Pulmonary Hypertension

1. See Also
   1. Pulmonary Hypertension
   2. Pulmonary Hypertension Diagnosis
   3. Cor Pulmonale
2. Definitions
   1. Pulmonary Hypertension
      1. Pulmonary artery systolic pressure >30 mmHg
      2. Pulmonary artery mean pressure >20 mmHg
   2. 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. Second to respiratory cause of Pulmonary Hypertension
3. Epidemiology: Primary Pulmonary Hypertension
   1. Incidence: 1-2 cases per 1 million
   2. Female predominance after Puberty in ratio of 1.7 to 1
   3. Most prevalent in ages 20 to 40 years old
4. Pathophysiology: Genetic associations
   1. BMPR2 gene
   2. ALK1 gene
   3. Endoglin (with or without Hereditary Hemorrhagic Telangiectasia)
5. Types (replaces old system of primary versus secondary Pulmonary Hypertension)
   1. Pulmonary artery Hypertension
      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
      1. Left-sided valvular heart disease
      2. Left-sided atrial or ventricular heart disease
   3. Pulmonary Hypertension associated with lung disease, Hypoxemia or both
      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
      1. Thromboembolism of proximal or distal pulmonary arteries
      2. Thromboembolism not due to thrombi (e.g. tumor, parasites)
   5. Miscellaneous Pulmonary Hypertension
      1. Sarcoidosis
      2. Pulmonary vessel compression
6. Causes
   1. See Pulmonary Hypertension Causes
7. 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
8. Symptoms
   1. Common
      1. Progressive Dyspnea on exertion
      2. Fatigue
      3. Syncope
   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%)
9. Signs
   1. Jugular Vein distention
   2. Prominent right ventricular impulse
   3. 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
10. Diagnosis
    1. See Pulmonary Hypertension Diagnosis
    2. Mean Pulmonary Artery Pressure (normally <15 mmHg)
       1. Rest: 25 mmHg or higher
       2. Exercise: 30 mmHg or higher
11. Labs
    1. See Pulmonary Hypertension Diagnosis
12. Management
    1. See Pulmonary Hypertension Management
13. Management: 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
14. 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
15. Prognosis: Primary Pulmonary Hypertension
    1. Median survival from diagnosis
       1. Prior medications: 2.8 years
       2. New medications: may exceed 10 years
16. 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