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BACKGROUND: Limited data exist on how trainees in paediatric cardiology are assessed among countries affiliated with the Association of European Paediatric and Congenital Cardiology. METHODS: A structured and approved questionnaire was circulated to educationalists/trainers in 95 Association for European Paediatric and Congenital Cardiology training centres. RESULTS: Trainers from 46 centres responded with complete data in 41 centres. Instructional design included bedside teaching (41/41), didactic teaching (38/41), problem-based learning (28/41), cardiac catheterisation calculations (34/41), journal club (31/41), fellows presenting in the multidisciplinary meeting (41/41), fellows reporting on echocardiograms (34/41), clinical simulation (17/41), echocardiography simulation (10/41), and catheterisation simulation (3/41). Assessment included case-based discussion (n = 27), mini-clinical evaluation exercise (mini-CEX) (n = 12), directly observed procedures (n = 12), oral examination (n = 16), long cases (n = 11), written essay questions (n = 6), multiple choice questions (n = 5), and objective structured clinical examination (n = 2). Entrustable professional activities were utilised in 10 (24%) centres. Feedback was summative only in 17/41 (41%) centres, formative only in 12/41 (29%) centres and a combination of formative and summative feedback in 10/41 (24%) centres. Written feedback was provided in 10/41 (24%) centres. Verbal feedback was most common in 37/41 (90 %) centres. CONCLUSION: There is a marked variation in instructional design and assessment across European paediatric cardiac centres. A wide mix of assessment tools are used. Feedback is provided by the majority of centres, mostly verbal summative feedback. Adopting a programmatic assessment focusing on competency/capability using multiple assessment tools with regular formative multisource feedback may promote assessment for learning of paediatric cardiology trainees.
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Cardiología , Aprendizaje , Humanos , Niño , Cateterismo Cardíaco , Simulación por Computador , EcocardiografíaRESUMEN
BACKGROUND: Limited data exist on training of European paediatric and adult congenital cardiologists. METHODS: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. RESULTS: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41). CONCLUSION: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.
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Cardiología , Humanos , Adulto , Niño , Cardiología/educación , Certificación , Curriculum , Becas , Europa (Continente)RESUMEN
OBJECTIVE: Cardiac involvement is the most important cause of mortality in juvenile systemic sclerosis (JSSc). Recent reports in adult patients underline that traditional techniques of imaging are inadequate to assess the subclinical cardiac involvement, while speckle tracking echocardiography (STE) is able to identify ventricular dysfunctions in the early stages. The aim of our study was to assess the role of STE in JSSc. METHODS: Demographic, clinical and laboratory data were collected from patients with JSSc. Cardiac investigations performed at baseline (T0) and 18 (T18) and 36 months (T36) follow-up included electrocardiography, conventional echocardiography with measurement of the ejection fraction (EF) and STE with assessment of left and right ventricular global longitudinal strain (LV-GLS and RV-GLS). Cardiac parameters have been compared with demographic characteristics and disease severity, assessed by the Juvenile Systemic Sclerosis Severity Score (J4S). RESULTS: A total of 18 patients, 12 (67%) females, entered the study. At T0, electrocardiography was abnormal in three patients, EF was reduced in one, LV-GLS was abnormal in three (16.7%) and RV-GLS was abnormal in five (27.8%). At T18, EF remained stable while at T36 the result decreased in seven of nine patients. At the same time, LV-GLS also worsened (from -21.6% to -18.2%, P = 0.01). LV-GLS and RV-GLS at baseline showed a significant correlation with J4S (P = 0.012 and P = 0.02, respectively). CONCLUSION: STE is more sensitive than standard echocardiography to identify cardiac involvement in JSSc. Over time, we observed a gradual worsening of LV-GLS, a sign of left ventricular dysfunction, that anticipated by several months the decrease of EF.
