Valve-Sparing Tetralogy of Fallot Repair With Intraoperative Dilation of the Pulmonary Valve. Mid-Term Results.

Deleterious long-term effects of chronic pulmonary regurgitation after repair of tetralogy of Fallot have become evident during the last decades. Subsequently, some groups have developed strategies to spare the pulmonary valve function at the time of repair with good early results. However, mid-term outcomes are scarce in the literature and in some cases controversial. The aim of our study is to report our results mid-term with valve-sparing repair of tetralogy of Fallot. We retrospectively reviewed patients undergoing tetralogy of Fallot repair and having preservation of the pulmonary valve with intraoperative dilation at our institution. From June 2009 through June 2017, 42 patients underwent valve-sparing tetralogy of Fallot repair. Median age and weight at surgery were 5.2 months and 7.2 kg. Median preoperative pulmonary valve diameters and Z scores by echocardiography were 6.4 mm (range 4.5-11 mm) and -2.3 (range -1.3 to -4.5). No patient died in our series. For a median follow-up of 45 months, the pulmonary valve has grown by Z score (P < 0.0001) as well as the pulmonary trunk (P= 0.00216). Significant pulmonary regurgitation has developed in 9 patients (21.4%). No patient has required reintervention/reoperation for recurrent right ventricular outflow tract obstruction. Patients with tetralogy of Fallot who had valve-sparing repair with intraoperative dilation of the pulmonary valve show good early and mid-term results with respect to right ventricular outflow tract obstruction. The pulmonary valve annulus and the pulmonary trunk grow through follow-up. Progressive development of significant pulmonary regurgitation is seen in more than 20% of patients. Long-term data with this approach and comparison with a population of patients undergoing a transannular patch repair are required to establish the real utility of this approach.

La importancia del estudio familiar y genético: la mutación p. L3778F en el receptor de la rianodina probablemente no cause un fenotipo tan grave / The importance of family-genetic screening: the phenotype caused by the p. l3778f ryanodine receptor mutation is likely less severe than previously thought

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Lower mini-sternotomy in congenital heart disease: just a cosmetic improvement?

OBJECTIVES: Lower mini-sternotomy represents a minimally invasive surgical technique that has been utilized for the repair of a wide variety of congenital heart defects with excellent surgical and cosmetic outcomes. However, clinical improvements provided for this technique beyond cosmetic results are controversial. The aim of our study is to report our results with lower mini-sternotomy for the repair of congenital heart malformations and compare them with a matched group with a full median sternotomy approach. METHODS: From 2010 through 2013, 105 consecutive congenital patients (81 paediatric) underwent lower mini-sternotomy at our centre (Group 1). We analysed in-hospital and follow-up outcomes, and compare them with an age-sex-diagnosis-type of surgery-matched group (Group 2). Both groups were managed following the same clinical protocols. RESULTS: In Group 1, age at the time of surgery was 12 ± 17 years (range from 0.2 to 64.6 years). In this group, 81 patients were paediatric and 62 were female. Operative techniques were atrial septal defect (n = 72), ventricular septal defect (n = 24) and atrioventricular canal repairs (n = 9). There were no deaths or major in-hospital complications. Two adult patients required conversion to full median sternotomy. For a medium follow-up of 1.5 years (range from 1 month to 5 years), there were no deaths, reinterventions or reoperations and no significant residual defects were found. Compared with Group 2, patients in Group 1 had longer cardiopulmonary bypass times (58.71 ± 19.08 vs 45.39 ± 20.45, P < 0.001) and cross-clamp times (32.75 ± 13.11 vs 23.22 ± 13.93, P < 0.001), higher rate of early extubation (96 vs 85%, P = 0.018) and lower rate of postoperative complications (11.6 vs 22.3%, P = 0.034). CONCLUSIONS: At our centre, lower mini-sternotomy represents a safe alternative for the repair of congenital heart defects in paediatric and adult populations. Cardiopulmonary bypass and cross-clamp times were longer in the mini-sternotomy group. However, these patients showed earlier extubation and less postoperative complications when compared with patients with a full sternotomy approach. Combined with improved cosmetic outcomes, lower mini-sternotomy could represent the technique of choice for these populations.

