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Pulmonary%20arterial%20hypertension Signs and Symptoms
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
- Precapillary PH defined as an increase in mean pulmonary arterial pressure (mPAP) >20 mmHg, pulmonary arterial wedge pressure (PAWP) of ≤15 mmHg, & pulmonary vascular resistance (PVR) of ≥3 Wood Units (WU), with all measurements taken at rest
- Isolated post-capillary PH is defined as mPAP >20 mmHg, PAWP >15 mmHg & PVR <3 WU
- Combined pre- & post-capillary PH is defined as mPAP >20 mmHg, PAWP >15 mmHg & PVR ≥3 WU
Signs and Symptoms
Typical Symptoms
- Progressive dyspnea on exertion, palpitations
- Fatigue, weakness, angina, syncope, abdominal distention
- Less commonly: Hemoptysis, Raynaud’s phenomenon
Risk Factors and Associated Conditions for Pulmonary Arterial Hypertension
Drugs & Toxins
- Definite association: Aminorex, Fenfluramine, Dexfenfluramine, Benfluorex, methamphetamines, Dasatinib, selective serotonin reuptake inhibitors, toxic grapeseed oil
- Possible association: Cocaine, Phenylpropanolamine, L-tryptophan, chemotherapeutic agents (Mitomycin C, Cyclophosphamide), Interferon α & β, St. John’s Wort, amphetamines, Bosutinib, direct-acting antiviral agents against hepatitis C virus infection (eg Sofosbuvir), Leflunomide & Indirubin
- Unlikely associated with PAH: Oral contraceptives, Estrogen therapy, cigarette smoking
- Definite association: Gender
- Possible association: Pregnancy, systemic hypertension
- Unlikely associated with pulmonary arterial hypertension: Obesity
- Definite association: HIV infection, severe symptomatic mitral or aortic valve disease
- Likely association: Portal hypertension or liver disease, collagen vascular diseases, congenital systemic-to-pulmonary cardiac shunts
- Possible association: 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)
