The role of the combination of echo-HRCT score as a tool to evaluate the presence of pulmonary hypertension in idiopathic pulmonary fibrosis

Abstract

Pulmonary hypertension (PH) is defined as an elevated mean pulmonary artery pressure at rest (mPAP ≥ 25 mmHg), evaluated by right heart catheterization (RHC). The aim of the present study was to evaluate HRCT findings in relation to transthoracic echocardiographic data to better characterize PH in IPF patients and to identify a non-invasive composite index with high predictive value for PH in these patients. 37 IPF patients were enrolled in this retrospective study. All patients underwent a complete assessment for PH, including transthoracic Doppler echocardiography, HRCT scan and right heart catheterization. Right heart catheterization was done in 19 patients (51.3%) as pre-lung transplant assessment and in 18 patients (48.6%) to confirm PH, suspected on the basis of echocardiography. Twenty out of 37 patients (54%) were confirmed to have PH by RHC. Multivariate regression showed that the combination of sPAP, PA area measured by HRCT and the ratio of the diameter of the segmental artery to that of the adjacent bronchus in the apicoposterior segment of the left upper lobe was strongly correlated with mPAP (R2 = 0.53; p = 0.0009). The ROC analysis showed that 931.6 was the ULN for PA area, with 86% sensitivity and 61% specificity (0.839 AUC); 20.34 was the ULN for the ratio of PA area to ascending aorta diameter, with 100% sensitivity and 50% specificity (0.804 AUC). The composite index proposed in the present study could help early detection of IPF patients suspected of PH requiring confirmation by RHC (if deemed clinically necessary).

Utility of Computed Tomographic Angiography for Pulmonary Hypertension Assessment in a Cohort of West Highland White Terriers With or Without Canine Idiopathic Pulmonary Fibrosis

“…In human medicine, HRCT is commonly used in patients with lower airway disease (17), and in addition to specifying the cause of the disease, HRCT with computed tomographic angiography (CTA) can identify comorbidities with repercussions on prognosis such as PH (18). In humans, diameter of the pulmonary trunk (PT), pulmonary trunk-to-aorta ratio (PT/Ao), and right ventricle-to-left ventricle ratio (RV/LV) are measured alone or in combination to assess the presence of PH (19)(20)(21).…”

Management of Giant Pulmonary Artery Aneurysm with Quadricuspid Valve Stenosis

“…6 There are various etiological factors responsible for PAAs such as congenital heart disease, vasculitis (like Behcet's disease), infections (bacterial, tuberculosis, syphilis), malignancy, connective tissue disorders (Marfan or Ehlers-Danlos syndrome), pulmonary valve abnormalities, chronic pulmonary embolism, pulmonary hypertension, and idiopathic dilatation. 7 In our patient, we observed a quadricuspid pulmonary valve, a rare congenital abnormality, that is reported in 0.2% of 3861 donor hearts. 8,9 About two-thirds of these valves have a hypoplastic supernumerary cusp 10 that causes pulmonary regurgitation and eventually may lead to a PAA.…”

“…There are various etiological factors responsible for PAAs such as congenital heart disease, vasculitis (like Behcet's disease), infections (bacterial, tuberculosis, syphilis), malignancy, connective tissue disorders (Marfan or Ehlers–Danlos syndrome), pulmonary valve abnormalities, chronic pulmonary embolism, pulmonary hypertension, and idiopathic dilatation. 7…”

Vascular remodeling of the small pulmonary arteries and measures of vascular pruning on computed tomography

“…This may include the integration of multiple imaging modalities, 41 as studies have demonstrated that combining CT-based measures of pulmonary hypertension with echocardiography, for example, may be superior to either method in isolation. 42,43 In conclusion, in this study of individuals who underwent resection for early-stage lung adenocarcinoma, more severe pulmonary vascular pruning on CT was associated with greater small vessel pulmonary arterial remodeling on histology. These findings provide additional insight into the relationship between the pulmonary vessels' radiographic appearance and underlying histologic vasculopathy and suggest CT imaging may be a non-invasive indicator of pulmonary vascular disease.…”

“…This may include the integration of multiple imaging modalities, 41 as studies have demonstrated that combining CT-based measures of pulmonary hypertension with echocardiography, for example, may be superior to either method in isolation. 42 , 43 …”