Pulmonary Hypertension in Chronic Lung Diseases: What Role Do Radiologists Play?
Adele Valentini,
Paola Franchi,
Giuseppe Cicchetti,
Gaia Messana,
Greta Chiffi,
Cecilia Strappa,
Lucio Calandriello,
Annemilia del Ciello,
Alessandra Farchione,
Lorenzo Preda,
Anna Rita Larici
Affiliations
Adele Valentini
Division of Radiology, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy
Paola Franchi
Department of Diagnostic Radiology, G. Mazzini Hospital, 64100 Teramo, Italy
Giuseppe Cicchetti
Advanced Radiodiagnostic Center, Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, 00168 Rome, Italy
Gaia Messana
Diagnostic Imaging Unit, Department of Clinical, Surgical, Diagnostic, and Pediatric Sciences, University of Pavia, 27100 Pavia, Italy
Greta Chiffi
Secton of Radiology, Department of Radiological and Hematological Sciences, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
Cecilia Strappa
Secton of Radiology, Department of Radiological and Hematological Sciences, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
Lucio Calandriello
Advanced Radiodiagnostic Center, Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, 00168 Rome, Italy
Annemilia del Ciello
Advanced Radiodiagnostic Center, Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, 00168 Rome, Italy
Alessandra Farchione
Advanced Radiodiagnostic Center, Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, 00168 Rome, Italy
Lorenzo Preda
Division of Radiology, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy
Anna Rita Larici
Advanced Radiodiagnostic Center, Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, 00168 Rome, Italy
Pulmonary hypertension (PH) is a pathophysiological disorder, defined by a mean pulmonary arterial pressure (mPAP) > 20 mmHg at rest, as assessed by right heart catheterization (RHC). PH is not a specific disease, as it may be observed in multiple clinical conditions and may complicate a variety of thoracic diseases. Conditions associated with the risk of developing PH are categorized into five different groups, according to similar clinical presentations, pathological findings, hemodynamic characteristics, and treatment strategy. Most chronic lung diseases that may be complicated by PH belong to group 3 (interstitial lung diseases, chronic obstructive pulmonary disease, combined pulmonary fibrosis, and emphysema) and are associated with the lowest overall survival among all groups. However, some of the chronic pulmonary diseases may develop PH with unclear/multifactorial mechanisms and are included in group 5 PH (sarcoidosis, pulmonary Langerhans’ cell histiocytosis, and neurofibromatosis type 1). This paper focuses on PH associated with chronic lung diseases, in which radiological imaging—particularly computed tomography (CT)—plays a crucial role in diagnosis and classification. Radiologists should become familiar with the hemodynamical, physiological, and radiological aspects of PH and chronic lung diseases in patients at risk of developing PH, whose prognosis and treatment depend on the underlying disease.
