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Introduction
- A syndrome resulting from restricted flow through the pulmonary arterial circulation resulting in increased pulmonary vascular resistance & remodeling, & ultimately leading to right heart failure
Definition
- Pulmonary arterial hypertension (PAH) is part of the spectrum of pulmonary hypertension (PH), which is a hemodynamic & pathophysiological condition defined as an increase in mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest
Signs and Symptoms
Typical Symptoms
- Progressive dyspnea on exertion, palpitations
- Fatigue, weakness, angina, syncope, abdominal distention
- Less commonly: Hemoptysis, Raynaud’s phenomenon
Risk Factors
| Risk Factors & Associated Conditions for Pulmonary Arterial Hypertension (PAH) | ||||
|---|---|---|---|---|
| Definite Causal Relationship | Likely Causal Relationship | Possible Causal Relationship | Unlikely Causal Relationship | |
| Drugs & Toxins | Aminorex Fenfluramine Dexfenfluramine Toxic grapeseed oil Benfluorex Selective serotonin reuptake inhibitor |
Amphetamines Dasatinib L-tryptophan Methamphetamine |
Amphetamine-like drugs Cocaine Phenylpropanolamine Chemotherapeutic agents (Mytomycin C, Cyclophosphamide) Interferon α & β St. John's Wort |
Oral contraceptives Estrogen therapy Cigarette smoking |
| Demographic Factors | Gender | Pregnancy Systemic hypertension |
Obesity | |
| Medical Conditions | HIV infection
Severe symptomatic mitral or aortic valve disease |
Portal hypertension or liver disease Collagen vascular diseases Congenital systemic-to- pumonary cardiac shunts |
Thyroid disorders
Hematological conditions (eg asplenia 2° to surgical splenectomy, sickle cell disease, β-thalassemia, chronic myeloproliferative disorders) Rare genetic or metabolic diseases (eg Type 1a glycogen storage disease, Gaucher's disease, hereditary hemorrhagic telangiectasia) |
|
