Dear Tatiyana,

Thank you for the interesting case. Given these ECG findings and episode of AF earlier it is likely that the patient has any cardiac structural damage. But what is its nature or origin? I think you have substantiation to exclude CAD. So, the most valuable methods are endomyocardial biopsy, cardiac MRT. By the way you should perform an available investigation for congenital channalopathies although I think the possibility of such dramatic presentation at this age is low.

Cardiac sarcoid should be a consideration. Noninvasive tests like cardiac MR should be performed earlier before considerimg biopsy.

you should think in Brugada's syndrome. the clinical findings are very suggestive as it is  nocturnal EKG. It has family implications as well

please, tell us yours ressults

It looks as channelopathy.... polymorphic VT during sleeping with bradycardia. .. mostly LQT3....TMT  or pharmacological steps test to see effecton QT interval... you already mentioned that by echo pt has normal heart.....

Coronary spasm /Prinzmetal angina/ should be discussed.

What an interesting case. The ST segments in the rhythm strip at the time of the polymorphic VT suggest ST depression different from the later recordings (I suspect you'd see ST elevation if a different lead were possible). The ST elevation with bradycardia is very dramatic. Coronary spasm I agree is a strong possibility, rather than a primary arrhythmia, although varying ST changes in Brugada would be considered. The degree of ST elevation in the first trace (assuming the STs are normal in other parts of the same trace) is very dramatic, and surely rather more than would be expected in a channelopathy. I'd review the coronary angiogram for plaque as a focus for spasm, try a nitrate or CCB, taking it at night (repeating the holter after stabilising the dose), do MR, review the resting XII lead carefully and consider provocative tests for Brugada (but I'd have to do a literature search to look for results of ST segment monitoring in Brugada). Check the family history carefully for sudden death, or device implantation. If he has risk factors for CAD I might well treat with statin and aspirin anyway, in case he has non-angiographic atherosclerosis (CT angio might look at arterial wall better than standard luminal coronary angiography in addition - calcium score may be revealing). Throw the book at him!

A very interesting and educational observation which rises many questions and possible answers about intimal patophysiology of this case. When  I wrote in 1972 about observed cases Prinzmetals variant angina of mine in "Serbian archives of medicine" I had an idea of some connection between exaggerated nocturnal vagotonia, spasm of "normal" coronary arteries  and a dangerous ventricular rhythm disturbances. Many years later the evolution of coronary artery investigations and of our knowledge about very fine mechanisms on the  level of transmembrane physiology of ion exchange, normal or disturbed, strengthened this idea. On the basis of given data I stronly suggest a possibility of a very complex cause of this case:

1. what is the cause of an exaggerated nocturnal vagotonia?,  2. Is it  there some not detected very fine plaque in "naissance" as a "locus minoris resistentiae" ? 3. Is it there some variant of channelopathy?

If I could lead this investigation, I would folow those steps, but sorry, I am retired for 26 yrs.  

. 

Hi Tatiyana,

Interesting ECG tracings. The upper image in the ECG panel is almost certainly spasm of the right coronary with characteristically ischaemic ST elevation in lead 2 &3. The Holter strip (I presume this is a lead 2 tracing and was not during an ST elevation episode) looks like monomorphic ecotopics causing PMVT due to R on T phenomenon  and transmural re-entry. 

I don't really see any evidence of channelopathy but of course you have given us only a little snapshot of info.

From the little information available I would hypothesise that the spasm caused myocardial ischaemia and post spasm reperfusion ectopy leading to pmvt.

In terms of investigations I think you need to consider ajmaline, particularly if there has been syncope. Perfusion MRI may be uselful.  Implantable loop recorder could be useful here also as sustained ventricular arrhythmias during sleep may indicate an ICD. 

I would treat for spasm here with CCB and nitrates in the first instance.

Would be interested to hear results!

Dear Сolleagues, thank you to all. We performed the following studies as recommended:

•  Serum potassium and calcium levels:ECG patterns in patients with hypercalcemia and hyperkalemia similar to that of Brugada syndrome. -Normal
• CK-MB and troponin levels: In patients with symptoms compatible with an acute coronary syndrome.-Normal
• Repeat coronary angiography.- Normal (ECG at the test in the attached file)
• MRI primarily to exclude arrhythmogenic right ventricular cardiomyopathy, as well as to assess for other potential causes of arrhythmias. - Normal
• Drug challenge with a sodium channel blocker (procainamide): 1-type BrS pattern ECG was induced. - View in the attached file below
• Genetic testing for a mutation in SCN5A (no mutation found in the hot spots by Sanger).- We plan to sequence of calcium channel genes (CACNA1C, CACNB2b, CACNA2D1); sodium channel β-subunit genes (SCN1B, SCN3B); glycereol-3 phosphate dehydrogenase 1-like enzyme (GPD1L) and MOG1, which affects trafficking of sodium channels; and in genes that affect transient outward current (Ito) (KCNE3, KCND3, KCNE5) in single patients and families with BrS.

Thus, interesting case turned out to be a late onset clinical manifestation of Brugada Syndrome associated with persistent atrial fibrillation;  pharmacological  inhibition (over-dose beta-blocker) of sinus node function is to trigger or exacerbate clinical manifestations.

Thank you all; the best, Tatiyana

thank you to let us  to know your findings and final diagnosis

best regards

Thank you for your interest and support.

Кind regards,

Tatiyana

Very nice case aswell.

I assume that the patient has a Prinzmetal- Angina. I would treat him with Diltiazem 

Hi Peter! This pattern is very similar to Рrinzmetal. This version was supposed  one of the first by us also. However, drug challenge with a sodium channel blocker (procainamide) was induced the 1st-type BrS pattern ECG . See attached file below,,,

Kind regards

Makes you wonder if Prinzmetal was actually describing Brugada! I haven't read the original manuscript.

Hi Philip! Your idea is very interesting and original. I was very impressed with it. Unfortunately, Dr. Myron Prinzmetal in original manuscript (Am J Med. 1959 Sep;27:375-88) variant angina pectoris really described: variant angina  was presented with clinical, electrocardiographic and autopsy findings (attacks of chest pain, reciprocal ST depression/elevation, coronary artery stenosis). In his landmark article in 1959, Dr. Myron Prinzmetal described a distinct type of “variant angina,” termed Prinzmetal’s angina. This chest pain tended to occur at rest (i.e. was not associated with increased cardiac work), waxed and waned cyclically, occurred at the same time each day, and could be accompanied by arrhythmias including ventricular ectopy, ventricular tachycardia, ventricular fibrillation, and various forms of AV block. The patient’s ECG during painful episodes typically showed ST segment elevations (occasionally accompanied by reciprocal ST depressions), whereas the ECG obtained after the pain had resolved showed resolution of these ST segment changes. Prinzmetal postulated that this separate clinical entity was due to transient spasm (“increased tonus”) of a large arteriosclerotic artery, causing temporary transmural ischemia in the distribution supplied by that artery.It was subsequently demonstrated that such episodes of coronary artery spasm can occur not only in patients with underlying fixed coronary artery obstruction but also in patients whose coronary arteries are anatomically normal.

But other case reports of the1960's representing the Prinzmetal`s ECG are very similar to BrS, since "reciprocal" depression of the ST segment in leads distant from those showing ST elevation are absent and the clinical picture described of attacks was blurred and nontypical.

Very interesting your opinion on this issue I have conquered.Thanks!

As you are suggesting we would count both these cases as of unstable angina/acute coronary syndrome, with atheroma causing transient coronary occlusion.

Yes, I suppose that is so