Pulmonary Hypertension Overview – Morrow

Based on 15 September 2016 AFP:

A patient has an echocardiogram as part of the evaluation for heart failure, and the PAP is 80.  What can the FP do?

Definition:  Pulm artery pressure > 25 mmHg.

Epidemiology:  20-80% of patients with L HF have PH.  Patients with acute PE, 4% have PH in the acute phase.

There is no role for screening for PH, except in some patients with systemic sclerosis (scleroderma).

Five types of pulmonary hypertension:

  1. Pulmonary arterial hypertension (PAH) – formerly known as primary pulmonary hypertension.  This is PH not caused by something else.
    1. The only type that the vasodilator drugs have strong evidence of efficacy for.
  2. Pulmonary hypertension secondary to L heart disease, systolic/diastolic dysfunction and valvular heart disease.
  3. Pulmonary hypertension secondary to lung disease (COPD, interstitial lung disease, OSA).
    1. If PAP is < 60mmHg, they should be on supplemental O2 to keep sats 92-94%.
  4. Pulmonary hypertension secondary to chronic VTE.
    1. Targeted treatment is pulmonary endarterectomy – can be curative.  Lifelong anticoagulation.
  5. Multifactorial PH
    1. sickle cell, systemic CTD, metabolic disorders, others…
    2. Tx:  hydroxyurea, transfusion

FPs can have the biggest role in optimizing treatment of Group 2 and 3 above!


  • Dyspnea (especially on exertion)
  • Fatigue
  • Angina
  • Syncope


  • Incidentally found on echo for other reason
  • Low SaO2
  • JVD
  • TR murmur
  • Ascites
  • Lower extremity edema
  • EKG:  RBBB, R axis deviation

Initial Evaluation:

  • CXR
  • EKG:  S1/Q3/T3, RBBB, RAE
  • PFTs
  • Sleep study
  • HIV, ANA, BNP, transaminases
  • Functional status:  6min walk

Make sure patients are up to date on their immunizations!

Do a good preoperative eval (functional status, recent echo, PFTs) prior to surgery!

Three big classes medication:

  1. Prostacyclins
  2. Endothelin receptor antagonists – bosentan, ambrisentan
  3. PDE-5 inhibitors:  sildenafil, tadalafil



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