Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension.

Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society

A B S T R AC T Background More than 30% of patients with pleural infection either die or require surgery. Drainage of infected fluid is key to successful treatment, but intrapleural fibrinolytic therapy did not improve outcomes in an earlier, large, randomized trial. Methods We conducted a blinded, 2-by-2 factorial trial in which 210 patients with pleural infection were randomly assigned to receive one of four study treatments for 3 days: double placebo, intrapleural tissue plasminogen activator (t-PA) and DNase, t-PA and placebo, or DNase and placebo. The primary outcome was the change in pleural opacity, measured as the percentage of the hemithorax occupied by effusion, on chest radiography on day 7 as compared with day 1. Secondary outcomes included referral for surgery, duration of hospital stay, and adverse events. Results The mean (±SD) change in pleural opacity was greater in the t-PA–DNase group than in the placebo group (−29.5±23.3% vs. −17.2±19.6%; difference, −7.9%; 95% confidence interval [CI], −13.4 to −2.4; P = 0.005); the change observed with t-PA alone and with DNase alone (−17.2±24.3 and −14.7±16.4%, respectively) was not significantly differ ent from that observed with placebo. The frequency of surgical referral at 3 months was lower in the t-PA–DNase group than in the placebo group (2 of 48 patients [4%] vs. 8 of 51 patients [16%]; odds ratio for surgical referral, 0.17; 95% CI, 0.03 to 0.87; P = 0.03) but was greater in the DNase group (18 of 46 patients [39%]) than in the placebo group (odds ratio, 3.56; 95% CI, 1.30 to 9.75; P = 0.01). Combined t-PA–DNase therapy was associated with a reduction in the hospital stay, as compared with placebo (difference, −6.7 days; 95% CI, −12.0 to −1.9; P = 0.006); the hospital stay with either agent alone was not significantly different from that with placebo. The frequency of adverse events did not differ significantly among the groups. Conclusions Intrapleural t-PA–DNase therapy improved fluid drainage in patients with pleural in fection and reduced the frequency of surgical referral and the duration of the hospital stay. Treatment with DNase alone or t-PA alone was ineffective. (Funded by an unrestricted educational grant to the University of Oxford from Roche UK and by others; Current Controlled Trials number, ISRCTN57454527.)

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Intrapleural use of tissue plasminogen activator and DNase in pleural infection

Pediatric Pulmonary Hypertension

Parapneumonic effusions occur in 20 to 40% of patients who are hospitalized with pneumonia. The mortality rate in patients with a parapneumonic effusion is higher than that in patients with pneumonia without a parapneumonic effusion. Some of the excess mortality is due to mismanagement of the parapneumonic effusion. Characteristics of patients that indicate that an invasive procedure will be necessary for its resolution include the following: an effusion occupying more than 50% of the hemithorax or one that is loculated; a positive Gram stain or culture of the pleural fluid; and a purulent pleural fluid that has a pH below 7.20 or a glucose below 60, or has a lactic acid dehydrogenase level of more than three times the upper normal limit for serum. Patients with pneumonia and an effusion of more than minimal size should have a therapeutic thoracentesis. If the fluid cannot be removed with a therapeutic thoracentesis, a chest tube should be inserted and consideration be given to the intrapleural instillation of fibrinolytics. If the loculated effusion persists, the patient should be subjected to video-assisted thoracoscopic surgery, and if the lung cannot be expanded with this procedure, a full thoracotomy with decortication should be performed. The definitive procedure should be performed within 14 d.

Parapneumonic effusions and empyema.

Respiratory Distress of the Term Newborn Infant

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare, fatal developmental lung disorder of neonates and infants. This review aims to address recent findings in the etiology and genetics of ACD/MPV and to raise awareness of this poorly known disease, which may also present as milder, unclassified forms. Successively discussed are what is known about the epidemiology, pathogenesis, pathophysiology, diagnostic indicators and approaches, genetic testing, treatment, and cases of delayed onset. The review concludes with suggestions for future directions to answer the many unknowns about this disorder.

Alveolar capillary dysplasia.

Traumatic brain injury: a primer for primary care physicians.

The treatment of complex parapneumonic effusions in children remains controversial, with some advocating less invasive, strictly medical management and others supporting a more aggressive approach of thoracotomy with or without decortication. Recent advances, including video-assisted thoracoscopic surgery and intrapleural fibrinolytic therapy, offer new options for effective treatment. We report the first case of successful resolution of a complex parapneumonic effusion in a 16-month-old girl with the use of tissue plasminogen activator (alteplase), infused via a catheter in the pleural space.

https://pediatrics.aappublications.org/content/pediatrics/111/2/e188.full.pdf

Alteplase in the treatment of complicated parapneumonic effusion: a case report.

The impact of gestational age on perioperative morbidity was examined using a novel construct, the resource utilization index (RUI). The medical records of subjects from birth to 1 year of age entered into a pediatric cardiothoracic surgery database from a major academic medical center between 2007 and 2011 were reviewed. The hypothesis tested was that infants born at 37–38 weeks (early-term infants) experience greater resource utilization after open heart surgery than those born at 39 completed weeks and that this association can be observed until 1 year of age. The results support the premise that resource utilization increases linearly with declining gestational age among infants at 0–12 months who undergo cardiac surgery. Five of the six variables comprising the RUI showed statistically significant linear associations with gestational age in the predicted direction. Multivariate linear regression analysis showed that gestational age was a significant predictor of an increased RUI composite. Further investigation is needed to test the concept and to expand on these findings.

The Impact of Gestational Age on Resource Utilization After Open Heart Surgery for Congenital Cardiac Disease From Birth to 1 Year of Age

Background: The pediatric intensive care unit (PICU) is an environment where seriously ill children receive complex care, delivered mostly by specialty-trained nurses (registered nurses [RNs]) who must perform multiple high-level tasks. With stressors on healthcare systems at an all-time high, design that optimizes RN workflow has taken on a renewed imperative. Objectives: To employ a multimodal approach (1) to identify environmental factors in the PICU patient room that contribute to caregiver workflow inefficiencies, (2) to optimize safety by identifying high-touch surfaces that cause hospital-acquired infections, (3) to develop human-centered design recommendations. Methods: This mixed-method case study was conducted in a 23-bed urban hospital PICU. The activities, movements, and workflows of 13 RNs were recorded using spatial movement mapping, behavioral mapping, and clinical activity mapping. Frequency of RN contact with surfaces was documented to assess relative infection transmission risk. Face-to-face interviews were conducted with RNs to elicit their views on care delivery and their physical work environment. Results: Direct patient care occupied 50% of RNs’ time. Of the direct patient care workflow activities recorded, 26% were to prepare for care around the bedside, while 27% were for random travel between clean and soiled areas. The surfaces most frequently touched were (1) patient bedrails, (2) intravenous pumps and poles, (3) tubing and medical equipment, and (4) vital sign monitors. Conclusion: Value-added tasks account for only about 20% of nurses’ work. Combining technology and strategic interior design to streamline workflow and enhance infection prevention optimizes efficiency and empowers frontline providers to maximize their time at the bedside performing value-added tasks.

Naomi B. Bishop

Papers

Alveolar capillary dysplasia.

Traumatic brain injury: a primer for primary care physicians.

Alteplase in the treatment of complicated parapneumonic effusion: a case report.

The Impact of Gestational Age on Resource Utilization After Open Heart Surgery for Congenital Cardiac Disease From Birth to 1 Year of Age

Lean Design of the Pediatric Intensive Care Unit Patient Room for Efficient and Safe Care Delivery