RESUMO
OBJECTIVE: To evaluate the prevalence of iron deficiency and its association with outcomes in children with heart failure. STUDY DESIGN: A single-center retrospective cohort study of patients with heart failure aged 1-21 years from July 2012 to June 2017 with available serum iron studies was performed. Subjects were analyzed in 2 groups: biventricular systolic heart failure (BiV) and single-ventricle congenital heart disease with systolic heart failure (SV). Iron deficiency was defined as ≥2 of the following: serum iron <50 µg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, or transferrin saturation <15%. The primary outcome was a composite adverse event (CAE) of ventricular assist device implantation, heart transplantation, or death, at 3 and 6 months from time of iron studies. RESULTS: Of the 107 subjects (77 BiV, 30 SV) included in the study, 56% were iron deficient. Demographics, etiology of heart failure, and chronicity of heart failure symptoms were not associated with iron deficiency. On multivariable analysis, in group BiV, iron deficiency was associated with CAE at 3 months (79% iron deficiency in CAE group vs 37% iron deficiency in non-CAE, P = .001, OR 7, 95% CI 2-21) and 6 months (76% iron deficiency in CAE vs 35% iron deficiency in non-CAE, P = .002, OR 7, 95% CI 2-24). In group SV, iron deficiency was associated with CAE at 6 months (79% iron deficiency in CAE vs 29% iron deficiency in non-CAE, P = .014, OR 8, 95% CI 2-32). CONCLUSIONS: Iron deficiency was present in 56% of the pediatric patients with heart failure who were evaluated with iron studies. Iron deficiency was associated with greater risk of ventricular assist device implantation, heart transplantation, or death.
Assuntos
Anemia Ferropriva/epidemiologia , Insuficiência Cardíaca/mortalidade , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Insuficiência Cardíaca/cirurgia , Transplante de Coração/estatística & dados numéricos , Coração Auxiliar/estatística & dados numéricos , Humanos , Tempo de Internação/estatística & dados numéricos , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVE: To determine frequency of missed heart failure diagnosis at first presentation among children with no known heart disease admitted with new-onset heart failure. STUDY DESIGN: Using a retrospective design, we reviewed electronic medical records of all patients aged <21 years with no known heart disease, hospitalized with new-onset heart failure during 2003-2015 at a tertiary-quaternary care institution. We assessed records for missed diagnosis of heart failure (primary outcome), associated process breakdowns, and clinical outcomes using a structured data collection instrument. RESULTS: Of 191 patients meeting inclusion criteria, 49% (94/191) were missed on first presentation. Most common incorrect diagnostic labels given to "missed" patients were bacterial infection (29%; 27/94), followed by viral illness (22%; 21/94) and gastroenteritis/hepatitis (21%; 20/94). On multivariable analysis, presentation to primary care provider (PCP), longer duration of symptoms (median 7 days), more than 2 symptoms of heart failure, and nausea/emesis were associated with missed diagnosis. On examining process breakdowns, 49% had errors in history-taking and 50% had no documentation of differential diagnoses. There was no difference in hospital mortality, length of stay, or mechanical circulatory support in missed vs not-missed cohorts. Unnecessary noninvasive and invasive tests were performed in 18% and 4% of patients, respectively. CONCLUSIONS: Nearly one-half of children with no known heart disease hospitalized with systolic heart failure were missed at first presentation and underwent significant nonrelevant treatment and testing. Initial presentation to the PCP, longer duration of symptoms before presentation, and nausea/emesis were associated with missed diagnosis.
Assuntos
Insuficiência Cardíaca Sistólica/diagnóstico , Diagnóstico Ausente/estatística & dados numéricos , Criança , Pré-Escolar , Registros Eletrônicos de Saúde , Feminino , Insuficiência Cardíaca Sistólica/epidemiologia , Mortalidade Hospitalar , Hospitalização , Humanos , Lactente , Tempo de Internação , Masculino , Análise Multivariada , Estudos Retrospectivos , Centros de Atenção Terciária , Procedimentos DesnecessáriosAssuntos
Anomalia de Ebstein/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/cirurgia , Coração Auxiliar , Imagem Multimodal/métodos , Adolescente , Anomalia de Ebstein/complicações , Ecocardiografia Doppler/métodos , Eletrocardiografia/métodos , Serviço Hospitalar de Emergência , Seguimentos , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/etiologia , Humanos , Masculino , Alta do Paciente , Radiografia Torácica/métodos , Medição de Risco , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
OBJECTIVES: The PediaFlow (HeartWare International, Inc, Framingham, Mass) is a miniature, implantable, rotodynamic, fully magnetically levitated, continuous-flow pediatric ventricular assist device. The fourth-generation PediaFlow was evaluated in vitro and in vivo to characterize performance and biocompatibility. METHODS: Supported by 2 National Heart, Lung, and Blood Institute contract initiatives to address the limited options available for pediatric patients with congenital or acquired cardiac disease, the PediaFlow was developed with the intent to provide chronic cardiac support for infants as small as 3 kg. The University of Pittsburgh-led Consortium evaluated fourth-generation PediaFlow prototypes both in vitro and within a preclinical ovine model (n = 11). The latter experiments led to multiple redesigns of the inflow cannula and outflow graft, resulting in the implantable design represented in the most recent implants (n = 2). RESULTS: With more than a decade of extensive computational and experimental efforts spanning 4 device iterations, the AA battery-sized fourth-generation PediaFlow has an operating range of 0.5 to 1.5 L/min with minimal hemolysis in vitro and excellent hemocompatibility (eg, minimal hemolysis and platelet activation) in vivo. The pump and finalized accompanying implantable components demonstrated preclinical hemodynamics suitable for the intended pediatric application for up to 60 days. CONCLUSIONS: Designated a Humanitarian Use Device for "mechanical circulatory support in neonates, infants, and toddlers weighing up to 20 kg as a bridge to transplant, a bridge to other therapeutic intervention such as surgery, or as a bridge to recovery" by the Food and Drug Administration, these initial results document the biocompatibility and potential of the fourth-generation PediaFlow design to provide chronic pediatric cardiac support.
