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Pulmonary Arterial Hypertension Management
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Pulmonary Arterial Hypertension Management
, Pulmonary Hypertension Management
See Also
Pulmonary Arterial Hypertension
Pulmonary Hypertension
Pulmonary Hypertension Causes
Pulmonary Hypertension Diagnosis
Precautions
Management strategies below are specific for
Pulmonary Arterial Hypertension
(WHO Group 1)
Other
Pulmonary Hypertension Causes
should be specifically treated
Pulmonary Hypertension
associated with left heart disease
Treat significant valvular disease (e.g.
Mitral Stenosis
)
Afterload
reduction for
Left Ventricular Dysfunction
Manage
Fluid Overload
with
Diuretic
s
Avoid vasodilators
Pulmonary Hypertension
associated with lung disease,
Hypoxemia
or both
Screen for
Sleep Apnea
CPAP for Obstructive Sleep Apnea
Maximize
COPD Management
Supplemental Oxygen
for
PaO2
<60 mmHg (<90%
Oxygen Saturation
)
Avoid vasodilators
Pulmonary Hypertension
associated with chronic thromboembolic disease (WHO Group 4)
Pulmonary endarterectomy (if surgical candidate, first-line if done at major center)
Lifelong
Anticoagulation
Miscellaneous
Pulmonary Hypertension
(WHO Group 5)
Maximize treatment of underlying cause (e.g.
Sarcoidosis
, pulmonary vessel compression)
Sickle Cell Anemia
may respond to
Hydroxyurea
, chronic transfusions
Management
Acute Presentation
See
Pulmonary Arterial Hypertension Crisis
Management
Chronic
Gene
ral measures
Prevent and promptly treat respiratory infections
Regular symptom-limited
Exercise
Avoid pregnancy (50% mortality)
Immunization
s
Influenza Vaccine
annually
Pneumococcal Vaccine
Perioperative Evaluation
Preoperative
Echocardiogram
Evaluate functional status
Consider surgical alternatives
Consult specialty care in the decision to approve, and the optimization before elective surgery
Anticoagulation
Coumadin
to keep INR between 1.7 to 2.2
Cardiac Output
maximization
Consider
Digoxin
to increase
Cardiac Output
or for tachyarrhythmias
Consider
Parenteral
inotropic medications in refractory inpatients
Decrease
Preload
Follow low-salt diet
Diuretic
s to reduce volume retention
Treat
Hypoxia
Oxygen Supplementation
to keep
Oxygen Saturation
>90-92% (60 mmHg)
Pulmonary Arterial Hypertension
medications
Low risk patient
See Vasodilators below
PDE-5 Inhibitors (
Sildenafil
) or
Endothelin receptor
Antagonist
s (Bosentan, Ambrisentan)
High risk patient (or low risk patient protocol fails)
Epoprostenol (Flolan) IV or
Selexipag (Uptravi) oral
Treprostinil (Remodulin, Orenitram) Oral, IV or SQ
Iloprost (Ventavis) Inhaled
Other medical management
Consider
Calcium Channel Blocker
Only indicated if positive vasoreactivity test (<5-20% of patients)
Experimental options
Nitric oxide
Surgery (if refractory to medical management)
Lung
Transplantation
Balloon Atrial Septoplasty
Management
Vasodilators (reduce vascular resistance) - Specific PAH Treatments
Endothelin receptor
Antagonist
s
Precautions
FDA pregnancy category X (and reduce OCP efficacy)
Liver Function Test
ing monthly (due to hepatotoxicity risk)
Drug Interaction
with
Warfarin
(increased INR)
Preparations
Bosentan (Tracleer) 62.5 mg orally twice daily (up to maximum of 250 mg/day)
Ambrisentan (Letairis) 5 mg daily (up to 10 mg/day)
Sitaxsentan
Phosphodiesterase-5 Inhibitors (PDE-5 Inhibitors)
Precautions
Avoid with nitrates (precipitous
Hypotension
risk)
Avoid with
CYP3A4
inhibitors (
Clarithromycin
,
Itraconazole
)
Adverse effects
Headache
Flushing
Epistaxis
Mechanism
Cyclic guanosine monophosphate (cGMP) is a vasodilator
cGMP is rapidly degraded by PDE5 (Phosphodiesterase 5) which present in right ventricle of PAH patients
Preparations
Sildenafil
(Revatio) 20 mg orally three times daily ($45/month)
Tedalafil (Adcirca) 40 mg daily (>$3000/month)
Prostenoids (Prostacyclin)
Efficacy: Highly effective
Precautions
Avoid stopping abruptly (risk of rebound
Pulmonary Arterial Hypertension
)
Adverse effects
Jaw pain
Diarrhea
Peripheral Edema
Headache
Mechanism
Prostacyclins reduce
Platelet
aggregation and causes vasodilation
Prostacylcins are reduced in PAH
Preparations
Epoprostenol (Flolan) 2 ng/kg/min IV (titrate 1-2 ng/kg/min every 15 min as needed)
Iloprost (Ventavis) 2.5 to 5 mcg nebulized every 2-4 hours (up to 9 doses per day)
Beraprost 20 mcg three times daily (titrate up to maximum 60 mcg, over several weeks)
Treprostinil (Remodulin)
IV/SQ: 1.25 ng/kg/min titrated once weekly
Inhaled: 18 mcg (3 breaths from 1.74 mg/2.9 ml
Inhaler
) four times daily
Increased by 18 mcg/dose (3 breaths) every 1-2 weeks
Maximum of 54 mcg/dose (9 breaths/dose)
Management
Calcium Channel Blocker
s
Do not use in non-vasodilator responders
Risk of
Hypotension
,
Syncope
and
Right Ventricular Failure
Effective longterm in only 5-20%
Effectiveness wanes over time
Requires vasodilation test as above (use only if responder)
If non-responder, then do not use
Calcium Channel Blocker
(use other vasodilators listed above)
Determine if patient responds to vasodilation (vasoreactivity study)
Perform right heart catheterization (mandatory prior to using
Calcium Channel Blocker
)
Administer vasodilator (e.g.
Adenosine
, epoprostenol)
Responder criteria
Pulmonary Artery Pressure decreases >10 mmHg and <40 mmHg
Cardiac Output
does not change or increases
Use responder status to direct therapy
Responder:
Calcium Channel Blocker
Non-responder: Use other vasodilators listed above
Preparations (use very high dose)
Diltiazem
ER (max 960 mg/day)
Nifedipine
ER (max 240 mg/day)
Amlodipine
(max 30 mg/day)
Complications
See
Pulmonary Arterial Hypertension Crisis
(
PAH Crisis
)
References
Meter (2013) Crit Dec Emerg Med 27(5): 2-10
Dunlap (2016) Am Fam Physician 94(6):463-9 [PubMed]
Gaine (2000) JAMA 284:3160-8 [PubMed]
Galie (2009) Eur Heart J 30(20): 2493-537 [PubMed]
Nauser (2001) Am Fam Physician 63(9):1789-98 [PubMed]
Rubin (1997) N Engl J Med 336:111-7 [PubMed]
Rubin (1993) Chest 104:236-50 [PubMed]
Ryerson (2010) Respir Res 11:12 [PubMed]
McLaughlin (2009) Circulation 119(16): 2250-94 [PubMed]
Stringham (2010) Am Fam Physician 82(4): 370-7 [PubMed]
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