WPWS AND LQTS

Two entities in a some electrocardiogram record.

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WPW-LQTS

El síndrome de Wolff-Parkinson-White (WPWS es una alteración cardíaca congénita (PRKAG2. mapa locus 7q36) con una despolarización prematura de los ventrículos (pre-excitación de los ventrículos a través de una conducción AV anómala, en este caso, a través de Kent paquete).

The Long QT's syndrome (LQTS) consists of an abnormal prolongation of the QT-interval in the ECG. El síndrome de QT largo (SQTL) consiste en una prolongación anormal del intervalo QT en el ECG. It has a special capacity to predispose to the appearance of malignant cardiac arrhythmias, especially, polymorphic ventricular tachycardia, ventricular fibrillation and torsade of pointes. Tiene una capacidad especial para predisponer a la aparición de arritmias cardiacas malignas, sobre todo, la taquicardia ventricular polimorfa, fibrilación ventricular y torsades de pointes.

If they already have little incidence separately, when they are united in an electrocardiogram, this incidence should be very much minor. Si ya tienen poca incidencia por separado, cuando están unidos en un electrocardiograma, esta incidencia debe ser mucho menor. In fact, we have not seen any publication about mentioned association in the current medical literature. Aim : Our aim has been to reveal the existence of an association of a WPWS alongside a LQTS in a same person. Methods : We have studied 17 members of three families (10 in Family 1, 5 in Family 2 and 2 in Family 3). De hecho, no hemos visto ninguna publicación sobre la mencionada asociación en la literatura médica actual. Objetivo: Nuestro objetivo ha sido revelar la existencia de una asociación de un WPWS junto a un LQTS en una misma persona. Métodos: Se estudiaron 17 miembros de tres familias (10 en la familia 1, 5 en la familia 2 y 2 en la familia 3). All patients were studied with ECG's test as fundamental test. They were also studied with EMMA genetical testing. Results : All affected members of the three families had a ventricular pre-excitation besides of others cardiac rhythm disturbances, concretely, Long QT's Syndrome (LQTS). Conclusion : By means of the following exposition, we show the existence of electrocardiographic patterns with a WPW's alongside a LQTS record. Todos los pacientes fueron estudiados con la prueba de ECG como prueba fundamental. También se estudió con la prueba genética EMMA. Resultados: Todos los miembros afectados de las tres familias tenía una preexcitación ventricular, además de otros trastornos del ritmo cardíaco, concretamente, el Síndrome de QT Largo (SQTL) . Conclusión: Por medio de la siguiente exposición, nos muestran la existencia de patrones electrocardiográficos con el GTM junto con un registro de LQTS.

con referencia a:

"El síndrome de Wolff-Parkinson-White (WPWS es una alteración cardíaca congénita (PRKAG2. mapa locus 7q36) con una despolarización prematura de los ventrículos (pre-excitación de los ventrículos a través de una conducción AV anómala, en este caso, a través de Kent paquete). The Long QT's syndrome (LQTS) consists of an abnormal prolongation of the QT-interval in the ECG. El síndrome de QT largo (SQTL) consiste en una prolongación anormal del intervalo QT en el ECG. It has a special capacity to predispose to the appearance of malignant cardiac arrhythmias, especially, polymorphic ventricular tachycardia, ventricular fibrillation and torsade of pointes. Tiene una capacidad especial para predisponer a la aparición de arritmias cardiacas malignas, sobre todo, la taquicardia ventricular polimorfa, fibrilación ventricular y torsades de pointes. If they already have little incidence separately, when they are united in an electrocardiogram, this incidence should be very much minor. Si ya tienen poca incidencia por separado, cuando están unidos en un electrocardiograma, esta incidencia debe ser mucho menor. In fact, we have not seen any publication about mentioned association in the current medical literature. Aim : Our aim has been to reveal the existence of an association of a WPWS alongside a LQTS in a same person. Methods : We have studied 17 members of three families (10 in Family 1, 5 in Family 2 and 2 in Family 3). De hecho, no hemos visto ninguna publicación sobre la mencionada asociación en la literatura médica actual. Objetivo: Nuestro objetivo ha sido revelar la existencia de una asociación de un WPWS junto a un LQTS en una misma persona. Métodos: Se estudiaron 17 miembros de tres familias (10 en la familia 1, 5 en la familia 2 y 2 en la familia 3). All patients were studied with ECG's test as fundamental test. They were also studied with EMMA genetical testing. Results : All affected members of the three families had a ventricular pre-excitation besides of others cardiac rhythm disturbances, concretely, Long QT's Syndrome (LQTS). Conclusion : By means of the following exposition, we show the existence of electrocardiographic patterns with a WPW's alongside a LQTS record. Todos los pacientes fueron estudiados con la prueba de ECG como prueba fundamental. También se estudió con la prueba genética EMMA. Resultados: Todos los miembros afectados de las tres familias tenía una preexcitación ventricular, además de otros trastornos del ritmo cardíaco, concretamente, el Síndrome de QT Largo (SQTL) . Conclusión: Por medio de la siguiente exposición, nos muestran la existencia de patrones electrocardiográficos con el GTM junto con un registro de LQTS."
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ELECTROCARDIOGRAPHY RECORDING OF "P on T" WITHIN A CONTEXT OF ACUTE PULMONARY OEDEMA RECOVERED.

