Resultados Precoces do Procedimento de Norwood em um Centro de Referência no Brasil / Early Outcomes of the Norwood Procedure in a Reference Center in Brazil

Resumo Fundamento Apenas dois artigos abordam os resultados precoces de pacientes com síndrome do coração esquerdo hipoplásico (SHCE) submetidos à operação de Norwood, no Brasil. Objetivos Avaliamos pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood para identificar os fatores preditivos de mortalidade precoce (nos primeiros 30 dias após a cirurgia) e intermediária (desde a sobrevida precoce até o procedimento de Glenn). Métodos Foram incluídos pacientes com SHCE submetidos em nosso serviço ao primeiro estágio da operação de Norwood de janeiro de 2016 a abril de 2019. Dados demográficos, anatômicos e cirúrgicos foram analisados. Os desfechos foram mortalidade precoce (nos primeiros 30 dias após a cirurgia), mortalidade intermediária (desde a sobrevida precoce até o procedimento de Glenn) e a necessidade de suporte pós-operatório com ECMO. Foram realizadas análises univariadas e multivariadas e calculados odds ratios, com intervalos de confiança de 95%. Um valor de p < 0,05 foi considerado estatisticamente significativo. Resultados Um total de 80 pacientes com SHCE foram submetidos ao primeiro estágio da operação de Norwood. A taxa de sobrevida em 30 dias foi de 91,3% e a taxa de sobrevida intermediária foi de 81,3%. Quatorze pacientes (17,5%) necessitaram de suporte com ECMO. Menor peso (p=0,033), estenose aórtica (vs atresia aórtica; p=0,036) e necessidade de suporte pós-operatório com ECMO (p=0,009) foram fatores preditivos independentes para mortalidade em 30 dias. A estenose da valva mitral ( vs atresia da valva mitral; p=0,041) foi um fator preditivo independente para mortalidade intermediária. Conclusão O presente estudo inclui a maior coorte brasileira de pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood na era recente. Nossas taxas de sobrevida foram comparáveis às mais altas taxas de sobrevida relatadas globalmente. Baixo peso corporal, estenose valvar aórtica e necessidade de suporte pós-operatório com ECMO foram preditores independentes para mortalidade em 30 dias. A estenose da valva mitral foi o único fator preditivo independente para mortalidade intermediária.

Abstract Background Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil. Objectives We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality. Methods Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled. Demographic, anatomical, and surgical data were analyzed. Endpoints were early mortality (within the first 30 days after surgery), intermediate mortality (from early survival up to the Glenn procedure) and the need for postoperative ECMO support. Univariate and multivariate analyses were performed, and odds ratios, with 95% confidence intervals, were calculated. A p-value <0.05 was considered statistically significant. Results A total of 80 patients with HLHS underwent the stage I Norwood procedure. The 30-day survival rate was 91.3% and the intermediate survival rate 81.3%. Fourteen patients (17.5%) required ECMO support. Lower weight (p=0.033), aortic stenosis (vs aortic atresia; p=0.036), and the need for postoperative ECMO support (p=0.009) were independent predictive factors for 30-day mortality. Mitral valve stenosis (vs mitral valve atresia; p=0.041) was an independent predictive factor for intermediate mortality. Conclusion The present study includes the largest Brazilian cohort of patients with HLHS undergoing the stage I Norwood procedure in the recent era. Our survival rates were comparable to the highest survival rates reported globally. Low body weight, aortic valve stenosis, and the need for postoperative ECMO support were independent predictors for 30-day mortality. Mitral valve stenosis was the only independent predictive factor for intermediate mortality.

Early Outcomes of the Norwood Procedure in a Reference Center in Brazil. / Resultados Precoces do Procedimento de Norwood em um Centro de Referência no Brasil.

