Pulmonary Hypertension

What is it?
Normally, pulmonary blood flow occurs in a low
pressure, high compliance system

• High blood pressure in the lungs

• The walls of the pulmonary arteries constrict
• The heart has to work harder to pump blood to the
lungs

“High resistance and low capacity”

 Why is it bad?
• Remodeling of the pulmonary vascular bed
– Intimal and medial hypertrophy with proliferation of smooth
muscle cells and eventual obliteration
– Pulmonary arteries constrict
– Right heart must pump against resistance
– Right heart becomes dilated and less efficient  TR
• Less blood gets out to the lungs and to the body
• Adaptation to stress, increased activity or growth become
impossible
 Clinical Classification: Where Is the Lesion?

VC RA RV PA PC PV LA LV Ao

Group V – Unclear multifactorial Mechanism :

sarcoidosis, Hematological disorders.

ALK-1, activin receptor-like kinase 1; Ao, aorta; BMPR2, bone morphogenetic receptor type 2; HHT, hereditary hemorrhagic telangiectasia; HIV, human immunodeficiency virus; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PC,
pulmonary capillary bed; PV, pulmonary vein; RA, right atrium; RV, right ventricle; VC, vena cava.
Simonneau et al. J Am Coll Cardiol. 2009;54(1 suppl S):S43-S54. Graphic adapted from http://cme.medscape.com/viewarticle/530730.
• Definition of PH:
– mPAP > 25 mm Hg at rest

• Definition of PAH:
– PWP < 15
– Normal LVEF
– No left-sided valvular disease
 Heath-Edwards Classification

• I – Medial hypertrophy
• II – Intimal hyperplasia
• III – Occlusive changes (by fibroelastic
tissue)
• IV – Dilation, medial thinning, occlusion
• V – Plexiform lesions
• VI – Necrotizing arteritis
IV through VI are irreversible!

Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S, Fishman A. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43:5S-12S.
 WHO Classification of Severity
• Class I: No limitation of usual physical activity; Activity doesn’t
cause dyspnea, fatigue, chest pain, or presyncope
• Class II: Mild limitation of physical activity; no discomfort at rest;
but activity causes dyspnea, fatigue, chest pain
• Class III: Marked limitation of activity; no discomfort at rest but
less than normal physical activity causes increased dyspnea,
fatigue, chest pain, or presyncope
• Class IV: Unable to perform physical activity at rest; may have
signs of RV failure; symptoms increased by almost any physical
activity

McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: A report of the American College of Cardiology Foundation Task
Force on Expert Consensus Documents and the American Heart Association: Developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension
Association. Circulation. 2009;119:2250–94
 Diagnosis – Physical Signs
• Cyanosis
• Low output
• Venous congestion
• Active right ventricular impulse
• Loud P2
• High frequency TR murmur
• High frequency diastolic PR murmur

Galiè N., Humbert M., Vachiery J.-L. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur. Heart J. August 2015;29
 Diagnosis - Testing
• Echo: Purely a screening tool
– Identify TR.
– Add mean RAP to the peak tricuspid jet velocity to get an estimate of
peak pulmonary pressure
• Definitive dx needs direct measure of PAP
– Normal mean PAP at sea level at rest = 12–16 mm Hg
– PHTN = mean PAP > 25 mmHg at rest and >30 mmHg with exercise
• Diagnosis requires the presence of above + 2 other conditions:
– Pulmonary artery occlusion pressure (PAOP or PCWP) < 15 mm Hg
– Pulmonary vascular resistance (PVR) > 3 Wood units
 RV, RA Enlargement on Echocardiogram

RV
LV

RA LA

Normal PH
Haddad F., Hunt S.A., Rosenthal D.N. Right ventricular function in cardiovascular disease, part I: anatomy, physiology, aging, and functional assessment of the right ventricle. Circulation. 2008;117(11):1436–1448.
 IVC’s

Rudski L.G., Lai W.W., Afilalo J. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography. J. Am. Soc. Echocardiogr. 2010;23(7):685–713.
 Therapy
• Treat any underlying disease.
• Treat cardiac disease (left sided).
• Treat lung disease and hypoxemia.
• Treat obstructive sleep apnea or any associated
sleep disorder.
• Treat thromboembolic disease.
• Remember: Groups 2-5 are managed differently
and vasodilator therapy is NOT recommended.

Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl 25):D34-D41
 Goals of Therapy

• Alleviate symptoms and improve quality of

life (exercise tolerance)
• Improve cardiopulmonary hemodynamics
and prevent right heart failure
• Delay time to clinical worsening
• Reduce morbidity and mortality
“It is not possible to vasodilate vessels that
do not exist”
 Treatment options
primary therapy
• Divided into two groups primary therapy and
advanced therapy.
• Primary therapy is directed at resolving the
underlying cause.
• Fix the underlying heart defect for those in
group 2
• Diagnosis and treat the underlying lung
condition for those in group 3
 Treatment options
primary therapy
• Anticoagulation with warfarin for those in class 4
(consider using with all classes) INR goal of 2
• Reversal of or treatment of underlying cause in
group 5
• Use diuretics for peripheral edema and hepatic
congestion.
• Oxygen 1-4 liters to maintain Sats. of 90% or
higher.
• Exercise to improve functional capacity
 Targets for Therapy

Humbert et al. New Engl J Med 2004

 How do you treat it?

• Vasoactive medications
– Prostacyclins
• Epoprostenol (synthetic prostacyclin (PGI2) aka Flolan®)
• Treprostinil (Remodulin®), Iloprost (Ilomedin®, Ventavis®)
– Endothelin receptor antagonists
• Bosentan (Tracleer®), Sitaxsentan (Thelin®), Ambrisentan
(Letairis®)
– Phosphodiesterase type 5 inhibitors
• Sildenafil (Revatio®), Tadalafil (Cialis®)
CRITICAL CARE MANAGEMENT
EFFECT OF PEEP

Effect of PEEP on preload

 Effect of PEEP on right ventricular afterload

Luecke, Thomas, and Paolo Pelosi. "Clinical review: positive end-expiratory pressure and cardiac output." Critical Care 9.6 (2005): 607
THANK YOU