Pulmonary Hypertension Basics
Recognize pulmonary hypertension, understand the groups, and know when (and to whom) to refer.
Learning Objectives
- 1.Recognize the presentation and screening of pulmonary hypertension.
- 2.Understand the WHO groups conceptually and why they matter.
- 3.Know the referral pathway and scope limits.
Overview
Pulmonary hypertension (PH) is elevated pressure in the pulmonary circulation with many causes. The APP role is recognition, basic workup, and timely referral — not starting advanced PAH therapy.
Why this matters
PH is often missed because dyspnea is non-specific. The cause (the WHO group) completely changes treatment, and Group 1 PAH needs specialist-directed therapy.
Recognizing PH
- •Progressive exertional dyspnea, fatigue, and later exertional syncope or edema.
- •Exam: loud P2, RV heave, signs of right heart failure (elevated JVP, edema, ascites).
- •Echo screens (estimated PA systolic pressure, RV size/function); confirmation requires right heart catheterization.
The WHO groups (concept)
- •Group 1: pulmonary arterial hypertension (PAH) — specialist-directed therapy.
- •Group 2: due to left heart disease (most common) — treat the heart disease.
- •Group 3: due to lung disease/hypoxia — treat the lung disease/hypoxia.
- •Group 4: chronic thromboembolic (CTEPH) — potentially treatable, needs specialist evaluation.
- •Group 5: multifactorial/other.
Workup & referral
- •Echo screen → if PH suspected, evaluate for left heart and lung causes, and consider CTEPH (V/Q scan) before labeling Group 1.
- •Refer suspected Group 1 PAH and Group 4 CTEPH to a PH specialist center.
- •Right heart catheterization confirms the diagnosis and hemodynamics — done by cardiology.
Scope note
Scope
Do not start advanced PAH-specific medications independently — these are specialist-directed and can be harmful in the wrong group. The APP recognizes, works up the cause, and refers.
Red flags / escalation
- •Signs of right heart failure / decompensation.
- •Exertional or warning-less syncope (high-risk in PH).
- •Rapidly progressive dyspnea.
Escalate
Syncope or right heart failure in suspected PH is high-risk — escalate and expedite specialist referral.
Common beginner mistakes
- •Labeling all PH as PAH and missing the common Group 2 (left heart) cause.
- •Starting PAH drugs without specialist confirmation of the group.
- •Missing CTEPH (a potentially curable cause).
- •Underestimating syncope risk in PH.
Mini cases
Dyspneic patient, echo shows elevated PA systolic pressure and a dilated, dysfunctional RV.
Next steps?
Show answer
Screen systematically for the cause — evaluate left heart disease (most common, Group 2) and lung disease (Group 3), and consider CTEPH (Group 4) with a V/Q scan before assuming Group 1. Refer to a PH specialist if Group 1/4 is suspected; confirmation needs right heart cath. Don’t start PAH drugs empirically.
A patient with suspected PH reports exertional syncope.
Significance?
Show answer
Syncope in PH is a high-risk feature suggesting significant disease — escalate and expedite specialist evaluation rather than routine follow-up.