II. Diagnosis: Step 1
- Tests
- Electrocardiogram (EKG)
- See Right Ventricular Strain EKG Pattern
- Right Axis Deviation
- Right Ventricular Hypertrophy
- Right atrial enlargement
- Right strain pattern (S1Q3T3)
- Sinus Tachycardia
- Atrial Fibrillation
- Anterior T Wave Inversion (leads V1-4)
- Chest XRay (abnormal in 90% of Pulmonary Arterial Hypertension)
- Primary pulmonary artery dilation
- Increased distal pulmonary vasculature markings
- Right atrial and ventricular enlargement
- Right Interlobar pulmonary diameter >16 mm
- Hilar to thoracic ratio >0.44
- Electrocardiogram (EKG)
- Interpretation: Pulmonary Hypertension suggested
- Proceed to Step 2 below
III. Diagnosis: Step 2
- Tests: Transthoracic Echocardiogram (most useful)
- Early findings
- Tricuspid regurgitation
- Peak pulmonary pressure estimated (Bernoulli equation)
- Mean Right Atrial Pressure added to Peak tricuspid jet velocity
- Systolic pulmonary artery pressure >35 to 40 mmHg consistent with Pulmonary Hypertension
- Late findings
- Right ventricular dilation
- Right Ventricular Hypertrophy
- Displaced interventricular septum
- Dilated main pulmonary artery
- Early findings
- Interpretation
- Precautions
- Peak pulmonary pressure has poor accuracy and is operator dependent
- Interpretation is best based on a general gestalt that there is high right sided heart pressure
- Findings not consistent with Pulmonary Hypertension
- No further evaluation needed
- Classification based on Echocardiogram findings
- Left ventricular disease
- Right Ventricular Failure with elevated pulmonary artery pressure
- Right Ventricular Failure without elevated pulmonary artery pressure
- Pericardial disease (Cardiac Tamponade)
- Findings consistent with Pulmonary Hypertension
- No structural heart disease suspected
- Follow Step 3 below
- Structural heart disease suspected
- Cardiac Catheterization (Gold standard)
- No structural heart disease suspected
- Precautions
IV. Diagnosis: Step 3
- Basic Tests (indicated in all cases)
- Complete Blood Count (CBC)
- Evaluate for Anemia (high output Heart Failure)
- Comprehensive Metabolic Panel (Electrolytes, Renal Function tests, Liver Function Tests)
- B-Type Natriuretic Peptide (BNP)
- Serum Troponin
- HIV Test
- Thyroid Stimulating Hormone (TSH)
- Evaluate for Hyperthyroidism (high output Heart Failure)
- Arterial Blood Gas (ABG) or Venous Blood Gas
- Supplemental Oxygen for all hypoxic patients
- Oximetry (6 Minute Walk Test)
- Pulmonary Function Tests
- Coagulation studies (PTT, INR)
- Preparation for Anticoagulation
- Complete Blood Count (CBC)
- Additional Tests as indicated
- Sleep Study
- Sickle Cell Anemia screening
- Imaging
- CT Chest
- Liver Ultrasound
- Autoimmune panel
- Anticentromere Antibody level
- Antinuclear Antibody level
- Anti-Scl-70 Antibody level
- Ribonucleoprotein Antibody level
- Interpretation of Pulmonary Function Tests (PFT)
- Severe Chronic Obstructive Lung Disease (COPD)
- See COPD Management
- Nearly normal PFT
- Go to Step 4 below
- Severe Restrictive Lung Disease
- Go to Step 5 below
- Severe Chronic Obstructive Lung Disease (COPD)
V. Diagnosis: Step 4 (Normal PFT Evaluation)
-
Ventilation Perfusion Scan (V-Q Scan)
- Exclude chronic or recurrent Pulmonary Embolism
- Preferred over CT due to higher sensitivity for smaller multiple distal emboli
- Interpretation of V-Q Scan
- No segmental defects
- Follow Step 5 below
- One or more segmental defects
- Obtain Pulmonary Angiogram
- Pulmonary Angiogram shows no proximal embolism
- Follow Step 5 below
- Pulmonary angiogram shows proximal embolism
- Treat Pulmonary Embolism
- No segmental defects
VI. Diagnosis: Step 5 (Restrictive Lung Disease Evaluation)
- Test: High resolution CT Chest
- Exclude Interstitial Lung Disease
- Exclude mediastinal fibrosis
- Interpretation
- Normal or mediastinal fibrosis
- Cardiac catheterization
- Interstitial Lung Disease
- Lung biopsy for tissue diagnosis
- Normal or mediastinal fibrosis