HEART DISEASE UPDATE 2006

By S. E. Brownlie

Heart testing sessions have been held twice during the summer of 2006. The whole weekend of 23rd to 25th June was devoted to heart-testing, on the Friday evening and Saturday at Peters Farm in Sussex and on the Sunday at the Grazely Rally.

A total of 31 hounds were examined that weekend, 24 bitches and seven dogs. Four hounds had atrial fibrillation but all had been previously diagnosed and were presented for check-up. As usual there were some hounds which were in the "equivocal" category, either because of mild ECG or ultrasound abnormalities, or because the examination was unsatisfactory or incomplete. The weather was glorious but this is not always good news for heart-testing as hot dogs pant a great deal, resulting in problems for the assessment.

In addition to examining dogs, a meeting was held to discuss heart-testing issues. The most difficult question is whether auscultation (listening with a stethoscope) and ECG examination is sufficient or whether ultrasound is also needed. The advantage of ECG only is that many more dogs can be seen in a day and the problems of operator experience and consistency of results are avoided. However we have seen a number of dogs with enlarged poorly contracting hearts on ultrasound which do not yet have ECG changes. I believe that ECG alone will not detect all affected dogs at screening sessions. It might be considered that the small number of affected dogs detected by ultrasound does not justify the effort involved, but for the individuals concerned and their breeding programmes, it may be devastating. The other main topic was whether regional testing sessions could be held, perhaps with the involvement of more cardiologists. More of this later.

The second testing session was held at the IWS Ch show at the Sports Connexion on 24th September 2006. On this occasion 16 hounds, nine dogs and seven bitches were examined and in addition one young dog was presented for auscultation only. Two males had atrial fibrillation and there were four hounds in the "equivocal" group. Testing was easier as the weather was cooler but a few hounds were very agitated with high heart rates and at shows the noisy conditions do not help. However generally the day went very well.

I should like to thank all those who helped to organise these events, especially Dagmar and Nick Kenis-Pordham, Jo Braine, Rebecca Peek and Alex Bennett.

News from the Veterinary Cardiovascular Society meeting at Loughborough, November 10th and 11th.

Over the past few years there has been considerable interest in the possibility of developing a genetic test for DCM in dogs. There have been many technical difficulties in carrying out this type of research but there has been a huge step forward because the dog genome has now been fully mapped. The completion of years of research was published in the journal "Nature" in December 2005.

One of the talks at Loughborough described a significant difference between DCM-affected and unaffected Dobermans in the gene coding for the large heart muscle protein titin. This is very exciting, but it must be stressed that this type of research is still in its infancy and this particular discovery is unlikely to apply to wolfhounds. It is probable that every breed will have a different gene defect, or possibly more than one, as numerous abnormal genes have been discovered in DCM in human families. However it is possible that before too long we may be able to apply these techniqueto wolfhounds and it is definitely time to think about storing DNA, especially from old hounds with good hearts, as these will be needed to compare with affected dogs.

The "PROTECT" study is now underway in Dobermans to see whether early drug treatment of symptomless dogs with DCM has any effect on their survival. Wolfhounds cannot yet be included in this study in UK, but a similar pilot project is underway in wolfhounds in Europe, so we shall await results.

I canvassed a few cardiologist colleagues to see if any would be interested in taking part in regional testing sessions. Some were unwilling to consider doing ultrasound examinations at shows but I have three definite volunteers - one in West Yorkshire, one in the New Forest and one in Wales.

It certainly seems that there is a renewed interest in heart-testing among wolfhound people. Unfortunately until we have a genetic test, there is no alternative to regular cardiac examinations in hounds of all ages.