Keywords: Acute Pulmonary Oedema; Electrocardiographic disturbances; “R on T phenomenon”; “P on T phenomenon”; First degree AV blockade; Long QT interval.

Abstract.

One of the disorders of cardiac rhythm most feared for all cardiologists is the "R on T phenomenon". Methods. – Valuation clinical and electrocardiographic. Results. – A “P on T phenomenon”. Conclusions. - According our experience when there is an acute pulmonary oedema, is very frequent the presence of an electrocardiographic recording as this.

From long time ago is well known that, the most vulnerable period of the heart (critical phase), is the ventricular repolarisation phase: T-wave. In fact, one of the disorders of cardiac rhythm most feared for all cardiologists is the "R on T phenomenon" ¹. In the "R on T phenomenon," there are premature ventricular beats that originate in the descending branch or at the peak of T- wave.

Any event that may alter the period of myocardial vulnerability (recuperation phase, also called T-wave) can also cause lethal cardiac arrhythmias, especially the dreaded onset of sustained ventricular fibrillation and the phenomenon denominated "torsades de pointes" ^{2, 3}.

One of these potentially lethal disorders can also be produced when the P- wave (auricle depolarisation) is interconnected to descending branch of T- wave before that these branches can arrive until the baseline of the electrocardiogram. Then we could talk of "P on T phenomenon”. This phenomenon is extremely common (in our experience) when they are presents a first-degree A-V blockade and a long QT- interval in the same ECG recording.

This image of “P- wave on T- wave" is more noticeable on D2-D3-aVF leads (See illustration).

The image resembles to a spacious "m " , where both the descending branch of the T- wave as the beginning of the ascending branch of the P- wave aren't in the baseline of the electrocardiogram. In our experience, all individuals, with these electrocardiographic characteristics, had presented serious problems of ventricular fibrillation, after to suffer several episodes of pulmonary embolism (fortunately, all were recovered). Besides of exhaustive studies (Battery), all this cases have also been studied for us with genomic study and have been positives for gene of Long QT Syndrome type 2 (gene HERG, also known as KCNH2) on chromosome 7) ⁴.

Ventricular tachycardia outflow tract in left ventricle may show similar layouts. Therefore, we made differential diagnosis with this condition and we have seen that this was a different entity (Echocardiography and Electrophysiology studies)

A brief medical history.

A 55 years-old adult male. First time he has been evaluated in any Hospital. Multi-risk patients: Hypertension not controlled. Diabetes type II not controlled. Severe smoker. Habitual drinker. He comes to our emergency department by ambulance the Intensive Care Unit with: Dyspnoea severe, audible respiratory crackling, Central cyanosis. Unconsciousness. Once recovered and conscious, patient has said us to have suffered several losses of consciousness. He thought were due to abusive ingestion of alcohol.

Familial history without clinical interest.

Reflexions.

We have seen other many events as this. The typical characteristic has been a patient with an acute pulmonary oedema and with an electrocardiographic recording as in presented clinical case (after its clinical recuperation). Nevertheless, after of look on medical literature of profuse way, we have not seen none type of reference of this picture in any of them. It is clear the presence of long QT interval alongside a first degree AV blockade, and they are creating a "P on T phenomenon"

RECOMMENDED REFERENCES

1. Albert NM. Ventricular dysrhythmias in heart failure. J Cardiovasc Nurs. 2004; 19(6 Suppl):S11-26.
2. Meyerfeldt U, Schirdewan A, Wiedemann M, Schutt H, Zimmerman F, Luft FC.. The mode of onset of ventricular tachycardia. A patient-specific phenomenon. Eur Heart J. 1997; 18:1956-65.
3. Denker S, Lehmann M, Mahmud R, Gilbert C, Akhtar M. Facilitation of ventricular tachycardia induction with abrupt changes in ventricular cycle length. Am J Cardiol. 1984; 53:508-15.
4. Steve J. Compton. Robert L. Lux. Matthew R. Ramsey. Katie R. Strelich. Genetically Defined Therapy of Inherited Long-QT Syndrome Correction of Abnormal repolarization by Potassium. Circulation. 1996;94:1018-1022

Figure 1

Conventional Electrocardiogram on D1 to aVF.

