Clinical-radiological-pathological correlation in pulmonary arterial hypertension

Area of Science:

• Cardiology
• Pulmonology
• Radiology

Background:

• Pulmonary hypertension (PH) is defined as mean pulmonary arterial pressure >20 mmHg.
• Current guidelines classify PH into five distinct groups based on shared pathophysiology and clinical presentation.
• This paper initiates a series reviewing all PH classification groups, focusing on pulmonary arterial hypertension (PAH).

Purpose of the Study:

• To review the clinical, radiological, and pathological features of pulmonary arterial hypertension (PAH).
• To differentiate between various forms of PAH, including idiopathic, heritable, drug-induced, and associated conditions.
• To highlight the importance of radiological investigations, particularly computed tomography, in diagnosing PAH.

Main Methods:

• Literature review of clinical, radiological, and pathological data pertaining to PAH.
• Analysis of histopathological features, including pulmonary arterial abnormalities like plexiform lesions.
• Evaluation of the role of imaging modalities in PAH diagnosis and subtyping.

Main Results:

• PAH encompasses diverse etiologies, including associated medical conditions, family history, and specific exposures.
• While histopathological features are common, variations in pulmonary arterial abnormalities and venous involvement exist among PAH subgroups.
• Radiological findings are crucial for accurate PH diagnosis and require systematic interpretation.

Conclusions:

• PAH is a complex condition with varied underlying causes and distinct pathological characteristics.
• Accurate diagnosis and subtyping of PAH rely heavily on integrating clinical, pathological, and radiological findings.
• A systematic approach to interpreting imaging, especially CT scans, is essential for effective PAH management.