicated studies are highly recommended.
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OBJECTIVES: Percutaneous patent ductus arteriosus (PDA) stenting is a therapeutic modality in patients with duct-dependent pulmonary circulation with reported success rates from 80-100%. The current study aims to assess the outcome and the indicators of success for PDA stenting in different ductal morphologies using various approaches. METHODS: A prospective cohort study from a single tertiary center presented from January 2018 to December 2019 that included 96 consecutive infants with ductal-dependent pulmonary circulation and palliated with PDA stenting. Patients were divided according to PDA origin into 4 groups: Group 1: PDA from proximal descending aorta, Group 2: from undersurface of aortic arch, Group 3: opposite the subclavian artery, Group 4: opposite the innominate/brachiocephalic artery. RESULTS: The median age of patients was 22 days and median weight was 3 kg. The procedure was successful in 78 patients (81.25%). PDA was tortuous in 70 out of 96 patients. Femoral artery was the preferred approach in Group 1 (63/67), while axillary artery access was preferred in the other groups (6/11 in Group 2, 11/17 in Group 3, 1/1 in Group 4, P <0.0001). The main cause of procedural failure was inadequate parked coronary wire inside one of the branch of pulmonary arteries (14 cases; 77.7%), while 2 cases (11.1%) were complicated by acute stent thrombosis, and another 2 cases with stent dislodgment. Other procedural complications comprised femoral artery thrombosis in 7 cases (7.2%). Patients with straight PDA, younger age at procedure and who had larger PDA at pulmonary end had higher odds for success (OR = 8.01, 2.94, 7.40, CI = 1.011-63.68, 0.960-0.99, 1.172-7.40,respectively, P = 0.048, 0.031,0.022 respectively). CONCLUSIONS: The approach for PDA stenting and hence the outcome is markedly determined by the PDA origin and morphology. Patients with straight PDA, younger age at procedure and those who had relatively larger PDA at the pulmonary end had better opportunity for successful procedure.
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Conducto Arterioso Permeable , Cateterismo Cardíaco/métodos , Conducto Arterioso Permeable/cirugía , Humanos , Lactante , Estudios Prospectivos , Circulación Pulmonar , Estudios Retrospectivos , Stents , Resultado del TratamientoRESUMEN
BACKGROUND: Interstitial lung disease (ILD) is a broad heterogeneous group of lung disorders that is characterized by inflammation of the lungs. Surfactant dysfunction disorders are a rare form of ILD diseases that result from mutations in surfactant protein C gene (SFTPC) with prevalence of approximately 1/1.7 million births. SFTPC patients are presented with clinical manifestations of ILD ranging from fatal respiratory failure of newborn to chronic respiratory problems in children. In the current study, we aimed to investigate the spectrum of SFTPC genetic variants as well as the correlation of the SFTPC gene mutations with ILD disease in twenty unrelated Egyptian children with diffuse lung disease and suspected surfactant dysfunction using Sanger sequencing. RESULTS: Sequencing of SFTPC gene revealed five variants: c.42+35G>A (IVS1+35G>A) (rs8192340) and c.43-21T>C (IVS1-21T>C) (rs13248346) in intron 1, c.436-8C>G (IVS4-8C>G) (rs2070687) in intron 4, c.413C>A p.T138N (rs4715) in exon 4, and c.557G>Ap.S186N (rs1124) in exon 5. CONCLUSION: The present study confirms the association of detecting variants of SFTPC with surfactant dysfunction disorders.