Assuntos
Fontes de Energia Elétrica , Insuficiência Cardíaca/terapia , Coração Auxiliar , Hemodinâmica , Implantação de Prótese/instrumentação , Função Ventricular , Fatores Etários , Animais , Animais Recém-Nascidos , Peso Corporal , Pré-Escolar , Fontes de Energia Elétrica/efeitos adversos , Insuficiência Cardíaca/fisiopatologia , Coração Auxiliar/efeitos adversos , Hemólise , Humanos , Lactente , Recém-Nascido , Teste de Materiais , Miniaturização , Desenho de Prótese , Carneiro DomésticoRESUMO
OBJECTIVE: To evaluate fibrosis and fibrosis-related gene expression in the myocardium of pediatric subjects with single ventricle with right ventricular failure. STUDY DESIGN: Real-time quantitative polymerase chain reaction was performed on explanted right ventricular myocardium of pediatric subjects with single ventricle disease and controls with nonfailing heart disease. Subjects were divided into 3 groups: single ventricle failing (right ventricular failure before or after stage I palliation), single ventricle nonfailing (infants listed for primary transplantation with normal right ventricular function), and stage III (Fontan or right ventricular failure after stage III). To evaluate subjects of similar age and right ventricular volume loading, single ventricle disease with failure was compared with single ventricle without failure and stage III was compared with nonfailing right ventricular disease. Histologic fibrosis was assessed in all hearts. Mann-Whitney tests were performed to identify differences in gene expression. RESULTS: Collagen (Col1α, Col3) expression is decreased in single ventricle congenital heart disease with failure compared with nonfailing single ventricle congenital heart disease (P = .019 and P = .035, respectively), and is equivalent in stage III compared with nonfailing right ventricular heart disease. Tissue inhibitors of metalloproteinase (TIMP-1, TIMP-3, and TIMP-4) are downregulated in stage III compared with nonfailing right ventricular heart disease (P = .0047, P = .013 and P = .013, respectively). Matrix metalloproteinases (MMP-2, MMP-9) are similar between nonfailing single ventricular heart disease and failing single ventricular heart disease, and between stage III heart disease and nonfailing right ventricular heart disease. There is no difference in the prevalence of right ventricular fibrosis by histology in subjects with single ventricular failure heart disease with right ventricular failure (18%) compared with those with normal right ventricular function (38%). CONCLUSIONS: Fibrosis is not a primary contributor to right ventricular failure in infants and young children with single ventricular heart disease. Additional studies are required to understand whether antifibrotic therapies are beneficial in this population.
Assuntos
Regulação para Baixo , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/patologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/patologia , Miocárdio/patologia , Criança , Pré-Escolar , Feminino , Fibrose , Marcadores Genéticos , Insuficiência Cardíaca/congênito , Insuficiência Cardíaca/genética , Insuficiência Cardíaca/patologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/genética , Síndrome do Coração Esquerdo Hipoplásico/patologia , Lactente , Recém-Nascido , Masculino , Reação em Cadeia da Polimerase em Tempo RealAssuntos
Dispneia/etiologia , Eletrocardiografia , Propranolol/uso terapêutico , Taquicardia Paroxística/diagnóstico por imagem , Taquicardia Supraventricular/diagnóstico por imagem , Taquicardia Supraventricular/tratamento farmacológico , Tosse/diagnóstico , Tosse/etiologia , Dispneia/diagnóstico , Serviço Hospitalar de Emergência , Seguimentos , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/terapia , Humanos , Lactente , Masculino , Radiografia Torácica/métodos , Medição de Risco , Índice de Gravidade de Doença , Taquicardia Paroxística/complicações , Taquicardia Paroxística/tratamento farmacológico , Taquicardia Supraventricular/complicações , Taquicardia Supraventricular/fisiopatologia , Resultado do TratamentoRESUMO
OBJECTIVES: Empiric treatment with milrinone, a phosphodiesterase (PDE) 3 inhibitor, has become increasingly common in patients with single ventricle heart disease of right ventricular (RV) morphology (SRV); our objective was to characterize the myocardial response to PDE3 inhibition (PDE3i) in the pediatric population with SRV. STUDY DESIGN: Cyclic adenosine monophosphate levels, PDE activity, and phosphorylated phospholamban (PLN) were determined in explanted human ventricular myocardium from nonfailing pediatric donors (n = 10) and pediatric patients transplanted secondary to SRV. Subjects with SRV were further classified by PDE3i treatment (n = 13 with PDE3i and n = 12 without PDE3i). RESULTS: In comparison with nonfailing RV myocardium (n = 8), cyclic adenosine monophosphate levels are lower in patients with SRV treated with PDE3i (n = 12, P = .021). Chronic PDE3i does not alter total PDE or PDE3 activity in SRV myocardium. Compared with nonfailing RV myocardium, SRV myocardium (both with and without PDE3i) demonstrates equivalent phosphorylated PLN at the protein kinase A phosphorylation site. CONCLUSIONS: As evidenced by preserved phosphorylated PLN, the molecular adaptation associated with SRV differs significantly from that demonstrated in pediatric heart failure because of dilated cardiomyopathy. These alterations support a pathophysiologically distinct mechanism of heart failure in pediatric patients with SRV, which has direct implications regarding the presumed response to PDE3i treatment in this population.