REGIONAL HEART TESTING - A DISCUSSION

Held at Peter's Farm, Rusper, West Sussex on Saturday 24th June 2006

Report by Rebecca Peek
Questionnaire compiled and analysed by Jo Braine

A meeting was held to discuss the current status of heart disease in Irish Wolfhounds and its research and what the next steps might be for the breed. This was an open forum to encourage an active exchange of views and was based on the results of a questionnaire that had been sent out. The questionnaire was sent to 55 breeders/owners and 22 replied, which was considered a good response rate at 40%. It was based on a previous document distributed by Serena Brownlie in 2004, requesting feedback from us all, but which had sadly produced very little response. For those that responded to the questionnaire, thank you and we hope the notes from this meeting will encourage you to take part in further correspondence or face to face meetings on the subject. We can only make progress with your input and commitment.

The meeting was held in the middle of a day of heart testing and lasted approximately 2 hours, but it was clear that the discussion could have lasted much longer and generated enough topics and information to warrant a further session dedicated only to discussion.

The following is a summary of the main points discussed and the recommendations that follow will be submitted to the Irish Wolfhound Health Group for consideration.

1. Questionnaire Responses: Q1-4 on a scale of 1-10, (rising in concern)
Q1 How concerned are you about heart disease… 19 x 10, 2 x 8 and 1 x 7.
Q2 How important is regular heart testing…15 x 10, 1 x 9, 1 x 8, 1 x 7, 3 x 5, and 1 x 3
Q3 How important is cost… 5 x 10, 1 x 9, 3 x 8, 2 x 6, 6 x 5, 1 x 4, and 3 x 1
Q4 How important is the convenience of travel… 3 x 10, 3 x 9, 1 x 8, 4 x 7, 3 x 6, 5 x 5, 1 x 4, and 1 x 2

So, all respondents have a high degree of concern about heart disease, and the majority place a high degree of importance on regular heart testing. The importance of cost is variable, but the majority lean towards cost being of greater concern. And convenience of travel is also widely spread, but with the majority placing greater importance on it.

The majority would ideally want to heart test all dogs with a couple testing breeding stock only and the majority would want to do that annually and certainly before breeding. Regarding the importance of confidentiality, 15 said it was not important and 7 said that such information would need to be managed, that it must be factual and not speculative, it depends on what is said, with some preferring to give the information themselves or wanting confidentiality in the first instance and a couple felt that confidentiality was very important. 17 were prepared to share their results with 4 sharing it conditionally from themselves to persons they identified themselves and 1 was not prepared to share.

Most thought setting up a Regional Heart Testing Scheme was a good/great idea, with all results and ECG tapes forwarded to Dr. Brownlie for her assessment. About half were in favour of using results to set up a risk assessment scheme to help when planning a breeding. A list of suggested vets that might be interested in taking part was provided.

2. Serena was asked what she recommends Irish Wolfhound breeders should be doing? When should we be starting to test? How often? Should we be doing ECG's and ultrasound all the time, or just auscultation and ECG's?
Serena's response was that that anything was better than nothing, but that for her auscultation and ECG were not the whole picture and things could show up on an ultrasound that were not apparent in the other two tests. So any heart testing in wolfhounds should include the ultrasound as well. She is happy to receive and interpret any results sent to her and the owner of the dog should make the vet carrying out the test aware of this.

Annual testing was thought to be sufficient, (unless a problem arose, in which case as directed by the specialist), and certainly prior to breeding. Although she recommends testing from 18 months - 2 years onwards, it was agreed that cases in younger hounds do occur and that if someone wanted to heart test a youngster, perhaps initially do an auscultation and ECG to see if there is anything concerning in that first.

Ideally Serena would like as much long-term data as possible from as many members/generations of a 'family' as possible, whether or not they are affected. Of particular interest are the older dogs without heart problems as a point of comparison, but they are often not taken to shows and unlikely to be taken for regular heart testing because of the expense &/or stress. Testing prior to breeding is important so that you know at the time of breeding that the dogs are 'unaffected', however, this is only a snapshot in time, and does not guarantee that the dog might not develop problems later. Regular testing on an annual basis will highlight early when/if a dog develops a problem.

