Evaluation of Suspected Pulmonary Hypertension

1. Evaluation of Suspected Pulmonary Hypertension Ron Workman CQC Presentation January 20th, 2008

2. Case Patient is a 44 year old male who presents to VA clinic with symptoms of increasing shortness of breath, chest heaviness, decreased exercise, tolerance and lower extremity swelling. He has no significant PMH or FMH, takes no medications at home, and denies tobacco use. An EKG was done in clinic showing RAE. An echocardiogram was done which revealed an elevated RVSP and right ventricular dilation.

3. Clinical Question What is the work up for a patient with suspected pulmonary hypertension?

4. Pulmonary Hypertension Overview Defined as mean PA pressure > 25mm Hg at rest or >30mm Hg with exertion Originally categorized by WHO in 1973 into just primary vs. secondary PH In 1998, WHO reclassified disease into 5 specific sub-categories based on specific disease process In 2003, categories slightly modified and updated to current Venice Classification System

5. WHO Classifications Pulmonary arterial hypertension (PAH) Idiopathic PAH (IPAH) Familial PAH (FPAH) Connective tissue diseases Congenital anomalies (i.e., shunts) Portopulmonary hypertension HIV infection Drugs/Medications

6. WHO Classifications (cont.) Pulmonary Venous Hypertension L sided heart/valvular disease Disorders of respiratory system/ chronic hypoxemia COPD OSA Interstitial lung disease Hypoventilation disorders Chronic pneumonia Other causes of hypoxemia (chronic altitude sickness)

7. WHO Classifications (cont.) Chronic thromboembolic disease Chronic PE (can affect proximal and distal PA) Sickle Cell Disease Infectious agents (ova/parasites, such as schistosomiasis) Other embolic diseases (tumor, foreign body, etc.) Miscellaneous causes Inflammatory conditions Langerhans Cell Histiocytosis Sarcoidosis Mechanical compression of pulmonary vasculature Tumor, foreign body, LAD Pulmonary capillary hemangiomatosis Pulmonary lymphangiomatosis

8. Initial EvaluationHistory & Physical HPI DOE most common, exercise intolerance, symptoms of RHF, sleep abnls, hoarseness, LE swelling (bilateral or unilateral) Past Medical/Surgical History Medications Anorectics, chemotherapy agents Family History Social/Occupational History Occupational exposure, tobacco/etoh/illicit drug use, sexual history Physical Exam Mostly localized to CV system Focus should be on identifying PE finding indicative for other possible causes

9. Initial Evaluation- Testing TTE w/ Doppler Preferred test for initial evaluation Estimates PASP using TV regurgitant flow Diagnostic test for L sided heart disease (Group 2 PH) Can detect presence of L->R shunts with “bubble” technique CXR Can see enlarged central pulmonary arteries, R heart enlargement Pruning of pulmonary arteries (PAH), interstitial infiltrates (ILD), hyperinflation (COPD) EKG Signs of PH such as RAE, RVH, RBBB, RV strain can be picked up on EKG

10. 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 RVH. Am Fam Physician. 2001 May 1;63(9):1789-98

11. Initial Evaluation- Testing Labwork ABG, CBC, BMP, LFTs, BNP, coags, thyroid panel, HIV/hepatitis serologies, autoimmune studies, antiphospholipid abs PFTs Assess for obstructive vs restrictive ventilatory deficits DLCO reduced in parenchymal disease, may be normal in chronic PE V/Q Scan Used to assess for chronic thromboembolic disease Normal test essentially rules out; positive test needs follow up with CT and/or pulmonary angiography

12. Initial Evaluation- Testing (cont.) Nocturnal Oximetry Screen for OSA Exercise Tolerance 6 minute walking test most commonly used Used to assign overall functional status classifications Also has prognostic significance

13. Further Initial Testing R heart catheterization Necessary if PAH is suspected Confirm PASP measurement Directly Measure CO and RAP Vasodilatory testing Recommended if PAH is suspected Decline in mean PAP > 10mm Hg and mean PAP < 40mm Hg Not recommended in extreme RHF Start with short-acting vasodilator (IV adenosine, inhaled NO) L heart catheterization Only required if L heart disease cannot be excluded

14. Additional Testing CT chest CT angiogram for non-invasive assessment of chronic thromboembolic disease High resolution CT for interstitial or bronchial disease Pulmonary arteriogram Definitive diagnosis of CTEPH Provides direct measurement of PA and RA pressures Polysomnography If nocturnal oximetry is positive 20-27% with OSA have mild PH, usually reversible w/ CPAP TEE Less accurate for PASP estimation More useful in detection of intracardiac shunts Can also detect PE in central pulmonary arteries

15. Additional Testing (cont.) Radionucleotide Ventriculography Non-invasive RV and LV functional assessment MRI Chest Investigation of cardiac anomalies; may be useful for longitudinal assessment of RV Cardiopulmonary Exercise Test Assess cardiopulmonary function with exercise; PH exhibits classic abnormalities Lung biopsy Only done if diagnosis requires histologic confirmation

17. Conclusion Pulmonary hypertension is a complex problem with many different, and often coexisting, etiologies. Taking an algorithmic approach to work up, guided by complete history and physical exam, can provide your patient with a thorough, yet cost effective, approach to diagnosis.

18. Sources Robyn J. Barst MD, et al., Diagnosis and differential assessment of pulmonary arterial hypertension. JACC vol. 43, June 2004, pp. S40-S47 Nauser TD, Stites SW, Diagnosis and treatment of pulmonary hypertension. Am Fam Physician. 2001 May 1;63(9):1789-98. Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D., Pulmonary Arterial Hypertension. N Engl J Med 2004;351:1655-65. Simonneau G, Galie N, Rubin LJ, et al., Clinical classification of pulmonary hypertension. JACC, 2004 Jun 16;43(12 Suppl S):5S-12S. Figure from Up to Date article Diagnostic evaluation of pulmonary hypertension, www.utdol.com