Association between pulmonary artery to aorta diameter ratio with pulmonary hypertension and outcomes in diffuse cystic lung diseases

• Published in: Medicine (Baltimore) 2021-06, Vol.100 (25), p.e26483-e26483
• Main Authors: Baldi, Bruno Guedes, Fernandes, Caio Júlio César dos Santos, Heiden, Gláucia Itamaro, Freitas, Carolina Salim Gonçalves, Sobral, Juliana Barbosa, Kairalla, Ronaldo Adib, Carvalho, Carlos Roberto Ribeiro, Souza, Rogério
• Format: Article
• Language: English
• Subjects: Adult; Aorta - diagnostic imaging; Aorta - pathology; Cross-Sectional Studies; Echocardiography; Female; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Lung - blood supply; Lung - diagnostic imaging; Lung - pathology; Lung Diseases - complications; Lung Diseases - mortality; Lung Diseases - pathology; Lung Diseases - surgery; Lung Transplantation - statistics & numerical data; Male; Middle Aged; Observational Study; Predictive Value of Tests; Prognosis; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - pathology; Risk Assessment - statistics & numerical data; ROC Curve; Tomography, X-Ray Computed; Walk Test
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000026483

To investigate the importance of pulmonary vascular measurements on computed tomography (CT) in predicting pulmonary hypertension (PH) and worse outcomes in diffuse cystic lung diseases (DCLDs).We conducted a cross-sectional study of patients with DCLDs. Patients underwent pulmonary function tests, a six-minute walk test (6MWT), chest CT, transthoracic echocardiography, and right heart catheterization. Pulmonary artery (PA) diameter and PA-ascending aorta ratio (PA-Ao ratio) were obtained from CT. Mean pulmonary artery pressure (mPAP) from right heart catheterization was correlated with tomographic, functional, and echocardiographic variables. The association between the PA-Ao ratio with outcomes was determined by Kaplan-Meier curves.Thirty-four patients were included (18 with pulmonary Langerhans cell histiocytosis and 16 with lymphangioleiomyomatosis, mean age 46 ± 9 years). Forced expiratory volume in the first second and lung diffusing capacity for carbon monoxide were 47 ± 20% and 38 ± 21% predicted, respectively. PA diameter and PA-Ao ratio were 29 ± 6 mm and 0.95 ± 0.24, respectively. PA-Ao ratio > 1 occurred in 38.2% of patients. PA-Ao ratio was a good predictor of PH. mPAP correlated best with PA-Ao ratio, PA diameter, oxygen desaturation during six-minute walk test, and echocardiographic variables. Patients with PA-Ao ratio > 1 had greater mPAP, and a higher risk of death or lung transplantation (log-rank, P