Pulmonary hypertension

Pulmonary Artery Pulmonary Artery HypertensionHypertension

Natalie Bermudez, RN, BSN, MS

Clinical Educator for Telemetry

Telemetry Course

StatisticsStatistics

PH is rare disease with an incidence of about 2-3 per million per year and a prevalence of about 15 per million.

Women, usually age 30s to 40s, are almost three times as likely to present

with idiopathic PAH than men.

Pulmonary Artery PressuresPulmonary Artery Pressures

In a healthy adult, normal pulmonary artery pressure (PAP) ranges from 12 to

16 mmHg3 (Vacca, 2009, p. 37)

For Pulmonary HTN

PAP > 25 mmHg at rest

PAP > 30 mmHg with activity

4 Classes of PAH4 Classes of PAHClass I – No symptom-induced limits on physical

activity

Class II – Slight symptom-induced limits on physical activity

Class III – Marked symptom-induced limits on physical activity

Class IV – Right-sided heart failure with dyspnea and fatigue at rest, and an inability to perform any

physical activity without symptoms

(Vacca, 2009, p. 38)

PAH PathophysiologyPAH Pathophysiology

The abnormally high blood pressure is associated with changes in the small blood vessels (capillaries) in

the lungs, resulting in an increased resistance to blood flowing through

the vessels

PAH & the EndotheliumPAH & the Endothelium

Changes are due to weakening of the endothelial cells (lining of the

vessels) which causes enlargement of the cells and

narrowing of lumen leading to increased resistance

Types of PAHTypes of PAH

1. Idiopathic PAH (IPAH)

2. Familial PAH (FPAH)

3. Associated PAH (APAH)

Idiopathic PAHIdiopathic PAH

“IPAH is diagnosed when the cause of PAH is undetermined

to be unknown and all other causative conditions have

been excluded.”

(Moser & Riegel, 2008, p. 1336)

Idiopathic PAHIdiopathic PAHAKA Primary PAH

“IPAH is mostly a disease of younger women, with female-to-male ratio of

2-3 to 1.” (Moser & Riegel, 2008, p. 1337)

Most common in women; ages 20 – 40

Mortality rate is high

Fatalities occur within 3 – 4 years

Idiopathic PAHIdiopathic PAHSTATISTICS

“IPAH has an estimated annual occurrence of 1 to 2 cases per

million.” (Moser & Riegel, 2008, p. 1337)

Idiopathic PAHIdiopathic PAHSURVIVAL RATES

The estimated median survival for untreated IPAH patients from diagnosis was 2.8 years, with group survival rates of:

• 68% at 1 year• 48% at 3 years• 34% at 5 years

(Moser & Riegel, 2008)


PATHOPHYSIOLOGY:PATHOPHYSIOLOGY:Primary PAH

Lining of Pulmonary Arteries Thicken

Lumens Become Narrow

Impair Ability to Become Distended

Vascular Resistance is increased


V/Q mismatch → Hypoxemia

Vasoconstriction Occurs

Further Increases Vascular Resistance

Familial PAHFamilial PAH• Associated with a mutation of the

bone morphogenetic protein receptor II gene (BMPR2)

• Autosomal dominant with little penetrance– Means that it does not manifest in all

persons carrying the gene– Makes is difficult to trace the family

history

Familial PAHFamilial PAH

“The incidence and prevalence is unknown because genetic

investigation is not considered routine in the diagnosis and treatment of

PAH” (Moser & Riegel, 2008, p. 1337)

Associated PAHAssociated PAH• Generally occurs later in life

secondary to another disease process– Connective tissue disease

– Portal HTN

– HIV infection

– Drugs (Fen-Phen, cocaine)

Associated PAHAssociated PAH• Other Causes

– Left heart disease/dysfunction

– Respiratory disease or hypoxemia

– Thromboembolic disease

– Sarcoidosis, histiocytosis X, lymphangiomatosis, and compression of pulmonary vessels

Associated PAHAssociated PAHSecondary PAHSecondary PAH

The most cause common is…

Left-Sided Heart Failure!!!

