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CMR in unusual pathologies from around the world (with ASCI)

Non-invasive Imaging: Magnetic Resonance Imaging

This globetrotting session was kicked off by Carlos Rochitte from the Heart Institute on Endomyocardial Fibrosis. We heard that it is a restrictive cardiomyopathy presenting with ventricular apical filling defects, constituted of fibrotic tissue with thrombus and/or calcification. On late gadolinium enhancement images the typical finding is an apical fibrosis pattern described as a bright linear image usually in a letter “V” shape pointing to the ventricular apex (single V sing), and often associated to a dark “V” like shape, possibly corresponding to thrombus and/or calcification.

Dr Rochitte went so far to say that this finding is pathognomonic of endomyocardial fibrosis.

We then stayed in South America for an update on Chagas Disease by Gustavo Volpe from the University of Sao Paulo. This Trypansoma Cruzi or kissing bug disease is actually falling in incidence but remains an important disease in endemic areas.It affects the heart by causing inflammation and fibrosis with heart block, tachyarrythmias and in some cases a classic apical aneurysm +/- thrombus.
Fibrosis by LGE is associated with falling ejection fraction,  worsening symptoms and adverse prognosis, predicting cardiac  events.

Carmen Chan from Queen Mary Hospital talked us through hypertrophic cardiomyopathy and the influence of Asian ethnicity. There are several morphologies of this diverse disease – the typical asymmetric septal hypertrophy, concentric, apical and obstructive variants. The overall incidence of the apical variant is 16% but in the asian population it accounts for as much as a quarter of cases. In general, apical HCM has a relatively benign course (86% 5 year event free) compared to other morphologies, but if associated with apical aneurysm there is a higher incidence of complications such as atrial fibrillation, arrhythmia, heart failure and stroke.

Ahmed Gharib of the National Institute of Health highlighted some of the cardiac complications of HIV disease and the antiretroviral therapies used to treat it. While the destructive cardiomyopathy associated with AIDS in the era pre-ART has decreased in frequency dramatically cardiac disease is still the cause of death in 6.5% of people with HIV in America and the EU. He showed that having HIV was a similar independant risk factor for myocardial infarction when compared to hypertension or hyperlipidaemia. There is a relationship between CD4 count and coronary plaque burden in the young and old and coronary thickening was worse in those with a longer duration of anti-retroviral therapy.
The postulated reason for all this has been summarised in the phrase ‘Inflammaging’.

 Continuing the world tour we next visited Japan for a review of Kawasaki disease by Hajime Sakuma.
Many more cases have been diagnosed and managed in Japan than in Europe or North America confirming that race and ethnicity play an important role. Modern treatment has decreased the prevalence of coronary aneurysms from up to 30% down to 5% after high dose IV immunoglobulins.
Mulitmodality imaging including MRA, stress perfusion, LGE and strain MRI along with CT coroanry angiogram are all useful tools in diagnosis and management of Kawasaki disease but the lack of radiation exposure in MRI is useful in this population that are frequently followed up for many years.

The session was closed in South Africa by Ntobeko Ntusi who showed some incredible images of some of the complications of tuberculosis in the heart. TB is a major killer around the world and has a complex interaction with HIV with which there is frequent co-infection and has proved a particular issue in Sub-saharan Africa. TB myopercardial syndromes occur in up to 10% of those co-infected with HIV. The cases included tuberculous myopercarditis, constrictice pericarditis and a left atrial tuberculoma diagnosed on CMR imaging.
He highlighted the lack of MR studies in this condition and
revealed plans for future CMR studies of pathophysiology and outcome in these myopercardial syndromes and closed with a simple message: ‘in 2015 no-one should die of tuberculosis’.

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.