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Pulmonary hypertension management decision support

Pulmonary Medicine

Pulmonary Hypertension Management

Workflow for the outpatient management of patients with pulmonary hypertension.

Contributors

Abhimanyu Chandel, MD MAS
Christopher Thomas, MD
Oksana Shlobin, MD
Vikramkit Khangoora, MD
Christopher S. King, MD
Steven D. Nathan, MD

Patient Inputs

Enter available data. The tool computes hemodynamics and risk strata, then proposes therapy options.

Assessment moment WHO clinical group mPAP (mmHg) PAWP (mmHg) PVR (WU) WHO functional class Class I: no limitation. Class II: slight limitation. Class III: marked limitation. Class IV: symptoms at rest or with minimal activity. 6MWD (meters) BNP (pg/mL) Usual non-acute reference is often <100 pg/mL (lab-dependent). NT-proBNP (ng/L) Usual non-acute reference is often <125 ng/L (<75 years) or <450 ng/L (>=75 years), lab-dependent. Systolic BP (mmHg) Platelets (x10^3/uL) Enter 0-1000. Example: 250 means 250,000/mm3.

Risk Score Inputs (WHO Group 1 only)

Choose a risk model, then use quick-click categories or exact values. Available options change by assessment stage.

Score strategy Initial: REVEAL 2.0 or simplified ESC/ERS-style 3-variable baseline estimate. Follow-up: REVEAL Lite 2, COMPERA 2.0 4-risk strata, or French noninvasive criteria.

WHO functional class

PAH subgroup

Age / sex

The lower-risk REVEAL age/sex category is selected by default; click the alternate box if the patient is a man older than 60 years.

Renal status

Clicking updates eGFR and the renal-status field below.

Heart rate

Clicking fills the exact heart-rate field used by REVEAL calculators.

Systolic BP

Hospitalization

6MWD

BNP

NT-proBNP

Pericardial effusion

DLCO

mRAP

PVR

This fills the exact hemodynamic PVR field above.

Advanced / exact values

Heart rate (bpm) eGFR (mL/min/1.73m2) DLCO (% predicted) Mean right atrial pressure (mRAP/RAP, mmHg)

Clinical Policy Layer

This controls how the app places sotatercept/Winrevair within escalation pathways. The active policy version is shown below the application.

Sotatercept placement mode Choose whether sotatercept is surfaced broadly after low-risk failure or held for higher-risk branches only.

Hepatic impairment Renal status Used for broad medication safety flags. Auto-filled from eGFR in REVEAL models: >=60 normal/mild, 30-59 moderate, <30 severe. Final dose decisions remain drug-specific.

Current Group 1 PAH Regimen

Follow-up escalation only. Select ongoing therapies to tailor add-on or escalation recommendations.

Group 4 CTEPH / CTED Workflow

Use the ALA/PHA guidance-to-guidelines CTEPH pathway to clarify operability, BPA candidacy, and the post-PTE branch before surfacing riociguat.

Operability status at expert CTEPH center Post-PTE status BPA eligible? PVR >4 WU when sequencing BPA? Per the ALA/PHA consensus CTEPH pathway, PVR >4 WU supports using medical therapy/riociguat before staged BPA.

Phenotype Modifiers

Use these to adjust pathway selection when phenotype is atypical or condition-specific.

Major cardiopulmonary comorbidity phenotype Typical scenario: older/mixed phenotype with significant cardiac or lung comorbidity where monotherapy-first escalation is often safer. Vasoreactivity testing indicated phenotype Use for IPAH/HPAH/drug-associated PAH scenarios where acute vasoreactivity testing is guideline-relevant. Positive vasoreactivity test Use only if a formal vasoreactivity test was positive; this opens the high-dose CCB branch. PH associated with ILD phenotype Enables the PH-ILD pathway. Inhaled treprostinil is only surfaced when mPAP is ≥25 mmHg and PVR is >3 WU. Severe ILD-PH phenotype Use with ILD-associated PH when severe disease is present; allows optional PH-center PDE5 consideration. Signs of right-heart failure Examples: worsening edema, ascites, elevated JVP, hepatomegaly, or decompensated RV failure features. Clinical volume overload / congestion Triggers decongestion guidance (diuresis) with electrolyte/renal monitoring recommendations. This is auto-checked when PAWP is >15 mmHg.

Safety and Interaction Modifiers

These directly affect contraindication checks and medication filtering.

Pregnant or trying to conceive Triggers pregnancy safety guardrails, including ERA/riociguat restrictions. Current nitrates or NO donor therapy Flags contraindications with PDE5 inhibitors and riociguat (for example nitroglycerin or isosorbide dinitrate). On strong CYP2C8 inhibitor (for example gemfibrozil) Used for interaction hard-stops, especially for selexipag. On clopidogrel Adds selexipag dose-frequency caution in the validation step.

Decision Output

Recommendations can be collapsed for viewing or cleared without resetting the patient form.

No analysis yet.

References & Evidence

Open references used to frame this build

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
2024 WSPH definition, classification and diagnosis of pulmonary hypertension
2024 WSPH treatment algorithm for pulmonary arterial hypertension
Sildenafil / Revatio prescribing information (DailyMed)
Bosentan prescribing information (DailyMed)
Apixaban / Eliquis prescribing information (DailyMed)
Rivaroxaban / Xarelto prescribing information (DailyMed)
2026 NEJM trial: apixaban vs rivaroxaban for acute venous thromboembolism
2021 NEJM INCREASE trial: inhaled treprostinil in PH-ILD
2019 CHEST guideline update for therapy of pulmonary arterial hypertension in adults
Sotatercept / Winrevair FDA label (2025 update)
American Lung Association / PHA guidance-to-guidelines document (Nov 2024)
Risk tool intent in this build: initial Group 1 uses REVEAL 2.0 or simplified ESC/ERS-style 3-variable baseline estimate; follow-up Group 1 uses REVEAL Lite 2, COMPERA 2.0 4-risk strata, or French noninvasive criteria.