Pulmonary Hypertension

Question 1

What things decreased impair vasodilation? (bad)

Answer 1

PGI2 decreased
NO decreased
VIP decreased
Kv channel decreased activity

Question 2

What things enhance vasoconstriction? (bad)

Answer 2

ET-1 increased
5-HT increased
TXA2 increased

Question 3

what anti-proliferative signals are decreased?

Answer 3

PGI1
NO
Kc channel activity

Question 4

what pro-proliferative signals are increased?

Answer 4

• ET-1, 5-HT, TXA2
• VEGF, PDGF, FGF
• BMPR2 / Alk1 mutations
• inflammatory cytokines
• Survivin, BAX, BCL

Question 5

PAH group 1:

Answer 5

pulmonary arterial hypertension (PAH)

Question 6

PAH group 2

Answer 6

PH due to left heart disease

Question 7

PAH group 3

Answer 7

PH due to lung disease or hypoxemia

Question 8

PAH group 4

Answer 8

chronic thromboembolic pulmonary hypertension

Question 9

PAH group 5

Answer 9

pulmonary hypertension with unknown cause

Question 10

what causes group 3: PH associate with lung disease/hypoxia?

Answer 10

hypoxia!!

• COPD = fibrosis, emphysema, severe lung disease
• High altitude
• Sleep apnea

Question 11

What are the stats on who gets PAH most likely? Age? Gender?

Answer 11

women 2x > men

age = 20-50yo

Question 12

What are the heritable genes associated with Group 1 HPAH?

Answer 12

BMPR2
ALK1
Endoglin

Question 13

What other diseases are associated with group 1 PAH?

Answer 13

connective tissue disease (scleroderma)
HIV infection
Portal hypertension (liver) 
congenital heart disease 
schistosomiasis
chronic hemolytic anemia

Question 14

what are the 5 major causes of group 1 PAH?

Answer 14

idiopathic (IPAH)
heritable (HPAH)
Drugs and toxin induced
Associated illnesses
Persistent pH of newborn

Question 15

What are they physical exam features that you have PAH?

Answer 15

increased second heart sound = delay in pulmonic valve closure
RV heave
Murmurs heard (usually tricuspid regurg.)

Question 16

What are they physical exam features that you have RV failure?

Answer 16

JVD
ascities
edema