Pulmonary hypertension & right heart failure

15 important questions on Pulmonary hypertension & right heart failure

How can pulmonary hypertension be measured?

By right heart catheterization (inserted in vein, flow-directed through the right atrium and ventrile, to reach the pulmonary artery).

What levels for the mean pulmonary arterial pressure (mPAP) are considered to be pulmonary hypertension?

MPAP > 20 mmHg

What distinction is being made in pulmonary hypertension?

Pre- and post-capillary hypertension.
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What types of pulmonary hypertension (HP) can be distinguished?

  • Arterial: pulmonary arterial hypertension (PAH) Rare, can be heritable
  • PH associated with lung disease (severe/mild) Common
  • PH associated with pulmonary artery obstructions
  • PH associated with left heart disease Very common
  • Unclear/multifactorial

When is pulmonary hypertension diagnosed as PAH?

- If the pulmonary artery pressure is increased
- If the LV is normal

What does PAH cause to the blood vessel?

It is more narrow.

How can PAH cause right heart problems?

PAH → increased pressure overload (RV has to put up to a higher pressure) → RV hypertrophy → RV dilatation → right heart failure

How can RV adaptation be assessed?

  • Right heart catheterization
  • Cardiac MRI
  • Echocardiography
  • Pressure volume analysis

What does increased RV pressure overload because of pulmonary hypertension cause on top of RV hypertrophy?

It also causes increased RV systolic and arterial elastance, and RV-arterial uncoupling in end-stage disease.

Why do PAH patients have no contractile reserve (despite the RV being hypercontractile, due to increased force-generating capacity of the cardiomyocytes)?

Due to
- RV-arterial uncoupling
- RV fibrosis → RV stiffness

What causes RV fibrosis in PAH patients?

Reduced phosphorylation of titin.

What is the consequence of RV diastolic stiffness for the right atrium?

The RA is dilated in PAH patients.

What causes the inefficient RA function in PAH patients?

The RA stroke work is increased (due to dilation), but there is no change in RV filling (due to stiffness).

What does eventually happen to the RA in PAH patients?

The RV stiffness causes RA stiffness (fibrosis) and lower contractility → RA uncoupling from RV → RA hypertrophy → RA dilatation becomes more pronounced than RV dilation.

What changes are there in RA sarcomeric function in PAH patients?

There are no changes.

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