Pulmonary HTN -Goya

Question 1

What is Pulmonary Hypertension?

Answer 1

mean pulmonary artery pressure (mPAP) > 25 mmHg

Question 2

What is Pulmonary artery hypertension (group 1)?

Answer 2

PAH

elevated mPAP at rest

pulmonar capillary wedge pressure (PCWP) < 15

no left sided heart valve disease

Question 3

A 35-year old female presents to the clinic with progressive dyspnea on exertion, and dry cough from last 1 year. Her vitals and physical exams are unremarkable. Her sister has similar “lung problems” from last 3 years as well. During the work up for her DOE you did echocardiogram, which of the following value for her mean pulmonary arterial pressure supports the diagnosis of pulmonary hypertension in her?
A. 10 mmHg
B. 20 mmHg
C. 30 mmHg

Answer 3

C. 30 mmHg

MAP > 25 for PH diagnosis

can’t determine PH from PAH from this–> need plum wedge pressure

Question 4

Where is the problem with the 5 groups of PH?

Answer 4

Group I=PAH=precapillary** (different from all others)

group II=valvular diseases of the heart

group III=post-capillary (lung disease)

Group IV=chronic thrombotic

Group V=misc. (sarcoidosis, etc)

Question 5

What acute and chronic pathophysiological changes will be seen in PH?

Answer 5

Acute: inc RV afterload, inc EDV, dec EF

Chronic: Progressive systolic pressure overload of RV dilates & hypertrophies (compensatory phase)

dec venous return compromises RV preload & pulmonary blood flow

Coexisting hypoxemia can impair the ventricle to compensate

Question 6

How do you classify PH based on severity?

Answer 6

mPAP* (mmHg)

Mild= 25-40

Moderate=41-55

Severe= > 55

Question 7

What history can point towards PH?

Answer 7

smoking

ETOH/recreational drug use

Systemic hypertension–> had LV dysfunction?

Cyanosis/murmur as a child

Joint/musculoskeletal pain

Raynaud’s syndrome

Previous history of VTE (blood clots) or family history of VTE

Use of appetite suppressant drugs

Question 8

What drugs are known to cause PAH?

Answer 8

Aminorex

Fenfluramine

Toxic rapeseed oil

Benfluorex

Question 9

What are some early signs of PH? Late signs?

Answer 9

Early: new onset dyspnea with exertion (out of proportion) and fatigue and weakness

late: syncope, CP, right heart failure

Question 10

What are some PE findings associated with PH?

Answer 10

May reveal increased P2 heart sound

Elevated JVD

Cor pulmonale with hepatomegaly, ascites & anasarca

Often will have clear lung sounds

Question 11

What will CXR show in PH?

Answer 11

enlargement of main pulmonary artery and its major branches with tapering of peripheral arteries

Question 12

What will EKG show in PH?

Answer 12

Right axis deviation

right atrial and right ventricular enlargement

right BBB

Question 13

What PFT changes are seen in PH?

Answer 13

No characteristic changes (must screen for restrictive or obstructive defect)

DLCO is reduced

Question 14

What is Echo used for in possible PH?

Answer 14

estimate PAP and assess right ventricular size

Question 15

What diagnostic test is necessary to confirm diagnosis of PH?

Answer 15

right heart catheterization

required before initiating treatment for PAH

Question 16

What is the order of testing in potential PH?

Answer 16

echo

if echo suggests PH–> evaluate for secondary causes of PH

right heart cath

Question 17

How is PAH different from PH?

Answer 17

PAH is progressive –> 50% survival at 2.8 years

can lead to sudden death

affects young males (mean age 45 yo)

Question 18

What is the treatment for PH (groups 2-5)?

Answer 18

treat the underlying disease (cardiac, lung disease, OSA, thromboembolic disease)

avoid pregnancy and high altitude

supportive (O2, oral anticoagulants, diuretics, calcium channel blockers)

CCB for PAH

Question 19

How do PAH pts who responded to CCBs do compared to those who did not respond?

Answer 19

if respond, much greater chance of 5 year survival

Question 20

What are the treatment options for PAH?

Answer 20

Endothelin receptor antagonist (ERAs):
-Oral treatment that act by blocking the binding of the endothelin to either one or both of its receptors

Synthetic Prostacyclins & Prostacyclin Analogues:

• Act by helping to correct the deficiency of endogenous prostacyclin seen in patients with PAH
• Administered by IV infusion, subcutaneous injection or by inhalation

Phosphodiesterase-5 (PDE-5) inhibitors:
Oral agents that act on NO pathway –> cause vasodilation

Question 21

Who should be considered for a lung transplant?

Answer 21

pts who are on IV prostacyclin who continue to show right heart failure