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Pulmonary Hypertension Kruti Jambula 4.15.11 Definition PH when a disease elevates pulmonary arterial pressure above normal. Pulmonary arterial pressure = LA pressure+ (Pulmonary blood flow x Pulmonary Vascular Resistance) PA pressure 25mm Hg is abnormal. Often progressive, if untreated RV dysfunction. Prognosis depends on reversibility of underlying disorder. Classification WHO classification, initially proposed in 1998 and revised in 2003 categorizes based on pathophysiology, clinical presentation and treatment. Classification PPHN Most common cause in newborns. 0.2% of all term infants. With or without acute respiratory conditions - persistently elevated pulmonary vascular resistance - R to L shunting across Foramen ovale/DA - significant hypoxemia. In the fetus systemic and pulmonary arterial pressures are almost equal because of a highly vascular placenta for gas exchange and constricted pulmonary vessels. At birth PAP decreases to 50% systemic and PBF increases by almost tenfold. Decline in pulmonary vascular resistance greatest in first 24 hours and continues to fall in the first 2 postnatal weeks. Multifactorial increase in PBF Increased arterial PH and oxygen tension physical pulling open of capillaries accompanying lung inflation local endogenous vasodilatory mediators- PGs and NO removal of the low systemic vascular resistance placenta following clamping of UC. Echo: exclude cyanotic heart disease. R to L shunting across foramen ovale or DA. Deviation of atrial septum from R to L. RA enlargement and TR. Treatment Aimed at preventing end-organ injury from hypoxia, ischemia and barotrauma. Correct any contributing disturbances: hypoglycemia, polycythemia, hypothermia or pneumothorax. Maintain systemic vascular resistance: colloids/crystalloids and inotropes Lower pulmonary vascular resistance: iNO which activates soluble guanylate cyclase and increases cGMP- activation of cascade causing calcium efflux and vascular SM relaxation. iNO: activates solu
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