© Copyright Annals of Internal Medicine, 2011Ann Int Med. 155 (3): ITC2-1.
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© Copyright Annals of Internal Medicine, 2011Ann Int Med. 155 (3): ITC2-1.
in the clinic
Pulmonary Hypertension
© Copyright Annals of Internal Medicine, 2011Ann Int Med. 155 (3): ITC2-1.
What is pulmonary hypertensionand 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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, 2011Ann 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