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Pulmonary Hypertension Pulmonary Module Steven W. Harris MHS, PA-C Pulmonary Hypertension Defined as pressure within the pulmonary arterial system elevated above the normal range. l greater than 25 mm Hg at rest or 30 mm Hg with exercise Pulmonary arterial pressure (PAP) l usually 12-15 mmHg Left atrial pressure l 6-10 mmHg * 2 Pulmonary hypertension develops when flow or resistance to flow across the pulmonary vascular bed increases Four Categories of Pulmonary Hypertension 1) Pulmonary hypertension from disorders of the respiratory system or hypoxemia. 2) Pulmonary venous hypertension. 3) Pulmonary hypertension from chronic thromboembolic disease. 4) Pulmonary arterial hypertension. (PAH) * 3 Chronic pulmonary hypertension is an important cause of right ventricular failure in the United States. Many of the individuals who die each year of chronic obstructive pulmonary disease (COPD) succumb secondarily to right ventricular failure resulting from pulmonary hypertension. * 4 In addition, greater than 200,000 deaths occur yearly from acute pulmonary embolism, the most common cause of sudden-onset pulmonary hypertension and acute right ventricular failure. * 5 * 6 * 7 * 8 In persons more than 50 years of age, Cor pulmonale the consequence of untreated pulmonary hypertension, is the third most common cardiac disorder (after coronary and hypertensive heart disease). * 9 Entities that cause increased pulmonary blood flow Congenital Heart Disease l Left to right shunt “Hyperkinetic“ pulmonary hypertension can be seen in patients with congenital heart disease who have extensive left-to-right cardiac shunts that produce a large pulmonary blood flow Increased cardiac output states l severe anemias * 10 Entities that cause an increased resistance to flow Pulmonary embolism In situ pulmonary embolism Pulmonary fibrosis Sarcoidosis, scleroderma, or extensive pulmonary resection Severe COPD Thoracic deformities Large tumor or infiltrate * 11 Entities that cause changes in arterioles Hypoxia (altitude) COPD Hypoventilation (sleep apnea) Acidosis Drugs Pulmonary arterial hypertension (PAH) * 12 Entities that cause venous pressure changes rule out underlying cardiac* 29 EKG Findings RVH (late finding) l RAD RBBB RV strain (acute ie Pulm embolus) l S wave in lead I l Q wave/ inverted T in Lead III * 30 * 33 * 34 Diagnostic Tests l Pulmonary angiography - should be done if segmental or larger defect on V/Q scan. Caution in pulmonary hypertension as can lead to hemodynamic collapse; use low osmolar agents, subselective angiograms. l Lung biopsy not recommended * 35 * 36 World Health Organization Classification of Functional Status of Patients with PH Class I - patients with PH who experience no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnea, fatigue, chest pain or presyncope Class II - patients with PH who have mild limitation of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnea, fatigue, chest pain or presyncope Class III - patients with PH who have marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increased dyspnea, fatigue, chest pain, or presyncope Class IV - patients with PH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure. Dyspnea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity * 37 Tx of Pulmonary HTN l Treat the cause! l Phosphodiesterase inhibitors (sildenafil) Revatio l Prosanoids (prostacyclin analogues) Flolan, Iloprost l Endothelin receptor antagonists Bosentan l Vasodilators L-arginine? l Coumadin * 38 Tx of Pulmonary HTN Phosphodiesterase inhibitors (sildenafil) Revatio l Nitric oxide stimulation of endothelium increases cGMP resulting in vasorelaxation l Phosphodiesterase breaks down cGMP * 39 Tx of Pulmonary HTN Prosanoids (prostacyclin analogues) Flolan, Iloprost l Prostacyclin is a potent vasodilator produced in the vascular endothelium . Also inhibits growth of smooth muscle cells * 40 Tx of Pulmonary HTN Endothelin receptor antagonists Bosentan l Endothelan-1 is a potent vasoconstrictor, induces fibosis and leads to proliferation of smooth-muscle cells * 41 Tx of Pulm HTN Treatment of heart failure (e.g., diuretics) Oxygen supplementation is indicated for rest, exercise, or nocturnal hypoxemia Calcium channel blockers (nifedipine, diltiazem l Only in selected patients that qualify via a vasoreactivity study. Digoxin (afterload reduction & increase cardiac output) Surgery of thrombolic emboli Low salt diet Cautious exercise Heart-lung transplant* 42 Prognosis Surviva

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