Figure 2.

Complete electrocardiogram with 12 leads.

SHORT PQ INTERVAL PLUS LONG QT INTERVAL IN SAME PERSON

SHORT PQ INTERVAL PLUS LONG QT INTERVAL ON SAME PERSON.

Keywords: Cycle cardiac. Short PR interval. Long QT interval. Tachycardia. Cardiac Rhythm.

ABSTRACT

For many authors (See References), there's a great relation between alteration of electrical cardiac system and the risk of serious cardiac disturbances and even of sudden cardiac death. We're according with them. We have the conviction of the existence of more electric cardiac problems than haven't been published yet. This presentation could be its corroboration. OBJECTIVE: Show the existence of new electrocardiographic parameters to clinicians. METHODS: Evaluation of electrocardiographic parameters with measurement of its length, both manually as of computerized way. RESULTS: A pattern of short PQ interval plus a long QT interval in the same person. CONCLUSIONS: Draw conclusions about a pattern, without previous support of bibliography would be a temerity and speculation. We just want to expose this clinical case. In none moment has been our intention make any type of conclusion; only has been show to doctors that these entities exist.

INTRODUCTION

Since more of 3000 year ago, we are studying the heart and in the two last centuries (XX and XXI), its electrical system. However, the last studies over its structure and there functions are related, almost all them, with entities already published (Variations on a same theme). This should not be necessarily so. Not only the entities as for example, short and long PQ interval or short and long QT interval (as well as its variations) are the only entities that have an own existence. They’re more than possible, the combinations between the values of duration of electrocardiographic intervals. The Heart may have altered its conduction system in many more occasions that in the mentioned entities, because also are manifold its mathematical possibilities. It would be great errors no consider these criteria.

METHODS and RESULTS

As methods for its valuation , we have used: Bazzet, Fridericia and Framingham formulas and the measures have been made with manual technique and digital technique (measure in pixel) in 12 leads. We have considered as normal values: PQ (or PR) interval equal or lesser 0.20 seconds and QT interval equal or lesser 0.45 seconds.

We have chosen, in order to simplify, the evaluation of values on orthogonal leads: D1, aVF, V2. These leads are more helpful for calculating the electrical axis of the heart. We also be have considered for this evaluation, the more equipotent derivations (lead); in this one case it is coincident with aVL and V2. Besides to the electrocardiograph, to this patient underwent a battery of tests: Holter, Ergometry, Analytical profiles, Echocardiogram. He was also tested for genetic study and electrophysiological study. In genetic studies there were not any specific alterations for gene of short PQ interval and nor for gene of long QT interval (alterations of channels of sodium or potassium).

In electrophysiological study there aren’t any alterations of cardiac conduction after of blocking with esmolol and posterior stimulation with adenosine. Why these normalities? We don’t know yet, but they’re already under actual study by our investigator team.

Brief History

He's a young man with 33 old-years; no taker usual medicines; in your family history there are two sudden deaths of possible cardiac origin. He came to emergency department by one access palpitations and tachycardias not related to the physical effort (which appeared suddenly during physiological sleep). In a first moment, your electrocardiogram was considered as normal, but, with a detailed interpretation, we can see it as there are electric alterations than could be explain the tachycardias and a possible sudden cardiac death. (We think that these cardiac events should be studied in more profundity than until to date).

From the point of view Epidemiological, we can expose, only, our clinical cases, position that when not having bibliographical support it is an impossible task in itself.

The Universe that we have at the present time, reaches the 32 clinical cases as the exposed here [Pertaining to 7 different families: North American 2 (2/7); Australian 1 (1/7); South American 2 (2/7); European 2 (2/7)]. The 100% of the cases are male and with age between the 12 and 40-old years. Absolutely all the patients have suffered, at least, three accesses of nocturnal tachycardia (inclusion criteria). In your familial antecedents, there are to the minus, one person with cardiac important symptomatology. As empirical treatment we have used a Beta Blocker (Sotalol).

Sotalol it's a very versatile drug, since it's structurally a beta blocker (antiarrhythmic class II) but it has also an action antiarrhythmic type I-A, also has been classified on the antiarrhythmic of group class III). ¹²

In some case, it seems have action both in the nodule auricle-ventricular, as in myocardial cells and seems to reduce number of accesses cardiological per year. All our clinical cases (32/32) were a diagnosis ambulatory of psychosomatic alterations.

DISCUSSION

In illustration exposed here, we can see as there are alterations of electrical systole not published until to date: We are in front of an electrical cardiac entity: short PQ interval (lesser than 0.12 seconds) plus a long QT interval (greater than 0.450 seconds), into same person.

And, however, the person hasn't had an important cardiac symptomatology, just slight symptoms.

