Pulmonary arterial hypertension: literature review

ABSTRACT

Pulmonary arterial hypertension [PAH] is a fatal disease as it results in a very bad damage to the pulmonary vasculature which progress gradually to cause right ventricular failure. In idiopathic PAH there is hypertrophy and hyperplasia of the pulmonary arteries which eventually obliterate the vessel, and in more advanced stages thrombosis The classification of PAH has undergone several revisions, the latest was during the 4[th] World Symposium held in 2008, few modifications were adopted concerning Group 1 and Group 4. Many pathways are involved in the abnormal proliferation and contraction of the smooth muscle cells of the pulmonary arteries in patients with PAH. Three of these pathways are important endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclin derivatives. The aim of medical treatment is to dilate the pulmonary vasculature and reverse the abnormal remodeling characteristic of pulmonary vascular disease. The practical difficulties encountered in treating children influence management and include their age, level of understanding, size, and in some, and the presence of other anomalies.Monitoring patients are normally monitored through commonly available tests such as pulse oxymetery, arterial blood gas tests, CXR, serial ECG's, serial echocardiograms, and spirometry or more advanced lung function tests. The prognosis of pulmonary hypertension varies in between different diagnostic groups. In general, the prognosis is poor with no definitive cure for it. Patients whose disease progresses and is unresponsive to medical treatments either undergo transplantation or die of progressive right-sided heart failure. Certain types of pulmonary venous hypertension has poorer prognosis especially in pediatric patients. Although the disease is * fatal, trials to get more effective treatments are being held in many centers, and great efforts should be done to clarify the complete patho-physiological process