Pulmonary Hypertension Workup Algorithm¶

flowchart TD
    A[Suspected PH<br/>TRJV >2.8 m/s or RV changes] --> B[Initial Evaluation]

    B --> B1[History & Physical]
    B --> B2[ECG]
    B --> B3[CXR]
    B --> B4[BNP/NT-proBNP]
    B --> B5[Echo with full PH protocol]

    B1 & B2 & B3 & B4 & B5 --> C{Echo suggests PH?}

    C -->|No - TRJV ≤2.8, normal RV| D[Low probability<br/>Consider other diagnoses]
    C -->|Yes - TRJV >2.8 or RV changes| E[Categorize by Echo]

    E --> F{PAWP estimate?}
    F -->|LA dilated, mitral disease| G[Suspect Group 2<br/>Left Heart Disease]
    F -->|LA normal, RV dominant| H[Suspect Pre-capillary PH]

    G --> G1[Optimize LV/Valve disease<br/>If refractory → Cath]

    H --> I[Additional Workup]
    I --> I1[PFTs + DLCO]
    I --> I2[V/Q Scan]
    I --> I3[CT Chest]
    I --> I4[Sleep Study if indicated]
    I --> I5[Autoimmune panel]
    I --> I6[HIV, Liver function]

    I1 & I2 & I3 & I4 & I5 & I6 --> J{Etiology identified?}

    J -->|Lung disease| K[Group 3 PH<br/>Treat underlying]
    J -->|V/Q mismatch| L[Group 4 CTEPH<br/>Anticoagulation, Consider surgery]
    J -->|None identified| M[Suspect PAH<br/>Right Heart Cath]

    M --> N[Right Heart Catheterization]

    N --> O{Hemodynamics}
    O -->|mPAP >20, PAWP ≤15,<br/>PVR >2 WU| P[Pre-capillary PH<br/>Confirmed PAH]
    O -->|mPAP >20, PAWP >15| Q[Post-capillary PH<br/>Group 2]

    P --> R[Vasoreactivity Testing]
    R --> S{Positive response?<br/>↓mPAP ≥10 to <40}
    S -->|Yes - Rare| T[Consider CCB therapy]
    S -->|No - Majority| U[PAH-Specific Therapy]

    U --> V[Risk Stratification]
    V -->|Low risk| W[Monotherapy<br/>PDE5i or ERA]
    V -->|Intermediate/High risk| X[Combination Therapy<br/>± Prostacyclin]

    style P fill:#ff6b6b
    style U fill:#ffd93d

📋 Text Version (if diagram doesn't render)

**PH Workup Algorithm** 1. **Initial Evaluation:** H&P, ECG, CXR, BNP, Echo with PH protocol 2. **Echo suggests PH?** (TRJV >2.8 or RV changes) - NO → Low probability, consider other diagnoses - YES → Categorize by echo 3. **PAWP Estimate:** - LA dilated/mitral disease → Group 2 (left heart) → Optimize, cath if refractory - LA normal/RV dominant → Suspect pre-capillary PH 4. **Additional Workup:** PFTs, V/Q scan, CT chest, sleep study, autoimmune panel, HIV/liver - Lung disease → Group 3 - V/Q mismatch → Group 4 CTEPH → Anticoagulation, consider surgery - None identified → Suspect PAH → Right heart cath 5. **RHC Hemodynamics:** - mPAP >20, PAWP ≤15, PVR >2 WU → Pre-capillary PAH confirmed - mPAP >20, PAWP >15 → Post-capillary (Group 2) 6. **Vasoreactivity Testing:** Positive (rare) → CCB; Negative (majority) → PAH-specific therapy

Classification Groups¶

Group	Cause	Examples
1	PAH	Idiopathic, heritable, CHD-associated
2	Left heart disease	HFpEF, HFrEF, valve disease
3	Lung disease/hypoxia	BPD, ILD, sleep apnea
4	CTEPH	Chronic thromboembolic
5	Unclear/multifactorial	Sarcoidosis, hematologic

Hemodynamic Definitions (2022)¶

Type	mPAP	PAWP	PVR
Pre-capillary	>20 mmHg	≤15 mmHg	>2 WU
Post-capillary	>20 mmHg	>15 mmHg	-

Pulmonary Hypertension Workup Algorithm¶

Classification Groups¶

Hemodynamic Definitions (2022)¶

PAH-Specific Medications¶

Pathway Targets¶

Monitoring Requirements¶