BACKGROUND: Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil. OBJECTIVES: We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality. METHODS: Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled. Demographic, anatomical, and surgical data were analyzed. Endpoints were early mortality (within the first 30 days after surgery), intermediate mortality (from early survival up to the Glenn procedure) and the need for postoperative ECMO support. Univariate and multivariate analyses were performed, and odds ratios, with 95% confidence intervals, were calculated. A p-value <0.05 was considered statistically significant. RESULTS: A total of 80 patients with HLHS underwent the stage I Norwood procedure. The 30-day survival rate was 91.3% and the intermediate survival rate 81.3%. Fourteen patients (17.5%) required ECMO support. Lower weight (p=0.033), aortic stenosis (vs aortic atresia; p=0.036), and the need for postoperative ECMO support (p=0.009) were independent predictive factors for 30-day mortality. Mitral valve stenosis (vs mitral valve atresia; p=0.041) was an independent predictive factor for intermediate mortality. CONCLUSION: The present study includes the largest Brazilian cohort of patients with HLHS undergoing the stage I Norwood procedure in the recent era. Our survival rates were comparable to the highest survival rates reported globally. Low body weight, aortic valve stenosis, and the need for postoperative ECMO support were independent predictors for 30-day mortality. Mitral valve stenosis was the only independent predictive factor for intermediate mortality.

FUNDAMENTO: Apenas dois artigos abordam os resultados precoces de pacientes com síndrome do coração esquerdo hipoplásico (SHCE) submetidos à operação de Norwood, no Brasil. OBJETIVOS: Avaliamos pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood para identificar os fatores preditivos de mortalidade precoce (nos primeiros 30 dias após a cirurgia) e intermediária (desde a sobrevida precoce até o procedimento de Glenn). MÉTODOS: Foram incluídos pacientes com SHCE submetidos em nosso serviço ao primeiro estágio da operação de Norwood de janeiro de 2016 a abril de 2019. Dados demográficos, anatômicos e cirúrgicos foram analisados. Os desfechos foram mortalidade precoce (nos primeiros 30 dias após a cirurgia), mortalidade intermediária (desde a sobrevida precoce até o procedimento de Glenn) e a necessidade de suporte pós-operatório com ECMO. Foram realizadas análises univariadas e multivariadas e calculados odds ratios, com intervalos de confiança de 95%. Um valor de p < 0,05 foi considerado estatisticamente significativo. RESULTADOS: Um total de 80 pacientes com SHCE foram submetidos ao primeiro estágio da operação de Norwood. A taxa de sobrevida em 30 dias foi de 91,3% e a taxa de sobrevida intermediária foi de 81,3%. Quatorze pacientes (17,5%) necessitaram de suporte com ECMO. Menor peso (p=0,033), estenose aórtica (vs atresia aórtica; p=0,036) e necessidade de suporte pós-operatório com ECMO (p=0,009) foram fatores preditivos independentes para mortalidade em 30 dias. A estenose da valva mitral ( vs atresia da valva mitral; p=0,041) foi um fator preditivo independente para mortalidade intermediária. CONCLUSÃO: O presente estudo inclui a maior coorte brasileira de pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood na era recente. Nossas taxas de sobrevida foram comparáveis às mais altas taxas de sobrevida relatadas globalmente. Baixo peso corporal, estenose valvar aórtica e necessidade de suporte pós-operatório com ECMO foram preditores independentes para mortalidade em 30 dias. A estenose da valva mitral foi o único fator preditivo independente para mortalidade intermediária.

Extracorporeal Membrane Oxygenation After Norwood Surgery in Patients With Hypoplastic Left Heart Syndrome: A Retrospective Single-Center Cohort Study From Brazil.

Background: Extracorporeal membrane oxygenation (ECMO) is increasingly being used to support patients after the repair of congenital heart disease. Objective: We report our experience with patients with a single functional ventricle who were supported by ECMO after the Norwood procedure, reviewing the outcomes and identifying risk factors for mortality in these patients. Methods: In this single-center retrospective cohort study, we enrolled 33 patients with hypoplastic left heart syndrome (HLHS) who received ECMO support after the Norwood procedure between January 2015 and December 2019. The independent variables evaluated in this study were demographic, anatomical, and those directly related to ECMO support (ECMO indication, local of initiation, time under support, and urinary output while on ECMO). The dependent variable was survival. A p < 0.05 was considered statistically significant. Results: The ECMO support was applied in 33 patients in a group of 120 patients submitted to Norwood procedure (28%). Aortic atresia was present in 72.7% of patients and mitral atresia in 51.5%. For 15% of patients, ECMO was initiated in the operating room; for all other patients, ECMO was initiated in the intensive care unit. The indications for ECMO in the cardiac intensive care unit were cardiac arrest in 22 (79%) of patients, low cardiac output state in 10 (18%), and arrhythmia in 1 patient (3%). The median time under support was 5 (2-25) days. The median follow-up time was 59 (4-150) days. Global survival to Norwood procedure was 90.9% during the 30-day follow-up, being 33.3% for those submitted to ECMO. Longer ECMO support (p = 0.004) was associated with a higher risk of death in the group submitted to ECMO. Conclusions: The mortality of patients with HLHS who received ECMO support after stage 1 palliation was high. Patients with low urine output were related to worse survival rates, and longer periods under ECMO support (more than 9 days of ECMO) were associated with 100% mortality. Earlier ECMO initiation before multiorgan damage may improve results.

