Abstract

Pulmonary artery hypertension causes remodeling of distal pulmonary arterial vasculature leading to increased resistance of the pulmonary arterial system, right ventricular dysfunction, and sudden cardiac death. The diagnosis of pulmonary arterial hypertension (PAH) diagnosis is made when mean pulmonary artery pressure during catheterization is ≥25 mmHg at rest, pulmonary vascular resistance (PVR) more than 3 wood units, a pulmonary capillary wedge pressure of <15 mmHg. One year survival rate is 86.3% and 5 year survival rate in PAH is 61.2%, and only 7 years of median survival. Although several breakthrough advances are made in the medical management for PAH, there are some patients who do not respond to medications and continue to detoriate despite optimal medical therapy. The non-responders to medical management are those patients whose right atrial pressure is >20 mmHg or cardiac index is <2.0 L/min/m2, which are pointers of poor prognosis. For medical refractory patients invasive procedures such as atrial septostomy, Potts shunt, and pulmonary artery denervation are a therapeutic or palliative strategy in the treatment of pulmonary artery hypertension and serve as a bridge before surgery and heart lung transplantation.

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