Pediatric Pulmonary Hypertension: A Pharmacotherapeutic Review

2009 ◽  
Vol 22 (2) ◽  
pp. 166-178
Author(s):  
Kimberly A. Pesaturo ◽  
Peter N. Johnson ◽  
E. Zachary Ramsey

Pulmonary hypertension in children is a disorder associated with increased pulmonary vascular resistance and arterial pressure, decreased cardiac output, and right-sided cardiac dysfunction that is caused by numerous etiologies. Although treatment will vary with underlying cause, pharmacological treatment has historically included inhaled nitric oxide and prostacyclin analogues. Over the past several years new agents have been added to the treatment armamentarium, including phosphodiesterase V inhibitors (eg sildenafil) and endothelin antagonists (eg bosentan). Further, more agents are currently under investigation for pulmonary hypertension in children including immunosuppressives and other endothelin antagonist entities. Limitations to treatment include the availability of appropriate, robust pediatric pharmacological data and constraints with dosage forms.

1998 ◽  
Vol 8 (2) ◽  
pp. 205-210 ◽  
Author(s):  
Fukiko Ichida ◽  
Kei-ichiro Uese ◽  
Shin-ichi Tsubata ◽  
Ikuo Hashimoto ◽  
Yuji Hamamichi ◽  
...  

AbstractTo determine whether a newly synthesized oral analog of prostacyclin, called beraprost sodium, could cause pulmonary vasodilation, we studied its hemodynamic effect on pulmonary hypertension of children, comparing it to other vasodilatory agents, such as nitric oxide and tolazoline. We studied 20 children (mean age 24 months) having pulmonary hypertension secondary to congenital heart disease. A single oral dose of beraprost sodium resulted in an appreciable reduction of pulmonary vascular resistance (mean 34%), which was comparable to that induced by inhalation of nitric oxide and intravenous delivery of tolazoline (mean 41% and 31%, respectively). The results suggest that beraprost sodium may serve as a novel and safe vasodilator for the screening of pulmonary vasoreactivity, as well as the treatment of pulmonary hypertension in children.


1996 ◽  
Vol 3 (6) ◽  
pp. 373-376 ◽  
Author(s):  
Robert M Kacmarek

A literature review on nitric oxide would identify thousands of citations on the biological implications of this molecule. From the perspective of respiratory care, the effect inhaled nitric oxide has on pulmonary vasculature is the most intriguing. Over the past five years inhaled nitric oxide has been shown to be useful in the management of oxygenation during acute respiratory distress syndrome, alternation of pulmonary vascular tone in persistent pulmonary hypertension in the newborn, and in the management of chronic pulmonary hypertension in both heart and lung transplant candidates, as well as other potential clinical uses. The key physioligical response is vasodilation of pulmonary vessels in communication with well ventilated lung units and the absence of systemic vascular effects by rapid binding to hemoglobin. Nitric oxide therapy is considered experimental. A delivery system is not commercially available. This has resulted in the development of makeshift delivery systems, many of which may have the potential for adverse effects.


2011 ◽  
Vol 10 (2) ◽  
pp. 104-108 ◽  
Author(s):  
Mary P. Mullen

Over the past decade, major progress has occurred in the care of children and adults with pulmonary arterial hypertension (PAH). Recent insights into molecular mechanisms implicated in the development of pulmonary vascular disease have led to revised clinical classification and diagnostic strategies.1 Data from multicenter pulmonary hypertension registries have provided increased understanding of the clinical course and natural history of many subtypes of disease.2–4 Additionally, the development of multiple therapies targeting important pathways of disease and the translation of those treatments to pediatrics have led to improved life experience and survival for many children with PAH.5


Circulation ◽  
2001 ◽  
Vol 103 (4) ◽  
pp. 544-548 ◽  
Author(s):  
Peter C. Rimensberger ◽  
Isabelle Spahr-Schopfer ◽  
Michel Berner ◽  
Edgar Jaeggi ◽  
Afksendiyos Kalangos ◽  
...  

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