Version July 2025
| Clinical variable | Lower risk | High risk |
| Clinical evidence of RV failure | No | Yes |
| Progressive symptoms | No | Yes |
| WHO functional class | I to II | III to IV |
| Growth | Normal | Poor weight gain and/or growth failure (ie, height or weight Z-score <-2) |
| BNP or NT-proBNP | Normal or minimally elevated | Markedly elevated and/or rising |
| 6MWD (for children >6 years old) | ≥350 meters | <350 meters |
| Echocardiography | Little to no RV enlargement or dysfunction No pericardial effusion | Considerable RA/RV enlargement Reduced LV size Increased RV/LV ratio Reduced TAPSE Low RV FAC Pericardial effusion |
| Cardiac MRI (if obtained) | RVEF ≥45% | RVEF <45% |
| Hemodynamics measured by cardiac catheterization | PVRI ≤10 WU•m2 mRAP <10 mmHg Systemic CI ≥2.5 L/min/m2 mPAP/mSAP ratio ≤0.75 Systemic venous saturation ≥65% PACI ≥0.9 mL/mmHg/m2 Reactive AVT | PVRI >11 WU•m2 mRAP >10 mmHg Systemic CI <2.5 L/min/m2 mPAP/mSAP ratio >0.75 Systemic venous saturation <65% PACI <0.9 mL/mmHg/m2 |
This stable summarizes the approach to severity assessment in children with PH based upon the clinical criteria listed above. Categorization as lower versus high risk is not precise and is based in part on the clinical judgment of the treating clinician. For the lower-risk category, most if not all of the criteria should be met. For the high-risk category, patients typically meet multiple criteria, though all criteria need not be met. Some patients have intermediate findings and do not fall clearly into a lower- or high-risk category.
This table is intended for use in conjunction with additional UpToDate content on PH in children. Refer to UpToDate topics on the evaluation and management of PH in children for additional details.
