Pulmonary Hypertension Overview And Treatment

PH has many underlying etiologies

The disease affects the left side of the heart

The hemodynamic definition of PH is a mean pulmonary arterial pressure (PAP) greater than or equal to 25 mmHg

Pulmonary arterial hypertension (PAH) is a subset of PH

The disease affects men much more than women

Non-specific symptoms such as fatigue and edema present clinically

Catheterization is required when PH is suspected

Underlying causes and PCWP or LVEDP are evaluated

Vasoconstriction is an issue

Vascular wall remodeling is an issue

There is a cure for PAH

The balance of vasoconstriction and vasodilation may indicate PAH

CCBs have a selective effect on vascular smooth muscle

Normal physiologic doses are sufficient for treatment purposes

Adverse effects must be considered

Long-term clinical response is less than 10%

The drug is a prostacyclin analog

There are two products: Flolan and Veletri

The drug improves mortality

The drug is selective

It has a longer half-life than epoprostenol

It is also shown to have a survival benefit

It has a higher cost than epoprostenol

It is stable at room temperature without a need for a back-up cassette.

It is a prostacyclin analog

True

False

It is a prostacyclin analog

It is approved for PAH WHO class 3 or 4 symptoms

Very few doses are given per day

There is a significant improvement in 6MWD and hemodynamics

It is an ET-1 receptor agonist

It is safe to use in pregnant women

It is highly selective for the endothelin A (ETa) receptor

It has many drug interactions

It is a non-selective ETa antagonist

Teratogenicity is an issue

Drug interactions are still concerning

It is safe to use in pregnant women

It is a tissue selective ET-1 receptor antagonist

Hepatotoxicity is a concern

Drug interactions are a cocnern

Sildenafil is shorter-acting while tadalafil is longer-acting

Sildenafil is TID dosing while tadalafil is QD dosing

Flushing and hypotension are two concerns with both drugs

Exercise ability is improved with both drugs

Nitrates can be safely used with both drugs

It is a soluble guanylate cyclase (sGC) stimulator

It can be used in patients with renal failure

Teratogenicity and hepatotoicity are concerns

There are many drug interactions

1.0 - 2.0

1.5 - 2.5

2.0 - 3.0

2.5 - 3.5

Medication safety

Optimizing medical regimens

Patient education and adherence

Quality improvement

There is a large amount of long-term data for the therapies of PAH

Prostacyclin dosage forms must be considered

Costs and convenience must be considered

Pharmacists are important in the therapy