Profile and consequences of children requiring prolonged mechanical ventilation in three Brazilian pediatric intensive care units.

OBJECTIVE: To describe the characteristics of children submitted to prolonged mechanical ventilation (MV), and evaluate their mortality, and associated factors as well as the potential impact at admissions to the pediatric intensive care unit (PICU). METHODS: We conducted a retrospective study enrolling all children admitted to three Brazilian PICUs between January 2003 and December 2005 submitted to MV > or =21 days. The three selected PICUs were located in university-affiliated hospitals. From the medical charts were reported anthropometric data, diagnosis, ventilator parameters on the 21st day, length of MV, length of stay in the PICU, specific interventions (e.g., tracheostomy), and outcome. RESULTS: One hundred eighty-four children (190 admissions) were submitted to prolonged MV (2.5% of all admissions to these 3 Brazilian PICUs), with a median age of 6 months. The mortality rate was 48% and the median time on MV was 32 days. Tracheostomy was performed on only 19% of the patients and, on average after 32 days of intubation. Mortality was associated with peak inspiratory pressure >25 cm H2O (odds ratio = 2.3; 1.1-5.1), fraction of inspired oxygen >0.5 (odds ratio = 6.3; 2.2-18.1), and vasoactive drug infusion (odds ratio = 2.6; 1.1-5.9) on the 21st day of MV. Seventy-six children (1% of the all admissions) were dependent on MV without other organ failures were 830 PICU admissions and were potentially prevented. CONCLUSIONS: A small group of children admitted to the PICU requires prolonged MV. The elevated mortality rate is associated with higher ventilatory parameters and vasoactive drug support on the 21st day of MV. Stable children requiring prolonged MV in the PICU potentially prevent additional admissions of a large number of acute and unstable patients.

22q11.2 deletion syndrome in patients admitted to a cardiac pediatric intensive care unit in Brazil.

The 22q11.2 deletion syndrome (22q11DS) is one of the most recognizable causes of congenital heart defects (CHDs), but the frequency varies in non-selected populations. The purpose of this study was to determine the incidence and clinical features of patients with CHD and 22q11DS admitted to a pediatric cardiology intensive care unit in Brazil. In a prospective study, we evaluated a consecutive series of 207 patients with a CHD following a clinical protocol and cytogenetic analysis by high resolution karyotype and fluorescent in situ hybridization (FISH). 22q11DS was identified in four patients (2%), a frequency similar to studies that evaluated subjects with major CHDs in other countries. Despite this similarity, we believe that the low rate of prenatal identification of CHDs and the limited access of these patients to appropriate diagnosis and care, which occur in our region, could have had an influence on this frequency. It is possible that 22q11DS patients with a severe CHD could have died before having a chance to access a tertiary hospital, leading to an underestimate of its frequency.

Hybrid management for hypoplastic left heart syndrome : an experience from Brazil.

Initial surgical reconstruction for hypoplastic left heart syndrome (HLHS) is associated with satisfactory outcomes only in a few referral centers. Moreover, there is a persistent high-risk period for sudden death while the patient waits for the next surgical procedure. The development of a less invasive approach, so-called "hybrid," postponing a major surgery outside the neonatal period, might reduce the immediate and late surgical burden on these patients. This is a retrospective study of a contemporary series of patients with HLHS seen in two separate institutions. Patients with HLHS or its variants who underwent a "hybrid" management were included in the study. Data are described as the mean and standard deviation or absolute numbers and percentage, as appropriate. From January 2004 to June 2006, 15 patients (10 male; 5 +/- 3.8 days old and 2.9 +/- 0.5 kg) were included in the study. Ten had both mitral and aortic atresia; the ascending aorta and atrial septal defect measured 2.5 +/- 1.4 and 4.9 +/- 1.2 mm, respectively. There were six hospital survivors after stage I (mortality rate 60%). During the interstage period, all but one patient needed additional procedures. One patient died of bacterial meningitis 4 months after stage I. Four patients were submitted to stage II operation at 6.6 +/- 0.5 months of age and one is waiting for the operation. All four required early reinterventions for pulmonary artery stenosis. Only one was discharged home and was not yet submitted to the third stage. The hybrid approach for HLHS was associated with poor results in this early experience from two independent institutions in a developing country. This might have been related to infrastructure and technical problems, as well as our own learning curve. Institutions working under the same conditions might face similar problems during their initial experience.