Learn what genetics can teach us about PAH
In associated pulmonary arterial hypertension, the PAH is associated with another disease or condition. PAH is commonly associated with connective tissue diseases (such as scleroderma, CREST syndrome and lupus), congenital heart disease, chronic liver disease, HIV, drugs and toxins, and more.
There are some other types of pulmonary arterial hypertension, but they aren’t quite as common.
Other Types of Pulmonary Hypertension
Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by old blood clots in the lungs (pulmonary embolism). Learn more about CTEPH
Explore the classifications of PH below for more information about the other types of PH.
Classifications of Pulmonary Hypertension
The World Health Organization (WHO) first defined the classifications of pulmonary hypertension in 1973 and the classifications have been revised over the years. The classifications of PH were most recently updated in Nice, France, in 2013.
Learn more about the classifications in the Pathlight article, “What’s Your PH? Understanding the Five PH WHO Groups,” written by Dr. John Kingrey of the Oklahoma Heart Hospital.
Updated clinical classification of PH (Nice 2013)
1. Pulmonary arterial hypertension (PAH)
1.2.2. ALK1, ENG, SMAD9, CAV1, KCNK3
1.3. Drug- and toxin-induced
1.4. Associated with:
1.4.1. Connective tissue diseases
1.4.2. HIV infection
1.4.3. Portal Hypertension
1.4.4. Congenital heart diseases
1’. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)
1’’. Persistent pulmonary hypertension of the newborn (PPHN)
2. Pulmonary hypertension due to left heart disease
2.1. Left ventricular systolic dysfunction
2.2. Left ventricular diastolic dysfunction
2.3. Valvular disease
2.4. Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
3. Pulmonary hypertension due to lung disease and/or hypoxia
3.1. Chronic obstructive pulmonary disease
3.2. Interstitial lung disease
3.3. Other pulmonary diseases with mixed restrictive and obstructive pattern
3.4. Sleep-disordered breathing
3.5. Alveolar hypoventilation disorders
3.6. Chronic exposure to high altitude
3.7. Developmental abnormalities
4. Chronic thromboembolic pulmonary hypertension (CTEPH)
5. Pulmonary hypertension with unclear multifactorial mechanisms
5.1. Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy
5.2. Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleimoyomatosis
5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
5.4. Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis, segmental PH