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© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
in the clinic
Pulmonary
Hypertension
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What is pulmonary hypertension
and what causes it?
 Pulmonary hypertension (PH)
 Mean pulmonary arterial pressure (PAP): >25 mm Hg
 Elevated PAP burdens normally thin-walled right ventricle
 Without treatment, right heart dysfunction = progressive
symptoms, often eventually death
 PH classified into 5 categories, each with a different…
 Mechanism for the elevated PAP
 Natural history
 Approach to treatment
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What causes pulmonary hypertension?
 PH due to left heart disease (most common cause)
 Systolic or diastolic left heart dysfunction
 Mitral or aortic valve disorders
 PH due to chronic hypoxemic lung disease
 Obstructive lung disorders; interstitial lung disease
 Sleep-disordered breathing
 PH due to embolic disease
 Miscellaneous causes
 Pulmonary arterial hypertension (PAH)
 Heritable / genetic abnormalities; idiopathic
 Risk factors: collagen vascular diseases, HIV infection,
liver disease, anorectic agent use
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
Who should be screened?
Patients with…
 Systemic sclerosis
 Family history of a heritable form of PAH
 Portal hypertension considered for organ transplant
 Increased perioperative mortality with elevated mean PAP
 Therapy may be needed prior to transplantation
 Annual screening recommended in this setting
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What are the symptoms?
 Progressive dyspnea (most common symptom)
 Fatigue
 Chest pain
 Presyncope / syncope
 Lower extremity edema
 Palpitations
 Hoarseness from Ortner syndrome (rare)
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What are the physical exam findings?
 Accentuated intensity of pulmonary second heart sound
 Tricuspid regurgitant murmur
 Pulmonary insufficiency murmur
 Right ventricular S3 or S4
 Parasternal heave or subxiphoid thrust
 Jugular venous distension
 Peripheral edema
 Hepatomegaly
 Ascites
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What is the role of echocardiography
patients with suspected PH?
 One of best tests to evaluate for possible PH
 May report an estimate of systolic PAP
 May provide information on cause of PH and symptoms
 ECHO evaluating dyspnea or cardiac murmur may find PH
 Indicators of more severe disease
 Right atrial or ventricular enlargement
 Hypertrophy; decreased right ventricular function
 Severe elevations in right ventricular pressure may cause
leftward deviation of interventricular septalum
 Pericardial effusion
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What other tests should be ordered in
the evaluation of PH?
 Autoantibody testing for collagen vascular disease
 Brain natriuretic peptide or N-terminal BNP
 Chest radiography; ECHO; EKG
 CBC; electrolytes / creatinine measurement
 HIV serologic testing
 Liver function testing
 Pulmonary function testing
 Oxyhemoglobin saturation at rest and with exertion
 Polysomnography
 Radionuclide ventilation-perfusion imaging
 Right heart catheterization
 Six-minute walking distance
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
Which patients require cardiac
catheterization?
 Right heart catheterization
 Required if PAH suspected (before advanced medical Rx)
 Also helps identify unrecognized left heart dysfunction and
pulmonary venous hypertension
 Left heart catheterization
 Often done concurrently, particularly if risk for CAD
 No right heart catheterization needed…
 PH known to be due to left heart or chronic pulmonary
disease AND and cath not needed to guide management
 Presence of PH itself doesn’t usually alter therapy
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
How should right heart catheterization
be done when PH is a consideration?
 Assess for possible left-to-right shunts
 Measure oxygen saturation in central veins, right atrium,
right ventricle, pulmonary artery
 Increased oxyhemoglobin saturation suggests oxygenated
blood being shunted to right-sided circulation
 Measure hemodynamics accurately
 At end of exhalation + level equipment at mid-thoracic line
 Unsure of wedge?  Measure left ventricular end-diastolic
pressure simultaneously
 Test pulmonary vasoreactivity in PAH
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What are the requirements for the
diagnosis of PAH?
 Presence of PH
 Mean PAP > 25 mm Hg
 Absence of pulmonary venous hypertension
 Left atrial or “wedge” pressure < 15 mm Hg
 Elevated pulmonary vascular resistance
 > 3 Wood units
 Exclusion of significant chronic hypoxemic lung disease
 Exclusion of chronic thromboembolic disease
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
When should a clinician consider
consultation with a specialist in
diagnosing pulmonary hypertension?
 Uncertainty regarding the diagnosis
 Multiple comorbid conditions that may complicate
diagnosis or treatment
 High-risk features or NYHA functional class III or IV
 Refer to a specialized center for evaluation
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
CLINICAL BOTTOM LINE: Diagnosis
and Screening…
 Confirm elevated pulmonary pressures
 Evaluate for potential causes
 ECHO; chest X-ray
 Ventilation-perfusion scanning
 Pulmonary function and blood testing
 Assess disease severity
 Measure oxyhemoglobin saturation
 6-minute walking distance
 Blood BNP
 Right heart catheterization
 Mandatory if therapy directed at PH itself (e.g., PAH)
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What is the approach to treatment of PH?
