Lecture 69 -Dx and Tx of Pulm HTN

Question 1

Normal Values vs PAH values –

what is normal mean pulmonary arterial pressure?
what is in PAH?

what is normal LVEDP?
what is it in PAH?

What is normal pulmonary vascular resistance ?
what is it in PAH?

Answer 1

• Mean pulmonary arterial pressure: ≥25 mm Hg
○ Normal: 12 – 18 mmHg

• PCWP, LVEDP: 3 Wood units
Normal:

Question 2

describe the general characteristics of the pulmonary circulation?

what is the net effect of exercise on the pulmonary circulation ?

Answer 2

low resistance, high compliance, vasodilated

low resistance, high compliance, vasodilated — due to

Question 3

Pathophysiology of PAH

– what are the main vascular changes?

Answer 3

Vasoconstriction

Cellular Proliferation –

Thrombosis

Plexiform Lesions – end stage PAH; tufts of cells replacing arteries and lumen

Question 4

Pathophysiology of PAH –

describe the neuro-hormonal imbalance in PAH?

• what factors are elevated?
• what factors are decreased?

what are their normal functions ?

Answer 4

Increase of vasoconstrictive/proliferative/prothrombic components

Endothelin, Angiopoeitin, Growth Factors

Decreases in vasodilating/cell control/anti-thrombotic components

Prostacyclin (PGI2) , Nitric Oxide

Question 5

how does PGI2 induce smooth muscle cell relaxation?

how does NO induced vasodilation decrease proflieratin?

Answer 5

cAMP

cGMP

Question 6

what Genetic and Cellular Mechanisms can faciliate changes in Pulm HTN?

Answer 6

Bone Morphogenic Protein Receptor II – Mutation causes changes in apoptosis that promotes cellular proliferation

Inflammation == autoantibodies, cytokines, sarcoidosis, etc

Coagulation – Pro-coagulation abnormalities

Question 7

Symptoms and Physical exam findings of PAH?

Answer 7

Symptoms: DOE, fatigue, dizziness, CP, Palpitations, Leg Edema, Syncope
– Lots of non specific symptoms.

Physical Exam Findings:
Loud Second Heart Sound

Heart Murmur – Tricuspid regurgitation, Pulmonary Regurgitation JVD, Hepatomegaly, LE Edema, Ascities

Question 8

what is seen on CXR in pulm HTN?

PA and Lateral

Answer 8

PA CXR — Fluffy, enlarged Pulmonary Arteries
Absence of the vessels that are normally seen (due to lack of blood flow)

Lateral CXR – Loss of the retrosternal clear space due to the enlargement of the RV

Question 9

EKG findings in PAH (in general)?

Answer 9

indings are usually later stage
Right axial Deviation —
Right atrial enlargement
Right Ventricular Hypertrophy

Right Ventricular strain

Question 10

Echocardiogram findings in PAH?

Answer 10

The most reliable heart exam and screening tool, but cannot diagnose with ECHO alone

Dilated RV 
	Dilated RA 
	Smaller than normal Left side of the heart 
	Can observe flow and visualize Tricuspid Regurgitation 
	Estimation of Lung Pressures is possible on ECHO ---

Question 11

what s the most definitive tool for Pulm HTN Dx?

In general, what are the findings?

Answer 11

Swan Ganz Catheter in order to determine the pressures of Right side, Lungs and Left side

In PAH we see high PA and Right Sided Pressures

Question 12

what are the different classifications of PAH ?

1 through 5

Answer 12

PAH Group 1 - Abnormalities in the Pulmonary Arteries themselves leading to PAH

Group 2 - Elevation of the Lung Pressures bc of Heart Disease (systolic dysfunciton, valve, Congenital)
Most common cause of PAH

Group 3 PHA – due to lung diseases and/or hypoxia

Group 4 – Chronic Thromboembolic PAH (CTEPH)

Group 5 – PH with Unclear Multifactoral

Question 13

all treatments aimed at PAH are geared towards what group # of PAH

what are the different classes of PAH treatment?

Answer 13

Group 1 PAH

Prostcyclin Derivatives – -increased cAMP

Endothelin Receptor Antagonists —

PD5 Inhibitors -

Soluble Guanylate Cyclase Stimulator

Question 14

what are the different classes of PAH treatment?

Answer 14

Prostcyclin Derivatives – -increased cAMP

Endothelin Receptor Antagonists —

PD5 Inhibitors -

Soluble Guanylate Cyclase Stimulator

Question 15

what is the only drug that has proven survival benefit for PAH?

what are some side effects of this drug?

what is the inhaled form of this drug?

Answer 15

Prostcyclin Derivatives – -increased cAMP
Flolan/Epoprostenol

Flushing, HA, N/V/D, Jaw pain, leg pain, Hypotension,—

Treprostinil – Iloprost, tyvaso

Question 16

what is the PD5 inhibitor ?

side effects?

Answer 16

PDE5 Inhibitors –

Sildenafil (Viagra, Revatio))

SE: Risk of hypotension, Cannot be used with nitrates

Question 17

what are the Endothelin Receptor Antagonists —?

Answer 17

Bosentan (Tracleer) –

side effects: increases in liver enzymes;