Pulmonary Hypertension

© Copyright Annals of Internal Medicine, 2011Ann Int Med. 155 (3): ITC2-1.

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in the clinic

Pulmonary Hypertension


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


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


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


What are the symptoms?

Progressive dyspnea (most common symptom)

Fatigue

Chest pain

Presyncope / syncope

Lower extremity edema

Palpitations

Hoarseness from Ortner syndrome (rare)


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


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


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


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


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


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


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


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)


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


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


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


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


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)


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


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


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


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


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


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


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

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Pulmonary Hypertension

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