Pulmonary hypertension classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Richard Channick, M.D.; Assistant Editor(s)-in-Chief: Ralph Matar, Lisa Prior, Ann Slater, R.N.

Overview

Pulmonary hypertension has been previously divided into two categories: primary (currently known as idiopathic pulmonary arterial hypertension [IPAH]) and secondary. However; given the fact that some subcategories of secondary pulmonary hypertension share several similarities with primary pulmonary hypertension in terms of pathology, progression and response to therapy, the WHO (World Health Organization) has based its reclassification of pulmonary hypertension on the mechanism of the disease.

Classification

Updated Clinical Classification of Pulmonary Hypertension

During the World Symposium on Pulmonary Hypertension (WSPH), five groups of disorders that cause pulmonary hypertension were identified: pulmonary arterial hypertension (Group 1); pulmonary hypertension due to left heart disease (Group 2); pulmonary hypertension due to chronic lung disease and/or hypoxia (Group 3); chronic thromboembolic pulmonary hypertension (Group 4); and pulmonary hypertension due to unclear multifactorial mechanisms (Group 5). This classification has been adopted by the Guidelines Committee of the European Society of Cardiology (ESC), European Respiratory Society (ERS), and International Society of Heart and Lung Transplantation (ISHLT), and is currently used by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the labelling of new drugs approved for pulmonary hypertension.

• 1. Pulmonary arterial hypertension (PAH)
  • 1.1. Idiopathic PAH
  • 1.2. Heritable PAH
    • 1.2.1 BMPR2
    • 1.2.2 ALK-1, ENG, SMAD9, CAV1, KCNK3
    • 1.2.3 Unknown
  • 1.3 Drug and toxin induced
  • 1.4 Associated with:
    • 1.4.1 Connective tissue disease
    • 1.4.2 HIV infection
    • 1.4.3 Portal hypertension
    • 1.4.4 Congenital heart diseases
    • 1.4.5 Schistosomiasis
• 1’ Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis
• 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 diseases 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 lung diseases

• 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, lymphangioleiomyomatosis
  • 5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
  • 5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH

Other Classification

The Venice 2003 Revised Classification System

In 2003, the 3rd World Symposium on Pulmonary Arterial Hypertension was convened in Venice to modify the classification based on the new understanding of disease mechanisms. The revised system developed by this group provides the current framework for understanding pulmonary hypertension.

The system includes several improvements over the former 1998 Evian Classification system. Risk factor descriptions were updated, and the classification of congenital systemic-to pulmonary shunts was revised. A new classification of genetic factors in PH was recommended, but not implemented because available data were judged to be inadequate.

The Venice 2003 Revised Classification system can be summarized as follows:

• WHO Group I - Pulmonary arterial hypertension (PAH)
• WHO Group II - Pulmonary hypertension associated with left heart disease
• WHO Group III - Pulmonary hypertension associated with lung diseases and/or hypoxemia
• WHO Group IV - Pulmonary hypertension due to chronic thrombotic and/or embolic disease
• WHO Group V - Miscellaneous

Venice Clinical Classification of Pulmonary Hypertension (2003)

• 1. Pulmonary arterial hypertension (PAH)
  • 1.1. Idiopathic (IPAH)
  • 1.2. Familial (FPAH)
  • 1.3. Associated with (APAH)
    • 1.3.1. Collagen vascular disease
    • 1.3.2. Congenital systemic-to-pulmonary shunts
    • 1.3.3. Portal hypertension
    • 1.3.4. HIV infection
    • 1.3.5. Drugs and toxins
    • 1.3.6. Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy)
  • 1.4. Associated with significant venous or capillary involvement
    • 1.4.1. Pulmonary veno-occlusive disease (PVOD)
    • 1.4.2. Pulmonary capillary hemangiomatosis (PCH)
  • 1.5. Persistent pulmonary hypertension of the newborn
• 2. Pulmonary hypertension with left heart disease
  • 2.1. Left-sided atrial or ventricular heart disease
  • 2.2. Left-sided valvular heart disease
• 3. Pulmonary hypertension associated with lung diseases and/or hypoxemia
  • 3.1. Chronic obstructive pulmonary disease
  • 3.2. Interstitial lung disease
  • 3.3. Sleep-disordered breathing
  • 3.4. Alveolar hypoventilation disorders
  • 3.5. Chronic exposure to high altitude
  • 3.6. Developmental abnormalities
• 4. Pulmonary hypertension owing to chronic thrombotic and/or embolic disease
  • 4.1. Thromboembolic obstruction of proximal pulmonary arteries
  • 4.2. Thromboembolic obstruction of distal pulmonary arteries
  • 4.3. Nonthrombotic pulmonary embolism (tumor, parasites, foreign material)
• 5. Miscellaneous

Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis