TABLE 1.
Scoring systems for detection of pulmonary hypertension (PH) in interstitial lung disease (ILD) and COPD
| First author, year [reference] | Subjects n | Scoring variables | Outcomes |
|---|---|---|---|
| ILD | |||
| Nathan, 2024# [132] | Derivation cohort: 481 IPF Validation cohort: 204 IPF |
FORD scoring system and FORD index: FVC%/DLCO% ratio Oxygenation nadir during 6MWT Race 6MWD |
PH (2022 ESC/ERS) AUC 0.75 Score ≥33 sensitivity 70% specificity 73% Score ≥53 specificity 98% |
| Joseph, 2023 [133] | 66 ILD | Gas exchange-derived PVC ΔETCO2 (CO measure) TTE sPAP Elevated FVC/DLCO |
PH (2022 ESC/ERS) AUC 0.94 sensitivity 0.86 specificity 0.93 |
| Parikh, 2022#
[134] Parikh, 2023 [135] |
Derivation cohort: 154 ILD Validation cohort: 161 ILD |
History Exam 6MWD DLCO CTA PA/A ratio BNP/NT-proBNP |
PH (6th WSPH) Score ≥6 (0–12) AUC 0.920 sensitivity 86.5% specificity 86.3% |
| Refini, 2021 [120] | 37 IPF | TTE sPAP PA area Diameter of the segmental artery to that of the adjacent bronchus in the apicoposterior segment of the left upper lobe ratio |
mPAP sensitivity 100% specificity 53% |
| Sobiecka, 2020 [136] | 93 ILD | Age 6MWD TLC/DLCO ratio |
Echocardiography sPAP AUC ROC 0.867 |
| Tello, 2019# [53] | 172 CLD 94 ILD 78 COPD |
TAPSE/sPAP ratio | Severe PH (6th WSPH) TAPSE/sPAP ratio 0.26 mm·mmHg−1 sensitivity 80.6% specificity 71.2% |
| Sonti, 2019 [42] | 105 IPF | sPAP FVC/DLCO PA/A ratio |
PH (6th WSPH) NPV 87.2% |
| Bax, 2018# [80] | Derivation cohort: 210 ILD Validation cohort: 61 ILD |
Stepwise echocardiographic algorithm | Severe PH (6th WSPH) Score ≥7 sensitivity 89% specificity 71% PPV 68% NPV 90% AUC 84.8% |
| Furukawa, 2018 [99] | 273 IPF |
DLCO PA/A ratio on CT ≥0.9 PaO2 <80 Torr |
mPAP AUC ROC 0.757 (95% CI 0.682–0.833) |
| COPD | |||
| Kovacs, 2022 [25] | 142 | TTE NT-proBNP PA/A diameter ratio |
Haemodynamically defined pre-capillary severe PH (6th WSPH) PPV 94% NPV 94% |
| Coste, 2019 [123] | 24 | CT-measured bronchial wall thickness % of cross-sectional area of PV <5 mm2 PaO2 |
Sensitivity of 87.5% for severe PH (6th WSPH) |
IPF: idiopathic pulmonary fibrosis; FORD: forced vital capacity (FVC)/diffusing capacity for of the lung for carbon monoxide (DLCO) ratio (F), oxygen saturation nadir during 6-min walk test (6MWT) (O), race (R) and distance ambulated during 6MWT (D); 6MWD: 6-min walk distance; ESC: European Society of Cardiology; ERS: European Respiratory Society; AUC: area under the curve; PVC: pulmonary vascular compliance; ΔETCO2: change in end-tidal carbon dioxide; CO: cardiac output; TTE: transthoracic echocardiography; sPAP: pulmonary artery systolic pressure; CTA: computed tomography angiogram; PA: pulmonary artery; A: aorta; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-BNP; WSPH: World Symposium on Pulmonary Hypertension; mPAP: mean pulmonary arterial pressure; TLC: total lung capacity; ROC: receiver operating characteristic; CLD: chronic lung disease; TAPSE: tricuspid annular plane systolic excursion; NPV: negative predictive value; PPV: positive predictive value; CT: computed tomography; PaO2: arterial partial pressure of oxygen; PV: pulmonary volume. #: includes a validation cohort.