Pulmonary hypertension

Transcription

1 Common Pulmonary Problems In Adults Todd A. May, M.D. Chief of the Medical Staff San Francisco General Hospital Clinical Professor Family & Community Medicine University of California, San Francisco Pulmonary Problems Pulmonary hypertension Secondary causes Pulmonary embolism Interstitial lung disease Sarcoidosis Obstructive sleep apnea COPD Asthma Lung cancer [Pleural effusion] Pulmonary Hypertension Pulmonary Hypertension Etiology Secondary most common Lung disease Heart disease Thromboembolic disease Vascular disease Liver disease Primary When no other cause found 1

2 Pulmonary Hypertension Symptoms Dyspnea and fatigue Lightheadedness, syncope, angina Signs JVD, loud P 2, murmur of TR, RV gallop, parasternal lift CLEAR lungs Pulmonary Hypertension Diagnosis CXR CXR Enlarged proximal pulmonary vessels, distal pruning ECG RAD, RAE, RVH most common Echo Estimate PA pressure Assess for shunts and valvular disease; ventricular function Pulmonary Hypertension Further work up PFTs Identify obstructive/restrictive lung disease High Resolution CT (HRCT) Identify interstitial lung disease VQ scan/ct Angiogram R/O chronic PE 2

3 Pulmonary Hypertension Selected labs ANA, RF, ESR LFTs, hepatitis serologies HIV antibody Drugs cocaine, anorexigens Right Heart Catheterization When work up is negative Measure pressures, RV fxn, shunts Test responsiveness to vasodilators Pulmonary Hypertension Treatment Treat underlying cause Tobacco cessation Supplemental oxygen Diuretics +/- Digoxin Anticoagulation Vasodilators select select cases Pulmonary Embolism Pulmonary Embolism Risk factors general, hypercoag- ulability, stasis, vascular injury Risk factors Symptoms *dyspnea, *pleuritic chest pain, cough, hemoptysis Symptoms Signs *tachypnea, tachycardia, JVD, murmur, signs of DVT Signs 3

4 Pulmonary Embolism Diagnosis initial initial tests CXR Usually non Usually non-specific or normal ECG Sinus tachycardia, non-specific changes ABG PaO 2 D-DimersDimers PaCO 2 Strong neg. predictive value Pulmonary Embolism Diagnosis confirmatory tests CT Angio Reported Reported sensitivity and specificity vary widely Improving technology, but not perfect LE Doppler Assess for DVT VQ Scan Normal high sensitivity High prob high spec. But, >60% non diagnostic Combining tests improves accuracy Pulmonary Embolism Diagnosis Pulmonary Angiography Gold standard Pursue when suspicion remains high Echocardiogram Assess critically ill Identify RV dysfunction 4

5 Pulmonary Embolism Treatment Heparin Warfarin Thrombolysis Thrombectomy/endarterectomy Vena cava filters Pulmonary Embolism Prevention Minimize risk factors Early ambulation SQ heparin Pneumatic compression devices Interstitial Lung Disease Interstitial Lung Disease Heterogeneous group of disorders Common histological features Alveolar derangement Inflammation Fibrosis 5

6 Interstitial Lung Disease Categories Idiopathic fibrotic Connective tissue disease Primary Drug induced Occupational/environmental Interstitial Lung Disease Symptoms Dry cough, insidious dyspnea Fatigue, malaise Signs Bibasilar dry rales, clubbing, cyanosis Interstitial Lung Disease Diagnosis CXR Early Normal Diffuse interstitial or alveolar changes Advanced honeycomb pattern HRCT Most sensitive, specific, and helpful Can help guide biopsy 6

7 Interstitial Lung Disease Diagnosis ABG Hypoxemia, esp. with exercise PFTs Restrictive pattern TLC FVC DLCO Biopsy Open lung Bx often required Interstitial Lung Disease Treatment Avoid exposures Tobacco cessation Corticosteroids Immunosuppressive and cytotoxic therapy O 2 and Bronchodilators Sarcoidosis Sarcoidosis Systemic granulomatous inflammatory disease Unknown etiology genetic susceptibility + environmental trigger Most common AA women, age Affects both genders, all races and ages 7

8 Sarcoidosis Symptoms and Signs Dyspnea, cough, dry rales Fever, arthralgias, lymphadenopathy Various skin lesions Colic, uremia Blurred vision, photophobia, uveitis Unusual neurologic syndromes Palpitations, arrhythmias Sarcoidosis Diagnosis CXR Bilateral hilar adenopathy +/- diffuse infiltrates, fibrosis HRCT Ground glass, nodular opacities, cystic air spaces PFTs FVC DLCO Sarcoidosis Diagnosis ACE levels in 60% of cases, but non-specific Biopsy Biopsy the most accessible site Path non-caseating granulomas Helps exclude infection, neoplasm 8

9 Sarcoidosis Treatment Corticosteroids Reduces symptoms Does not modify course of disease Do not treat asymptomatic patients Steroid sparing agents Methotrexate, hydrochloroquine TNF α inhibitors Sarcoidosis Prognosis Variable Many remain asymptomatic Spont. resolution common Some slowly progress Overall mortality ~5% Some permanent organ damage common At least 2-4% of U.S. adults Repetitive episodes of upper airway obstruction during sleep O 2 desaturation and disrupted sleep 9

