Pulmonary Hypertension


Etiology (5th World Symposium Classification; Nice, France 2013)

Group 1: Pulmonary Arterial Hypertension

1.1: Idiopathic Pulmonary Arterial Hypertension (IPAH) (see Idiopathic Pulmonary Arterial Hypertension)

1.2: Heritable Pulmonary Arterial Hypertension

1.3: Drug and Toxin-Induced: Definite Association

1.3: Drug and Toxin-Induced: Likely Association

1.3: Drug and Toxin-Induced: Possible Association

1.3: Drug and Toxin-Induced: Unlikely Association

1.4: Associated Pulmonary Arterial Hypertension (APAH)

1′: Other

1”: Other

Group 2: Pulmonary Arterial Hypertension Due to Left Heart Disease

Group 3: Pulmonary Arterial Hypertension Due to Lung Disease and/or Hypoxemia

3.1: Chronic Obstructive Pulmonary Disease (COPD) and Obstructive Lung Diseases

3.2: Interstitial Lung Disease (ILD)

3.3: Other Pulmonary Disease with Mixed Restriction-Obstruction

3.4: Sleep-Disordered Breathing

3.5: Respiratory Failure Due to Chronic Hypoventilation (see Respiratory Failure)

3.6: Chronic High Altitude

3.7: Developmental Lung Diseases

Group 4: Pulmonary Arterial Hypertension Due to Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Other Pulmonary Artery Obstruction

Group 5: Pulmonary Arterial Hypertension Due to Unclear Multifactorial Mechanisms

5.1: Hematologic Disorders

5.2: Systemic Disorders

5.3: Metabolic Disorders

5.4: Other

Other Disorders Not Included in Classification


Normal Pulmonary Artery Pressure

Hypoxic Pulmonary Vasoconstriction


Acute Hypoxic Pulmonary Vasoconstriction

Chronic Hypoxic Vasoconstriction

Pathophysiology of Pulmonary Hypertension by Group


Chest X-Ray (see Chest X-Ray)

Clinical Findings

Electrocardiogram (EKG) (see Electrocardiogram)

Clinical Findings

Arterial Blood Gas (ABG) (see Arterial Blood Gas)

Serum Brain Natriuretic Peptide (BNP) (see Serum Brain Natriuretic Peptide)

Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests)

Echocardiogram (see Echocardiogram)

Clinical Utility

Clinical Findings

Clinical Efficacy

Ventilation/Perfusion (V/Q) Scan (see Ventilation-Perfusion Scan)

Computed Tomography (CT) Pulmonary Angiogram (see Computed Tomography Pulmonary Angiogram)

Clinical Efficacy

Pulmonary Artery Magnetic Resonance Imaging (MRI) (see Magnetic Resonance Imaging)

Swan-Ganz (Pulmonary Artery) Catheterization (see Swan-Ganz Catheter)

General Comments

Pulmonary Hypertension with Elevated Pulmonary Capillary Wedge Pressure (PCWP)

Pulmonary Hypertension with Normal Pulmonary Capillary Wedge Pressure (PCWP)

Vasodilator (Vasoreactivity) Testing

Pulmonary Artery Angiogram (see Pulmonary Artery Angiogram)

Lower Extremity Venous Doppler Ultrasound (see Lower Extremity Venous Doppler Ultrasound)

6-Minute Walk Test (see 6-Minute Walk Test)

Clinical Classification

World Health Organization (WHO) Functional Classification of Pulmonary Hypertension

Clinical Manifestations

General Comments

Cardiovascular Manifestations

Gastrointestinal Manifestations

Neurologic Manifestations

Otolaryngologic Manifestations

Pulmonary Manifestations

Rheumatologic Manifestations

Reproductive Manifestations

Increased Risk of Death with Pregnancy (see Pregnancy)

Increased Risk of Small-for-Gestational Age Infants

Increased Risk of Congenital Anomalies

Increased Risk of Death with Mechanical Ventilation, General Anesthesia, and Surgical Procedures


General Measures

Recommended Therapies by WHO Functional Class (Chest 2014 Guidelines for Pharmacologic Therapy of Pulmonary Hypertension in Adults) (Chest, 2014) [MEDLINE]

WHO Functional Class II

WHO Functional Class III

WHO Functional Class IV

WHO Functional Class III or IV with Unacceptable Clinical Status Despite Established Pulmonary Hypertension-Specific Monotherapy

Calcium Channel Blockers (see Calcium Channel Blockers)

Prostacyclin Analogues (Prostanoids)

General Comments

Epoprostenol (PGI2, Prostacyclin, Flolan, Veletri) (see Epoprostenol)

Inhaled Iloprost (Ilomedin, Ventavis) (see Iloprost)

Treprostinil (Remodulin, Tyvaso) (see Treprostinil)

Endothelin Receptor Antagonists (see Endothelin Receptor Antagonists)

General Comments

Ambrisentan (Letairis) (see Ambrisentan)

Bosentan (Tracleer) (see Bosentan)

Macitentan (Opsumit) (see Macitentan)

Phosphodiesterase Type 5 Inhibitors (see Phosphodiesterase Type 5 Inhibitors)

General Comments

Sildenafil (Viagra, Revatio) (see Sildenafil)

Tadalafil (Adcirca, Cialis) (see Tadalafil)

Riociguat (Adempas) (see Riociguat)


Inhaled Nitric Oxide (iNO) (see Nitric Oxide)

Lung Transplantation (see Lung Transplant)

Specific Management of Pulmonary Hypertension During Pregnancy (see Pregnancy)

Specific Management of Hypotension Associated with Right Ventricular Failure

Strategies to Decrease Pulmonary Artery Pressures and/or Decrease RV Afterload

Clinical Efficacy


Overall Mortality Rate for Pulmonary Hypertension

Group 1 Pulmonary Hypertension

Groups 2-5 Pulmonary Hypertension