pulmonary%20arterial%20hypertension
PULMONARY ARTERIAL HYPERTENSION
Pulmonary arterial hypertension is a syndrome resulting from restricted flow through the pulmonary arterial circulation resulting in increased pulmonary vascular resistance and ultimately leading to right heart failure.
It is a part of the spectrum of pulmonary hypertension, which is hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure ≥25 mmHg at rest.
Typical symptoms include progressive dyspnea on exertion, palpitations, fatigue, weakness, angina, syncope and abdominal distention.

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
Demographic Factors
  • Definite association: Gender
  • Possible association: Pregnancy, systemic hypertension
  • Unlikely associated with pulmonary arterial hypertension: Obesity
Medical Conditions
  • 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)
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