pulmonary hypertension

Question 1

what are the causes of group 1 phtn

Answer 1

idiopathic, hereditable, drug/toxin,

associated with - hiv, CTD, portal hypertension, congenital heart disease, schistosomiasis

PVOD (which can be idiopathic, heritable, drug/toxin/radiation, HIV, CTD)

newborn

Question 2

what are the known genes associated with phtn

Answer 2

bmpr2

alk-1, eng, smad9, cav1, kcnk3

Question 3

what are the causes of group 2 phtn

Answer 3

heart

LV systolic or diastolic dysfunction, valvular disease, or congenital abnormalities

Question 4

what are the causes of group 3 phtn

Answer 4

lung

copd, ild, OSA, hypoventilation, high altitiude

Question 5

what are the causes of group 4 phtn

Answer 5

chronic thromboembolic obstruction

CTEPH,

angiosarcoma, other intravascular tumors, arteritis

Question 6

what are the causes of group 5 phtn

Answer 6

unclear mechanisms

hematologic - chronic hemolytic anemia, myeloproliferative, splenectomy
systemic - sarcoid, pulmonary histiocytosis,
metabolic - glycogen storage, gaucher, thyroid

Question 7

what does the CHEST consensus guideline suggest as treatment for patients with functional class 1 PH

Answer 7

no PH specific therapy, aggressive comorbidity reversal and then close monitoring

Question 8

what do the CHEST consensus guidelines suggest as the first-line evaluation for symptomatic patients w/ PH?

Answer 8

they suggest vasoreactivity testing to determine candidacy of CCB therapy.

Individuals at high risk include FC4, low BP, low CO, PVOD and can cause hypotension.

Right heart failure is a contraindication

A trial of CCB is 3 months of nifedipine or amlodipnie (at very high doses - 120 MG for nifedepine, 20 mg for amlodipine)

If there is no evidence of vasoreactivity, CCBn should not be used empirically.

Question 9

what do the CHEST consensus guidelines suggest for treatment naive patients w/ FC 2 or 3 symptoms who are not candidate for or did not tolerate CCB?

Answer 9

for FC 2 - combination therapy w/ ambrisentan and tadalafil

Question 10

what are the classes available to treat phtn (4), what is their mechanism, and what are the example medications?

Answer 10

endothelin receptor antagonists
- blocks pulmonary vasconstriction and proliferation
- bosentan, ambrisentan, macitentan

nitric oxide - cGMP enhancers
- increases cGMP causing vasodilation and blocking proliferation
- there are 2 subtypes in this group
- 1) phosphodiesterase 5 inhibitors - sildenafil, tadalafil
- 2) soluble-guante cyclase stimulator - riociguat (Adempas)

prostacycline pathway agonsists
- increase cAMP > vasodilation and decreased broliferation
- 2 subclasses
- 1) analogues - epoprostenol, treprostinil, iloprost
- 2) receptor agonist - selexipag

CCB