 Identify the cause
 Chronic cardiac or pulmonary disease
 Treat the underlying condition
 Oxygen therapy
 Maintain oxygen saturation ≥90%
 Right heart dysfunction
 Minimize fluid overload and dyspnea
 Use diuretics
 Restrict salt
 Monitor weight
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
How should patients with PH due to
left heart disease be treated?
 Systolic HF
 Use: ACE inhibitors; β-blockers; diuretics
 Possibly: cardiac resynchronization, implantable
cardioverter defibrillator placement, digitalis
 HFpEF
 Control BP + heart rate with β-blockers, diuretics
 Left HFrEF
 Differentiate from PAH
 Don’t use prostacyclin analogues, endothelin antagonists
 PH secondary to left valvular heart disease
 Evaluation for correction of valvular disease
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
How should patients with PH due to
lung disease be treated?
 Optimize treatment of underlying cause
 Sleep apnea (minimize nocturnal desaturation)
 COPD
 Idiopathic lung disease
 Use supplemental oxygen to avoid hypoxia
 Enroll in pulmonary rehabilitation
 Don’t use PAH therapy in PH due to lung disease
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
How should patients with chronic
thromboembolic PH be treated?
 Prevention of recurrent clot / embolism
(anticoagulation)
 Pulmonary thromboendarterectomy (PTE)
 Refer to center experienced in procedure and postop
 Improves symptoms in most patients
 Surgical mortality at experienced centers: <5%
 Use medical therapy directed at PH only when…
 PTE impossible due to distal location of disease
within pulmonary vasculature
 Therapeutic bridge needed until PTE performed
 Patient decides not to have PTE
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What drugs are available for the
treatment of PAH?
 Diuretics
 Supplemental oxygen
 Calcium-channel blockers (trial only if demonstrated
vasoreactivity)
 Anticoagulants
 Digoxin
Advanced therapies
 Endothelin Antagonists (for lower risk patients)
 Phosphodiesterase-5 Inhibitors (for lower risk patients)
 Prostacyclins (for higher risk patients)
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
Is there a role for combination therapy
in PAH?
 Sequential addition of advanced therapies: current model
 Initial therapy: based on illness severity + functional class
 If PAH worsens: add therapies until treatment goals met
 Approach shown to improve 6-min walking distance and
delay clinical worsening
 Combination therapy
 Optimum and safest approaches not yet established
 Investigations ongoing
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What is the role of lung transplantation?
 Treats underlying pulmonary condition in patients with…
 Late-stage respiratory disease: severe PH + COPD or ILD
 PAH: consider transplant when progressive disease requires
parenteral therapy
 Defer until deterioration occurs despite max medical Rx
 Double lung transplantation: preferred procedure for PAH
 Reduces right ventricular afterload: right heart often recovers
 PAH: 23% unadjusted 3-mo mortality after procedure
 Heart-lung transplantation
 For uncorrectable congenital heart lesions
 For concomitant primary cardiac failure
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What is the role of exercise?
 Exercise improves exercise capacity and QOL
 Important adjunct to medical therapy
 Avoids deconditioning from being sedentary
 PH isn’t a contraindication to judicious exercise
 Encourage patients to remain active within symptom limits
 Mild breathlessness is acceptable
 Avoid severe breathlessness, exertional dizziness, near
syncope, or chest pain
 Isometric exercises discouraged due to exertional syncope
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
CLINICAL BOTTOM LINE: Treatment…
 Right HF
 Use diuretics, salt restriction
 PH due to left heart or chronic hypoxemic lung disease
 Treat underlying disorders (not PH per se)
 Chronic thromboembolic PH
 Use anticoagulants + possible thromboendarterectomy
 PAH
 Perform right heart catheterization with vasodilator test
 Don’t treat empirically with calcium-channel blockers
 Advanced therapies: prostacyclins, endothelin-receptor
antagonists, PDE5 inhibitors
 Evaluate need for supplemental oxygen
 Lung transplantation: in nonresponsive advanced disease
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What is the prognosis of PH?
 Negative prognostic sign in many conditions (HF, COPD)
 In particular, PAH prognosis
 Worse in presence of advanced NYHA/WHO functional
class, shorter 6-min walking distance
 Worse with connective tissue disease
 Worse with portopulmonary hypertension
 Untreated PAH: median survival 2.8 years
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
What should patients be taught about
pulmonary hypertension?
 How PH is distinct from systemic hypertension
 How PH often connotes significant systemic disease
 Regardless of the cause
 Requires ongoing closely coordinated medical care
 Requires monitoring salt intake, fluid balance, weight
 That patients with PAH may need to…
 Self-administer medications
 Monitor themselves for AEs or progression of disease
 Know how other health issues might compromise PAH care
 Where to find information and peer support
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.
CLINICAL BOTTOM LINE: Prognosis…
 PH a negative prognostic sign in many conditions (HF, COPD)
 Treatment can delay complications
 Ongoing closely coordinated medical care is important
 Monitor salt intake, fluid balance, weight
 PH almost always connotes significant systemic disease
© Copyright Annals of Internal Medicine, 2011
Ann Int Med. 155 (3): ITC2-1.