Pulmonary%20arterial%20hypertension Management
Monitoring
- 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
Prevention
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