It was generally agreed that to ensure that as many dogs as possible are tested on a regular basis, it is important to provide a scheme that is accessible, both in terms of geography and cost. Providing heart testing at shows is invaluable, but has several downsides - it's a noisy and potentially stressful environment; most people will only be bringing a few, younger dogs, and not all that they might otherwise book in; there is a tighter limit to how many you can get through, as it is quite time consuming; it can be a very stressful time for the owners as well as the dogs and potentially a very public arena should the news be upsetting.

3. So what form should a Regional Heart Testing Scheme take? It was generally agreed that we'd like to see regional heart screening set up in as many locations as possible, but recognise that this may be very limited by who might come on board with this. We discussed the need, ideally, for centres in Scotland, Wales, Northern Ireland, Ireland, the Midlands, the North, South Central, East Anglia, and the South East.

Travelling is important to people, as is cost. The main aim is to make heart testing available to as many people as possible at as reasonable a rate as possible to encourage as many dogs to be tested as possible. So, perhaps, as with the shows and testing with Serena, we might share a daily rate between several dogs on set 'Clinic Days', say two sessions a year, Autumn and Spring, which might be at the weekend and run over two days. It could be one day every quarter. Testing with ultrasound takes 20-30 minutes per dog, so that would determine the maximum number of dogs you could get through in a day. Or perhaps we might have access to the cardiologist at any time of the year, but at a preferential rate; the cost would have to be bearable for regular testing and testing of all dogs, other than just those to be bred from. This would possibly be less workable and less attractive to the participating vet, but we can only know this by asking them.

So, what's in it for the cardiologist? Apart from goodwill, what will they get out of it? Probably good ongoing business; if a dog that is affected is referred to them for treatment, and if they are the preferred clinic for wolfhounds, they will pick up good referral business. They will also have the benefit of an ongoing history of the dogs, so that should they need to be treated they will have a greater chance of improving the dogs lifespan by picking it up early and with that will go a reputation for good 'success' rates. They will also be associated with ongoing, leading edge research into this area and benefit from the latest developments.

If they don't lose out financially then it will be worth their while, so Day Clinics, sharing the daily rate cost may be the way forward. However, if people want a more flexible, call-any-time approach, they may need to pay more for a specific appointment. We discussed the argument that it costs less than a puppy or stud fee to get a dog or bitch checked out before breeding, but if you want to check all your stock, then they wont necessarily be 'producing' to cover the cost and the costs for most people can be prohibitive if you go down the usual route.

There needs to be consistency across all screening centres, in terms of cost and availability.

Breeders would probably make more use of the facility than pet owners, however, it should be available to everyone who owns a wolfhound and if any hound needed a referral from their own vet, the closest regional centre should be their first port of call.

Information on Regional Testing Schemes must be made available to everyone, through all available media, such as breed club/society websites and magazines, and the IWHG.

4. Who should we be using?
It was agreed that the initiative would need to be done under the auspices of Serena with all results being copied back to her and co-ordinated by her. Guidelines should be set by Serena to establish a standardised testing format. It was agreed that ultrasound scanning on the table was not necessary and someone like Serena has the authority and weight to impress this upon the cardiologist! If possible, establish a uniform agreed cost for doing it, and make the guideline info available to breeders for what has been agreed as well.

The cardiologist involved then also has the benefit of direct access to Serena if he/she needs to discuss a particular case. Serena should 'approve' or recommend those cardiologists taking part.

Any testing should be conducted by experienced canine cardiologists on VCVAss List and also accredited in Echo Doppler and on the Doppler List. On the list provided by us several were already known to Serena, but several were not. Several new names were suggested at the meeting.

Another option discussed is the possibility of 'buddying-up' with any existing breeder screening programmes for other breeds, such as the Dobermans, Boxers, Cockers, Newfoundlands etc. How are they managing to get preferential rates, are they being funded by club subsidies or sponsorship by drug companies? Where are they being tested?

Also, Veterinary Colleges might well be interested in setting up such initiatives, especially if they are interested in research in this area.

If there is to be DNA Research carried out, would the AHT be interested in being one of the screening centres and the DNA Research?

