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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 doesnt 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; -blockers; diuretics
Possibly: cardiac resynchronization, implantable
cardioverter defibrillator placement, digitalis

HFpEF
Control BP + heart rate with -blockers, diuretics

Left HFrEF
Differentiate from PAH
Dont 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
Dont 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 isnt 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
Dont 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.