Initial Workup
- Labs: CBC, BMP, LFTs, TSH, HIV, ANA, BNP, troponin
- V/Q scan: rule out CTEPH (Group 4)
- PFTs, Oximetry, HRCT: if ILD or OSA suspected
- Diuresis
- Oxygen goal: >92%
PH Classification
| Group | Etiology | Examples | Workup | Treatment |
|---|---|---|---|---|
| 1 | PAH | Idiopathic, CTD, HIV, drugs | ANA, HIV, LFTs, RHC (pre-capillary), V/Q scan | ERA, PDE5i, prostacyclins, diuretics, O2, CCB if vasoreactive |
| 2 | Left Heart Disease | HFrEF, HFpEF, valvular disease | TTE, BNP, RHC (post-capillary, wedge > 15) | Optimize HF, ACEi/ARB, BB, diuretics, valve repair/replacement |
| 3 | Lung Disease / Hypoxia | COPD, ILD, OSA, altitude | PFTs, oximetry, ABG, HRCT | Oxygen if hypoxic, treat lung disease, PAH meds usually not indicated |
| 4 | CTEPH | Prior PE, thrombophilia | V/Q scan, CT PA, RHC | Anticoagulation, PTE surgery, riociguat, BPA if inoperable |
| 5 | Multifactorial | Sarcoid, sickle cell, CKD | Tailored labs, imaging, biopsies | Treat underlying disease, PAH therapies case-by-case |
Gold standard to define PH is a mPAP > 20mmHg on right heart cath, but TTE has its utility - it is around 85% sensitive and 70% specific for detecting the presence of PH. TTE parameters that are measured are the following:
- Elevated systolic PA pressure (sPAP)
- Right heart dilation or dysfunction
- TR jet velocity >2.8 m/s
- Signs of right heart strain
PH Severity by RHC & TTE
| Severity | mPAP (RHC) | sPAP (TTE) |
|---|---|---|
| Normal | ≤ 20 mmHg | < 35 mmHg |
| Mild PH | 21–30 mmHg | 35–44 mmHg |
| Moderate PH | 31–40 mmHg | 45–59 mmHg |
| Severe PH | > 40 mmHg | ≥ 60 mmHg |
TTE Measurements
- Estimated RAP: 3-15mmHg based on IVC diameter and collapsibility: If <2.1cm and >50% collapse = 3mmHg, If >2.1cm or <50% collapse = 8mmHg, if >2.1cm and <50% collapse = 15mmHg
- TR peak velocity: ≥2.8 m/s → Suggests elevated pulmonary pressures
- Estimated RVSP: RVSP ≈ 4 × (TR peak velocity)² + RAP
- PH severity: Mild (35–44 mmHg), moderate (45–59 mmHg), or severe (≥60 mmHg)
RVSP Calculator
RVSP Severity Reference
| RVSP | Interpretation |
|---|---|
| < 35 mmHg | Normal |
| 35–44 mmHg | Mild PH |
| 45–59 mmHg | Moderate PH |
| ≥ 60 mmHg | Severe PH |
RHC Measurements
- PCWP (wedge pressure)
- ≤15 mmHg = pre-capillary (Groups 1, 3, 4)
- 15 mmHg = post-capillary (Group 2)
- PVR
- ≥3 Wood units + high PAWP = combined pre- and post-capillary (Group 2/5 overlap)
Pulmonary HTN Severity + PVR Calculator
mPAP Severity Reference
| mPAP (RHC) | Interpretation |
|---|---|
| ≤ 20 mmHg | Normal |
| 21-30 mmHg | Mild elevation |
| 31-40 mmHg | Moderate elevation |
| > 40 mmHg | Severe elevation |
PVR Severity Reference
| PVR | Interpretation |
|---|---|
| < 2 WU | Normal |
| 2.0-2.9 WU | Mild elevation |
| 3.0-5.0 WU | Moderate elevation |
| > 5.0 WU | Severe elevation |
PVR = (mPAP - PCWP) / CO
Group 1 (Pulmonary Arterial Hypertension – PAH)
Etiologies: idiopathic, connective tissue disease, HIV, drugs
Treatment:
