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.

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)
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