Uncommon Causes of Pulmonary Hypertension With Associated Cardiomyopathy: Computed Tomography and Magnetic Resonance Imaging of Cardiothoracic Manifestations

Abstract

Pulmonary hypertension (PH) is a disease characterized by pathologically increased pressure in the pulmonary arteries, defined by a mean pulmonary arterial pressure (mPAP) >20 mmHg at rest measured with right heart catheterization (RHC). This definition encompasses pathologies with very different pathological backgrounds, ultimately resulting in PH. For this reason, the latter can be possibly (though seldom) accompanied by cardiomyopathies, pathologies characterized by a structural and functionally abnormal myocardium not secondary to coronary disease, hypertension, valvular disease, or congenital heart disease. Notable examples of these diseases are sarcoidosis (a multi-systemic inflammatory granulomatous disease, possibly involving the lung and the heart), systemic sclerosis (SSc) (a connective tissue disease [CTD], possibly causing interstitial lung disease [ILD], direct as well indirect involvement of the cardiovascular system), and chronic kidney disease (CKD) (a progressive pathological process involving the kidneys, with multi-systemic involvement and possible development of a peculiar form of cardiomyopathy, i.e., uremic cardiomyopathy [UC]). The diagnostic work-up of patients with coexistent PH and cardiomyopathies implies the use of multiple imaging techniques, with computed tomography (CT) and cardiovascular magnetic resonance (CMR) being among the most important. The knowledge of CT and MRI findings, together with a suggestive clinical picture, forms the basis for a correct diagnosis, therefore it is important for the radiologist to recognize them in complex clinical scenarios. The advent of new technologies (e.g., photon counting detectors) and the development of new artificial intelligence (AI) algorithms will further pave the way for improved diagnostic processes (also regarding this kind of pathologies) as well as allowing to perform a better prognostic evaluation.