Modified Hybrid Procedure in Hypoplastic Left Heart Syndrome: Initial Experience of a Center in Northeastern Brazil.

INTRODUCTION:: Although it only corresponds to 2.5% of congenital heart defects, hypoplastic left heart syndrome (HLHS) is responsible for more than 25% of cardiac deaths in the first week of life. Palliative surgery performed after the second week of life is considered an important risk factor in the treatment of HLHS. OBJECTIVE:: The aim of this study is to describe the initial experience of a medical center in Northeastern Brazil with a modified off-pump hybrid approach for palliation of HLHS. METHODS:: From November 2012 through November 2015, the medical records of 8 patients with HLHS undergoing hybrid procedure were retrospectively evaluated in a tertiary private hospital in Northeastern Brazil. The modified off-pump hybrid palliation consisted of stenting of the ductus arteriosus guided by fluoroscopy without contrast and banding of the main pulmonary artery branches. Demographic and clinical variables were recorded for descriptive analysis. RESULTS:: Eight patients were included in this study, of whom 37.5% were female. The median age and weight at the time of the procedure was 2 days (p25% and p75% = 2 and 4.5 days, respectively) and 3150 g (p25% and p75% = 3077.5 g and 3400 g, respectively), respectively. The median length in intensive care unit stay was 6 days (p25% and p75% = 3.5% and 8 days, respectively). There were no in-hospital deaths. Four patients have undergone to the second stage of the surgical treatment of HLHS. CONCLUSION:: In this series, the initial experience with the modified off-pump hybrid procedure showed to be safe, allowing a low early mortality rate among children presenting HLHS.

Modified hybrid procedure in hypoplastic left heart syndrome: initial experience of a center in northeastern Brazil

Abstract Introduction: Although it only corresponds to 2.5% of congenital heart defects, hypoplastic left heart syndrome (HLHS) is responsible for more than 25% of cardiac deaths in the first week of life. Palliative surgery performed after the second week of life is considered an important risk factor in the treatment of HLHS. Objective: The aim of this study is to describe the initial experience of a medical center in Northeastern Brazil with a modified off-pump hybrid approach for palliation of HLHS. Methods: From November 2012 through November 2015, the medical records of 8 patients with HLHS undergoing hybrid procedure were retrospectively evaluated in a tertiary private hospital in Northeastern Brazil. The modified off-pump hybrid palliation consisted of stenting of the ductus arteriosus guided by fluoroscopy without contrast and banding of the main pulmonary artery branches. Demographic and clinical variables were recorded for descriptive analysis. Results: Eight patients were included in this study, of whom 37.5% were female. The median age and weight at the time of the procedure was 2 days (p25% and p75% = 2 and 4.5 days, respectively) and 3150 g (p25% and p75% = 3077.5 g and 3400 g, respectively), respectively. The median length in intensive care unit stay was 6 days (p25% and p75% = 3.5% and 8 days, respectively). There were no in-hospital deaths. Four patients have undergone to the second stage of the surgical treatment of HLHS. Conclusion: In this series, the initial experience with the modified off-pump hybrid procedure showed to be safe, allowing a low early mortality rate among children presenting HLHS.

Diagnóstico pré-natal de atresia tricúspide tipo IIC com atresia aórtica / Prenatal diagnosis of tricuspid atresia IIC associated with aortic atresia

Introdução: A atresia tricúspide é uma cardiopatia rara, sendo sua associação com discordância atrioventricular e atresia aórtica ainda mais rara. Objetivo: Relatar caso de associação cardíaca rara, reiterando o papel do ecocardiograma fetal no diagnóstico precoce das malformações cardíacas complexas. Relato: Recém-nascido com diagnóstico pré-natal de ausência de conexão direita, ventrículo direito hipoplásico, via de saída única pulmonar emergindo do ventrículo esquerdo e arco aórtico hipoplásico foi submetido à cirurgia de Norwood-Sano, com 48 horas de vida, com boa evolução. Conclusão: Os achados ecocardiográficos no feto predisseram a necessidade de intervenção neonatal precoce e asseguraram o manejo pós-natal adequado.