Associated PAHAssociated PAH• Connective Tissue Disease

– Scleroderma: affects 6% to 60% – Lupus: affects 4% to 14%

• Drug Use– Amphetamines/Appetite suppressants:

affects 25 to 50 cases per million annually

• HIV: incidence is 0.5%– irrespective of viral load

Associated PAHAssociated PAHSURVIVAL STATISTICS

• Congenital heart disease: 5 years

• Portopulmonary HTN: 2 years

• HIV: 1 to 2 years

• Sickle Cell Disease: 1 to 2 years

• Amphetamines/Appetite Suppressants: 2 to 3 years

(Moser & Riegel, 2008)

Associated PAHAssociated PAHMORTALITY RATE VARIABLES

Of the Natural History of Untreated PAH

• Mean PAP

• Right Atrial Pressure

• Cardiac Index

• NYHA Functional class– New York Heart Association classification of

dyspnea(Moser & Riegel, 2008)

Associated PAHAssociated PAHPATHOPHYSIOLOGY:PATHOPHYSIOLOGY:

AKA Secondary PAH

Depends on Underlying Etiology


“Patients with PAH benefit from oral, inhaled, or intravenous medications

targeted to inhibit vascular growth and proliferation.” (Moser & Riegel, 2008, p. 1336)


If a patient does not receive treatment the following complications will occur:

Pulmonary HypertensionPulmonary Hypertension

Hypertrophy of medial smooth muscle layer of the arterioles – worsens distensibility


Increased pressure in the lungs spreads to right ventricle (which supplies the

pulmonary artery)


The ventricle becomes hypertrophic and eventually fails (Cor Pulmonale)


Impaired distensibility due to hypertrophy can lead to arrhythmias

Pulmonary HypertensionPulmonary HypertensionSIGNS & SYMPTOMS:SIGNS & SYMPTOMS:

Early signs and symptoms of PAH/PH are similar to many other signs and symptoms

of other disease processes

May remain misdiagnosed until disease process has irreversibly progressed

Pulmonary HypertensionPulmonary HypertensionSIGNS & SYMPTOMS:SIGNS & SYMPTOMS:

SOB/TachypneaFatigue/WeaknessNonproductive coughAngina pectorisSyncopePeripheral edemaHemoptysis (rarely)

Orthopnea or PND (with pulmonary venous hypertension)

ClubbingHepatojugular reflexLoud P2 (pulmonic

closure sound)


CARDIAC SIGNS & SYMPTOMS:CARDIAC SIGNS & SYMPTOMS:

TachycardiaDecreased Peripheral B/P

S/S of Right-Sided HF (JVD, ascites)Displaced PMI

Pulmonary HypertensionPulmonary HypertensionDIAGNOSTIC EVALUATION:DIAGNOSTIC EVALUATION:

Echocardiography • It is often the 1st screening test for Dx of PAH• Estimates PAP• ventricular wall motion and possible valvular

dysfunction/regurgitation (tricuspid); right atrial/ventricular enlargement

Radionuclide Imaging• abnormal right & left ventricular functioning


To RULE OUT Thromboembolic PAH:To RULE OUT Thromboembolic PAH: Perfusion Lung Scanning → multiple patchy and diffuse filling or normal results; inconsistent with findings associated with

pulmonary embolism


Pulmonary Angiography → detect filling defects in pulmonary vasculature and if

obstruction is operable

PA Catheterization → ↑ PAP SBP > 30

Pulmonary Function Studies → ↓ Flow Rates, ↑ Residual Volume, ↓ Total Lung

Capacity


DIAGNOSTIC EVALUATION:DIAGNOSTIC EVALUATION:ABG’s → hypoxemia (↓ PaO2) EKG Δ’s

• RV hypertrophy is seen in 87% of patients w/ IPAH

• Right axis deviation 79% of patients w/ IPAH• ST-segment depression and T-wave inversion

in right precordial leads• RA enlargement

• Tall/Peaked P waves (known as Cor Pulmonale) in inferior leads (II, III, aVF)

Treatment of PAHTreatment of PAH

O2 Therapy (hypoxemia): For all types of PAH

Fluid Restriction (↓ preload and ↓ workload) Heart-Lung Transplant (irreversible Δ’s)

Medication Regimen

Treatment of PAHTreatment of PAH

Inotropics (ie, Digoxin) → ↑ CO Diuretics → ↓ Intravascular & Venous Return CCBs & other vasodilators → ↓ Myocardial

Workload and O2 consumption Bronchodilators → Relax smooth muscle and

↑ airway patency β-blockers → ↓ cardiac workload and improve

oxygenation Anticoagulant Therapy → avoid blood clots

Common Medications Common Medications Specifically for Treatment Specifically for Treatment

of IPAHof IPAH

Tracleer (bosentan) - POTracleer (bosentan) - PO

Revatio (sildenafil citrate) - PORevatio (sildenafil citrate) - PO

Flolan (epoprostenol) - IVFlolan (epoprostenol) - IV

Idiopathic PAHIdiopathic PAHTracleer (bosentan)Tracleer (bosentan)

Most effective drug in treating IPAH

Very, very, very expensive!!!

