Dr Vicky Kelly is a Consultant Clinical Psychologist and Service Lead for the Barts Heart and Thorax Centre Psychological Services at St Bartholomew’s Hospital. She set up this service in 2017, after working in paediatric cardiac psychology services at Great Ormond Street Hospital from 2009. Prior to this she worked in Paediatric Palliative Care, Cystic Fibrosis, Diabetes, and Child and Adolescent Mental Health Services.

Her research interests include anxiety, decision making around cardiac devices, and patient and family experience of invasive treatment. She is also interested in developing support structures for staff working in acute physical health settings. She enjoys collaborating with patients, families, and staff to develop the provision of psychologically informed healthcare.

Inherited cardiac conditions present unique psychosocial challenges for young people and their families (Caleshu et al., 2016). Diagnosis can occur against the distressing backdrop of a sudden death in the family meaning patients must grapple with a new diagnosis at the same time as grieving for a loved one. Or it can happen suddenly and traumatically in the context of an out of hospital cardiac arrest, leaving a family reeling with shock whilst experiencing on-going trauma, and having to come to terms with a new life-long condition.

It can also bring about the need for screening and genetic testing as other family members might be affected which can lead to additional worry and guilt for all affected (Potterton et al., 2024). Many patients might experience no, or mild symptoms yet suddenly find themselves plunged into having conversations about preventing the risk of sudden cardiac death by withdrawing from competitive sports and intense exercise, needing to take new medication, or having an implantable cardioverter defibrillator (ICD) inserted (Priori at al., 2013). And all of this is usually happening at a time when the focus for the young person concerned, is on having fun with friends and not thinking too far into the future. This can inevitably and completely understandably lead to a sort of “checking out” or avoidance of any conversation related to the heart which can result in increased anxiety from the family and medical systems that surround the young person.

To understand the psychological impact of these challenges, it is helpful to consider the developmental context. Late adolescence and young adulthood represent a significant developmental phase in our lives. According to Havighurst (1973), young adults are tasked with the daunting job of achieving autonomy and independence, establishing their identity, establishing emotional maturity, and starting to plan in terms of career choices, relationships, and considering parenthood. No easy task and I would hazard a guess that most of us in our forties and beyond can still not say we have achieved all those things! Discovering they have a cardiac condition can force young people to be more dependent on others (e.g. parents and family) at a time when they want to be testing out their independence. It can also bring about more uncertainty about what a cardiac condition means for their identity in amongst other questions that they might already be having about their gender identity, ethnicity, and sexual identity to name but a few.

From a cognitive perspective, the pre-frontal “brakes” are not yet fully developed and risk taking and pleasure-seeking behaviours are a big part of most people’s late teens and twenties. Having a cardiac condition increases the harm some of these behaviours might cause to physical health such as experimenting with drugs and alcohol, which can lead to increased feelings of isolation at a time when partying the night away feels like the norm for most of their peer group. Other executive functions including planning, organisation and the maturity of judgment may not fully develop until the mid-thirties. This can make complex decisions about medication, whether to have an ICD, and even just turning up to clinic appointments more of a challenge for this age group.

But that’s just what we know from clinical experience and the literature, what do the experts, i.e. the patients, say about all of this?

Imran is a 27-year-old man and has kindly agreed to co-author this article with me by letting me interview him about his experiences as a patient with Hypertrophic Cardiomyopathy (HCM).

VK: By way of introduction, can you tell us a bit about you and your cardiac condition please? What is it and when did you first get diagnosed?

IK: I have Hypertrophic Cardiomyopathy (HCM) which is a genetic heart condition that causes the walls of the heart to thicken and effectively reduces its ability to pump blood efficiently around the body. I was diagnosed with this condition in 2013 when I was 15 years old.

VK: And what can you remember about that time?

IK: It was quite a pivotal moment in my life, not only due to the medical changes, but the whole uncertainty with everything…it was a very surreal mixture of different feelings. I felt completely adrift with my thoughts, and I felt a lot of confusion from just trying to process everything. I didn’t know what the diagnosis meant for me, what kind of changes I would have to make, how would it impact me for the future, and what it meant for the people around me. I didn’t really have time to consider any of that.

VK: Yes, it sounds like it was, understandably, a very overwhelming time. How would you describe the psychological impact of having an inherited heart condition, particularly on young people?

IK: Ultimately, it differs for different people. I can’t speak for others, but I can give my insights. The most challenging psychological hurdle that I had to face (which I am still dealing with now) is just coming to terms with it and managing my expectations. I think that was the most difficult thing. As well as that, for me personally, I didn’t want to speak to anyone about this condition, there was a stigma around it, I kept things to myself.

