Pulmonary Hypertension

Group 1: idiopathic, HIV, drug induced (meds for weight loss), connective tissue disease, portal hypertension, congenital heart dz, schistosomiasis Group 2: Related to left heart failure (HFpEF, HFrEF), valvular heart disease Group 3: Related to copd, restrictive lung dz, ILD, OSA, hypoxia without lung dz Group 4: Related to chronic thrombo-embolic disease Group 5: Unclear etiology, multifactorial, other: e.g. sarcoid, hematologic (e.g. p vera, essential thrombocytosis), other

Your Path

Pulmonary Hypertension

Symptoms

Can include: SOB, DOE, chest pain, syncope, other related to underlying process/associated disorder

Physical Exam Findings

Findings related to underlying disorders (if present)
Sometimes low O2 sat, abnormal VS (if uncompensated)
Elevated JVP, edema, murmur TR

Sub-Diseases

Pulmonary Arterial Hypertension (idiopathic)

HIV

Portal Hypertension

Lupus (SLE)

Mixed Connective Tissue D/o (MCT)

Rheumatoid Arthritis (RA)

Scleroderma

Heart Failure

Obstructive Sleep Apnea (OSA)

COPD

Diffuse Parenchymal Lung Disease - DPLD (ILD)

Pulmonary Emboli

Tests

TTE is typically first test to identify PAH
Chem, LFTs, cbc, bnp, TSH, ANA, HIV
PFTs, EKG
CXR, CT (high resolution to assess for parenchymal dz)
V/Q if concern chronic VTE
Sleep study if concern OSA
Serologies for specific connective tissue disorders (as indicated), heaptitis
Right heart cath for definitive dx, assess impact vasodilator therapies