- PDE-5 inhibitors: sildenafil 20 mg TID, tadalafil 40 mg daily
- Endothelin receptor antagonists: ambrisentan, macitentan
- sGC stimulator: riociguat 1–2.5 mg TID (avoid with PDE5i)
- Prostacyclins: epoprostenol (IV), treprostinil
- Consider vasoreactivity testing → CCBs if positive
- Refer to PH specialty center for advanced therapies
References
- Humbert, M., Kovacs, G., Hoeper, M. M., Badagliacca, R., Berger, R. M. F., Brida, M., Carlsen, J., Coats, A. J. S., Escribano-Subias, P., Ferrari, P., Ferreira, D. S., Ghofrani, H. A., Giannakoulas, G., Kiely, D. G., Mayer, E., Meszaros, G., Nagavci, B., Olsson, K. M., Pepke-Zaba, J., ... Rosenkranz, S. (2022). 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart Journal, 43(38), 3618–3731. https://doi.org/10.1093/eurheartj/ehac237
- Kovacs, G., Bartolome, S., Denton, C. P., Gatzoulis, M. A., Gu, S., Khanna, D., McLaughlin, V. V., Naeije, R., Preston, I. R., Sourani, H. A., Souza, R., Torbicki, A., Vonk Noordegraaf, A., & Humbert, M. (2024). Definition, classification and diagnosis of pulmonary hypertension. European Respiratory Journal, 64(4), 2401324. https://doi.org/10.1183/13993003.01324-2024
- McLaughlin, V. V., Archer, S. L., Badesch, D. B., Barst, R. J., Farber, H. W., Lindner, J. R., Mathier, M. A., McGoon, M. D., Park, M. H., Rosenson, R. S., Rubin, L. J., Tapson, V. F., & Varga, J. (2009). ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Journal of the American College of Cardiology, 53(17), 1573–1619. https://doi.org/10.1016/j.jacc.2009.01.004
- Mukherjee, M., Rudski, L. G., Addetia, K., Barker, P. C., Berkowitz, R., Branch, K. R., Dudzinski, D. M., Hahn, R. T., Hung, J., Khandheria, B. K., Lang, R. M., Mor-Avi, V., Muraru, D., Nagueh, S. F., Porter, T. R., Raza, F., Selton-Suty, C., Shah, S. J., & Tsang, W. (2025). Guidelines for the echocardiographic assessment of the right heart in adults and special considerations in pulmonary hypertension. Journal of the American Society of Echocardiography, 38(4), 295–342. https://doi.org/10.1016/j.echo.2025.01.007
- Rudski, L. G., Lai, W. W., Afilalo, J., Hua, L., Handschumacher, M. D., Chandrasekaran, K., Solomon, S. D., Louie, E. K., & Schiller, N. B. (2010). Guidelines for the echocardiographic assessment of the right heart in adults: A report from the American Society of Echocardiography. Journal of the American Society of Echocardiography, 23(7), 685–713. https://doi.org/10.1016/j.echo.2010.05.010
- Kim, N. H., Delcroix, M., Jais, X., Madani, M. M., Matsubara, H., Mayer, E., Ogo, T., Tapson, V. F., Ghofrani, H. A., Jenkins, D. P., & Lang, I. M. (2019). Chronic thromboembolic pulmonary hypertension. European Respiratory Journal, 53(1), 1801915. https://doi.org/10.1183/13993003.01915-2018
- Tunariu, N., Gibbs, S. J. R., Win, Z., Gin-Sing, W., Graham, A., Gishen, P., & Al-Nahhas, A. (2007). Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. Journal of Nuclear Medicine, 48(5), 680–684. https://doi.org/10.2967/jnumed.106.039438