VK: And how did that affect you, keeping things to yourself?

IK: It added to the feelings of isolation. I didn’t want to speak to anyone else because I didn’t want to burden them with what I was going through, but that meant I had to deal with that all by myself. Thinking about it now, that felt quite tough, especially to have to deal with that on your own at that age (15 years) with all the emotional weight of it. With a thing like a heart condition, it’s not something you can see, it’s all on the inside. This makes it harder to convey and people don’t really see everything that’s going on for you mentally and physically.

VK: And what skills, approaches and ideas have you been able to draw upon to manage this impact?

IK: I don’t think I managed it particularly well in the beginning! My coping mechanism was to just forget about it. I didn’t know how to process everything, nor did I want to talk to anyone about it, so I thought “I will just forget about it as best as I can”. And to an extent it worked a bit with some of the psychological aspects because I wasn’t actively thinking about it, but I don’t think it was the best approach… More recently I have had some psychological therapy and that was one of the biggest factors that helped me manage the condition, because it was just a space to reflect on everything and talk about things openly without being judged. And it helped me process things which I never had the opportunity to do before, in all honesty. And I think that’s helped me embrace all of this and think about what it really means.

VK: It sounds like it gave you that space, whereas before it felt too overwhelming. And are there other things that you did that helped?

IK: I think I have always tried to maintain a positive outlook on life. It gives you that hope and drive to overcome things.

VK: Yes, absolutely maintaining hope and a positive outlook sound like they are very helpful for you.

IK: And following on from the psychology sessions I had, it led me to have more open conversations with the people around me, which I hadn’t done before, and that kind of led me to building a good support network around me. Now I know I have people who I can reach out to if I am having difficult periods and that has made me feel less isolated compared to before.

VK: That is such lovely learning you are describing about the importance of reaching out. And it’s interesting because nothing has changed with what you are dealing with, but it sounds like you have more resources to draw on now.

What would you say now to young people who have just been diagnosed with an inherited heart condition?

IK: The first thing I would say, is it is honestly ok to feel whatever you might be feeing. You won’t have all the answers and don’t expect yourself to have all the answers. It’s a process of acceptance with no specific timeframe. Take your time, reach out to people. Try not to feel isolated.

VK: Is there anything you would do differently?

IK: I wish I had opened up more to those around me. I worried I was being a burden, but I probably wouldn’t have been, and I think that would have really helped me at the time, to work things through and help cope with everything. Because you have got to appreciate that you’re taking all that in and juggling life at the same time. It’s a lot.

VK: Yes, there are so many changes happening at that age.

And how can teams and charitable organisations help more?

IK: I think that there are some very helpful organisations out there but in terms of improvements, I found you must really go out and seek out information for yourself. That can feel overwhelming. Maybe the information sharing/signposting information could be better structured or more accessible. Maybe there needs to be more specific support, tailored for young people.

VK: Yes, navigating the support out there can be very challenging.

Maybe it would be better for the idea of support to be introduced as standard at the point of diagnosis?

For example, for cardiac teams to provide some sort of support structures that go hand in hand with your treatment journey, such as meeting support services at specific time points in your medical journey.

IK: Yes, I think that would be helpful.

I would like to take this opportunity to thank Imran for taking the time to contribute to this article and for articulating his experience of being diagnosed with HCM as a young person. He has provided useful insights into the types of understandable psychological responses that young people might expect to experience and ideas about how to help with them.

References

Caleshu C., Kasparian N.A., Edwards K.S., Yeates L., Semsarian C., Perez M., Ashley E., Turner C.J., Knowles J.W., Ingles J. (2016) Interdisciplinary psychosocial care for families with inherited cardiovascular diseases. Trends Cardiovasc Med. Oct;26(7):647-53.

Havighurst, R. J. (1973)

Chapter 1 – History of Developmental Psychology: Socialization and Personality Development through the Life Span. Life-Span Developmental Psychology (pp 3-24). BALTES, P. B. and SCHAIE, K. W. (Eds.)

Potterton, A., Shaughnessy, L., Wootton, M., Sharma, S., Wray, J. (2024) The psychological impact of the diagnostic pathway for inherited cardiac conditions in children and adolescents: A systematic review of the literature. Progress in Paediatric Cardiology (72) 1-12.

Priori, S.G., Wilde, A.A., Horie, M., et al. (2013) HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes: Document Endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm, 10, 1932-1963.