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1.
JAMA Cardiol ; 2019 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-31411652

RESUMO

Importance: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk. Objective: To develop and validate an SCD risk prediction model that provides individualized risk estimates. Design, Setting, and Participants: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017. Exposures: The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping. Main Outcomes and Measures: A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise). Results: Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95%, CI 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years. Conclusions and Relevance: This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

2.
Pediatr Dev Pathol ; : 1093526619865641, 2019 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-31335286

RESUMO

Human parvovirus B19 represents the most common etiology of myocarditis in the pediatric population. Although it usually causes a benign exanthematic viral infection, parvovirus B19 may also present as disseminated disease with tropism for the myocardium, causing heart failure with high mortality. We present the case of a 2-year-old patient with fulminating acute myocarditis in whom the histological, immunophenotypic, and microbiological findings in necropsy showed multiorgan involvement caused by parvovirus B19. The autopsy revealed changes due to infection with parvovirus B19 as well as hypoxic-ischemic and secondary autoimmune changes. Medullary aplasia was observed, transmural lymphocyte myocarditis, lymphocytosis in the dermis with endothelial cells positive for parvovirus B19 in immunohistochemistry, cholestatic hepatitis due to ischemia and autoimmune hepatitis, lymphadenitis, and signs of hemophagocytosis. We also found hypoxic-ischemic encephalopathy.

5.
Pediatr Cardiol ; 39(1): 51-56, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28986648

RESUMO

Chest pain is a typical symptom of acute myocarditis in adolescents. It may be indistinguishable from myocardial ischemia so it is called "infarct-like pattern." Cardiovascular magnetic resonance has an important role as a non-invasive diagnostic tool. The aim of our study is to provide a description of an acute myocarditis series with infarct-like pattern and to evaluate the cardiovascular magnetic resonance role in a pediatric population. We included all pediatric patients (0-16 years) admitted to our hospital (May 2007-May 2016) with clinical diagnosis of acute myocarditis and infarct-like presentation (chest pain, EKG alterations, and released cardiac biomarkers). Diagnosis was confirmed with cardiovascular magnetic resonance using Lake Louise criteria. Seven patients (five males, two females) with a median age of 14 years (12.5-15.2) were included. All patients showed ST-segment changes and released cardiac biomarkers. Three patients had left ventricular hypertrophy and two presented mild systolic left ventricular dysfunction. All patients had at least two positive Lake Louise criteria. Late gadolinium enhancement was positive in all of them. With a median follow-up of 23 months (8-47), all of them are alive, with no cardiac symptoms and normal ventricular function. Infarct-like pattern is a typical presentation of acute myocarditis in adolescents. CMR should be performed in this population and may be considered as a first-line diagnostic tool. Its high sensitivity in infarct-like acute myocarditis may allow us to avoid endomyocardial biopsy. Unlike what was described in adults, late gadolinium enhancement does not imply worse outcome in our series.


Assuntos
Imagem Cinética por Ressonância Magnética/métodos , Miocardite/diagnóstico , Miocárdio/patologia , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Meios de Contraste , Diagnóstico Diferencial , Eletrocardiografia , Feminino , Gadolínio , Humanos , Lactente , Masculino , Infarto do Miocárdio/diagnóstico , Estudos Retrospectivos
12.
Pediatr Cardiol ; 37(4): 623-8, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26687178

RESUMO

Acute kidney injury (AKI) is frequent in the postoperative period of pediatric heart surgery and leads to significant morbidity and mortality. Renal replacement therapies (RRTs) are often used to treat AKI; however, these therapies have also been associated with higher mortality rates. Earlier initiation of RRT might improve outcomes. This study aims to investigate the relationship between the RRT and morbidity and mortality after pediatric heart surgery. We performed a single-center retrospective study of all children undergoing pediatric heart surgery between April 2010 and December 2012 at a tertiary children's hospital. A total of 480 patients were included. Of those, 109 (23 %) were neonates and 126 patients (26 %) developed AKI within the first 72 postoperative hours. Patients who developed AKI had longer PICU admissions [12 days (4-37.75) vs. 4 (2-11); p < 0.001] and hospital length of stay [27 (11-53) vs. 14 (8-24) p < 0.001] and higher mortality [22/126 (17.5 %) vs. 13/354 (3.7 %); p < 0.001]. RRT techniques were used in 32 (6.6 %) patients [18/109 (16 %) neonates and 14/371 (3.8 %) infants and children; p < 0.01], with 25 (78 %) receiving peritoneal dialysis (PD) and 7 (22 %) continuous RRT (CRRT). Patients who received PD within the first 24 postoperative hours had lower mortality compared with those in whom PD was initiated later [4/16 (25 %) vs. 4/9 (44.4 %)]. Mortality among patients who received CRRT was 28.6 % (2/7). No deaths were reported in patients treated with CRRT within the first 24 postoperative hours. Postoperative AKI is associated with higher mortality in children undergoing cardiac surgery. Early initiation of RRT, both PD in neonates and CRRT in pediatric patients, might improve morbidity and mortality associated with AKI.


