Pulmonary Hypertension 1

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Review of Pulmonary Hypertension

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Pulmonary Hypertension 1

  1. 1. Pulmonary Hypertension James Ratliff MD 27 March 2008
  2. 2. Pulmonary Hypertension <ul><li>Normal pressures </li></ul><ul><ul><li>25 mmHg systolic; 10 mmHg diastolic </li></ul></ul><ul><li>PHT </li></ul><ul><ul><li>> 30 to 35 mmHg systolic </li></ul></ul><ul><ul><li>> 20 mmHg diastolic </li></ul></ul><ul><li>Right ventricular dilatation (acute) </li></ul><ul><li>Right ventricular hypertrophy (chronic) </li></ul><ul><ul><li>Corpulmonale (2 o to a pulmonary cause) </li></ul></ul>
  3. 3. PHT Cycle
  4. 4. Pathology <ul><li>Small muscular arteries & arterioles </li></ul><ul><ul><li>Hypertrophy of the media (smooth muscle) </li></ul></ul><ul><ul><li>Hyperplasia of the intimal layer </li></ul></ul><ul><ul><li> luminal diameter  lumen may be obliterated </li></ul></ul><ul><li>Pulmonary arteries (with  ing hypertension) </li></ul><ul><ul><li>Thickening of the media </li></ul></ul><ul><ul><li>Atherosclerotic plaques </li></ul></ul><ul><ul><li>In situ thrombosis  webs and bands </li></ul></ul>
  5. 5. Pathology
  6. 6. Pulmonary Hypertension Pathophysiology <ul><li>Pre-capillary (arterioles and arteries) </li></ul><ul><ul><li> Systolic and diastolic pressures </li></ul></ul><ul><ul><li>Capillary pressure normal </li></ul></ul><ul><li>Post-capillary (pulmonary venous hypertension) </li></ul><ul><ul><li> Capillary pressure </li></ul></ul><ul><li> PHT   RV pressure </li></ul><ul><li>RV hypertrophy; Flattening of the IV septum </li></ul><ul><li>RV failure </li></ul><ul><ul><li> RV end diastolic pressure   RA pressure </li></ul></ul><ul><ul><li> cardiac output </li></ul></ul>
  7. 7. Clinical Manifestations <ul><li>Symptoms </li></ul><ul><ul><li>Dyspnea </li></ul></ul><ul><ul><li>Chest Pain </li></ul></ul><ul><ul><li>Syncope </li></ul></ul><ul><li>Signs </li></ul><ul><ul><li> JVP (prominent a wave) </li></ul></ul><ul><ul><li>RV heave </li></ul></ul><ul><ul><li>R sided S3 </li></ul></ul><ul><ul><li>Tricuspid regurgitation </li></ul></ul><ul><ul><li> P2 </li></ul></ul><ul><ul><li>Palpable P2 </li></ul></ul><ul><ul><li>Peripheral edema </li></ul></ul><ul><ul><li>Pulsatile Liver </li></ul></ul><ul><li>EKG </li></ul><ul><ul><li>R axis deviation </li></ul></ul><ul><ul><li>R ventricular strain </li></ul></ul><ul><ul><li>S 1 Q 3 (pulm embolism) </li></ul></ul><ul><ul><li>P pulmonale </li></ul></ul><ul><ul><li>Incomplete RBBB </li></ul></ul><ul><li>ChestX-ray </li></ul><ul><ul><li>Prominent pulm arteries </li></ul></ul><ul><ul><li>Etiology of PHT </li></ul></ul>
  8. 8. Primary Pulmonary Hypertension <ul><li>Unknown etiology </li></ul><ul><li>Females 20 to 40 years of age </li></ul><ul><li>Exclude secondary causes (including drugs) </li></ul><ul><li>Raynaud’s syndrome is common </li></ul><ul><li>Prognosis is poor </li></ul><ul><li>Treatment  vasodilators </li></ul><ul><ul><li>Limited by effect on systemic circulation </li></ul></ul><ul><ul><ul><li>Usually cardiac output is low and fixed </li></ul></ul></ul><ul><ul><li>Response is variable </li></ul></ul><ul><ul><li>Should always be done in an ICU </li></ul></ul><ul><ul><li>Prostacyclin may be useful (continuous IV infusion) </li></ul></ul><ul><li>Anticoagulate </li></ul><ul><li>Oxygen </li></ul>