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Ecocardiografía/métodos , Esclerodermia Sistémica/complicaciones , Disfunción Ventricular/etiología , Adolescente , Niño , Preescolar , Diagnóstico Precoz , Femenino , Humanos , Masculino , Estudios Retrospectivos , Esclerodermia Sistémica/diagnóstico por imagen , Disfunción Ventricular/diagnóstico por imagen , Adulto JovenRESUMEN
AIM: In complex congenital heart diseases (CHD), patients may remain affected by significant morbidity and mortality after surgery. We analysed the end-of-life (EoL) care in children with severe CHD who died in our institution and investigated perspectives of parents and health-care professionals (HCPs). METHODS: Medical records of all children (age < 18 years old) affected by a severe CHD who died in a tertiary cardiac care centre were reviewed. Subsequently, a cross-sectional questionnaire-based study of parents and HCPs of children involved in the study was designed. RESULTS: In total, 30 children died (median age: 45 days; range: 15 days to 3.4 years). Of them, 97% (31/32) died in an intensive care unit setting and were intubated and sedated at EoL. A total of 77% (23/30) died without parents being present at bedside. Eighteen families and 10 HCPs were interviewed. For 61% of the parents (11/18) and 70% of the clinicians (7/10), the goal of therapy at the EoL was 'to lessen your child's suffering as much as possible'. Overall, 44% of parents (8/18) and 50% of HCPs recognised that their child had no chance of survival 'a few days before the child died'. CONCLUSIONS: We believe that these data suggest an unconscious reluctance to change goals of care in EoL, shifting from intensive care to comfort and quality of life.
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Cardiopatías Congénitas , Cuidado Terminal , Adolescente , Cuidadores , Niño , Estudios Transversales , Cardiopatías Congénitas/cirugía , Humanos , Persona de Mediana Edad , Padres , Percepción , Calidad de VidaRESUMEN
The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making.
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Antiarrítmicos/uso terapéutico , Anticoagulantes/uso terapéutico , Antivirales/uso terapéutico , Arritmias Cardíacas/tratamiento farmacológico , Tratamiento Farmacológico de COVID-19 , Cardiopatías Congénitas/terapia , Factores Inmunológicos/uso terapéutico , Adenosina Monofosfato/análogos & derivados , Adenosina Monofosfato/uso terapéutico , Alanina/análogos & derivados , Alanina/uso terapéutico , Arritmias Cardíacas/epidemiología , Arritmias Cardíacas/fisiopatología , Síndrome de Brugada/tratamiento farmacológico , Síndrome de Brugada/epidemiología , Síndrome de Brugada/fisiopatología , COVID-19/epidemiología , COVID-19/fisiopatología , Procedimientos Quirúrgicos Cardíacos , Cardiología , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/fisiopatología , Trasplante de Corazón , Humanos , Transmisión Vertical de Enfermedad Infecciosa , Síndrome de QT Prolongado/tratamiento farmacológico , Síndrome de QT Prolongado/epidemiología , Síndrome de QT Prolongado/fisiopatología , Miocarditis/epidemiología , Miocarditis/fisiopatología , Miocardio , Pediatría , Medición de Riesgo , SARS-CoV-2 , Sociedades Médicas , Síndrome de Respuesta Inflamatoria Sistémica/tratamiento farmacológico , Síndrome de Respuesta Inflamatoria Sistémica/epidemiología , Síndrome de Respuesta Inflamatoria Sistémica/fisiopatología , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Ventricular/epidemiología , Taquicardia Ventricular/fisiopatologíaRESUMEN
Percutaneous treatment of aortic coarctation is based on angioplasty and/or stenting of the isthmus. We report a case of a 28-year-old girl suffering from aortic coarctation syndrome (coarctation + ventricular septal defect + bicuspid aorta). She underwent coarctectomy with subclavian flap and pulmonary bandage followed by ventricular septal defect closure and bandage removal in her first year of life. When she was 27 years old, a follow-up echocardiography detected an isthmic pressure gradient and a demodulated Doppler in abdominal aorta. A cardiac catheterisation confirmed the diagnosis of aortic re-coarctation. An AndraStent® XL 48 mm was implanted with a resolution of the isthmic gradient. One year later, because of the reappearance of demodulated Doppler in abdominal aorta, a chest X-ray was performed, which showed a stent third-grade fracture. The fracture was corrected by positioning a covered stent cheatham platinum 45 mm through the fragments. The rarest complication after stenting procedures is the fracture of the device with an incidence between 0.01% and 0.08%. Pressure overload beyond the elastic threshold of the material and the pulsatile tension exerted by the blood flow on the walls of the stent are the main mechanisms at the base of the fracture, together with the compliance of the tissue. A vessel that underwent multiple surgical rearrangements could interfere with and complicate the physiopathology at the basis of the fracture. In conclusion, stenting is a safe technique to treat aortic coarctation; stent fracture is a rare event, and different anatomical and haemodynamic factors are related to this complication.
