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Overlapping Phenotypes

Challenging cases

Non-invasive Imaging: Magnetic Resonance Imaging

This session was one the most well attended sessions of Euro CMR 2014, and a well-received addition to the programme.
The clinical focus of the session aimed to shed light on the challenges we face as imagers on a daily basis – are the images we are reviewing demonstrating a normal variant or are they indicative of a structural cardiomyopathy?
Frequently the findings are subtle, and often the reason the patient has been referred for CMR is for a definitive answer based on an inconclusive or abnormal echo- is this HCM or simply hypertensive heart disease; is this dilated heart due to athletic remodelling or mild DCM; and could this be ARVC or even mild LVNC.
The implications of any one of these diagnoses on a medical report are vast and a false positive or false negative diagnosis carries huge implications.
The 1st of the presentation was given by Dr Rory O’ Hanlon of Ireland, who has a particular interest in the field of sports cardiology and cardiomyopathy. He highlighted a number of key points in his practice to help differentiate an athlete’s heart from either mild DCM, or HCM- the high resting SV, symmetrical dilatation of all 4 chambers, low normal EF, and of course the absence of LGE. When doubt remains, detraining may be an option and re scan. Clinical information is key however- the ethnicity of the athlete, the volume of training, the age of the athlete, and of course whether the athlete has presented with symptoms or a known family hx vs a routine pre participation screen, as all of this ensures a more complete interpretation of the scan.
This fact was highlighted throughout the meeting and in particular by Professor Bill Mc Kenna, that imaging for many of the cardiomyopathies has to be interpreted in the clinical context. Dr O’ Hanlon showed some lovely cases of athletes with pathology with enthusiastic audience interaction.
The next presentation was by Dr James Moon discussing the many challenges of differentiating “true” left ventricular non compaction from hypertrabeculation due to ethnicity, ventricular remodelling, or simply normal variants. There were two key points he raised. The first was that the advent of higher resolution imaging, we are seeing detail in trabeculae never seen before, potentially leading to a vast overcall of LVNC. He summarised a large number of studies on the topic from echo and CMR with large numbers of proposed criteria, a fact, he said, that reflects the challenges we face to separate normal from abnormal. Novel work from his unit by Dr Gaby Captur, is likely to move the field on significantly. James again emphasised the important point of restraint in putting a diagnosis of LVNC in a report owing to the familial and clinical implications this may have for a patient.
The 3rd of the presentation was given by Dr Oliver Bruder on how CMR can be used to differentiate HCM from hypertensive heart disease. The audience were fortunate to get a sneak peak at the updated numbers from Dr Bruder’s EuroCMR registry, which now has data on over 34K patients. The clinical question around cardiomyopathy/myocarditis accounts for over a third of referrals for CMR across Europe. Dr Bruder highlighted work from his and other units on the prognostic importance of LGE in HCM. There remains overlap between HCM and hypertensive patients and differentiating them remains challenging- where family hx, the ECG, the presence of symptoms, are all helpful additions in the referral to aid an optimal CMR interpretation. One study by Rudolph et al, has demonstrated fibrosis in HTN patients, but for others, it would be the presence of mid wall fibrosis in a thick heart that is most likely to point towards HCM rather than hypertensive heart disease.
The last of the speakers was Massimo Lombardi, whose relaxed and entertaining presenting style was a pleasure to be around. He presented a case of biventricular dilatation and reduced LV and RV ejection fraction in a high level basketball player scanned twice over a 5 year period and challenged the audience many times to give a diagnosis- ARVC? ALVC? Biventricular arrhythmogenic cardiomyopathy? Or athletic remodelling? The case raised much discussion from the co-chair Cristina Basso regarding the patient’s ECG interpretation, which we were assured was completely normal by Massimo. Dr Moon, co-chair, then raised an interesting hypothesis- why not label simply as DCM, and therefore open up options for proven medical therapies like ACEI and BB for the patient.
This talk generated a lot of audience participation and debate, once again emphasising the challenges we face on a daily basis in scan interpretation in subtle and overlapping conditions.


Overlapping Phenotypes - Challenging cases

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.