  9. 9. Secondary Pulmonary Hypertension <ul><li>Pathogenesis </li></ul><ul><li>Ppa = (Q x PVR) + Ppv </li></ul><ul><li>Volume of pulmonary blood flow (Q) </li></ul><ul><ul><li>Cardiac shunts – eg, ASD, VSD </li></ul></ul><ul><ul><ul><li>Initially L  R shunt </li></ul></ul></ul><ul><ul><ul><li>If converts to a R  L shunt - Eisenmenger’s syndrome </li></ul></ul></ul><ul><li>• Resistance in the pulmonary vascular bed (PVR) </li></ul><ul><ul><li>Hypoxia (COPD, sleep apnea) </li></ul></ul><ul><ul><li>Emphysema </li></ul></ul><ul><ul><li>Chronic Pulmonary Emboli </li></ul></ul><ul><li>• Pulmonary venous pressure (Ppv) </li></ul><ul><ul><li>Mitral stenosis </li></ul></ul><ul><ul><li>LV overload </li></ul></ul>
  10. 10. Differential Diagnosis <ul><li> Resistance to blood flow </li></ul><ul><ul><li>1 o PHT </li></ul></ul><ul><ul><li>Connective Tissue Disease – Scleroderma </li></ul></ul><ul><ul><li>Chronic PE </li></ul></ul><ul><ul><li>Emphysema </li></ul></ul><ul><li>Hypoxic Vasoconstriction </li></ul><ul><ul><li>Any underlying lung disease  hypoxia </li></ul></ul><ul><ul><li>Sleep apnea </li></ul></ul><ul><li>Cardiac </li></ul><ul><ul><li> Blood Flow (shunts – ASD, VSD) </li></ul></ul><ul><ul><li> Pulmonary venous pressure </li></ul></ul><ul><ul><ul><li>Mitral valve stenosis </li></ul></ul></ul><ul><ul><ul><li>LV failure </li></ul></ul></ul>
  11. 11. Detection of PHT by Echo TR = Tricuspid Regurgitant Peak Velocity (m/sec)  p = 4(TR) 2 RV sys = RA est +  p TR Tricuspid valve Echo transducer
  12. 12. Clinical Approach to Pulmonary Hypertension
  13. 13. <ul><li>Pulmonary Arterial HTN </li></ul><ul><ul><li>Primary pulmonary hypertension Sporadic disorder Familial disorder Related conditions </li></ul></ul><ul><ul><li>Collagen vascular disease </li></ul></ul><ul><ul><li>Congenital systemic-to-pulmonary shunt </li></ul></ul><ul><ul><li>Portal hypertension </li></ul></ul><ul><ul><li>Human immunodeficiency virus infection </li></ul></ul><ul><ul><li>Drugs and toxins </li></ul></ul><ul><ul><li>Anorectic agents (appetite suppressants) </li></ul></ul><ul><ul><li>Persistent pulmonary hypertension of the newborn </li></ul></ul><ul><li>Pulmonary Venous HTN </li></ul><ul><ul><li>Left-sided atrial or ventricular heart disease Left-sided valvular heart disease </li></ul></ul><ul><ul><li>Extrinsic compression of central pulmonary veins </li></ul></ul><ul><ul><li>Fibrosing mediastinitis </li></ul></ul><ul><ul><li>Adenopathy and/or tumors </li></ul></ul><ul><ul><li>Pulmonary veno-occlusive disease </li></ul></ul>WHO Diagnostic Classification of Pulmonary Hypertension
  14. 14. WHO Diagnostic Classification of Pulmonary Hypertension <ul><li>Pulmonary HTN assoc. w/ disorders of the respiratory system </li></ul><ul><ul><li>Chronic obstructive pulmonary disease Interstitial lung disease </li></ul></ul><ul><ul><li>Sleep-disordered breathing Alveolar hypoventilation disorders </li></ul></ul><ul><ul><li>Chronic exposure to high altitudes </li></ul></ul><ul><ul><li>Neonatal lung disease </li></ul></ul><ul><ul><li>Alveolar-capillary dysplasia </li></ul></ul><ul><li>Pulmonary HTN assoc. w/ chronic thrombotic / embolic disease </li></ul><ul><ul><li>Thromboembolic obstruction of proximal pulmonary arteries </li></ul></ul><ul><ul><li>Obstruction of distal pulmonary arteries Pulmonary embolism (thrombus, tumor, ova and/or parasites, foreign material) </li></ul></ul><ul><ul><li>In-situ thrombosis Sickle cell disease </li></ul></ul><ul><li>Pulmonary HTN assoc w/pulmonary vasculature Inflammatory conditions </li></ul><ul><ul><li>Schistosomiasis Sarcoidosis </li></ul></ul><ul><ul><li>Pulmonary capillary hemangiomatosis </li></ul></ul>
