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 Tissue composition changes such as fibrosis, edema, or infiltration are frequent features in myocardial diseases  Cardiac imaging modalities offer the ability to characterize myocardial tissue to varying extent  Cardiovascular magnetic resonance offers comprehensive myocardial tissue characterization providing various diagnostic and prognostic imaging biomarkers  Advanced cardiac imaging is expected to become an integral part in risk-stratification and personalized medicine ABSTRACT Myocardial fibrosis, either focal or diffuse, is a common feature of many cardiac diseases and is associated with a poor prognosis for major adverse cardiovascular events. While histological analysis remains the gold standard for confirming the presence of myocardial fibrosis, endomyocardial biopsy is invasive, suffers from sampling errors, and is not practical in the routine clinical setting. Cardiac imaging modalities offer non-invasive surrogate biomarkers not only for fibrosis but also for myocardial edema and infiltration to varying degrees, and have important roles in the diagnosis and management of cardiac diseases. This review summarizes important pathophysiologic features in the development of commonly-encountered cardiac diseases, and the principles, advantages and disadvantages of various cardiac imaging modalities (echocardiography, single photon emission computer tomography, positron emission tomography, multi-detector computer tomography, and cardiovascular magnetic resonance imaging) for myocardial tissue characterization, with an emphasis on imaging focal and diffuse myocardial fibrosis.

Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. Despite early detection of CHD and major advances in paediatric cardiac surgery, ES is still prevalent and requires a multidisciplinary approach by adult CHD experts in tertiary centres. Central cyanosis is the primary clinical manifestation leading to secondary erythrocytosis and various multiorgan complications that increase morbidity and affect quality of life. Close follow-up is needed to early diagnose and timely manage these complications. The primary goal of care is to maintain patients’ fragile stability. Although the recent use of advanced PAH therapies has substantially improved functional capacity and increased life expectancy, long-term survival remains poor. Progressive heart failure, infectious diseases and sudden cardiac death comprise the main causes of death in patients with ES. Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. Endothelin receptor antagonists are used as first-line treatment in symptomatic patients, while phosphodiesterase-5 inhibitors may be added. Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. Additional well-designed trials are required to assess the comparative efficacy of various PAH agents and the benefit of combination therapy. Finally, the development of a risk score is of utmost importance to guide clinical therapy.