Pulmonary HTN and Pulmonary arterial hypertension

Question 1

Pulmonary HTN definition

Answer 1

mean pulmonary arterial pressure >25 mmHg

Question 2

pulmonary capillary wedge pressure (PCWP)

Answer 2

corresponds to pressure in left atrium.

>15 suggestive of left sided heart failure

Question 3

how can you develop pulmonary hypertension from heart failure with preserved EF?

Answer 3

concentric hypertrophy of LV leads to impaired diastolic filling and increased left atrial pressure

this causes pulmonary edema and transmission of increased hydrostatic pressure back to the pulmonary arteries.

Question 4

WHO Group 1` PHTN

Answer 4

pulmonary arterial hypertension (inherited)

scleroderma

systemic sclerosis

rheumatoid arthritis

HIV

drug/stimulant use

portopulmonary HTN

Question 5

WHO group 2 PHTN

Answer 5

due to left sided heart dx

Question 6

WHO group 3 PHTN

Answer 6

due to chronic lung dx (COPD, ILD)

Question 7

WHO group 4 PHTN

Answer 7

due to chronic thromboembolic dx

Question 8

WHO group 5 PHTN

Answer 8

sarcoidosis

thyroid disease

histiocytosis

glycogen storage disease

Question 9

symptoms of pulmonary HTN

Answer 9

dyspnea, fatigue, weakness

exertional angina, syncope

abdominal distension and pain

Question 10

signs of pulmonary HTN

Answer 10

left parasternal life and RV heave loud P2 and right sided S3 Pansystolic murmur of tricuspid regurgitation JVD, ascites, peripheral edema and hepatomegaly.

Question 11

treatment of pulmonary arterial HTN (WHO 1)

Answer 11

bosentan - endothelin receptor antagonist

Question 12

on pulmonary HTN pts there may be

Answer 12

moderately decreased FEV1 and FVC and see low DLCO

Question 13

presentation of pulmonary HTN is

Answer 13

dypsnea, fatigue, syncope that shows inability to increase CO in activity

Question 14

Clinical features of pulmonary HTN

Answer 14

Question 15

pulmonary HTN evaluation

Answer 15

Question 16

pulmonary HTN definition is:

Answer 16

mean pulmonary artery pressure >25 mm Hg at rest

Question 17

chronic thromboemoblic pulmonary HTN is

Answer 17

elevated pulmonary arterial HTN due to prior VTE. There is failure of plasma mediated lysis of initial clot in pulmonary artery causing altered resistance and peripheral pulmonary vascular bed.

NOTE: 50% of pts will not have history of PE.

This can be seen years to months after syndrome.

Need TTE and V/Q scan more than a CT pulm angiogram. Normal V/Q scan excludes CTEPH.

Question 18

how to treat chronic thromboembolic pulmonary HTN

Answer 18

need life long anticoagulation with INR 2-3

Need surgical thromboendarterectomy is the only cure and should be referred even if mild dx. This helps prevent long term complications and chronic cardiac conditions.

Best candidates are people who have central dx rather than peripheral.

Peripheral dx people should get lung transplant.

Question 19

in the evaluation of suspected pulmonary HTN, if the TTE didn’t show signs of pulmonary HTN but still suspect it what do you order?

Answer 19

exercise TTE to see if this will show it.

Question 20

Treatment of group 1 pulmonary HTN involves

Answer 20

vasodilators, (phosphodiesterase 5 inhibitors - tadalafil, sildenafil)

soluble guanylate cyclase activators (riociguat) and

endothelial antagonists (bosentan and ambrisentan)

prostanoids - treprostinil and epoprostenol

prostacycline receptor agonists (selexipag)

Also treat with maintaining euvolumia with diuretics if necessary and O2 supplementation if sPO2<88 or <90 with signs of right heart failure

Response to see if pt will benefit from vasodilators can be predicted by the vaso-reactivity testing in the Right heart catherization

positive when vasodilator like inhaled NO or IV adenosine causes a reduction of >10 mmHg of mean pulmonary arterial pressure reduction without significantly reducing the cardiac output or BP.

Longterm may need lung or heart-lung transplant.

Question 21

standard treatment for type 1 primary pulmonary HTN

Answer 21

Also treat with maintaining euvolumia with diuretics if necessary and O2 supplementation if sPO2<88 or <90 with signs of right heart failure

Response to see if pt will benefit from vasodilators can be predicted by the vaso-reactivity testing in the Right heart catherization

positive when vasodilator like inhaled NO or IV adenosine causes a reduction of >10 mmHg of mean pulmonary arterial pressure reduction without significantly reducing the cardiac output or BP.

Question 22

primary treatment of primary arterial pulmonary HTN with positive vasoreactivity test

Answer 22

Treat with nifedipine or CCB

HR>100 use dilitazem

HR<100 use nifedipine

do not use CCB in patient has cardiac index <2 L/min

do not use verapamil. only useful for people who have don’t positive acute vasoreactivity to a vasodilator test

Positive vasoreactivity test to vasodilator challenge is when pulmonary vascular resistance was reduced by >20% after administration of epoprostenol and nitric oxide and adenosine

Question 23

what intervention can help with improve functional capacity for type 1 primary hypertension?

Answer 23

supervised exercise traning.

Question 24

can pulmonary HTN type 1 women get pregnant?

Answer 24

experts recommend against getting pregnant.

Question 25

what medical complication is associated with type 1 primary pulmonary HTN

Answer 25

seen with a higher risk for vascular thrombosis and embolism

And if being treated, current drug of choice is warfarin. Little experience with DOACs