II. Diagnosis: Step 1

  1. Tests
    1. Electrocardiogram (EKG)
      1. See Right Ventricular Strain EKG Pattern
      2. Right Axis Deviation
      3. Right Ventricular Hypertrophy
      4. Right atrial enlargement
      5. Right strain pattern (S1Q3T3)
      6. Sinus Tachycardia
      7. Atrial Fibrillation
      8. Anterior T Wave Inversion (leads V1-4)
    2. Chest XRay (abnormal in 90% of Pulmonary Arterial Hypertension)
      1. Primary pulmonary artery dilation
      2. Increased distal pulmonary vasculature markings
      3. Right atrial and ventricular enlargement
      4. Right Interlobar pulmonary diameter >16 mm
      5. Hilar to thoracic ratio >0.44
  2. Interpretation: Pulmonary Hypertension suggested
    1. Proceed to Step 2 below

III. Diagnosis: Step 2

  1. Tests: Transthoracic Echocardiogram (most useful)
    1. Early findings
      1. Tricuspid regurgitation
      2. Peak pulmonary pressure estimated (Bernoulli equation)
        1. Mean Right Atrial Pressure added to Peak tricuspid jet velocity
        2. Systolic pulmonary artery pressure >35 to 40 mmHg consistent with Pulmonary Hypertension
    2. Late findings
      1. Right ventricular dilation
      2. Right Ventricular Hypertrophy
      3. Displaced interventricular septum
      4. Dilated main pulmonary artery
  2. Interpretation
    1. Precautions
      1. Peak pulmonary pressure has poor accuracy and is operator dependent
      2. Interpretation is best based on a general gestalt that there is high right sided heart pressure
    2. Findings not consistent with Pulmonary Hypertension
      1. No further evaluation needed
    3. Classification based on Echocardiogram findings
      1. Left ventricular disease
      2. Right ventricular failure with elevated pulmonary artery pressure
      3. Right ventricular failure without elevated pulmonary artery pressure
      4. Pericardial disease (Cardiac Tamponade)
    4. Findings consistent with Pulmonary Hypertension
      1. No structural heart disease suspected
        1. Follow Step 3 below
      2. Structural heart disease suspected
        1. Cardiac Catheterization (Gold standard)

IV. Diagnosis: Step 3

  1. Basic Tests (indicated in all cases)
    1. Complete Blood Count (CBC)
      1. Evaluate for Anemia (high output Heart Failure)
    2. Comprehensive Metabolic Panel (electrolytes, Renal Function tests, Liver Function Tests)
    3. B-Type Natriuretic Peptide (BNP)
    4. Serum Troponin
    5. HIV Test
    6. Thyroid Stimulating Hormone (TSH)
      1. Evaluate for Hyperthyroidism (high output Heart Failure)
    7. Arterial Blood Gas (ABG) or Venous Blood Gas
      1. Supplemental Oxygen for all hypoxic patients
    8. Oximetry (6 Minute Walk Test)
    9. Pulmonary Function Tests
    10. Coagulation studies (PTT, INR)
      1. Preparation for Anticoagulation
  2. Additional Tests as indicated
    1. Sleep Study
    2. Sickle Cell Anemia screening
    3. Imaging
      1. CT Chest
      2. Liver Ultrasound
    4. Autoimmune panel
      1. Anticentromere Antibody level
      2. Antinuclear Antibody level
      3. Anti-Scl-70 Antibody level
      4. Ribonucleoprotein Antibody level
  3. Interpretation of Pulmonary Function Tests (PFT)
    1. Severe Chronic Obstructive Lung Disease (COPD)
      1. See COPD Management
    2. Nearly normal PFT
      1. Go to Step 4 below
    3. Severe Restrictive Lung Disease
      1. Go to Step 5 below

V. Diagnosis: Step 4 (Normal PFT Evaluation)

  1. Ventilation Perfusion Scan (V-Q Scan)
    1. Exclude chronic or recurrent Pulmonary Embolism
    2. Preferred over CT due to higher sensitivity for smaller multiple distal emboli
  2. Interpretation of V-Q Scan
    1. No segmental defects
      1. Follow Step 5 below
    2. One or more segmental defects
      1. Obtain Pulmonary Angiogram
      2. Pulmonary Angiogram shows no proximal embolism
        1. Follow Step 5 below
      3. Pulmonary angiogram shows proximal embolism
        1. Treat Pulmonary Embolism

VI. Diagnosis: Step 5 (Restrictive Lung Disease Evaluation)

  1. Test: High resolution CT Chest
    1. Exclude Interstitial Lung Disease
    2. Exclude mediastinal fibrosis
  2. Interpretation
    1. Normal or mediastinal fibrosis
      1. Cardiac catheterization
    2. Interstitial Lung Disease
      1. Lung biopsy for tissue diagnosis

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