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Coartación Aórtica , Adulto , Angioplastia , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico , Coartación Aórtica/cirugía , Cateterismo Cardíaco , Femenino , Hemodinámica , Humanos , Stents , Resultado del TratamientoRESUMEN
The recommendations of the Association for European Paediatric and Congenital Cardiology for basic training in paediatric and congenital cardiology required to be recognised as a paediatric cardiologist by the Association for European Paediatric and Congenital Cardiology are described below. Those wishing to achieve more advanced training in particular areas of paediatric cardiology should consult the training recommendations of the different Association for European Paediatric and Congenital Cardiology Working Groups available on the Association for European Paediatric and Congenital Cardiology website (www.aepc.org) and the respective publications 1-6. The development of training requirements is the responsibility of the Educational Committee and the Association for European Paediatric and Congenital Cardiology Council in collaboration with the Working Groups of the Association for European Paediatric and Congenital Cardiology. Trainees should be exposed to all aspects of general paediatric and congenital cardiology from fetal life to adolescence and adulthood. Centres performing generalised and specialised work in paediatric and congenital cardiology should be committed to deliver postgraduate training. At each training institute, trainers should be appointed to supervise and act as mentors to the trainees. Association for European Paediatric and Congenital Cardiology will provide basic teaching courses to supplement the training process.
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Cardiología , Cardiopatías Congénitas , Adolescente , Adulto , Niño , HumanosRESUMEN
Aims: Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results: We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P < 0.001) and pulmonary hypertension (P < 0.001). Most patients were asymptomatic at last follow-up (279/451, 62%); STPs were more frequently asymptomatic than CMPs (73% vs. 47%, P < 0.001), with fewer cardiac [odds ratio (OR) 0.42, 95% CI 0.22-0.82] and respiratory symptoms (OR 0.08, 95% CI 0.02-0.28). Many STPs (63/254, 25%) had stenosis/occlusion of the scimitar drainage, and this was associated with a younger age at surgery (OR 0.4, CI 0.21-0.78). Conclusion: Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age.
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Procedimientos Quirúrgicos Cardíacos/métodos , Venas Pulmonares/cirugía , Sistema de Registros , Síndrome de Cimitarra/cirugía , Preescolar , Ecocardiografía Doppler , Europa (Continente)/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Síndrome de Cimitarra/diagnóstico , Síndrome de Cimitarra/epidemiología , Tasa de Supervivencia/tendencias , Factores de Tiempo , Resultado del TratamientoRESUMEN
The relationship between myocardial iron load and eccentric myocardial remodeling remains an under-investigated area; it was thought that remodeling is rather linked to fibrosis. This study aims to determine whether or not measures of remodeling can be used as predictors of myocardial iron. For this purpose, 60 patients with thalassemia were studied with 3D echocardiography and myocardial relaxometry (T2*) by Cardiac MRI. 3D derived sphericity index was significantly higher in patients with myocardial iron load. It was correlated with T2* with a 100% sensitivity and specificity (cut-off value of 0.34) to discriminate between patients with and without myocardial iron overload.
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Cardiomiopatías/diagnóstico por imagen , Sobrecarga de Hierro/diagnóstico por imagen , Talasemia beta , Adolescente , Niño , Estudios Transversales , Ecocardiografía Tridimensional/métodos , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Miocardio/patología , Sensibilidad y Especificidad , Remodelación VentricularRESUMEN
BACKGROUND AND AIMS: Preterm newborns undergo hemodynamic challenges in the postnatal period. The aim of this study was to investigate myocardial mechanics changes in the postnatal period in preterm infants using speckle tracking echocardiography (STE). MATERIAL AND METHODS: Thirty-nine preterm infants ≤34 weeks' gestation underwent cardiac ultrasound evaluation during the first 96 hours of life. A repeated echocardiogram at 3 weeks of age was performed. Echocardiographic assessment involved left ventricular ejection fraction, mitral E/A ratio, S' and E' velocities, E/E' ratio, tricuspid annular plane systolic excursion (TAPSE), left atrium-to-aorta ratio, ductal diameter and ductal shunt pattern. Left ventricular longitudinal, circumferential and radial strain, apex-basal rotation and twist were measured from the apical 4-chamber and short-axis views using STE. RESULTS: The mean gestational age was 30 ± 2.7 weeks with a mean birth weight of 1318 ± 485 g. Apical segments demonstrated higher longitudinal strain than basal and mid-ones. In all gestational ages, endocardial longitudinal strain was higher than the epicardial. Epicardial longitudinal strain significantly increased during the first 3 weeks, resulting in the change in basal rotation from counterclockwise to clockwise and thus in the acquisition of twist. Deformation parameters were higher in infants with a hemodynamic significant patents ductus arteriosus. CONCLUSIONS: Echocardiographic assessment of myocardial deformation parameters is feasible in preterm infants. Our data suggest that the maturational process of the myocardium is due to the development of the epicardial layer after birth, which allows the acquisition of the twist.
