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.


  • A sustained response is considered when treated patients continue in World Health Organization (WHO) functional class I or II with near-normal hemodynamics after several month of treatment


General Care

Maintenance of Intravascular Volume

  • Near-normal intravascular volume is important in the long-term management of idiopathic pulmonary arterial hypertension (IPAH)
    • Sodium-restricted (<2400 mg/day) diet, fluid restriction & judicious use of diuretics reduces volume overload in patients with pulmonary hypertension (PH) & right ventricular hypertension
  • Monitor renal function & blood biochemistry in patients to avoid hypokalemia & the effects of decreased intravascular volume leading to pre-renal failure

Hemoglobin (Hb) Levels

  • PAH patients are extremely sensitive to decreases in hemoglobin (Hb) levels
    • Anemias should be promptly treated
  • Phlebotomies should be done if hematocrit (Hct) >65% in symptomatic patients (headache, poor concentration)

Concomitant Medications

  • Avoid drugs that interfere with oral anticoagulants or increase risk of GI bleeding
  • Empiric use of angiotensin-converting enzyme (ACE) inhibitors or beta-blockers should be discouraged as this may result in hypotension & right heart failure

Prevention of Infection

  • Due to potentially devastating effects of resp tract infection in patients with pulmonary hypertension, immunization with influenza & pneumococcal vaccine is warranted
  • Respiratory tract infection should be treated aggressively
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