Scleroderma Patient Education
Event
Pulmonary Arterial Hypertension Overview

Michael J. Cuttica MD
Assistant Professor of...
Outline
• 1. Pulmonary Arterial Hypertension Defined
• 2. Diagnosis of PAH
• 3. Treatment of PAH
What is Pulmonary Hypertension?
• PH is high blood pressure in the blood vessels
in the lungs.
• The blood pressure in you...
What is the Anatomy?
Systemic BP:
Systolic 120 mmHg
Diastolic 80 mmHg
Mean 65 mmHG

Pulmonary Artery Pressure:
Systolic 20...
Updated Clinical Classification of Pulmonary
Hypertension
(Dana Point 2008)
Class 1:
Pulmonary Arterial Hypertension

Clas...
Where is the disease?
Class 4: CTEPH

Class 3: PH

Class 2: PVH
Class 1: PAH

Class 5: PH – Multifact.
Clinical Classification of Pulmonary
Hypertension (Dana Point, 2008)
Class 1: PAH
mPAP at rest

> 25mmHg

Class 2: PVH
mPA...
What Causes PAH?
• Four Factors are thought to cause the
increased pulmonary vascular resistance that
characterizes the di...
What is “remodeling” of a pulmonary
vessel?
PAH
Normal
Medial hypertrophy

• Abnormal Smooth
Muscle Cell growth
• Extensio...
What is the Clinical Presentation of
PAH?
Breathlessness
Without overt signs of heart and lung disease

Fatigue

Weakness
...
Why do Patients with PAH get short of
breath?

Your lungs are the source of oxygen that your body needs for physical activ...
Reduced Blood Flow Means Less
Oxygen

If you have PAH, it is harder for blood to reach your lungs and pick up oxygen becau...
Right Heart Strain

As the right heart is strained over time we see it dilate/enlarge and we see the
function/squeeze of t...
How Do We Diagnose PAH?
• Typically, you undergo a series of tests to
determine if you have PAH or another illness with
sy...
What is an Echocardiogram?
• Uses sound waves to create a
picture of your heart and
measure blood flow
• Provides useful i...
What is Pulmonary Function Testing?
• Determine the flow of air in
and out of the lung
– ? Obstructive lung disease

• Det...
What is a Right Heart
Catheterization (RHC)?
• RHC is the gold standard test for making the
diagnosis of PAH.
– It is abso...
What is a RHC?
• A thin catheter is inserted
through a large IV either in the
neck or groin.
• This catheter is then guide...
What is the Anatomy?

Left Atrium:
6-10mmHg

Right Atrium:

Left Ventricle:

2-8 mmHg

Systolic 120 mmHG
Diastolic 6-12mmH...
What Other Tests Might be ordered?
• EKG/Electrocardiogram

Looks for signs of Right heart
strain

• CT scan of chest
• Ch...
How do we treat PAH?
• The goal of treatment for PAH is to relieve symptoms
and slow the progression of disease.
• Initial...
Humbert M et al. N Engl J Med 2004;351:1425-1436
Conclusions
• Pulmonary hypertension is high blood
pressure in the blood vessels in the lungs
• Patients with scleroderma ...
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Pulmonary Arterial Hypertension Overview for Scleroderma Patients

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Pulmonary Arterial Hypertension Overview
Michael J. Cuttica MD Assistant Professor of Medicine Northwestern Pulmonary Hypertension Program
Northwestern University

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Pulmonary Arterial Hypertension Overview for Scleroderma Patients

