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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)
Idiopathic PAHIdiopathic PAH
PATHOPHYSIOLOGY:PATHOPHYSIOLOGY:Primary PAH
Lining of Pulmonary Arteries Thicken
Lumens Become Narrow
Impair Ability to Become Distended
Vascular Resistance is increased
Idiopathic PAHIdiopathic PAH
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
Pulmonary Artery Pulmonary Artery HypertensionHypertension
“Patients with PAH benefit from oral, inhaled, or intravenous medications
targeted to inhibit vascular growth and proliferation.” (Moser & Riegel, 2008, p. 1336)
Pulmonary Artery Pulmonary Artery HypertensionHypertension
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
Pulmonary HypertensionPulmonary Hypertension
Increased pressure in the lungs spreads to right ventricle (which supplies the
pulmonary artery)
Pulmonary HypertensionPulmonary Hypertension
The ventricle becomes hypertrophic and eventually fails (Cor Pulmonale)
Pulmonary HypertensionPulmonary Hypertension
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)
Pulmonary HypertensionPulmonary Hypertension
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
Pulmonary HypertensionPulmonary HypertensionDIAGNOSTIC EVALUATION:DIAGNOSTIC EVALUATION:
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 HypertensionPulmonary HypertensionDIAGNOSTIC EVALUATION:DIAGNOSTIC EVALUATION:
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
Pulmonary HypertensionPulmonary Hypertension
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
Idiopathic PAHIdiopathic PAH
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
Idiopathic PAHIdiopathic PAH
Tracleer (bosentan)Tracleer (bosentan)
Drug-Drug Interactions
Glyburide & Cyclosporine A
Cause retention of Tracleer resulting in increased risk of liver damage
Idiopathic PAHIdiopathic PAH
Tracleer (bosentan)Tracleer (bosentan)
Safe to Use With…
Coumadin (warfarin)
Lanoxin (digoxin)
Diuretics
Calcium Channel Blockers
Idiopathic PAHIdiopathic PAH
Tracleer (bosentan)Tracleer (bosentan)
If interested in more info, visit the Tracleer website!!!
www.tracleer.com
Idiopathic PAHIdiopathic PAH
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.
Idiopathic PAHIdiopathic PAH
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.
Idiopathic PAHIdiopathic PAH
Flolan (epoprostenol)Flolan (epoprostenol)
A prostacyclin (prostaglandin)
Flolan directly vasodilates (expands, relaxes) blood vessels.
Flolan also prevents blood cells from clumping together (antiplatelet agent).
Pulmonary HypertensionPulmonary Hypertension
Flolan (epoprostenol)Flolan (epoprostenol)
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.
Pulmonary HypertensionPulmonary Hypertension
Flolan (epoprostenol)Flolan (epoprostenol)
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.
Pulmonary HypertensionPulmonary Hypertension
Flolan (epoprostenol)Flolan (epoprostenol)
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.