10 Risk Factors Obesity Male gender 3:1 Advancing age Craniofacial abnormalities Family history Alcohol, sedative hypnotics, tobacco Major Symptoms & Signs Excessive daytime sleepiness Impaired concentration Loud snoring Witnessed apneas, gasping Obesity Crowded pharynx *Neurocognitive Consequences* Excessive daytime sleepiness Decreased cognitive performance Increased automobile accidents Decreased quality of life Cardiovascular Consequences *Systemic hypertension* Possible associations Coronary artery disease Cerebrovascular disease Pulmonary hypertension Arrhythmias Increased mortality 10

11 Diagnosis Polysomnography ( sleep study ) Full night, gold standard Split night polysomnography Most common format Oximetry overnight Sensitive, but not specific Home monitoring systems Treatment Conservative Weight loss if obese Positioning lat. decubitus Sleep hygiene; avoid alcohol, sedatives, caffeine, tobacco Treatment Continuous Positive Airway Pressure (CPAP) Primary therapy, proven efficacy apnea, hypoxia, sleep disruption; cognitive function Poorly tolerated by many 11

12 Treatment Oral Appliances Helps snoring, little in apnea Patients prefer over CPAP Palatal implants Modestly effective for mild/mod OSA Surgery Many procedures employed Lack of adequate clinical trials Obstructive Lung Diseases Chronic Obstructive Lung Diseases Emphysema Chronic Bronchitis Asthma 12

13 COPD Diagnosis PFT Hallmarks FEV 1 /FVC < 70% (most sensitive) FEV 1 best measure of severity Greater DLCO with emphysema COPD Chronic Management Tobacco cessation! Bronchodilators Anti-cholinergics (e.g., ipratropium) ß 2 -Agonists short short or long-acting Theophylline Oxygen When PaO 2 < 55mmHg (SaO 2 < 88%) COPD Chronic Management Anti-inflammatory inflammatory agents Inhaled steroids not routine Consider trial with severe disease or frequent exacerbations Systemic steroids helpful for acute exacerbations only Avoid chronic use 13

14 COPD Acute exacerbations Antibiotics Small benefit when sputum Bronchodilators Anti-cholinergics and short-acting ß 2 -agonists Corticosteroids Short term systemic steroids Non-Invasive Pos. Press. Vent. Asthma Asthma Asthma is a chronic inflammatory disorder of the airways Bronchial edema Prominent bronchspasm Guidelines: Expert Panel Report 3 (EPR-3) Asthma EPR-3 NIH Classification Impairment Intermittent Persistent Mild Persistent Moderate Persistent Severe Symptoms 2x d/week > 2x d/week daily throughout day Nighttime awakenings 2x/month 3-4x/month > 1x/week, not nightly Short-acting β-agonist use 2x d/week >2x d/week, not >1x/d Interference with normal activity Lung function FEV 1 or PEF Risk Assessing Severity daily often 7x per week Several times daily none minor limitation some limitation extremely limited FEV 1 80% FEV 1 /FVC nl FEV 1 80% FEV 1 /FVC nl FEV 1 >60%, but <80%; FEV 1 /FVC 5% FEV 1 <60%; FEV 1 /FVC >5% Exacerbations requiring oral steroids 0-1 per year 2 per year 2 per year 2 per year 14

15 Asthma EPR-3 NIH Classification Impairment Well Controlled Not Well Controlled Very Poorly Controlled Symptoms 2x d/week > 2x d/week throughout the day Nighttime awakenings 2x/month 1-3x/week 4x/week Short-acting β-agonist use 2x d/week >2x d/week Several times daily Interference with normal activity none minor limitation extremely limited FEV 1 or PEF FEV 1 80% FEV 1 /FVC normal Risk Assessing Control and Adjusting Rx Exacerbations requiring oral steroids FEV 1 80% FEV 1 /FVC normal FEV 1 <60%; FEV 1 /FVC >5% 0-1 per year 2 per year 2 per year Asthma EPR-3 Guideline Highlights Assess severity/control Spirometry emphasized Stepwise approach Now 6 steps rather than 4 Preferred and Alternative Rx options ICS remain bedrock of Rx Regular f/u, reassessment Partner with patients action action plans Asthma Long-term Controllers Inhaled corticosteroids Leukotriene modifiers Long-acting ß 2 -agonists Cromolyn/nedocromil Methylxanthines Asthma Rescue meds Short-acting ß 2 agonists Systemic corticosteroids Severe exacerbations (ED) Anti-cholinergics Magnesium sulfate Helium-oxygen mixtures 15

16 Lung Cancer Lung cancer 80% Non-Small Cell very bad 20% Small Cell worse Non Small Cell Lung Cancer Various histological types Adeno, squamous, large cell Potential for surgical cure Non Small Cell Lung Cancer 14% cured Surgery offers best chance Stage I-IIII 86% die Late stage at diagnosis is typical 10% stage I and II; 50% stage IV Screening ineffective 16

17 Non Small Cell Lung Cancer Staging by TNM system Determines prognosis and treatment options Tumor size/location Assess nodes in mediastinum Search for metastasis brain, bone, liver Non Small Cell Lung Cancer Treatment modalities Surgery Chemotherapy Radiation therapy Small Cell Lung Cancer Systemic disease from outset Traditional staging system not used; surgery is not an option; grim prognosis Limited SCLC Disease limited to one radiation port Combination radiation and chemorx Extensive SCLC Disease spread beyond hemithorax Chemotherapy only 17