Pulmonary Hypertension

1. See Also
   1. Pulmonary Arterial Hypertension
   2. Pulmonary Hypertension Causes
   3. Pulmonary Hypertension Diagnosis
   4. Pulmonary Hypertension Management
   5. Cor Pulmonale
2. Definitions
   1. Pulmonary Hypertension
      1. Pulmonary artery systolic pressure >30 mmHg
      2. Pulmonary artery mean pressure >20 mmHg
   2. Pulmonary Arterial Hypertension (Previously known as Primary Pulmonary Hypertension)
      1. Idiopathic Pulmonary Hypertension
   3. Secondary Pulmonary Hypertension
      1. Secondary to one of Pulmonary Hypertension Causes
   4. Cor Pulmonale
      1. Right ventricular failure
      2. Secondary to respiratory cause of Pulmonary Hypertension
3. Types (replaces old system of primary versus secondary Pulmonary Hypertension)
   1. Pulmonary artery Hypertension (WHO Group 1)
      1. Idiopathic or familial
      2. Includes persistent Pulmonary Hypertension of the newborn
      3. Collagen vascular disease
      4. HIV Infection
   2. Pulmonary Hypertension associated with left heart disease (WHO Group 2)
      1. Left-sided valvular heart disease
      2. Left-sided atrial or ventricular heart disease
   3. Pulmonary Hypertension associated with lung disease, Hypoxemia or both (WHO Group 3)
      1. Chronic Obstructive Lung Disease
      2. Interstitial Lung Disease
      3. Sleep Apnea
      4. Chronic high altitude exposure
   4. Pulmonary Hypertension associated with chronic thromboembolic disease (WHO Group 4)
      1. Thromboembolism of proximal or distal pulmonary arteries
      2. Thromboembolism not due to thrombi (e.g. tumor, parasites)
   5. Miscellaneous Pulmonary Hypertension (WHO Group 5)
      1. Sarcoidosis
      2. Pulmonary vessel compression
4. Causes
   1. See Pulmonary Hypertension Causes
5. Associated Conditions
   1. Chronic Hemolytic Anemia
   2. Congenital Heart Disease
   3. Connective tissue disease
   4. Human Immunodeficiency Virus (HIV Infection)
   5. Portal Hypertension
   6. Persistent Pulmonary Hypertension of the newborn
6. Symptoms
   1. Common
      1. Progressive Dyspnea on exertion
      2. Fatigue
      3. Syncope (especially Syncope on exertion)
   2. Less Common
      1. Hoarseness (Oertner Syndrome)
         1. Pulmonary artery compress left recurrent laryngeal
      2. Angina-type exertional Chest Pain
   3. Rare
      1. Cough
      2. Hemoptysis
      3. Raynaud's Phenomenon (2%)
7. Signs
   1. Jugular Vein distention
   2. Prominent right ventricular impulse
   3. Fixed Split S2 Heart Sound
      1. Accentuated second pulmonic valve component (P2)
         1. Louder than the aortic second sound (A2)
      2. A2 remains louder as stethoscope moved to apex
   4. Right Ventricular Fourth Heart Sound (S4 Heart Sound)
   5. Right-sided Third Heart Sound (S3 Heart Sound)
      1. Indicates advanced disease
      2. Associated with poor prognosis
   6. Tricuspid insufficiency murmur
      1. More prominent as right ventricle dilates
   7. Hepatomegaly
   8. Peripheral edema
8. Diagnosis
   1. See Pulmonary Hypertension Diagnosis
   2. Mean Pulmonary Artery Pressure (PAP)
      1. Normal: <15 mmHg
      2. Pulmonary Hypertension
         1. Rest: 25 mmHg or higher
         2. Exercise: 30 mmHg or higher
   3. Pulmonary capillary wedge pressure (PCWP)
      1. PCWP <15 mmHg: Pre-capillary Pulmonary Hypertension
         1. All Causes of Pulmonary Hypertension EXCEPT those due to left heart disease (WHO Groups 1,3,4,5)
      2. PCWP >15 mmHg: Post-capillary Pulmonary Hypertension
         1. Left heart disease related causes (WHO Group 2 Pulmonary Hypertension)
9. Diagnostics
   1. See Pulmonary Hypertension Diagnosis
   2. Electrocardiogram (EKG)
      1. Right Ventricular Hypertrophy
   3. Evaluate WHO functional class status
      1. Six minute walk test
10. Evaluation: Screening of high risk groups
    1. Protocol
       1. Annual Echocardiogram
       2. Reflex to right heart catheterization if positve Echocardiogram for pulmonary artery Hypertension
    2. Indications
       1. BMPR2 gene positive (screen first degree relatives for gene)
       2. HIV Infection
       3. Portal Hypertension (if considering liver transplantation)
       4. Prior appetite suppressant medication such as Fenfluramine if symptoms
       5. Sickle Cell Disease
       6. Systemic Sclerosis
       7. Congenital Heart Disease with shunt
       8. Recent Acute Pulmonary Embolism with persistent symptoms at 3 months
          1. Consider ventilation-perfusion scan with reflex to Pulmonary Angiography if positive
11. Management
    1. See Pulmonary Hypertension Management
12. Complications: Right Ventricular Failure
    1. Secondary to persistent Pulmonary Hypertension
    2. Cor Pulmonale: Subtype of right ventricular failure
       1. Second to respiratory cause of Pulmonary Hypertension
13. References
    1. Gaine (2000) JAMA 284:3160-8
    2. Nauser (2001) Am Fam Physician 63(9):1789-98
    3. Rubin (1997) N Engl J Med 336:111-7
    4. Rubin (1993) Chest 104:236-50
    5. McLaughlin (2009) Circulation 119(16): 2250-94
    6. Stringham (2010) Am Fam Physician 82(4): 370-7