Volume 42, Issue 2 e70103
REVIEW

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

Emanuele Muscogiuri

Corresponding Author

Emanuele Muscogiuri

Department of Radiology, UZ Leuven, Leuven, Belgium

Correspondence: Emanuele Muscogiuri ([email protected])

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Valerie Van Ballaer

Valerie Van Ballaer

Department of Radiology, UZ Leuven, Leuven, Belgium

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Walter De Wever

Walter De Wever

Department of Radiology, UZ Leuven, Leuven, Belgium

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Emanuele Di Dedda

Emanuele Di Dedda

Department of Radiology, UZ Leuven, Leuven, Belgium

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Adriana Dubbeldam

Adriana Dubbeldam

Department of Radiology, UZ Leuven, Leuven, Belgium

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Laurent Godinas

Laurent Godinas

Department of Pneumology, UZ Leuven, Leuven, Belgium

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Marion Delcroix

Marion Delcroix

Department of Pneumology, UZ Leuven, Leuven, Belgium

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Jan Bogaert

Jan Bogaert

Department of Radiology, UZ Leuven, Leuven, Belgium

Department of Imaging and Pathology, KU Leuven, Leuven, Belgium

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First published: 14 February 2025

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.

Conflicts of Interest

The authors declare no conflicts of interest.

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