Vitamin D deficiency at pediatric intensive care admission / Deficiência de vitamina D em internações na unidade de terapia intensiva pediátrica

OBJECTIVE: to assess whether 25hydroxivitaminD or 25(OH)vitD deficiency has a high prevalence at pediatric intensive care unit (PICU) admission, and whether it is associated with increased prediction of mortality risk scores. METHOD: prospective observational study comparing 25(OH)vitD levels measured in 156 patients during the 12 hours after critical care admission with the 25(OH)vitD levels of 289 healthy children. 25(OH)vitD levels were also compared between PICU patients with pediatric risk of mortality III (PRISM III) or pediatric index of mortality 2 (PIM 2) > p75 [(group A; n = 33) vs. the others (group B; n = 123)]. Vitamin D deficiency was defined as < 20 ng/mL levels. RESULTS: median (p25-p75) 25(OH)vitD level was 26.0 ng/mL (19.2-35.8) in PICU patients vs. 30.5 ng/mL (23.2-38.6) in healthy children (p = 0.007). The prevalence of 25(OH)vitD < 20 ng/mL was 29.5% (95% CI: 22.0-37.0) vs. 15.6% (95% CI: 12.2-20.0) (p = 0.01). Pediatric intensive care patients presented an odds ratio (OR) for hypovitaminosis D of 2.26 (CI 95%: 1.41-3.61). 25(OH)vitD levels were 25.4 ng/mL (CI 95%: 15.5-36.0) in group A vs. 26.6 ng/mL (CI 95%: 19.3-35.5) in group B (p = 0.800). CONCLUSIONS: hypovitaminosis D incidence was high in PICU patients. Hypovitaminosis D was not associated with higher prediction of risk mortality scores. .

OBJETIVO: avaliar se a deficiência da 25-hidroxivitamina D, ou 25 (OH) vitD, tem prevalência elevada em internações na unidade de terapia intensiva pediátrica, e se estaria relacionada à previsão de escores de risco de mortalidade. MÉTODO: estudo observacional prospectivo comparando níveis de 25 (OH) vitD de 156 pacientes, mensurados nas primeiras 12 horas da internação em terapia intensiva, com níveis de 25 (OH) vitD de 289 crianças saudáveis. Os níveis de 25 (OH) vitD também foram comparados entre pacientes na UTIP com escore PRISM III ou PIM 2 > p75 (Grupo A; n = 33), e o restante, (Grupo B; n = 123). A deficiência de vitamina D foi definida como níveis < 20 ng/mL. RESULTADOS: o nível médio (p25-p75) de 25 (OH) vitD foi 26,0 ng/mL (19,2-35,8) em pacientes internados na UTIP, em comparação a 30,5 ng/mL (23,2-38,6) em crianças saudáveis (p = 0,007). A prevalência de 25 (OH) vitD < 20 ng/mL foi de 29,5% (IC 95%, 22,0-37,0), em comparação a 15,6% (IC 95%,12,2-20,0) (p = 0,01). Os pacientes em terapia intensiva pediátrica apresentaram uma razão de chance (RC) para hipovitaminose D de 2,26 (IC 95%, 1,41-3,61). Os níveis de 25 (OH) vitD foram 25,4 ng/mL (IC 95%, 15,5-36,0) no grupo A, em comparação a 26,6 ng/mL (IC 95%, 19,3-35,5) no grupo B (p = 0,800). CONCLUSÕES: a incidência de hipovitaminose D foi elevada em pacientes em terapia intensiva pediátrica, mas não foi associada à maior previsão de escores de risco de mortalidade. .

Vitamin D deficiency at pediatric intensive care admission.

