Transcript Pulmonary Hypertension Stephen M. Akers, MD Associate Chief, Pulmonary Critical Care

Pulmonary Hypertension
Stephen M. Akers, MD
Associate Chief, Pulmonary Critical Care
Associate Professor Medicine
CMSRU
Pulmonary Hemodynamics
• Pressure = Flow X Resistance
• Ppa - Ppv = Q X PVR
• Ppa = Q x PVR + Ppv
Ppa = Pulmonary Arterial Pressure
Ppv = Pulmonary Venous Pressure
Q = Pulmonary Blood Flow
PVR = Pulmonary Vascular Resistance
Pulmonary Hypertension
Increased Flow
• Congenital Heart Disease
VSD, ASD, Patent Ductus
• Liver Disease
• Arteriovenous Shunts
Pulmonary Hypertension
Increased Venous Pressure
• Systolic Dysfunction
• Diastolic Dysfunction
• Ischemic Heart Disease
• Valvular Disease
• Constrictive Pericarditis
• Restrictive Cardiomyopathy
Pulmonary Hypertension
Increased Vascular Resistance
• Pulmonary Vasculopathy
• Decreased Cross Sectional Area
of Pulmonary Vascular Bed
• Hypoxic Vasoconstriction
of Pulmonary Arteries
Pulmonary Arteriopathy
Pulmonary Vasculopathy
Pulmonary Hypertension
1ary
Pulm HTN
Dx Exclusion
2ary Pulm HTN
Mult. Causes)
Pulmonary Hypertension
Classification
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I Pulmonary Arterial HTN
II Pulmonary Venous HTN
III Pulmonary HTN Hypoxia
IV Pulmonary HTN Thrombotic
Obstruction
• V Pulmonary HTN Miscellaneous
I Pulmonary Arterial HTN
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Idiopathic, Familial
HIV, Portal HTN, CV Disease
Anorexigens, Veno Occlusive
Pul Cap Hemangioma
Congen Heart Dis, Shisto
Cocaine, Amphetamines
II Pulmonary Venous HTN
• Systolic Heart Disease
• Diastolic Heart Disease
• Valvular Heart Disease
• Ischemic Heart Disease
• Restrictive/Constrictive Disease
III Pulmonary Hypertension
Associated with Hypoxia
• COPD
• Interstitial Lung Disease
• Obstructive Sleep Apnea
• Obesity Hypoventilation
• High Altitude
Cor Pulmonale
• Lung Disease Pulm HTN
• Hypoxia, Obliteration of
Pulmonary Vasculature
• COPD, Interstitial Disease,
Hypoventilation
Cor Pulmonale
Cor Pulmonale RV
Hypertrophy
Cor Pulmonale
RV Hypertrophy
Cor Pulmonale
• Symptoms
Dyspnea, Angina, RUQ
Fullness, Lightheadedness
• Signs
JVD, Edema, Ascites
IV Pulmonary Hypertension
Chronic Thromboembolism
• Central Obstruction of Large
Pulmonary Arteries
• Distal Arteriopathy (remodeling) of
Smaller Arterioles
Chronic Thromboembolic
Obstruction
V Miscellaneous Causes
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Histiocytosis X, LAM
Sarcoid
Sickle Cell Disease, Vasculitis
Myeloproliferative Disorders
Compression Pulmonary Vessels
Metabolic, Neurofibromatosis
Transpulmonary Gradient
• Mean Ppa – Ppv (normal < 13)
• Ppa = 24 / 9 = 6 + 8 = 14
• Ppv = 5
• TPG = 14 - 5 = 9 mm Hg
(Mean PA = 2/3 Diastolic + 1/3 Systolic)
Normal Hemodynamic
Values
• Mean Pulmonary Artery Pressure
Normal 15 to 20 mmHg
Borderline 20 to 24
Pulmonary Hypertension 25
• Pulmonary Venous Pressure < 15
• Transpulmonary Gradient <13
Transpulmonary Gradient
Normal
Left Heart Failure
Post Capillary
Elevated