Assuntos
Lesão Renal Aguda/terapia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Intervenção Médica Precoce , Complicações Pós-Operatórias/terapia , Terapia de Substituição Renal , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/mortalidade , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Análise Multivariada , Diálise Peritoneal/estatística & dados numéricos , Complicações Pós-Operatórias/mortalidade , Estudos Retrospectivos , Espanha , Fatores de Tempo
15.
World J Pediatr Congenit Heart Surg ; 6(3): 438-42, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26180162

RESUMO

BACKGROUND: Correlation between ventricular end-diastolic pressure and pulsed Doppler and tissue Doppler-derived E/e' ratio has been widely reported in adults but scarcely studied in children with congenital heart diseases. This ratio is defined as the relationship between diastolic transmitral flow velocity (cm/s; E) and myocardial diastolic relaxation velocity (cm/s; e') in the lateral aspect of the mitral annulus. Our main objective was to ascertain whether a correlation existed between direct measurement of left atrial pressure and echocardiographic E/e' ratio in children after heart surgery. METHODS: Prospective study including 27 consecutive children after pediatric heart surgery. Data were analyzed according to whether they were obtained within the first 72 hours following surgery or later on. Sensitivity, specificity, positive and negative predictive values, and areas under the receiver-operating characteristics curve of E/e' ratio in detection of left atrial pressure values ≥13 mm Hg were evaluated. RESULTS: Forty-eight studies were conducted in 27 patients. Thirty-two studies were performed during the first 72 hours after heart surgery and 16 beyond the third day. Median patient age was 0.82 years (5 days-16 years). Median left atrial pressure values and E/e' measurements of the whole cohort (N = 48) were 12.0 and 10.2, respectively. Intraclass correlation index between left atrial pressure values and echocardiographic E/e' ratio was 0.35, 0.25 for studies performed within 72 hours, but 0.78 (P < .01) for those performed later. There was also a high positive predictive value, since in 13 (87%) of 15 studies with an E/e' ratio ≥13, the left atrial pressure was ≥13 mm Hg. CONCLUSION: While echocardiographic E/e' ratio did not show a good correlation with left atrial pressure in the immediate postoperative period, the positive predictive value may suffice to aid clinicians in predicting elevated pressures.


Assuntos
Pressão Atrial/fisiologia , Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardíacos/métodos , Ecocardiografia Doppler , Átrios do Coração/fisiopatologia , Cardiopatias Congênitas/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos
16.
World J Pediatr Congenit Heart Surg ; 6(3): 480-3, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26180171

RESUMO

Transposition of the great arteries with intact ventricular septum and persistent pulmonary hypertension (TGA-IVS PPHN) is a rare association with a poor prognosis. We report the case of a term newborn with TGA-IVS PPHN successfully managed with perioperative extracorporeal membrane oxygenation (ECMO) and aggressive pulmonary vasodilation therapy that underwent successful arterial switch procedure. A lung biopsy obtained during the surgical procedure showed pulmonary interstitial glycogenosis, a reversible condition. Concerns over left ventricle deconditioning after ECMO could be minimized with appropriate management and monitoring of the ductus arteriosus and appropriate timing of surgery.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Permeabilidade do Canal Arterial/cirurgia , Oxigenação por Membrana Extracorpórea/métodos , Doença de Depósito de Glicogênio/etiologia , Síndrome da Persistência do Padrão de Circulação Fetal/cirurgia , Transposição dos Grandes Vasos/cirurgia , Doença de Depósito de Glicogênio/terapia , Humanos , Recém-Nascido
17.
Eur J Pediatr ; 174(7): 957-63, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25652766

RESUMO

UNLABELLED: Surgical site infection (SSI) remains a major source of morbidity, mortality, and increased health care costs in children undergoing heart surgery. The aim of this study was to assess the effectiveness of an intervention program designed to reduce the high incidence of SSI observed at our center in pediatric patients. An interdisciplinary infection control program including pre-, intra-, and postoperative measures was introduced for children undergoing heart surgery with cardiopulmonary bypass. We conducted a quasi-experimental interventional study comparing a pre-intervention cohort (June 2009 to March 2010) and a post-intervention cohort (July 2011 to July 2012). A significant drop in SSI incidence from 10.9 % (95 % CI 4.7-18.8) to 1.92 % (95 % CI 0.4-5.52) was observed. Variables significantly associated with infection risk were median age (14 days in infected vs 2.3 years in non-infected patients; p<0.01), hospitalization unit (10.3 % SSI cumulative incidence in the neonatal intensive care unit vs 0 cases in the pediatric intensive care unit; p<0.01), and median preoperative hospital stay (14 days in infected vs 1 day in non-infected patients; p=0.03). CONCLUSIONS: The implementation of a new intervention program was associated with an 82 % (95 % CI 34-94) reduction in SSI incidence in children undergoing heart surgery at our center. WHAT IS KNOWN: • Surgical site infection (SSI) is associated with significant morbidity and mortality following pediatric cardiac surgery. • Younger patients and longer cardiopulmonary bypass times are associated with higher SSI rates. What is New: • Comprehensive infection control program including preoperative, intraoperative and postoperative nonpharmacologic measures is a key factor for the prevention of SSI. • A significant reduction in SSI rates can be achieve despite a narrower-spectrum antibiotic usage.


Assuntos
Ponte Cardiopulmonar , Controle de Infecções , Esternotomia , Infecção da Ferida Cirúrgica/prevenção & controle , Fatores Etários , Estudos de Coortes , Feminino , Hospitalização , Humanos , Incidência , Lactente , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Unidades de Terapia Intensiva Pediátrica , Masculino , Equipe de Assistência ao Paciente , Fatores de Risco , Espanha/epidemiologia , Infecção da Ferida Cirúrgica/epidemiologia
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