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Pulmonary Hypertension

Pulmonary hypertension occurs when there's an increase in mean pulmonary arterial pressure (NR ~ 15mmHg), which puts strain on the right side of the heart as it tries to pump blood through all that resistance. This can be caused by:

  • Parenchymal lung disease - COPD, Asthma, ILD, Bronchiectasis, CF

  • Pulmonary vascular disease - Idiopathic pulmonary htn, Pulmonary vasculitis, PE, Portal hypertension

  • Hypoventilation - Sleep apnoea, kyphosis/scoliosis, neuromuscular conditions (e.g. MG)

  • Left heart disease: LVF, mitral valve disease

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“An illustration depicting pulmonary hypertension” © BruceBlaus (Licensed under CC-BY 4.0) https://creativecommons.org/licenses/by/4.0/


Presentation

The main presenting symptom is SOB. Patients may also present with fatigue and syncope.


O/E - Tachycardia, Raised JVP, Peripheral oedema, Hepatosplenomegaly


Investigations

Key investigation to do here is an ECG, which will show changes consistent with R-sided heart strain and RVH. These include:

  • RAD

  • P Pulmonale - Big, tall, peaked P waves

  • ST depression and T-wave inversion (V1-4 and III)

  • Sinus tachycardia

  • RBBB

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Right ventricular strain pattern due to RVH - LITFL - https://litfl.com/right-ventricular-strain-ecg-library/

Gold-standard diagnostic test is Right Heart Catheterisation, which measures the mean pulmonary arterial pressure. Diagnosis made if pressure > 25mmHg.


Other investigations to do include:

  • Echo

  • CXR – Dilated pulmonary arteries, RVH


Management

The main aim here is to reduce pulmonary vascular resistance. To do this, patients can be given:

  • IV Prostanoids (e.g. epoprostenol)

  • Endothelin receptor antagonists (e.g. bosentan)

  • PDE-5 inhibitors (e.g. sildenafil)

  • Dihydropyridine CCBs (e.g. Nifedipine)


N.B. Dihydropyridine CCBs (e.g. amlodipine) work more on blood vessels, whilst Non-dihydropyridine CCBs (e.g. verapamil) work more on the heart.




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