5. What should we be doing with the data?
If Serena is the central co-ordinator of data, what is she going to do with it and who will have access to it? How can it be made available to breeders in a meaningful way?

We discussed that although most people had expressed little or no concern about sharing this information, we should not ignore that not everyone with access to it might be benign in their intentions. Data Protection applies to all areas, so disclosure of any information has to be with consent and also we have to be careful about how any information is shared/accessed/managed for a beneficial purpose. We discussed that it might be unwise to allow people to be able to access all your health or other information for no valid reason other than curiosity, but disclosing an affected dog's name to benefit another's breeding programme or research is, for most, acceptable.

We also touched on the issue that such information is potentially of interest to the KC and/or Insurance companies which might want access to it with a view to 'requiring' all dogs to be status checked before insuring/registering.

As with the Risk Analysis, the information might be co-ordinated centrally and accessed on request, probably free of charge, but for breeding purposes, so maybe breeders would need to 'register' with the database in some way as proof of validity. Also, there is always the possibility of joining forces with Risk Analysis, where a database format already exists and is global in its remit, which adds further value to the information and saves the time in designing and creating such a programme.

An important aspect of a programme like the Risk Analysis is that you are given a percentage risk and dogs are only named by permission of the owner/breeder. This allows us to have access to vital information without affecting some people's need for confidentiality and the risk is calculated so you can make an informed and balanced assessment.

Some present felt that access to this information should be left to the breeder's discretion and trust that they would share the info they needed to with those parties that needed to know. We also discussed the need for a breeder to feel that they could share information without any sense of blame, but as a responsibility to the breed as a whole and develop a culture of openness without fear.

It was generally agreed that in the absence of DNA tests to help us make breeding choices, the sharing of information has to be the next best way forward. We also discussed how this data might link in with/be beneficial to other heart research programmes already in existence, be that breed specific or generally. There may be human programmes available to us where the candidacy approach might benefit all.

DNA Research was generally supported as an avenue to pursue, and the possibility of linking in with the AHT for storage of blood taken from Livershunt tests and possible gene research. Again it raises the question of how much might it cost. Who owns it? Who has access to it? How is it protected? And being sure that the DNA is that of the dog specified…

There is also gene research going on in the States that we might be able to work with.

The bottom line was that any results generated and co-ordinated must be put to good use and have a purpose and that there must be regular feedback and contact with the breeders/owners.

6. Key Issues - also discussed

Who should drive this? Serena has been proposed to oversee the initiative and collate and interpret the results and generate meaningful data. However, someone/a body needs to create the initiative and sort out the logistics and make it happen. So far individuals have been making smaller things happen and could continue in this way, but ideally to speed things up and have some weight there needs to be a working party tasked to make it happen. In the first instance this will go to the IWHG as a proposal which we hope they will pick up and run with, but it will need specific people made responsible for doing this to ensure clear lines of communication. It is also critical that any progress is communicated back to all interested parties on a regular basis. It is important that any momentum already generated in the breed and with Serena is maximised.

How enforceable is regular heart testing? It was generally agreed that you cannot force breeders to do anything at this stage, nor would anyone want to. However, there does appear to be a sea change going on where more people seem willing and open to sharing information. Peer pressure and a change of behaviour in breeders will be more likely to have a greater effect. We discussed the Continental model that grades the heart tested dogs and publishes the information and has a more 'policed' approach and it was generally seen as culturally less acceptable to us. It is also still open to abuse and misrepresentation and all policing seemed to do was force people to find ways around the draconian measures. It was also agreed that making heart testing more available at a more accessible cost would probably be more likely to have a major impact on people's behaviour and attitudes.