Insurance will not cover unless ALL other meds have been proven to be ineffective

May take up to one month before insurance approves


Tracleer (bosentan)Tracleer (bosentan)

Vasodilator

62.5 mg PO b.i.d. for 4 weeks, then

125 mg PO b.i.d.

May take 1 month to see results

Idiopathic PAHIdiopathic PAHTracleer (bosentan)Tracleer (bosentan)

Major Side Effects:

Liver Damage (hepatotoxicity)Monitor LFT’s

Hypotension Monitor B/P



Drug-Drug Interactions

Glyburide & Cyclosporine A

Cause retention of Tracleer resulting in increased risk of liver damage



Safe to Use With…

Coumadin (warfarin)

Lanoxin (digoxin)

Diuretics

Calcium Channel Blockers



If interested in more info, visit the Tracleer website!!!

www.tracleer.com

http://www.tracleer.com/


Revatio (sildenafil citrate)Revatio (sildenafil citrate)

The FDA approved the main ingredient in Viagra (sildenafil citrate) June 7, 2005 under

the name Revatio.

It dilates (relaxes) blood vessels and improves exercise ability in PH patients.


Revatio (sildenafil citrate)Revatio (sildenafil citrate)

The approved dose for PH is 20 mg T.I.D.

(different than in Viagra – P.R.N.)

Just like Viagra, people taking nitrates (like nitroglycerin or Isordil [isosorbide dinitrate] )

should avoid Revatio.

Taking both can cause fatal blood pressure changes.


Flolan (epoprostenol)Flolan (epoprostenol)

A prostacyclin (prostaglandin)

Flolan directly vasodilates (expands, relaxes) blood vessels.

Flolan also prevents blood cells from clumping together (antiplatelet agent).



It increases cardiac output and lowers afterload, improving PH symptoms, and it

also improves survival time.

This prostacyclin is similar to the natural prostacyclin produced by the cells lining

(endothelium) our blood vessels.



Flolan is an IV drug which is only active in your blood for about 5 minutes, so you have to take it as a continuous IV infusion 24 hours a day.

The delivery system is complex and requires you to learn sterile preparation, operation of

the pump, and care of the IV catheter.

Flolan must be kept cold after mixing but cannot be frozen.

http://community.healthgate.com/getcontent.asp?siteid=jhealthsystem&docid=/usp/203429b

http://www.chfpatients.com/glossary_2.htm#iv



VERY, VERY, VERY EXPENSIVE:

Costs anywhere from $25,000 a year and up.

ReferencesDonofrio, J., Haworth, K, Schaeffer, L. & Thompson, G., (Eds.). (2005).

Cardiovascular care made incredibly easy. Ambler, PA: Lippincott Williams & Wilkins.

Jacobs, M., & Meyer, T. (2006). The push is on pulmonary hypertension. Nursing Made Incredibly Easy, 4(3), pp. 42-52.

McCance, K. L., & Huether, S. E. (2006). Pathophysiology: The biologic basis for disease in adults and children, (5th ed.). Philadelphia, PA: Elsevier Mosby.

Moser, D. K., & Riegel, B. (2008). Cardiac nursing: A companion to braunwald’s heart disease. Saunders Elsevier: St. Louis, MO.

Smeltzer, S. C., Bare, B. G., Hinkle, J. L., & Cheever, K. H. (2008). Brunner and suddarth’s textbook of medical-surgical nursing, (11th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.

Vacca, V. M. (2009). On the alert for pulmonary artery hypertension. Nursing 2009, 39(12), pp. 36-40.

Woods, S. L., Froelicher, E. S., Underhill Motzer, S., & Bridges, E. J. (2005). Cardiac nursing, (5th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.

Pulmonary hypertension

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