RESUMO
OBJECTIVES: To evaluate the clinical utility of pulmonary artery capacitance index (PACi) in the assessment of disease severity and prognostic value in children with idiopathic and heritable pulmonary arterial hypertension (PAH). STUDY DESIGN: PACi is defined as the ratio of stroke volume index over pulmonary pulse pressure. A retrospective study was performed to compare PACi, brain natriuretic peptide (BNP), 6-minute walk distance, New York Heart association (NYHA) functional class, and adverse outcomes (hospitalization due to heart failure, lung transplantation, and cardiac mortality) in 72 Japanese children (10 ± 3.6 years) with idiopathic and heritable PAH. RESULTS: PACi had significant correlations with pulmonary vascular resistance index (r =-0.73, P < .0001), BNP levels (r = -0.40, P = .0008), and 6-minute walk distance (r = 0.57, P < .05). Statistically significant differences in PACi were observed between NYHA functional class II vs combined III and IV (median; 1.1 vs 0.6 mL/mm Hg/m2, respectively, P < .05). There were 25 of 72 (35%) children who had an adverse event including initiation of hospitalization due to heart failure, lung transplantation, and death. Cumulative event-free survival rate was significantly lower when PACi was <0.85 mL/mm Hg/m2 (log-rank test, P < .0001). CONCLUSIONS: PACi correlated with BNP and NYHA functional class and may serve as a strong prognostic marker in children with idiopathic and heritable PAH.
Assuntos
Hipertensão Pulmonar Primária Familiar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Capacitância Vascular , Criança , Feminino , Humanos , Masculino , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
OBJECTIVE: To determine whether the N-terminal fragment of B-type natriuretic peptide (NTproBNP) was a biomarker of clinical, laboratory, and echocardiographic abnormalities in children with homozygous sickle cell disease. STUDY DESIGN: We conducted a single-center retrospective study that consisted of analysis of data from November 2007 to December 2010. We correlated serum NTproBNP with clinical and laboratory findings, echocardiographic data, and New York Heart Association (NYHA) functional class. RESULTS: NTproBNP levels from 42 children (median age, 9 years; 52% female) had significant correlations with hemoglobin (r = -0.63, P < .05), and echocardiographic measurements including tricuspid regurgitant velocity (r = 0.46, P < .05), lateral E' (r = -0.52, P < .05), and lateral E/E' ratio (r = 0.60, P < .05), suggesting diastolic dysfunction. In addition, NTproBNP levels increased from NYHA functional class I to class III and had a significant linear correlation with the NYHA functional class (r = 0.69, P < .05). CONCLUSIONS: NTproBNP correlated with low hemoglobin and tissue Doppler data as indicators of diastolic dysfunction. Elevated NTproBNP may be a prognostic biomarker for the presence of diastolic dysfunction related to anemia in children with sickle cell disease.