Mode of inheritance - This was agreed as most urgent and still the most difficult aspect for us to deal with as breeders. Although most experts in this canine field suspect that it is likely to be an autosomal dominant with incomplete penetrance, we are still very unclear as to how that behaves and how that information can be made meaningful in our breeding plans. Most said they would like to see gene research in this area and subsequently a test made available in the future, if possible, to identify which dogs are safe for breeding and which are not. It is hoped that a test would allow us to breed away from the problem in an informed and effective way, without having to throw the baby out with the bathwater - it is unlikely that all dogs in a litter would be affecteds; if we could identify this we could hopefully breed safely from unaffected siblings, and retain valuable lines. However, as it currently stands, we need guidance based on the data already available to us - what else can we do to help ourselves in the absence of hard scientific evidence?

Ongoing research is essential. It was stressed that for any research to be meaningful and significant, ideally we should be establishing a 'Lifetime Study' approach to it, whereby a dog is monitored throughout its life. Setting up Regional Heart Testing is likely to make this more possible. What is the next stage for us with the information we are generating already and which programmes already underway might also benefit from it , and in so doing, benefit us as well? What are the small moves we need to make to have maximum impact?

Open Register - A published list of dogs either clear or known affecteds? As in Holland? Score based, what does it tell us, people will work around it, it's not unequivocal - is it workable/desirable?

Stud dogs - yearly MOTs if at public stud? This was discussed and generally thought a good practice as it is in keeping with the recommendation for annual heart testing anyway.

Recommendations The way ahead…
1. Regional Heart Testing -We've identified the need, now we need to identify how we can do it.
Approach IWHG with proposals
Set up a working party as a central driving body.
Identify regions and names of cardiologists in those areas, (with Serena's guidance)
Approach them and assess response
Report back with results and recommendations to IWHG and Serena
Roll out a regional heart testing programme across the UK and NI, and Ireland, if they so wish (I make it sound soooo easy!!)
Communicate the new initiative to the general breed membership
Establish regular feedback from and to Serena

2. In the interim continue with testing at shows, ideally the two Open and two Championship shows, or Rallies, and then reduce the dependence on these as the regional testing gets underway.

People are informally approaching Serena for heart testing sessions and organising it amongst themselves. Again, in the short term, this is very valuable and if we can spread Serena's trips regionally, so that people in different areas have access to her expertise, so much the better.

3. Education of breeders - Breeders Guidelines There is still a great deal of confusion about what should happen as far as breeding is concerned, when there is a problem in your lines and also how this is inherited. It would be helpful to produce something for breeders that explains as clearly as possible, what we do know and how to breed with that knowledge. At the AGM the Club proposed an educational leaflet for pet owners, but actually there is an argument that we need to educate ourselves and the veterinary profession first.

In the absence of DNA tests and the Risk Analysis could Serena make available to us some sort of published Breeders Guidelines, based on her information to date? By this I mean, based on what she thinks is the likely mode of inheritance, or the patterns of incidences that she has seen over the years, how we can breed on, knowing that some or several dogs in the pedigree are affected dogs. What would she recommend? For Example, if the parent/s are affected or have died from late onset or early onset, is it safe to breed from their offspring, and under what conditions. At what stage is an affected dog in the pedigree not considered a threat? How far back does it have to be in the pedigree? What are the risks that we can take and definitely should not take? If a dog has very late onset, is it considered to be geriatric and not a threat, or if it has been affected young, is it likely to be inherited? If there are known lines that make it more likely/inevitable, but are bound by confidentiality, is there a way Serena can look at a trial pedigree and give an indication of risk?

This could all be done within the existing bounds of confidentiality.

4. Set up a further Open Forum for an update/further exploration

5. Risk Analysis - Approach Anne Janis to see if there is any way of using our data within her programme?

6. Investigate other research areas for collaboration

LINKS
http://www.iwfoundation.org/articles_detail.html?item_id=46&year=2014 - article on Cardiomyopathy in Irish wolfhounds on IWF website
http://www.iwfoundation.org/articles_detail.html?item_id=44&year=2014 - article on Heart Disease in the Irish wolfhound on IWF website
http://www.fdc.to/canine.htm - Canine Dilated Cardiomyopathy Research Project
http://www.iwfoundation.org/ - The Irish Wolfhound Foundation website

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Updated 8/11/2015