I don't know any study about exercise test in Eisenmenger. I think that exercise test is contraindicated in this patology.

Dear Dr Babu,

Actually in many studies, exercise test has been performed to patients with Eisenmenger syndrome with a modified Bruce protocol or simply a symptom limited exercise test. In this population, an exercise test joined to a 6-min walk test, quality of life test and some biomarkers, are really useful to assess the functional class before and after starting the treatment (sidenafil+/-bosentan).

Here you can find some more information:

- Int J Cardiol. 2011 Sep 15;151(3):307-12.

- Int J Cardiol. 2011 Jul 15;150(2):177-81.

- Int J Cardiol. 2010 Mar 18;139(3):241-7

Kind regards,

Dr G Isasti

Thank you for your comment and references Dr. Jose and Dr.Isasti

I agree with Dr. Isasti about the 6-min walk test, but I am affraid with the modified exercise test, because depend of the experience of the executer with the Eisenmenger syndrome and complications.

Dr. Jose - thank you! I agree that there is a need for operator experience while conducting exercise tests especially in eisenmenger syndrome.

Is necessary to know that the test is frequently performed by a not expert in this theme. Best regards

I have been doing cardiopulmonary exercise stres test for almost 9 years. I have done many adults with CHD. I have one patient with Eisenmenger syndrome who died after 3 minutes into exercise due to ventricular arrhythmia. I strongly recommend not do cardiopulmonary exercise stress test in patients with Eisenmenger syndrome especially in adults because, the prognostic value of peak oxygen consumption in these group of patients is limited, at best. It may be that the 6 min walk test is more suitable for the routine outpatient assessment of these patients, providing information on their overall cardiovascular status, disease progression, and response to therapy.These patients have fixed PVR, unable to increase cvardiac output through an increase in pulmonary blood flow, but can increase right to lefet shunting resulting in severe cyanosis. They are however different than IPAH because they may have preserved RV function. More work is needed in this area before recommending any guidelines for doing exercise testing in this population.

Thanks for the comment Dr. Jose and Bibhuti. Ventricular arrhythmias are indeed a serious risk during CPX. Like you mention, I agree that the 6MWT probably stresses the body to a great extent in these already severely de-conditioned patients. The pathophysiology of Eisenmenger's is definitely to be considered during testing and we should work in this area to establish some facts.

Dr. Das, if I may ask, what method and protocol of testing did you use for the patient you mentioned in your comment? Was a 6MWT done prior to the exercise test?

Dr Babu:

Thanks for your comments. The patient I mentioned wasa 33 yr old with Eissemneger. Functional class NYHA II. We did a treadmill with Naughton protocol. It was unfortunate. Recently, a urvey showed very low indcidence of major cardiovascular events with exercise training. (Ref: www.acsm.org) This patient had never ventricular arryhtmia. But because of hemodynamics, patient edeveloped destauration, wxwercise stopped immediately. Subsequently fast ventricular arrhythmia related hypoxia and acidosis.

The risks of maximal symptom-limited testing are indeed higher than expected in all patients with pulmonary arterial hypertension, including Eisenmenger physiology. Nonetheless, large series have showed an acceptable absolute risk. Therefore, I think we should weigh the risks and benefits of maximal testing (preferably cardiopulmonary testing) compared to the simple six minute test we routinely perform in the follow-up of pah patients. I think in most instances the answer is "no, we do not need CP testing to support clinical decisions"; however, I suppose this can be of help in pts with "grey zone" six minute or unclear symptom status, when decisions regarding "hard therapies" (ie prostanoids and transplant) have to be made.

Dr Guido:

There are studies in literature for CPX testing in patients with CHD and PAH (severity varies). There is a group of patients where it can be done. REF:

Duffels MGJ et al. AJC 2009:103:1309-1315

Apostolopoulou SC et al Heart 2005;91:1447-1452

In children with primary PAH there are also CPX studies done. Ref:

Yetman A et al AJC 2005

Garofano RR et al Pedaitr Cardiol 1999;20:61-64

There is no systematic study on patients with Eisenmenger Syndrome and there is no guidelines. I would not recommend to do this. I think 6 min walk test will serve the purpose for general functional status evaluation. But for long term whether the medications are really working, we have to have better non-inavsive assessment modality and end-point should be better than just showing improvement in 6-min walk test criteria. Future study on a large scale is warranted on this special population.

These are indeed very interesting views. Dr. Bibhuti thanks for sharing the details of the case. As noted, even with a very conservative protocol, it is interesting to see that the problems occured post exercise rather than during. I had a patient a few months back (around the time I posted this question) who had a severe bradycardia following exercise. From a peak HR of 179 it dropped to 43 with no signs of heart block or ectopics.Her HR resolved to a baseline of around 60 within 5 min. - I guess we need more studies in this area.

The risk of testing Eisenmenger's is indeed debatable.

Dr.Guido, thank you for your comments. I am of the opinion that CPX (cardiopulmonary exercise testing) should form part of the clinical decision making as studies in PAH have shown that these CPX parameters do have prognostic implications. However, its use in children is still not known. Perhaps till we have solid evidence for the use of CPX in these populations, it will be the 6MWT that we would end up using safely.

Like Dr. Bhibuti says, this is indeed worth investigating in future trials.

To my knowledge, these should be other large experiences:

http://circ.ahajournals.org/content/113/24/2796.long#T2

http://circ.ahajournals.org/content/125/2/250.full

They do not mention an uncommon incidence of major complications related to the test

The numbers are relatively small, however only very large centers may recruit more than 30-40 Eisenmenger patients who are suitable for maximal CPET...."large scale" is a relative concept in these disease entities

Anyway I agree with you - as I pointed out above - that in a vast majority of patients 6 minute data will suffice for clinical decisions to be taken. However, I do not feel that published data support an absolute contraindication for CPET.

Dr. Guido, Thanks you for the references and your comments

Dear Dr. Babu. The conclusion about my opinion is: the exercise test is not necessary for clinical decision on Eisenmenger syndrome. So it is a unnecessary aditional risk for a patient with a serious disease. If there are not absolute contraindication, also there are not clear indication. I prefer the sentence: Primum non Nocere.

Dear Dr. Jacob, I agree " Primum non Nocere" Clinical judgement should dictate the need for a formal exercise test

Agree exercise testing in Eisenmenger is not necessary for clinical decision

I agree with you, Sébastian.