OBJECTIVE: to assess whether 25hydroxivitaminD or 25(OH)vitD deficiency has a high prevalence at pediatric intensive care unit (PICU) admission, and whether it is associated with increased prediction of mortality risk scores. METHOD: prospective observational study comparing 25(OH)vitD levels measured in 156 patients during the 12 hours after critical care admission with the 25(OH)vitD levels of 289 healthy children. 25(OH)vitD levels were also compared between PICU patients with pediatric risk of mortality III (PRISM III) or pediatric index of mortality 2 (PIM 2) > p75 [(group A; n = 33) vs. the others (group B; n = 123)]. Vitamin D deficiency was defined as < 20 ng/mL levels. RESULTS: median (p25-p75) 25(OH)vitD level was 26.0 ng/mL (19.2-35.8) in PICU patients vs. 30.5 ng/mL (23.2-38.6) in healthy children (p = 0.007). The prevalence of 25(OH)vitD < 20 ng/mL was 29.5% (95% CI: 22.0-37.0) vs. 15.6% (95% CI: 12.2-20.0) (p = 0.01). Pediatric intensive care patients presented an odds ratio (OR) for hypovitaminosis D of 2.26 (CI 95%: 1.41-3.61). 25(OH)vitD levels were 25.4 ng/mL (CI 95%: 15.5-36.0) in group A vs. 26.6 ng/mL (CI 95%: 19.3-35.5) in group B (p = 0.800). CONCLUSIONS: hypovitaminosis D incidence was high in PICU patients. Hypovitaminosis D was not associated with higher prediction of risk mortality scores.

Pro-adrenomedullin, pro-endothelin-1, procalcitonin, C-reactive protein and mortality risk in critically ill children: a prospective study.

INTRODUCTION: We tested the hypothesis that higher mid-regional pro-adrenomedullin (MR-proADM), carboxy-terminal pro-endothelin-1 (CT-proET-1), procalcitonin (PCT) and C-reactive protein (CRP) plasma concentrations would be associated with increased prediction of mortality risk scores. METHODS: Prospective observational study set in two pediatric intensive care units (PICUs). Two-hundred-thirty-eight patients were included. MR-proADM, CT-proET-1, PCT and CRP levels were compared between children with PRISM III and PIM 2 > p75 (Group A; n = 33) and the rest (Group B; n = 205). RESULTS: Median (range) MR-proADM levels were 1.39 nmol/L (0.52-12.67) in group A versus 0.54 (0.15-3.85) in group B (P < 0.001). CT-proET-1 levels were 172 pmol/L (27-500) versus 58 (4-447) (P < 0.001). PCT levels were 7.77 ng/mL (0.34-552.00) versus 0.28 (0.02-107.00) (P < 0.001). CRP levels were 6.23 mg/dL (0.08-28.25) versus 1.30 mg/dL (0.00-42.09) (P = 0.210). The area under the ROC curve (AUC) for the differentiation of group A and B was 0.87 (95% CI:0.81-0.821) for MR-proADM, 0.86 (95% CI:0.79-0.92) for CT-proET-1 and 0.84 (95% CI:0.74-0.94) for PCT. A MR-proADM > 0.79 nmol/L had 93% sensitivity and 76% specificity to differentiate groups, whereas a CT-proET-1 > 123 pmol/L had 77% sensitivity and 84% specificity, and a PCT concentration > 2.05 ng/mL had 80% sensitivity and specificity. CONCLUSIONS: In critically ill children, high levels of MR-proADM, CT-proET-1 and PCT were associated with increased prediction of mortality risk scores. MR-proADM, CT-proET-1 and PCT concentrations higher than 0.80 nmol/L, 123 pmol/L and 2 ng/mL, respectively, could be used by clinicians to identify critically ill children at higher prediction of risk death scores.

La expresión del miedo. Relato biografico de una joven anoréxica / The expression of fear. A biographic tale of an anorexic young lady

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