Pulmonary HTN Flashcards
WHO grouping (5) of Pulm HTN
- Pulmonary arterial HTN
- Left heart disease
- Lung disease / hypoxia (COPD, ILD)
- Chronic thromboemboli (PE)
- Misc (sarcoidosis)
The mean pulmonary artery pressure must be >___mmHg at rest to dx pulm HTN.
(ON EXAM)**
>25mmHg
Which world health group?
- Pulm Arterial HTN secondary to various disorders
- Diseases that localize directly to the pulm arteries leading to structural changes, smooth muscle hypertrophy, & endothelial dysfunction
Group 1
Which World Health group?
- Schistosomiasis
- Drugs / toxins
- HIV
Group 1
Which drugs will DEFINITELY cause pulmonary HTN?
Appetite suppressants (aminorex, fenfluramine, dexfenfluramine)
Which drugs/toxins will POSSIBLY be risk factors for pulm arterial HTN? (PAH)
- amphetamines
- L-tryptophan
- meth
- cocaine
- St. John’s Wort
What drug is:
- associated w/ development of persistent pulm HTN of the newborn when taken by pregnant mothers?
- associated w/ poor prognosis in those established w/ PAH?
SSRI
Which group?
- Pulmonary venous HTN secondary to left heart disease
- Often referred to as “pulmonary venous HTN” or “post capillary pulm HTN”
Group 2
Which group?
- Pulmonary HTN secondary to lung disease or hypoxemia
- Caused by advanced obstructive and restrictive lung disease (COPD, ILD, fibrosis, bronchiectasis)
Group 3
- What is shown here?
- Which group?

- PE (bilateral) probably from DVT
- group 4 (clotting/emboli/thrombo)
Which group?
- Pulm HTN secondary to chronic thromboembolic occlusion of proximal and distal pulm arteries
- This classification no longer includes pts w/ non-thrombotic occlusion, such as tumors/foreign objects
Group 4
Group 5 is pulm arterial HTN secondary to what 4 things?
- Hematologic disorders
- Metabolic disorders
- Systemic disorders (sarcoidosis)
- Misc (tumor embolization***)
- Which organization classified severity of pulm HTN?
- What 2 things is severity based on?
- NYHA, and modified by WHO
- Sxs & functional status
Which class of NYHA severity?
- without limitation of physical activity
- no dyspnea, fatigue, CP, or near syncope w/ exertion
Class 1
Which class of NYHA severity?
- Slight limitation of physical activity
- no sxs at rest, but ordinary physical activity causes dyspnea, fatigue, CP, or near syncope
Class 2
Which class of NYHA severity?
- “Marked” limitation of physical activity
- no sxs at rest, but LESS THAN ORDINARY activity causes sxs
- Pts usually present for tx with this class!
Class 3
Which class of NYHA severity?
- Inability to perform any physical activity w/o sxs
- Evidence of right heart failure
- Dyspnea / Fatigue at rest and worsening of sxs w/ any activity
Class 4
What is the #1 symptom of Pulm HTN?
Dyspnea on Exertion (DOE)
- What murmur is heard w/ Pulm HTN?
- Where?
- Louder with what?
- Tricuspid regurg (holosystolic)
- heard along left parasternal line
- Louder w/ inspiration = Carvallo’s Sign
ALL patients w/ Pulm HTN should be screened for what 2 things?
- HIV
- Collagen vascular disease
Pts w/ idiopathic PAH often have normal PaO2 at rest, but show evidence of _____ with a ______ in PaCO2.
- Hyperventilation
- Decrease in PaCO2
W/ echocardiography, Doppler flow can estimate what?
Right Ventricular Systolic Pressure (RVSP)
***What is the gold standard test to dx PAH?**
Right sided cardiac catheterization
(RHC = right heart cath)
Cardiac catheterization is helpful in differentiating _____ from ______ by assessment of drop in pressure across the pulmonary circulation also known as __________. ****
- Pulmonary arterial hypertension
- Pulmonary venous hypertension
- Transpulmonary gradient
Are FVC and TLC on PFT testing elevated, low, or normal for a pt w/ pulm HTN?
Both normal
What test differenitiates chronic thromboembolic pulmonary HTN from idiopathic pulmonary arterial HTN?
V/Q lung scan
(V=ventilation Q=perfusion)
What is the most definitive diagnostic procedure for defining the distribution and extent of disease in chronic thromboembolic pulmonary HTN?
Pulmonary Angiography
What diagnostic procedure is preferred for pts w/ renal failure bc the dye of angiography/CT will damage their kidneys?
Ventilation Quotient (V/Q) Scan
What is first line therapy for Group 1?
Oral CCB such as Diltiazem or Nifedipine
Group 1 tx***
- Oral CCB (Diltiazem or Nifedipine) should only be given to pts w/ ____ _____ _____ ______ bc they are harmful otherwise.
- (ON EXAM)
Positive Acute Vasodilator Response
What do you need to treat for Group 2 pts?
Left Heart Failure
Tx for Group 3 pts w/ hypoxemia
Supplemental Oxygen
Tx for group 4 (3 things)
- Anticoagulants
- Thromboendarterectomy (special/rare surgery)
- Riociguat (DON’T GIVE TO PREGNANT WOMEN)
Tx for group 5
Tx underlying etiology
Which medication for PAH have a short medication half life requiring a reliable continuous infusion, difficulty in titration, and high cost of therapy?
(DO NOT suddently stop this med. Must titer or pt will die!)
(ON EXAM)
Endothelin Receptor Antagonist
Prostacyclins (Epoprostenol)