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Home > EB Store > Pediatric Pulmonary Hypertension: Diagnosis And Management In The Acute Care Setting
Pediatric Pulmonary Hypertension: Diagnosis And Management In The Acute Care Setting - $30.00
This issue includes 4 AMA/ACEP category 1, AAP Prescribed CME credits
Authors:
Peter E. Oishi, MD and
Jeffrey R. Fineman, MD
Peer Reviewers:
Dolores Hoey, MD, and
Ghazala Q. Sharieff, MD, FAAP, FACEP, FAAEM
Publication Date:
January 1, 2008, Volume 5, Number 1
Excerpt from the issue…
A 10-year-old girl is brought to the pediatric emergency department after collapsing at school during her physical education class. She is well known to the emergency physicians, having been treated in the ED on multiple occasions over the past year for asthma exacerbations. Most recently, she was admitted for inpatient treatment and was discharged after 24 hours with nebulized bronchodilators.
As the paramedics transfer her to the ED staff, the patient’s mother reports that her daughter has experienced increasing shortness of breath with exercise over the past few days - even with walking up stairs at home - and, in general, has appeared to be fatigued. The patient has been using her prescribed inhaler frequently, without clear relief. The mother also mentions that on the evening prior to this event, her daughter complained of abdominal pain.
Conclusion to the above case study...
This patient was suffering from arterial pulmonary hypertension, and her previous presentations to the ED were incorrectly diagnosed as reactive airway disease. In the ED, she had significant dyspnea that improved somewhat with 100% oxygen delivered by facemask. CXR revealed clear lung fields with pruned distal pulmonary vessels and dilated central pulmonary arteries. ECG revealed right ventricular hypertrophy. Echocardiography revealed supra-systemic systolic pulmonary artery pressures. She was intubated, and mechanical ventilation was adjusted to achieve respiratory alkalosis. She was maintained on an inspired oxygen concentration of 100% and started on 20 ppm of inhaled nitric oxide and intermittent inhaled prostacyclin. The child was then transferred to the pediatric intensive care unit. A pulmonary hypertension specialist was consulted and the patient was scheduled for a diagnostic right heart catheterization. Genetic testing and family history subsequently confirmed a diagnosis of familial arterial pulmonary hypertension. The patient improved and was eventually discharged home on intravenous prostacyclin and bosentan.
About this article:
Pediatric pulmonary hypertension is a relatively rare, often rapidly progressive disease with varied etiologies that is associated with significant morbidity and mortality. In fact, until recently, virtually all patients with this condition died within a few years of diagnosis.1-3 Fortunately, fundamental advances in vascular biology over the last several decades have translated into a vast expansion of our understanding about pulmonary hypertension, and an increasing number of promising therapies have emerged to treat this condition. Because the potential consequences can be so devastating, this issue of Pediatric Emergency Medicine Practice will provide a comprehensive overview of pediatric pulmonary hypertension, with particular attention to diagnosis and acute management.