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Diagnóstico Precoz , Ecocardiografía/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Enfermedades del Prematuro/diagnóstico , Recien Nacido Prematuro , Disfunción Ventricular Izquierda/diagnóstico , Femenino , Estudios de Seguimiento , Edad Gestacional , Ventrículos Cardíacos/fisiopatología , Humanos , Recién Nacido , Masculino , Estudios Prospectivos , Sístole , Disfunción Ventricular Izquierda/fisiopatología , Función Ventricular IzquierdaRESUMEN
Surgical re-implantation of the left coronary artery (LCA) is the treatment of choice in anomalous origin of left coronary artery from pulmonary artery (ALCAPA). Despite normalization of left ventricular function after surgery, residual coronary lesions or myocardial fibrosis may be found. The aim of this study was to detect regional left ventricular dysfunction predictive of coronary lesions or residual myocardial fibrosis using speckle tracking. We enrolled ten patients treated with surgical re-implantation of LCA for ALCAPA. All patients were asymptomatic, and ejection fraction (EF) was normal. Using S-SR imaging, we studied longitudinal, radial, and circumferential function. A cardiac MRI was performed to assess myocardial fibrosis and the anatomy of the coronaries. In case of suspected coronary stenosis, a coronary angiography was performed. Finally, 20 normal subjects were enrolled as control group. Median age at surgery was 188 days, and mean follow-up was 8.7 ± 4.7 years. Longitudinal and circumferential functions were reduced in LCA territory compared to RCA territory and normal. MRI showed LCA stenosis in three of ten patients, confirmed by coronary angiography: these patients had the lowest longitudinal strain values in LCA territories (-11.7, -14.7 and -14.8%). Radial strain was preserved (Normal 45.6 ± 12.1, ALCAPA 43.5 ± 10.7%, p = ns), while circumferential strain was mildly depressed (-23.5 ± 3.8 vs. -20.3 ± 2.0%, p < 0.05). After LCA re-implantation, ALCAPA patients showed a residual sub-endocardial damage in LCA territories. Despite a normal systolic and diastolic function, the prevalence of residual coronary lesions was high. A mean longitudinal strain >-15% in LCA territories was able to identify those patients.
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Síndrome de Bland White Garland/diagnóstico por imagen , Síndrome de Bland White Garland/cirugía , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Vasos Coronarios/diagnóstico por imagen , Disfunción Ventricular Izquierda/diagnóstico por imagen , Síndrome de Bland White Garland/complicaciones , Niño , Preescolar , Enfermedad de la Arteria Coronaria/etiología , Vasos Coronarios/cirugía , Ecocardiografía , Fibrosis , Humanos , Lactante , Imagen por Resonancia Magnética , Miocardio/patología , Procedimientos Quirúrgicos VascularesRESUMEN
In August 2010, the Nit-Occlud® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24-48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud® Lê VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.
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Cateterismo Cardíaco/métodos , Fluoroscopía , Defectos del Tabique Interventricular/terapia , Hemólisis , Dispositivo Oclusor Septal , Adolescente , Niño , Preescolar , Europa (Continente) , Femenino , Defectos del Tabique Interventricular/clasificación , Humanos , Lactante , Masculino , Tempo Operativo , Sistema de Registros , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: The objective of this investigation is to evaluate the safety, the impact of endomyocardial biopsy (EMB) results in myocarditis management and the incidence of different etiologies of myocarditis in a pediatric population. BACKGROUND: Although EMB is an established diagnostic tool to evaluate suspected myocarditis, there is lack of clear diagnostic and management guidelines for myocarditis in pediatric patients, particularly in infants. METHODS: We performed a retrospective database review and subsequent outcomes analysis from five Italian pediatric cardiology centers to identify patients aged 0-18 years who underwent EMB for suspected myocarditis or inflammatory cardiomyopathy (ICMP) between 2009 and 2011. RESULTS: EMB was performed in 41 children, of which 16 were male. The population ranged between 16 days of age to 17 years (mean age at EMB = 5.2 ± 4.9 years). The overall incidence of EMB-related complications was 15.5% (31.2% in infants, and 6.8% in children > 1 year of age; P = 0.079) while the incidence of EMB-driven treatment changes was 29.2%. Histological examination together with PCR on heart biopsy specimens allowed an etiological diagnosis in 26/41 patients (63%). Among the 15 patients (36.5%) with diagnosis of dilated cardiomyopathy (DCM) 11 had idiopathic DCM. Finally, we found an overall incidence of death/cardiac transplantation of 24%. CONCLUSIONS: In a pediatric population with suspected myocarditis/ICMP, EMB was useful in confirming the diagnosis only in 41% of cases but showed an overall diagnostic power of 63%. As complications of EBM are not negligible, particularly in infants, the risk/benefit ratio should be taken into account in each patient.
