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A Guide for Pediatric Pulmonary Hypertension

A Guide for Pediatric Pulmonary Hypertension

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and children. The disease affects nearly two of every 1,000 babies born each year in the United States, and children can suffer with health issues throughout their lives or die prematurely. However, with a proper diagnosis, rigorous evaluation, and comprehensive treatment, the prognosis for many of these children can be excellent, depending on the underlying cause of the pulmonary hypertension. The American Heart Association and American Thoracic Society convened a panel of experts to review the current literature and make recommendations on diagnosing, evaluating, and treating pediatric pulmonary hypertension. Published in Circulation and available for free online at http://circ.ahajournals.org, the guidelines are the result of extensive literature reviews of over 600 studies, extensive discussions, and formal scoring of recommendations. “These first-ever guidelines were rigorously developed in collaboration with a group of diverse experts that cross the traditional silos of several disciplines,” says Steven H. Abman, MD, the guidelines committee co-chair. “To optimize the care of pediatric patients with pulmonary hypertension, increased awareness of at-risk patients and the use of multidisciplinary care teams are critical.”   Key Highlights According to Dr. Abman, the guidelines provide clinicians with practical advice on classifying the many types of pulmonary hypertension, a key step in determining treatment. The document also discusses proven and emerging medical and surgical therapies as well as treatments that are approved for children with pulmonary hypertension and which drugs and dosages should be used. In addition, guidance is provided for long-term monitoring.   Specialized Care “It’s important to note that the causes, clinical presentations, and treatment...
The Current State of Pediatric Pulmonary Hypertension

The Current State of Pediatric Pulmonary Hypertension

Research has shown that pulmonary hypertension is associated with substantial morbidity and mortality in children. Registry data from the United Kingdom and the Netherlands estimate an incidence of idiopathic pulmonary hypertension of 0.48 to 0.70 cases per million, respectively. However, such data on pediatric pulmonary hypertension remain unknown in the United States. Studies assessing the inpatient care of pediatric pulmonary hypertension in the U.S. have been small or focused on select subgroups of patients. Reviewing the Data To determine trends in volume, demographics, procedures performed during admission, and resource use, Bryan Maxwell, MD, MPH, Melanie Nies, MD, and colleagues examined national data on hospitalizations among pediatric patients with pulmonary hypertension. The study, published in Pediatrics, reviewed data from 1997 to 2012 in the Kids’ Inpatient Database, the largest publicly available database of inpatient pediatric care in the United States, according to Dr. Nies. Beginning in 1997, the database has released discharge data every 3 years from thousands of hospitals throughout the U.S. Overall, children with pulmonary hypertension accounted for 0.13% of 43 million pediatric hospitalizations included in the registry during the study period. Discharges for pediatric pulmonary hypertension doubled from 1997 to 2012. Cumulative, inflation-adjusted national hospital charges associated with pediatric pulmonary hypertension hospitalizations increased from $926 million in 1997 to $3.12 billion in 2012. “It is important to note that although all-cause, in-hospital mortality associated with the condition remains high, it decreased from 11.3% of hospitalizations in 1997 to 5.9% in 2012,” Dr. Nies says. The study investigators also found a shift in the type of children who accounted for the majority of pediatric pulmonary hypertension cases. “Children...
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