Levels of N-terminal-pro-brain natriuretic peptide in congenital heart disease surgery and its value as a predictive biomarker.

OBJECTIVES: To explore the value of N-terminal-pro-brain natriuretic peptide (NT-ProBNP) as a predictive biomarker of postoperative cardiovascular surgery. METHODS: A prospective study of 68 patients (0-15 years), submitted to open-heart surgery was conducted. NT-ProBNP and other biochemical and clinical markers were measured preoperatively and during the first 48 postoperative hours. RESULTS: NT-ProBNP preoperative reduced one hour after surgery, increased significantly later, and remained without change between 12 hours and 48 hours postoperatively. Peak values (24 hours) were correlated with preoperative levels (R=0.73; P<0.001), risk adjustment congenital heart surgery-1 (R=0.37; P<0.002), length of cardiopulmonary bypass (CPB) (R=0.57; P<0.001); age (R=-0.55; P<0.001) and weight of patients (R=-0.46; P<0.001). Independent predictors of NT-ProBNP-peak were preoperative value (ß=0.42) and CPB length (ß=0.24; R(2) of model 0.63; P<0.001). The peak values were correlated to a maximum inotropic score (R=0.46; P<0.001), duration of inotropic therapy (R=0.44; P<0.001), duration of mechanical ventilation support (R=0.39; P<0.001) and length of stay in the Pediatric Intensive Care Unit (R=0.45; P<0.001). Independent predictors of enhanced intensive care unit stay, controlling by risk adjustment score, were high preoperative NT-ProBNP (OR 5.5, 95% CI 1.2-25.5), and high postoperative troponine (OR 10.5, 95% CI 2.2-49.2). CONCLUSIONS: NT-ProBNP concentration is dependent on time during the perioperative period, it peaks at 24 hours and depends on the preoperative value and CPB length. A high peptide level before surgery is an independent predictor of prolonged stay in intensive pediatric care.

Craniofacial dyssynostosis: description of the first four Spanish cases and review.

Craniofacial dyssynostosis (CD) is characterized by premature fusion of the lambdoid and posterior part of the sagittal sutures, and short stature. Thus, the skull shape becomes dolichocephalic with protuberant forehead and either bulging or flat occiput. Facial changes are secondary to the skull defects, and some additional findings have also been described. We report on the first four known Spanish patients. They were unrelated and had Spanish ancestors. In the three previous reports about this syndrome, the authors hypothesized that the frequency of the gene causing CD must be rather high in the Spanish population, and relatively common in areas with Spanish ancestry. We have estimated the minimal birth prevalence of the syndrome in 0.51 per million livebirths. It has been previously suggested that the syndrome is inherited as an autosomal recessive trait, since there were two affected sisters among the nine published cases. Phenotypic variability is discussed in detail in this paper. We also underline several aspects for the anticipatory guidance of affected individuals, especially recommending a neurologic evaluation taking into account the radiologic findings in order to plan early interventions to avoid undesirable consequences of craniosynostosis. It is also recommended to perform additional studies (ophthalmologic, cardiologic, among others) to rule out the existence of associated anomalies, which are more frequent than previously considered.