Inflammatory markers in Eisenmenger syndrome and their association with clinical outcomes. A cross-sectional comparative study.

BACKGROUND: Inflammation may be an important factor contributing to the progression of Eisenmenger syndrome (ES). The purpose of the current study was to: characterize the inflammatory profile in ES patients and compare measures to reference values for congenital heart disease and pulmonary arterial hypertension (CHD-PAH); and investigate whether inflammatory markers are associated with other clinical markers in ES. METHODS: Twenty-seven ES patients were prospectively selected and screened for systemic inflammatory markers, including interleukin (IL)-1ß, tumor necrosis factor-alpha (TNF-α) and IL-10. Clinical data and echocardiographic parameters were obtained, with concomitant analysis of ventricular function. Functional capacity was assessed using the 6-min walk test (6MWT). Renal function and blood homeostasis were evaluated by the level of blood urea nitrogen (BUN), creatinine, and plasma electrolytes. RESULTS: Patients with ES expressed higher IL-10, IL-1ß and TNF-α compared to reference values of patients with CHD-PAH. IL-10 was negatively associated with BUN (r = -0.39,p = 0.07), creatinine (r = -0.35, p = 0.002), sodium (r = -0.45, p = 0.03), and potassium (r = -0.68, p = 0.003). IL-10 was positively associated with bicarbonate (r = 0.45, p = 0.02) and trended toward a positive association with right ventricular fractional area change (RVFAC) (r = 0.35, p = 0.059). IL-1ß was negatively associated with potassium (r = -0.5, p = 0.01). TNF-α demonstrated positive association with creatinine (r = 0.4,p = 0.006), BUN (r = 0.63,p = 0.003), sodium (r = 0.44, p = 0.04), potassium (r = 0.41, p = 0.04), and was negatively associated with RVFAC (r = -0.38,p = 0.03) and 6MWT distance (r = -0.54, p = 0.004). CONCLUSION: ES patients exhibit a more severe inflammatory profile compared to reference values for CHD-PAH. Furthermore, inflammatory markers are related to renal dysfunction, right ventricular impairment and poorer functional capacity.

Effects of acute oxygen supplementation on functional capacity and heart rate recovery in Eisenmenger syndrome.

BACKGROUND: Eisenmenger syndrome (ES) precipitates the extreme manifestation of pulmonary hypertension, which leads to severe functional limitation and poor quality of life. The propose of the current study was: 1) examined the acute effects of 40% oxygen supplementation during the 6-minute walk test (6MWT); and 2) evaluate the relation between exercise capacity and clinical cardiac parameters in patients with ES. METHODS: Thirty subjects were prospectively included; all were submitted to a 6MWT with compressed air and with 40% of oxygen. Heart rate recovery at the first minute (HRR1) and perceived effort Borg scale for dyspnea and lower limb fatigue were recorded in both tests scenarios. RESULTS: The 6MWT distance was modestly, negatively associated with pulmonary vascular resistance (PVR) [r=0.46, p=0.02]. Patients improved 6MWT distance (p<0.001) and exhibited a faster HRR1 (p<0.001) with 40% supplemental oxygen compared to compressed air. With 40% supplemental oxygen, subjects revealed lower dyspnea and lower limb fatigue compared to 6MWT without oxygen supplementation (p<0.001). The amount of change in the 6MWT distance from air to oxygen was moderate, positively associated with tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) [r=0.50, p=0.03; r=0.64, p<0.001, respectively]. CONCLUSION: Acute 40% oxygen supplementation in patients with Eisenmenger syndrome led to an improvement in 6MWT distance, faster HRR1 and lower dyspnea and lower limb fatigue perception. Moreover, functional capacity was positively associated with right ventricular parameters.

Echocardiographic parameters and brain natriuretic peptide in patients after surgical repair of tetralogy of Fallot.

