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 Management


  • 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 and judicious use of diuretics reduces volume overload in patients with pulmonary hypertension (PH) and right ventricular hypertension
  • Monitor renal function and blood biochemistry in patients to avoid hypokalemia and 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 and right heart failure

Prevention of Infection

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

Follow Up

  • Ensure compliance of patients to their follow-up schedule, especially those belonging to WHO FC I
  • Follow-up visit at 3-6 months and 6-12 months after initial follow-up is advised
  • Baseline measurements of the following should be established during the initial follow-up, and in all patients showing signs of worsening clinical status:
    • Medical history
    • WHO-FC
    • ECG
    • 6MWT/Brog dyspnea score
    • Basic blood exams (eg CBC, INR, serum creatinine, sodium, potassium, AST/ALT, bilirubin, BNP/NT-proBNP)
    • CPET
    • Echocardiography
    • Specific laboratory tests (eg TSH, troponin, uric acid, iron levels)
    • Blood gas analysis
    • Right heart catheterization (RHC)
  • Patient’s medical history and functional class should be assessed using ECG, 6MWT/Borg dyspnea score and basic laboratory exams (eg CBC, INR, serum creatinine, sodium, potassium, AST/ALT, bilirubin, BNP/NT-proBNP levels) every follow-up visit of 3-6 months
  • CPET, echocardiography, specific laboratory tests (eg TSH, troponin, uric acid, iron levels), blood gas analysis, and RHC should be added at 6-12 months follow-up visit
  • At 3-6 months after every treatment adjustment, repeat evaluation of the patient’s complete medical history, functional class, ECG, 6MWT, echocardiography, basic blood tests, blood gas analysis and RHC are recommended
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