  15. 15. Functional Assessment of Patients with Pulmonary Hypertension <ul><li>Class I: </li></ul><ul><ul><li>Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope. </li></ul></ul><ul><li>Class II: </li></ul><ul><ul><li>Patients with pulmonary hypertension resulting in slight limitation of physical activity. These patients are comfortable at rest, but ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope. </li></ul></ul><ul><li>Class III: </li></ul><ul><ul><li>Patients with pulmonary hypertension resulting in marked limitation of physical activity. These patients are comfortable at rest, but less than ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope. </li></ul></ul><ul><li>Class IV: </li></ul><ul><ul><li>Patients with pulmonary hypertension resulting in inability to perform any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may be present at rest, and discomfort is increased by any physical activity. </li></ul></ul>
  16. 16. Risk Factors for Pulmonary Hypertension <ul><li>Drugs and toxins </li></ul><ul><ul><li>Definite causal relationship Aminorex Fenfluramine Dexfenfluramine Toxic rapeseed oil </li></ul></ul><ul><ul><li>Amphetamines L-Tryptophan </li></ul></ul><ul><ul><li>Meta-amphetamines Cocaine Chemotherapeutic agents </li></ul></ul><ul><ul><li>Antidepressants Oral contraceptives Estrogen therapy Cigarette smoking </li></ul></ul><ul><li>Demographic factors and medical conditions </li></ul><ul><ul><li>Gender </li></ul></ul><ul><ul><li>Pregnancy Systemic hypertension </li></ul></ul><ul><ul><li>Obesity </li></ul></ul><ul><li>Diseases </li></ul><ul><ul><li>Human immunodeficiency virus infection </li></ul></ul><ul><ul><li>Portal hypertension and/or liver disease Collagen vascular diseases Congenital systemic-to-pulmonary cardiac shunts </li></ul></ul><ul><ul><li>Thyroid disorders </li></ul></ul>
  17. 17. EKG Findings in PHT <ul><li>FIGURE 1. Electrocardiogram demonstrating the changes of right ventricular hypertrophy (long arrow) with strain in a patient with primary pulmonary hypertension. Right axis deviation (short arrow) , increased P-wave amplitude in lead II (black arrowhead) , and incomplete right bundle branch block (white arrowhead) are highly specific but lack sensitivity for the detection of right ventricular hypertrophy.12 </li></ul>
  18. 18. Indications to Aide in Diagnosis of PHT <ul><li>FIGURE 2. Anatomy of the thorax, along with indication of the upper limits of normal pulmonary vascular dimensions. A right interlobar pulmonary diameter of greater than 16 mm or a hilar-to-thoracic ratio of greater than 0.44 is specific but not sensitive for the diagnosis of pulmonary hypertension.12 </li></ul>
  19. 19. Pulmonary Hypertension Possible Treatments <ul><li>Correct underlying cause: </li></ul><ul><ul><li>Surgical treatment of mitral stenosis </li></ul></ul><ul><ul><li>left to right shunt </li></ul></ul><ul><ul><li>accessible chronic thromboemboli </li></ul></ul><ul><li>Afterload reduction, </li></ul><ul><ul><li>Digoxin (Lanoxin) and Diuretics for left ventricular dysfunction </li></ul></ul><ul><ul><li>Prevention and treatment of respiratory infection </li></ul></ul><ul><ul><li>Avoidance of anorectic agents </li></ul></ul><ul><li>Decrease pulmonary vascular resistance: </li></ul><ul><ul><li>Vasodilators / Oxygen / Calcium channel blockers </li></ul></ul><ul><ul><ul><li>Diltiazem (Cardizem), Nifedipine (Procardia) Prostacyclin (epoprostenol [Flolan]) or prostacyclin analogs Nitric oxide (investigational) </li></ul></ul></ul><ul><ul><ul><li>Anticoagulants for primary pulmonary hypertension and chronic thromboembolism </li></ul></ul></ul><ul><li>Increase cardiac output: </li></ul><ul><ul><li>Short-term parenteral inotropes Digoxin </li></ul></ul><ul><li>Reduce volume overload: </li></ul><ul><ul><li>Low-salt diet Diuretics </li></ul></ul><ul><li>Perform lung transplantation or atrial septosotomy (investigational) </li></ul>

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