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Biopsia , Miocarditis/patología , Miocardio/patología , Adolescente , Factores de Edad , Biopsia/efectos adversos , Niño , Preescolar , Bases de Datos Factuales , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Italia/epidemiología , Masculino , Miocarditis/epidemiología , Miocarditis/terapia , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Medición de Riesgo , Factores de RiesgoRESUMEN
OBJECTIVES: At the moment there are no standard guidelines for the treatment of autoimmune congenital heart block (CHB). We set out to carry out a prospective cohort study to evaluate the benefits, limits, and safety of a combined therapy protocol to treat antibody-related CHB. METHODS: Twelve consecutive pregnant patients positive to anti-SSA/Ro ± anti-SSB/La antibodies in whom CHB was detected were prospectively evaluated from 2009 to 2014. The treatment protocol consisted of: weekly plasmapheresis, fortnightly intravenous immunoglobulins (IVIG), and daily 4 mg betamethasone from CHB detection until delivery; IVIG was administered to the neonates soon after birth. RESULTS: At the time CHB was detected, six of the foetuses presented atrioventricular blocks of 2(nd) degree type and six of 3(rd) degree type. Two of the foetuses with a 2(nd) degree block reverted to a 1st degree block and one to a normal atrioventricular conduction. The condition was stable throughout the pregnancy in the other three cases of 2(nd) degree block. All six 3(rd) degree blocks were stable during pregnancy and confirmed at birth. After a mean of 37.6 months ± 19.6 SD post-birth, the infants with 1st, normal sinus rhythm, and 2(nd) degree blocks at birth were all found to be stable. During the follow-up (29 months ± 19.8 SD), pacemakers were implanted in three of the six infants with 3(rd) degree blocks. CONCLUSIONS: This combined therapy seems to be effective and safe in treating 2(nd) degree CHB, while its efficacy in treating 3rd degree CHB remains to be established.
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Enfermedades Autoinmunes/terapia , Betametasona/administración & dosificación , Glucocorticoides/administración & dosificación , Bloqueo Cardíaco/congénito , Inmunoglobulinas Intravenosas/administración & dosificación , Plasmaféresis , Adulto , Anticuerpos Antinucleares/sangre , Enfermedades Autoinmunes/sangre , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/inmunología , Betametasona/efectos adversos , Biomarcadores/sangre , Terapia Combinada , Esquema de Medicación , Quimioterapia Combinada , Ecocardiografía Doppler , Femenino , Edad Gestacional , Glucocorticoides/efectos adversos , Bloqueo Cardíaco/sangre , Bloqueo Cardíaco/diagnóstico , Bloqueo Cardíaco/inmunología , Bloqueo Cardíaco/terapia , Humanos , Inmunoglobulinas Intravenosas/efectos adversos , Recién Nacido , Masculino , Plasmaféresis/efectos adversos , Embarazo , Estudios Prospectivos , Factores de Tiempo , Resultado del Tratamiento , Ultrasonografía Prenatal/métodosRESUMEN
During the last decade interest in preserving pulmonary valve (PV) function has stimulated a few surgeons to apply valve-sparing techniques in repairing tetralogy of Fallot (TOF), with the aim of preserving long-term right ventricular function. Since June 2007, we embarked upon a program for preserving PV function in selected patients during TOF repair. More recently, the introduction of more complex PV plasty techniques allowed us to further extend the applicability of PV preservation techniques. We believe that preservation of PV function during early repair of TOF, by combining different intraoperative surgical maneuvers, can be extended to almost all patients with classic TOF.