Reproducibility of ambulatory blood pressure monitoring in hypertensive patients with type 2 diabetes mellitus.

OBJECTIVE: To evaluate the reproducibility of ambulatory blood pressure monitoring (ABPM) (SpaceLabs-90207) and placebo effect on ABPM. METHODS: Blood pressure was measured in the office and over two ABPM periods with an interval from one to ten months (mean 4.9 months), in 26 patients with type 2 diabetes mellitus and hypertension. Eleven patients (G1) had two ABPMs without taking antihypertensive drugs for 15 days, whereas G2 (N=15) had the second ABPM after administration of a placebo for 15 days. RESULTS: In the evaluation of the coefficient of variation (CV) of diurnal (awake) systolic BP (DSBP), of diurnal (awake) diastolic BP (DDBP), of 24-hour systolic BP (24hSBP) and of 24-hour diastolic BP (24hDBP), the values found were 4.6%, 3.9%, 5.0%, 4.0% for G1 and 4.3%, 5.1%, 3.7%, 5.1% for G2 respectively. We also determined the CV of nocturnal (sleep) systolic and diastolic BP (NSBP and NDBP) for G1 (7.7%; 8.2%) and G2 (5.6%; 6.3%). Heart rate CV during alertness and sleep were: G1=5.9% and 9.0%; G2=6.9% and 5.8% respectively. When the total number of 'patients was analyzed, all variables showed a strong correlation between the first and second ABPM measurements (DSBP, r = 0.76; P < 0.001; DDBP, r = 0.65; p < 0.001; 24hSBP, r = 0.77; p < 0.001; 24hDBP, r = 0.70; p < 0.001; NSBP, r = 0.62; p < 0.001; NDBP, r = 0.52; p < 0.01). Office systolic and diastolic BP and 24hSBP and 24hDBP also showed correlation (r = 0.65; p < 0.001; r = 0.57; p < 0.01). CONCLUSION: Mean of pressure levels measured by ABPM presented good reproducibility and were not affected by placebo.

Reprodutibilidade da medida ambulatorial da pressão arterial em pacientes hipertensos com diabete melito tipo 2 / Reproducibility of ambulatory blood pressure monitoring in hypertensive patients with type 2 diabetes mellitus

OBJETIVO: Avaliar a reprodutibilidade e o efeito placebo sobre a monitorização ambulatorial da pressão arterial (MAPA) (SpaceLabs-90207). MÉTODOS: Mensurou-se a PA no consultório e por meio de duas MAPA, realizadas em um intervalo de 1 a 10 meses (média de 4,9 meses), de 26 pacientes com diabetes tipo 2 e hipertensão. Onze pacientes (G1) realizaram as duas MAPA sem medicação anti-hipertensiva por 15 dias, enquanto o G2 (N = 15) fez a segunda MAPA em uso de placebo pelo mesmo período. RESULTADOS: Ao avaliarmos os coeficientes de variação (CV) da PA sistólica na vigília (PASV), PA diastólica na vigília (PADV), PA sistólica nas 24h (PAS24h) e PA diastólica nas 24h (PAD24h), encontramos valores de 4,6 por cento, 3,9 por cento, 5,0 por cento e 4,0 por cento no G1 e 4,3 por cento, 5,1 por cento, 3,7 por cento e 5,1 por cento no G2, respectivamente. Quanto ao CV da PA sistólica e diastólica durante o sono (PASS e PADS), encontramos 7,7 por cento e 8,2 por cento para G1, e 5,6 por cento e 6,3 por cento para G2, respectivamente. O CV da freqüência cardíaca na vigília e durante o sono foram: G1 = 5,9 por cento e 9,0 por cento, G2 = 6,9 por cento e 5,8 por cento, respectivamente. Analisando o total dos pacientes, todas as variáveis mostraram fortes correlações entre a primeira e a segunda MAPA (PASV, r=0,76; P<0,001; PADV, r=0,65; p<0,001; PAS24h, r=0,77; p<0,001; PAD24h, r=0,70; p<0,001; PASS, r=0,62; p<0,001; PADS, r=0,52; p<0,01). Ocorreram também correlações entre a PA sistólica e a diastólica de consultório e a PAS24h e PAD24h (r=0,65; p<0,001; r=0,57; p<0,01). CONCLUSÃO: A média dos níveis pressóricos avaliados pela MAPA apresentou boa reprodutibilidade e esses não foram afetados pelo efeito placebo.