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Background: Inborn errors of metabolism (IEMs) commonly present with pediatric cardiomyopathy. Identification of the underlying cause is necessary as it may lead to improved outcomes. Objectives: We aimed to investigate the diagnostic rate, the clinical, and biochemical spectra of IEMs among Egyptian pediatric patients presenting with cardiomyopathy, and their outcome measures. Methods: We retrospectively analyzed the clinical, biochemical, and radiological data of 1512 children diagnosed with cardiomyopathy at Cairo University Children's Hospital over a 5-year duration. Results: Two hundred twenty-nine children were clinically suspected as IEMs and underwent metabolic workup. Nineteen different IEMs were confirmed in 57 (24.4%) of the suspected children. Their median age at presentation was 2.6 years and the majority had extra-cardiac manifestations. Hypertrophic cardiomyopathy represented 43/57 (75.4%) of confirmed cases, while dilated cardiomyopathy represented 13/57 (22.8%), and one patient presented with a mixed phenotype. Twenty- six patients (45.6%) survived, while 31 patients (54%) either died or were lost to follow up and assumed deceased. Conclusions: We developed for the first time a database and a diagnostic scheme for metabolic cardiomyopathies in Egyptian children. With the recent introduction of enzyme replacement therapy, many metabolic disorders became treatable, thus establishing an early and accurate diagnosis is extremely important.
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Cardiomiopatías , Egipto , Humanos , Evaluación de Resultado en la Atención de Salud , Estudios RetrospectivosRESUMEN
Background: Several reports of unheeded complications secondary to the current mass international rollout of SARS-COV-2 vaccines, one of which is myocarditis occurring with the FDA fully approved vaccine, Pfizer, and others. Main body of the abstract: Certain miRNAs (non-coding RNA sequences) are involved in the pathogenesis in viral myocarditis, and those miRNAs are interestingly upregulated in severe COVID-19. We hypothesize that the use of mRNA-based vaccines may be triggering the release of host miRNAs or that trigger the occurrence of myocarditis. This is based on the finding of altered host miRNA expression promoting virus-induced myocarditis. Short conclusion: In conclusion, miRNAs are likely implicated in myocarditis associated with mRNA vaccines. Our hypothesis suggests the use of miRNA as a biomarker for the diagnosis of mRNA vaccine-induced myocarditis. Additionally, the interplay between viral miRNA and the host immune system could alter inflammatory profiles, hence suggesting the use of therapeutic inhibition to prevent such complications.
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Two paediatric patients suffering from recurrent coarctation after native coarctation surgery were scheduled for cardiac catheterisation in a hybrid setting by the age of two and three years. Through a right anterior mini-thoracotomy, unmounted stents were successfully placed in the coarctant segment. One-year follow up did not show echocardiographic improvement but the patients were controlled on medical therapy. Five-year follow up showed stent breakage in the first case, while the second showed stent stenosis that needed balloon dilatation.
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Angioplastia de Balón , Coartación Aórtica , Angioplastia de Balón/efectos adversos , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/cirugía , Cateterismo Cardíaco/efectos adversos , Niño , Preescolar , Estudios de Seguimiento , Humanos , Recurrencia , Stents , Resultado del TratamientoRESUMEN
BACKGROUND: Evaluation of certain biomarkers could be used to predict left ventricular (LV) and right ventricular (RV) function impairment in children with type 1 diabetes mellitus. The aim of this study was to determine the best cardiac biomarker for prediction of diabetic cardiomyopathy. METHODOLOGY: This study was designed as case-control study. A total of 55 children with type 1 diabetes mellitus (group/G1) and 55 healthy controls (G2) were subjected to echocardiography including 3D-Speckle Tracking Echocardiography and tissue Doppler imaging for assessment of RV and LV systolic and diastolic functions. As well as HbA1c, troponin I, brain natriuretic peptide (BNP), plasma cardiotrophin (CT-1), activin-A, transforming growth factor-ß, and human insulin-like growth factor binding protein-7 (IGFBP-7) measurements. RESULTS: Diabetic patients showed RV and LV systo-diastolic dysfunction compared to controls, the best predictor of LV systolic dysfunction was CT-1 (sensitivity: 69%, while IGFBP-7 was found to be the best predictor of RV systolic dysfunction (sensitivity: 63%). BNP was found to the best predictor of diastolic RV and LV dysfunction (sensitivity: 82% for both). CONCLUSION: CT-1 has proven to be a diagnostic superiority in LV systolic dysfunction whilst BNP continues to prove every day through our study and through many others that it is the chief marker of diastolic dysfunction and HFpEF. This potential accuracy and the increasing availability of BNP in the outpatient setting make it clear that it should be used as a screening test for diabetic patients.