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Valvuloplastia con Balón/métodos , Válvula Pulmonar , Tetralogía de Fallot/cirugía , Humanos , Lactante , Selección de Paciente , Función Ventricular DerechaRESUMEN
BACKGROUND AND AIM OF THE STUDY: Congenital mitral valve (MV) dysplasia is a relatively rare and highly complex cardiac disease. We present our results and illustrate the techniques used to repair these valves. METHODS: Between 1972 and 2014, 100 consecutive patients underwent surgical repair of congenital MV dysplasia at our institution. Predominant MV regurgitation was present in 53 patients (53%) whereas mitral stenosis was prevalent in 47 (47%). RESULTS: There were five early (5%) and eight late deaths (9%). Actuarial survival was 95%, 94%, and 93% at 5, 10, and 20 years, respectively. Sixteen patients (18%) required reintervention due to subsequent MV dysfunction. Actuarial freedom from reintervention for MV dysfunction was 95%, 92%, and 89% at 5, 10, and 20 years, respectively. CONCLUSIONS: The mechanism underlying the valve dysfunction in congenital mitral valve dysplasia is multifactorial and requires the application of a variety of surgical techniques for repair. doi: 10.1111/jocs.12743 (J Card Surg 2016;31:352-356).
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Procedimientos Quirúrgicos Cardíacos/métodos , Enfermedades de las Válvulas Cardíacas/cirugía , Válvula Mitral/cirugía , Adolescente , Adulto , Anciano , Niño , Preescolar , Femenino , Estudios de Seguimiento , Enfermedades de las Válvulas Cardíacas/congénito , Enfermedades de las Válvulas Cardíacas/diagnóstico , Humanos , Lactante , Masculino , Persona de Mediana Edad , Válvula Mitral/anomalías , Válvula Mitral/diagnóstico por imagen , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
Children affected by hemodynamically significant congenital heart disease (HSCHD) experience severe respiratory complications that can increase the frequency of hospitalizations. The aim of the SINERGY study was to describe the incidence of respiratory diseases and to collect information on active and passive immunoprophylaxis in the first 2 years of life. In this retrospective, multicenter, and epidemiologic study, children with HSCHD were enrolled across 11 Italian sites. Children born between December 31, 2007, and December 31, 2012, were observed during their first 2 years of life. Data were collected through hospital database searches and parent interviews. Four hundred twenty children were enrolled: 51.7 % were female, 79.5 % were born full-term (≥37 weeks), and 77.6 % weighed >2500 g at birth. The most frequent heart defects were ventricular septal defect (23.1 %) and coarctation of the aorta (14.3 %). The incidence of respiratory diseases was 63.1 %. Frequent respiratory diseases not requiring hospitalization were upper respiratory tract infections (76.4 %), acute bronchitis (43.3 %), and influenza (22.1 %), while those requiring hospitalization were bronchitis and bronchiolitis (8.3 % each one). While active immunoprophylaxis was applied with wide compliance (diphtheria/pertussis/tetanus, 99.5 %; Haemophilus influenzae type b, 72.5 %; pneumococcus, 79.9 %; meningococcus, 77.4 %), only 54 % of children received respiratory syncytial virus (RSV) passive prophylaxis (palivizumab). Of the 35 hospitalizations due to bronchiolitis, 27 (77.1 %) did not receive prophylaxis against RSV, compared with 8 (22.9 %) who received prophylaxis (P < 0.0001). Children with HSCHD are at major risk of respiratory diseases. Passive immunoprophylaxis can help to prevent hospitalizations for bronchiolitis.
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Cardiopatías Congénitas , Anticuerpos Monoclonales , Anticuerpos Monoclonales Humanizados , Antivirales , Niño , Femenino , Hospitalización , Humanos , Incidencia , Italia , Masculino , Infecciones por Virus Sincitial Respiratorio , Estudios RetrospectivosRESUMEN
BACKGROUND: Clinical utility of remote monitoring of implantable cardiac devices has been previously demonstrated in several trials in the adult population. The aim of this study was to assess the clinical utility of remote monitoring in a pediatric population undergoing pacemakers implantation. METHODS: The study population included 73 consecutive pediatric patients who received an implantable pacemaker. The remote device check was programmed for every 3 months and all patients had a yearly out-patient visit. Data on device-related events, hospitalization, and other clinical information were collected during remote checks and out-patient visits. RESULTS: During a mean follow-up of 18 ± 10 months, 470 remote transmissions were collected and analyzed. Two deaths were reported. Eight transmissions (1.7%) triggered an urgent out-patient visit. Twenty percent of transmissions reported evidence of significant clinical or technical events. All young patients and their families were very satisfied when using remote monitoring to replace out-patient visits. CONCLUSIONS: The ease in use, together with satisfaction and acceptance of remote monitoring in pediatric patients, brought very good results. The remote management of our pediatric population was safe and remote monitoring adequately replaced the periodic out-patient device checks without compromising patient safety.
