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Risk Factors and Associated Conditions for Pulmonary Hypertension

The exact mechanism by which the risk factors produce PPH has not been established. Given the fact that the absolute risk is generally low, factors of individual susceptibility are likely to play an important role.

The following risk factors have been categorized by the WHO conference (Evian 1998) based on the strength of the association with PPH and their probable causal role. "Definite" indicates an association based on several concordant observations, including a major controlled study or a clear epidemic. Definite  risk factors are considered to play a causal role in the development of the disease. "Very likely" indicates several concordant observations (including large case series and studies) that are not attributable to considered biases, or a general consensus among experts. "Possible" indicates as association based on case series, registries, or expert opinions. "Unlikely" indicates risk factors that have been proposed but have not been found to have any association from controlled studies.

A. Drugs and Toxins
    1. Definite
        Aminorex
        Fenfluramine
        Dexfenfluramine
        Toxic Rapeseed Oil

    2. Very Likely
        Amphetamines
        L-tryptophan

    3. Possible
        Meta-amphetamines
        Cocaine
        Chemotherapeutic Agents

    4. Unlikely
        Antidepressants
        Oral Contraceptives
        Estrogen Therapy
        Cigarette Smoking

B. Demographic and Medical Conditions
    1. Definite
        Gender

    2. Possible
        Pregnancy
        Systemic Hypertension

    3. Unlikely
        Obesity

C. Diseases
    1. Definite
        HIV Infection

    2. Very likely
        Portal Hypertension / Liver Disease
        Collagen Vascular Diseases
        Congenital Systemic-Pulmonary Cardiac Shunts

    3. Possible
        Thyroid disorders

Source: Rich S (Editor): Primary pulmonary hypertension. Executive Summary from the World Symposium on Primary Pulmonary Hypertension 1998: www.who.int/en