BACKGROUND: Although the residual lesions after surgical correction of tetralogy of Fallot (TOF) can be evaluated by Doppler echocardiography (DE), the relation of DE parameters with the proBNP level, a potential biomarker of right ventricle overload, is not well known. The objective of this study was to evaluate the DE parameters and their relation to proBNP levels. METHODS: proBNP plasma level and Doppler echocardiography parameters were obtained on the same day in 49 patients later after repair of TOF (mean age of 14.7 years, 51% female, mean PO time of 9.5 years). The DE parameters studied were the dimensions of the right atrium (RA) and ventricle (RV), RV diastolic and systolic function, and residual pulmonary lesions. The relation between them and proBNP levels were analyzed and the cutoff values of DE parameters for elevated proBNP determined. RESULTS: proBNP was elevated in 53% and correlated with RV diastolic diameter (r = 0.41; P = 0.003), RA longitudinal (r = 0.52; P = 0.0001) and transversal (r = 0.47; P = 0.001) diameters, pressure half time of pulmonary regurgitation (PR) velocity (PHT) (r =-0.42; P = 0.005), and the PR index (r =-0.60; P < 0.001). By multivariate analysis, the PR index (r =-597; P = 0,001; CI: -913.2 to -280.8) and RA longitudinal (r = 7.74; P < 0,001; CI 4.18 to 11.31) were independent predictors of elevated proBNP. PHT lower than 64 msec (0.76) and PRi lower than 0.65 (0.81) had the best accuracy for elevated proBNP. CONCLUSION: proBNP may be increased in patients after surgical repair of TOF, correlated with the size of right cardiac chambers and the severity of PR.

Padrões de pressão e de velocidade de fluxo num modelo in vitro de anastomose sistêmico-pulmonar. Importância para análise do estado funcional / Patterns of pressure and flow velocity distribution in a in vitro model of systemic to pulmonary artery anastomosis. Importance for functional evaluation

A função das anastomoses sistêmicos-pulmonares(S-P) e a detecção de obstrução parcial são dificeis pela ecocardiografia. Objetivo: Analisar os padrões de pressão e de distribuição espacial da velocidade do fluxo num modelo in vitro de S-P com ou sem obstrução parcial. Método: Foi utilizado um modelo de fluxo contínuo: dois tubos - circulações sistêmica(S) e pulmonar(P), foram conectados por outro - anastomose sistêmico-pulmonar (S-P). O modelo foi estudado sem obstrução e com uma obstrução longa na S-P, com medida direta das pressões em S,P e na S-P antes e após a obstrução, e análise simultânea com Doppler contínuo (DC) e mapeamento de fluxo em cores (MFC). Resultados: Sem obstrução a pressão de 5 (36,6 mmHg) foi maior do que em P(9,4mmHg) e S-P(6,3 mmHg)(p menor 0,05), sem diferença significante entre S-P e P. O maior gradiente de pressão por cateter (30,4mmHg) e por DC(27,9mmHg) foi na junção de S com S-P com sinais de aceleração de fluxo proximal ao MFC. Com obstrução, as pressões em S(95,8 mmHg) e S-P(75,4 mmHg) antes da obstrução não foram diferentes entre si, mas maiores do que as pressões de P(35,9 mmHg) e S-P(33 mmHg) após a obstrução (p menor 0,05), com gradientes de pressão pelo DC semelhantes e com sinais de aceleração proximal à junção de S com S-P, e antes da abstrução em S-P ao MFC. Conclusão: Com obstrução de S-P observou-se gradiente de opressão baixo ao DC na junção de S com S-P, e altos no trajeto da S-P com sinais de aceleração de fluxo, sem obstrução os maiores gradientes de pressão e aceleração de fluxo ocorreram na junção de S com S-P.

Estenose aórtica severa em recém-nascido / Severe aortic stenosis in newborn infants

A estenose aórtica severa apresenta mortalidade elevada no período neonatal. Os autores apresentam o caso de um recém-nascido com estenose aórtica severa diagnosticada intra-útero, que, ao nascimento, se apresentava hemodinamicamente como uma síndrome hipoplásica do ventrículo esquerdo. Os autores tamém fazem a revisäo dos aspectos fisiopatológicos e tratamento

Ecodopplercardiografia nas principais cardiopatias congenitas acianogenicas e cianogenicas / Doppler-echocardiography in the main acyanotic and cyanotic congenital heart diseases

A ecodopplercardiografia ocupa lugar de destaque, como metodo nao-invasivo no diagnostico das cardiopatias. Neste trabalho, procuramos rever os principais aspectos ecocardiograficos das cardiopatias congenitas acianogenicas e cianogenicas mais frequentes. Foi dada a enfase as caracteristicas anatomicas, aos plano ecocardiograficos utilizados nos diversos diagnosticos e as principais limitacoes do metado