pulmonary HTN

Question 1

define?

Answer 1

elevated mean pulmonary arterial pressure >20 mmHg at rest (normal is between 10-14)

Question 2

etiology?

Answer 2

1. pulmonary arterial hypertension: hereditary (BMPR2 loss of function mutation)
2. left heart disease: CHF, valvular heart disease
3. chronic lung diseases: COPD, obstructive sleep apnea, interstitial lung disease
4. chronic thromboembolic pulmonary hypertension due to recurrent PE

Question 3

clinical features?

Answer 3

common: syncope, SOB on exertion, chest pain

high JVP, signs of RHF, loud and palpable second heart sound, parasternal heave due to RVH

Question 4

diagnosis?

Answer 4

non invasive initial test from high clinical suspicion: echocardiogram to identify markers of high pulmonary artery pressure

invasive confirmatory: right heart catheterization will determine mPAP >20 mmHg is diagnostic

Question 5

treatment?

Answer 5

assess for shock and treat acute decompensated cases.

long term management focuses on treating underlying cause
PAH: give CCB for pulmonary vasodilation
left heart disease: treat CHF accordingly (diuretics, ARNI, ACEI) and treat valvular disease
chronic lung disease: long term oxygen therapy, consider lung transplant
CTEPH: pulmonary endarterectomy