OBJECTIVE: To evaluate the reproducibility of ambulatory blood pressure monitoring (ABPM) (SpaceLabs-90207) and placebo effect on ABPM. METHODS: Blood pressure was measured in the office and over two ABPM periods with an interval from one to ten months (mean 4.9 months), in 26 patients with type 2 diabetes mellitus and hypertension. Eleven patients (G1) had two ABPMs without taking antihypertensive drugs for 15 days, whereas G2 (N=15) had the second ABPM after administration of a placebo for 15 days. RESULTS: In the evaluation of the coefficient of variation (CV) of diurnal (awake) systolic BP (DSBP), of diurnal (awake) diastolic BP (DDBP), of 24-hour systolic BP (24hSBP) and of 24-hour diastolic BP (24hDBP), the values found were 4.6 percent, 3.9 percent, 5.0 percent, 4.0 percent for G1 and 4.3 percent, 5.1 percent, 3.7 percent, 5.1 percent for G2 respectively. We also determined the CV of nocturnal (sleep) systolic and diastolic BP (NSBP and NDBP) for G1 (7.7 percent; 8.2 percent) and G2 (5.6 percent; 6.3 percent). Heart rate CV during alertness and sleep were: G1=5.9 percent and 9.0 percent; G2=6.9 percent and 5.8 percent respectively. When the total number of 'patients was analyzed, all variables showed a strong correlation between the first and second ABPM measurements (DSBP, r = 0.76; P < 0.001; DDBP, r = 0.65; p < 0.001; 24hSBP, r = 0.77; p < 0.001; 24hDBP, r = 0.70; p < 0.001; NSBP, r = 0.62; p < 0.001; NDBP, r = 0.52; p < 0.01). Office systolic and diastolic BP and 24hSBP and 24hDBP also showed correlation (r = 0.65; p < 0.001; r = 0.57; p < 0.01). CONCLUSION: Mean of pressure levels measured by ABPM presented good reproducibility and were not affected by placebo.

Impaired reduction of nocturnal systolic blood pressure and severity of diabetic retinopathy.

The aim of the present study was to evaluate the influence of elevated levels of nocturnal blood pressure (BP) on diabetic retinopathy (DR). A total of 88 diabetic hypertensive patients were divided according to the stage of DR. They underwent 24 h ambulatory BP monitoring and ophthalmological evaluation, and their average level of fasting blood glucose as well as their glycemic control index (percentage of fasting blood glucose higher than 11.2 mmol/L over the previous four years) were calculated. When diabetic patients with retinopathy (n=29) (group 1) were compared with patients without retinopathy (n=59) (group 2), a significant difference was observed in diabetes duration (124 months [range six to 460 months] versus 43 months [range six to 365 months], respectively; P<0.05). In addition, group 1 showed higher levels of nocturnal systolic BP (NSBP) (141 +/- 22 mmHg versus 132+/-18 mmHg; P<0.05). However, no significant differences were found between the two groups (group 1 and group 2) when diurnal pressoric levels were compared (diurnal systolic BP, 153+/-19 mmHg versus 146+/-19 mmHg, P not significant; and diurnal diastolic BP, 91+/-9 mmHg versus 91+/-13 mmHg, P not significant). DR correlated with diabetes duration (r=0.26; P<0.05) and with glycemic control index (r=0.24; P<0.01). Multivariate regression analysis showed NSBP to be an independent predictor of DR (r(2)=0.12; P<0.01). Moreover, patients with severe stages of DR (preproliferative, proliferative or macular edema) showed a lower decrease of NSBP than the other patients (3.9+/-6.0 mmHg versus 9.2+/-6.0 mmHg; P<0.05). The present study suggests that the absence of 24 h normal pressoric rhythm can interfere with the prevalence and severity of DR.