Asunto(s)
Estimulación Cardíaca Artificial/métodos , Cardiopatías Congénitas/terapia , Monitoreo Fisiológico/métodos , Tecnología de Sensores Remotos/métodos , Adolescente , Niño , Preescolar , Femenino , Cardiopatías Congénitas/fisiopatología , Humanos , Lactante , Masculino , Estudios ProspectivosRESUMEN
AIMS: During the last decade the cooperation between surgeons and cardiologists has further expanded by combining surgical and interventional techniques (CCBSI) performed in the operating room, without the use of fluoroscopy. We sought to evaluate the results of our experience with CCBSI. METHODS: All children with congenital heart disease (CHD) who underwent a CCBSI in the operating room between June 2007 and January 2014 were enrolled. RESULTS: Sixty-eight patients were included. Median age at CCBSI was five months (range 1-48 months). The three main diagnoses leading to surgery included: (1) tetralogy of Fallot (TOF) (n = 40), (2) muscular ventricular septal defects (VSD) (n = 12), (3) single ventricle with pulmonary artery branch stenosis (n = 4). There were 72 catheter-based procedures associated with surgical maneuvres, including: (1) transatrial balloon dilation (BD) of the pulmonary valve (n = 45), (2) transinfundibular BD of the main pulmonary artery trunk (n = 12), (3) perventricular VSD closure with septal occluder (n = 8), (4) BD of pulmonary artery branches (n = 5), and other less common procedures (n = 2). There were no procedure-related complications and no hospital deaths. Median follow-up time was four years (range 0.95-7.9 years). There was one late death for respiratory distress after transapical balloon dilation of the aortic valve. One patient required BD and stenting of the left pulmonary artery branch 3.6 years after intraoperative BD for residual stenosis. CONCLUSIONS: The CCBSI represents a safe and effective treatment for selected patients with complex CHD. It will be helpful in minimizing patients' surgical trauma and in shortening or avoiding the use of cardiopulmonary bypass.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Cardiopatías Congénitas/terapia , Cateterismo Cardíaco/métodos , Puente Cardiopulmonar , Preescolar , Terapia Combinada , Dilatación/métodos , Proteínas de Escherichia coli , Femenino , Humanos , Lactante , Masculino , Tempo Operativo , Arteria Pulmonar , Válvula Pulmonar , Dispositivo Oclusor Septal , Stents , Tetrahidrofolato Deshidrogenasa , Factores de Tiempo , Resultado del TratamientoRESUMEN
Increasingly, more patients with univentricular heart reach adulthood. Therefore, long-term psychological features are an important concern. The aim of this study was to evaluate the clinical and psychological profile of post-Fontan adult patients and to identify the most significant determinants of quality of life. In this retrospective cross-sectional study, we reviewed the surgical and medical history of post-Fontan adult patients. Patients underwent a 24-h electrocardiogram, echocardiography and exercise testing. Self-report questionnaires were used to assess the Work Ability Index, quality of life (Satisfaction with Life Scale), perceived health status (SF-36 questionnaire), coping strategies (Brief Cope questionnaire) and presence of mood disorders (Hospital Anxiety and Depression Scale). Thirty-nine patients aged between 18 and 48 years (mean 27.5 years) were enrolled. The mean follow-up was 21.5 years. Most patients were unmarried (82.9 %), had a high school diploma (62.9 %) and were employed (62.9 %). Twenty-nine patients (82.3 %) had at least one long-term complication. The median single ventricle ejection fraction was 57 %, and the median maximal oxygen consumption was 26.8 ml/min/kg. This population tended to be anxious and to use adaptive coping strategies. Quality of life was perceived as excellent or good in 57.2 % of cases and was not related to either cardiac function or exercise capacity. Both quality of life and SF-36 domains were related to the Work Ability Index. This cohort of post-Fontan adult patients enjoyed a good quality of life irrespective of disease severity.