Pulmonary hypertension (PH) in children is a heterogenous disease of the small pulmonary arteries characterized by a progressive increase in pulmonary vascular resistance. Despite adequate medical therapy, long-term pressure overload is frequently associated with a progressive course leading to right ventricular failure and ultimately death. Invasive hemodynamic assessment by cardiac catheterization is crucial for initial diagnosis, risk stratification and therapeutic strategy. Although echocardiography remains the most important imaging modality for the assessment of right ventricular function and pulmonary hemodynamics, cardiovascular magnetic resonance (CMR) has emerged as a valuable non-invasive imaging technique that enables comprehensive evaluation of biventricular performance, blood flow, morphology and the myocardial tissue. In this review, we summarize the principles and applications of CMR in the evaluation of pediatric PH patients and present an update about novel CMR based concepts and imaging biomarkers that may provide further diagnostic, therapeutic and prognostic information.
Keywords: Pediatric pulmonary hypertension (pediatric PH); cardiovascular magnetic resonance imaging (CMR imaging); right ventricular dysfunction.
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