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What is Pulmonary Hypertension?

mean pulmonary artery pressure (mPAP) > 25 mmHg


What is Pulmonary artery hypertension (group 1)?


elevated mPAP at rest

pulmonar capillary wedge pressure (PCWP) < 15

no left sided heart valve disease


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

C. 30 mmHg

MAP > 25 for PH diagnosis

can't determine PH from PAH from this--> need plum wedge pressure


Where is the problem with the 5 groups of PH?

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)


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

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


How do you classify PH based on severity?

mPAP* (mmHg)

Mild= 25-40


Severe= > 55


What history can point towards PH?


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


What drugs are known to cause PAH?



Toxic rapeseed oil



What are some early signs of PH? Late signs?

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

late: syncope, CP, right heart failure


What are some PE findings associated with PH?

May reveal increased P2 heart sound

Elevated JVD

Cor pulmonale with hepatomegaly, ascites & anasarca

Often will have clear lung sounds


What will CXR show in PH?

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


What will EKG show in PH?

Right axis deviation

right atrial and right ventricular enlargement

right BBB


What PFT changes are seen in PH?

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

DLCO is reduced


What is Echo used for in possible PH?

estimate PAP and assess right ventricular size


What diagnostic test is necessary to confirm diagnosis of PH?

right heart catheterization

(required before initiating treatment for PAH)


What is the order of testing in potential PH?


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

right heart cath


How is PAH different from PH?

PAH is progressive --> 50% survival at 2.8 years

can lead to sudden death

affects young males (mean age 45 yo)


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

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

avoid pregnancy and high altitude

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



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

if respond, much greater chance of 5 year survival


What are the treatment options for PAH?

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


Who should be considered for a lung transplant?

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