Pulmonary HTN -Goya Flashcards
What is Pulmonary Hypertension?
mean pulmonary artery pressure (mPAP) > 25 mmHg
What is Pulmonary artery hypertension (group 1)?
PAH
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
Moderate=41-55
Severe= > 55
What history can point towards PH?
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
What drugs are known to cause PAH?
Aminorex
Fenfluramine
Toxic rapeseed oil
Benfluorex
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?
echo
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)
CCB for PAH
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