Other Causes
Pre Capillary
Transpulmonary Gradient
Category I
• Ppa = 75/30 = 45
• Ppv = 5
• TPG = 45 – 5 = 40 mm Hg
Transpulmonary Gradient
Category II
• Ppa = 60/24 = 36
• Ppv = 20
• TPG = 32 – 20 = 12 mm Hg
Pulmonary Vascular
Resistance
• (Ppa mean – Ppv)/Cardiac Output
• Ppa = 21/9; Ppv = 3; CO = 5
• 13 mm – 3 mm / 5 L/m
• PVR = 2 mm Hg.min/Liters
Pulmonary Vascular
Resistance Units
• Wood Units mmHg.min/Liters
Normal 0.5 t0 2
• Metric Units dyn.s.cm-5
(Wood Units x 80)
Normal 20 - 130
Typical PVR Values
• Normal Heart Lungs
12 – 7 / 5 = 1 (80)
• Left Heart Failure
30 – 20 / 5 = 2 (160)
• Scleroderma
60 -10 / 2.5 = 20 (1600)
Treatment Options
• Prostacyclins
• Endothelin Antagonists
• Phosphodiesterase Inhibitors
vances in Pulmonary Hypertension Winter 2006 Vol 5 No 4,
Prostacyclins
• Potent Vasodilators
• Inhibit
• Smooth Muscle Proliferation
• Platelet Aggregation
• Improve
• Hemodynamics
• Exercise Tolerance
• Survival
Endothelin Antagonists
• Block Endothelin Receptors
• Vasodilatory Anti Proliferative Effects
• Oral Drug Minimal Side Effects
• Improve
• Hemodynamics
• Exercise Tolerance
• Survival
Phosphodiesterase
Inhibitors
• Increased Cyclic GMP
• Vasodilatory Anti Proliferative
• Improve
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Hemodynamics
Exercise Tolerance
• END
Treatment Pulmonary
Hypertension
• World Health Organization
Functional Class
• Results of Vasodilator Trial
WHO Functional Classification
Class I
asymptomatic
Class II
SOB ordinary activity
Class III
SOB < normal activity
Class IV
SOB any activity or rest
vances in Pulmonary Hypertension Winter 2006 Vol 5 No 4,
Normal Hemodynamics
Multiple Hit Hypothesis
• Genetic Predisposition
BMP mutation
• Second Hit
HIV HSV 8
Scleroderma
Schistosomiasis
Hemodynamic Variant Left Heart
Failure
• Long-standing Pulm Venous HTN 
Pulmonary Arterial Vasculopathy
Increased LAP e.g. 20 mm Hg
Increased Mean PAP e.g. 55 mmHg
Increased TPG e.g. 35 mm Hg
• Pulmonary HTN “Out of Proportion”
Pulmonary Vasculopathy
Left Heart Failure
• Mechanical Factors Induce
Vasoconstriction
Vascular Remodeling
• Usually Resolves with Elimination
Pulmonary Venous Hypertension
Transpulmonary Gradient
CHF
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Dilated LV, EF 10-15%, CI 2.0
PA 100/45 (65) PCWP 32
PA-PCWP (65-32=33 (nl 12)
Normal Pulmonary Artery
Role of Endothelin in
Pulmonary HTN
• Potent Vasoconstrictor, Smooth Muscle
Mitogen and Pro-inflammatory/fibrotic
Mediator
•  Circulating Levels in PA HTN
•  Staining Muscular Pulmonary Arteries
Bosentan Improves Survival
McLaughlin Am J Respir Crit Care Med 2003;167:A442
Prostacyclin Improve
• Hemodynamics
• Exercise capacity
• Survival
Rubin, et al Ann Int Med 1990;112:485-91
Barst, et al NEJM1996;334:296-301
Bosentan
• Improves Hemodynamics
• Improves Exercise Capacity
• Improves Survival
McLaughlin Am J Respir Crit Care Med 2003;167:A442
Pulmonary HTN
• Mean PA 25 at Rest
? 30 with Exercise
• Diagnosis
Echocardiogram
Cardiac Catheterization