  1. 1. Scleroderma Patient Education Event Pulmonary Arterial Hypertension Overview Michael J. Cuttica MD Assistant Professor of Medicine Northwestern Pulmonary Hypertension Program Northwestern University October 18, 2013
  2. 2. Outline • 1. Pulmonary Arterial Hypertension Defined • 2. Diagnosis of PAH • 3. Treatment of PAH
  3. 3. What is Pulmonary Hypertension? • PH is high blood pressure in the blood vessels in the lungs. • The blood pressure in your lung is normally very low: – about 1/5 your systemic blood pressure • Many disease processes can cause the pulmonary blood pressure to rise.
  4. 4. What is the Anatomy? Systemic BP: Systolic 120 mmHg Diastolic 80 mmHg Mean 65 mmHG Pulmonary Artery Pressure: Systolic 20-30 mmHg Diastolic 5-15 mmHg PA (mean) <25 mmHG
  5. 5. Updated Clinical Classification of Pulmonary Hypertension (Dana Point 2008) Class 1: Pulmonary Arterial Hypertension Class 1. Pulmonary (IPAH) 1.1 Idiopathic Arterial Hypertension 1.2 Heritable: BMPR2, ALK1/endoglin, Unknown 1.3 Drug and toxin induced: Class 2. Pulmonary Hypertension with Left Heart 1.4 Associated with: 1.4.1: Disease Connective Tissue Disease 1.4.2: HIV infection 1.4.3: Portal HTN Class 3. Pulmonary Hypertension associated with lung 1.4.4: Congenital heart disease disease and/or hypoxemia 1.4.5: Schistosomiasis 1.4.6: Chronic hemolytic anemia Class 4. Pulmonary Hypertension due to chronic 1.5 Persistent PH of the newborn thromboticorand/or embolic disease 1’ PVOD and PCH Class 5. Pulmonary Hypertension with unclear or multifactorial mechanisms (sarcoid, MPD, CRF on HD)
  6. 6. Where is the disease? Class 4: CTEPH Class 3: PH Class 2: PVH Class 1: PAH Class 5: PH – Multifact.
  7. 7. Clinical Classification of Pulmonary Hypertension (Dana Point, 2008) Class 1: PAH mPAP at rest > 25mmHg Class 2: PVH mPAP at rest PCWP mPAP with exercise > 30mmHg Pulmonary Vascular Resistance (PVR): PCWP < 15mmHg PVR >3 Wood units Change in pressure/ flow Right heart Pressure – Left heart Pressure Cardiac Output Class 3: PH associated with lung disease mPAP at rest > 20mmHg Underlying chronic lung disease: COPD, ILD, Alveolar Hypoventilation Syndromes, Sleep disordered breathing > 25mmHg > 15mmHg
  8. 8. What Causes PAH? • Four Factors are thought to cause the increased pulmonary vascular resistance that characterizes the disease: 1.Vasoconstriction • Under expression of vasodilators like NO and prostacyclin • Over expression of vasoconstrictors endothelin-1 2.Remodeling of pulmonary vessel wall 3.Thrombosis in situ 4. Inflammation
  9. 9. What is “remodeling” of a pulmonary vessel? PAH Normal Medial hypertrophy • Abnormal Smooth Muscle Cell growth • Extension of smooth muscle into small PA’s • Abnormal Endothelial cell growth – monoclonal origin • Inflammatory Cell infiltration around plexiform lesions. • In situ thrombosis– clots.
  10. 10. What is the Clinical Presentation of PAH? Breathlessness Without overt signs of heart and lung disease Fatigue Weakness Angina/Chest Pain Edema/Swelling Syncope/passing out Clinical Suspicion of PAH
  11. 11. Why do Patients with PAH get short of breath? Your lungs are the source of oxygen that your body needs for physical activity Oxygen from your lungs enters your blood as it flows through microscopic blood vessels called capillaries that surround tiny air sacs called alveoli.
  12. 12. Reduced Blood Flow Means Less Oxygen If you have PAH, it is harder for blood to reach your lungs and pick up oxygen because the pulmonary arteries become so narrow, the resistance increases, and blood can not flow quickly through them. Over time, this increased resistance to blood flow puts a strain on the right side of the heart as it has to work extra hard to push blood through the lungs.
  13. 13. Right Heart Strain As the right heart is strained over time we see it dilate/enlarge and we see the function/squeeze of the right heart decrease. With less oxygen in the blood stream and the right heart pumping less forcefully patients feel tired more often and get short of breath quickly, especially when exerting themselves.
  14. 14. How Do We Diagnose PAH? • Typically, you undergo a series of tests to determine if you have PAH or another illness with symptoms similar to PAH. • Making this determination is very important as treatments for different conditions are very different. • Primary tests usually done first are: – echocardiogram and pulmonary function testing.
  15. 15. What is an Echocardiogram? • Uses sound waves to create a picture of your heart and measure blood flow • Provides useful information about the structure and function of your heart • Can provide an estimation of the pressures in your pulmonary artery. Dilated Right ventricle with reduced function
  16. 16. What is Pulmonary Function Testing? • Determine the flow of air in and out of the lung – ? Obstructive lung disease • Determine the volume of air in the lung – ? Restrictive lung disease • How well does oxygen go from your lungs and into your blood? – ? Vascular health of the lung
  17. 17. What is a Right Heart Catheterization (RHC)? • RHC is the gold standard test for making the diagnosis of PAH. – It is absolutely necessary before initiation of any PAH specific therapy • This test allows us to measure the blood pressure in your lungs directly. • It allows us to calculate how much blood your heart is pumping in every minute.
  18. 18. What is a RHC? • A thin catheter is inserted through a large IV either in the neck or groin. • This catheter is then guided through the right heart and into the pulmonary artery • Typically, a RHC can be performed in less than 1 hour. • Occasionally, an additional catheter will be inserted into an artery to measure pressures on the left side of the heart.
  19. 19. What is the Anatomy? Left Atrium: 6-10mmHg Right Atrium: Left Ventricle: 2-8 mmHg Systolic 120 mmHG Diastolic 6-12mmHg Pulmonary Artery Pressure: Systolic 20-30 mmHg Diastolic 5-15 mmHg PA (mean) <25 mmHG PCWP: 6-12mmHg (must be <PA diastolic)
  20. 20. What Other Tests Might be ordered? • EKG/Electrocardiogram Looks for signs of Right heart strain • CT scan of chest • Chest X-ray Imaging of the chest to look for lung disease. • V/Q scan Looking for blood clots • Sleep study Sleep issues like sleep apnea are associated with PH
  21. 21. How do we treat PAH? • The goal of treatment for PAH is to relieve symptoms and slow the progression of disease. • Initial treatment decisions are based on how far the disease has progressed and how symptomatic a patient is. • Treatment options include: – – – – Oral therapies: pills Inhaled therapies Continuous IV or subcutaneous infusion Surgery: lung transplant
  22. 22. Humbert M et al. N Engl J Med 2004;351:1425-1436
  23. 23. Conclusions • Pulmonary hypertension is high blood pressure in the blood vessels in the lungs • Patients with scleroderma are at an increased risk for developing this complication • The diagnosis of this disease is made by excluding other causes of symptoms and by right heart catheterization • Great advances in treatment have been made in the last 10-15 years

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