If we give as true that the myocardium is more unstable and vulnerable when there are changes in the length of intervals (PQ and QT in this case. See references), it’s clear that this electrocardiogram meets those criteria. Therefore, the risk of serious heart rhythm disturbances (torsades de pointes, ventricular fibrillation, etc...) could be very high in this patient.

In this presentation, our questions have been:

• Is there a short PQ interval? Yes, there is.
• Is there a long QT interval? Yes, there is.
• Is there a high risk of events of lethal arrhythmias? Don't is known for us this problematic yet.

We’re then in front of a cardiac entity, that can have a high risk of serious arrhythmias, and therefore at high risk of sudden cardiac death.

CONCLUSIONS

It’s extremely difficult to draw conclusions without committing some sort of bias. Make some conclusion over this electrocardiographic pattern would be only make speculations because there aren't any antecedents as references. Our only intention with this exhibition has been to make public its existence. At no time has been to assert that there is a categorical relation, cause (electrocardiogram) - effect (symptoms).

If we give like certain all commentaries of cited authors (see references), we can to draw the following reflexions:

• Has this patient any risk of serious arrhythmias? We don't know yet. But it’s more than probable.
• Has this patient any risk of sudden cardiac death? We don't know yet. But it’s more than probable.

The only certain is than we’re before a pattern unknown and without any references and, therefore, is practically impossible say if may have risk of serious ventricular disturbances and/or sudden cardiac death.

MANAGEMENT AND TREATMENT

If since it is very difficult the unification of criteria among doctors, at this time, to decide what is the most effective treatment in the QT prolonged and in short PQ separately, when both are present in the same person (as the present case), such treatment can only be empirical, given his status unknown until today.

For some authors, the fundamental differences between QTL1 (KCNQ1 (KvLQT1) and QTL2 (KCNH2 -HERG) with QTL3 (SCN5A), besides the gene responsible, is the appearance of clinical and response to treatment with beta blockers. In QTL3, symptoms usually appear during sleep and are poorly controlled by the administration of beta-blockers. In clinical case presented, the symptoms are primarily nocturnal (tachycardias and palpitations). But, the most serious symptoms appear without relation with the physical effort.

Sotalol (a beta blocker), it appears that controls the duration of symptomatic phases in this patient.

There's a scheme Risk Stratification based on the genetic defect, the duration of the QTc and sex ^{8, 12}. The categories were divided into high-risk (50%: QTc> 500 ms, LQT1, LQT2, and men with LQT3), intermediate risk (30-40%: QTc <500> 500 ms: in women with LQT3) and low risk (<30%:>

There're authors who come to categorize a prolonged QT according to the morphology of the wave T.

RECOMMENDED REFERENCES

1. Cowan JC, Yusoff K, Moore M. Importance of lead selection in QT interval measurement. Am J Cardiol. 1988; 62: 83-87.

2. Zabel M, Franz MR, Klingenheben T. Rate-dependence of QT dispersion and the QT interval:

comparison of atrial pacing and exercise testing. J Am Coll Cardiol. 2000; 36: 1654-1658.

3. Glancy JM, Garratt CJ, Woods KL. Three-lead measurement of QTc dispersion. J Cardiovasc

Electrophysiol. 1995; 6: 987-992.

4. Gussak I, Brugada P, Brugada J. Idiopathic short QT interval: a new clinical

syndrome? Cardiology .2000; 94: 99–102.

5. Gaita F, Giustetto C, Bianchi F. Short QT syndrome: a familial cause of sudden death. Circulation. 2003; 108: 965–970.

6. Breijo-Márquez FR. Decrease of electrical cardiac systole. Int J Cardiol. 2008; 23; 126(2):e36-38.

7. DeBruyne, MC, Hoes, AW, Kors JA. QTc dispersion predicts cardiac mortality in the elderly: the Rotterdam Study. Circulation. 1998; 97: 467-472.

8. Nynke H, Arthur A.M. Wilde, Stefan K. Diagnostic criteria for congenital long QT syndrome in the era of molecular genetics: do we need a scoring system? European Heart Journal 2007; 28 (5): 575-580.

9. Nathaniel W. Taggart, MD; Carla M. Diagnostic Miscues in Congenital Long-QT Syndrome.

Circulation. 2007; 115: 2613-2620.

10. Recommendations for the Standardization and Interpretation of the Electrocardiogram Part I: The Electrocardiogram and Its Technology: A Scientific statement From the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society Endorsed by the International Society for Computerized Electrocardiology. Circulation. 2007; 115:1306-1324.

11. Behrens S. Franz MR. Interaction’s substrate-trigger: the role of ventricular repolarization. In: Dunbar S, Ellenbogen KA, Epstein AE, Eds. Sudden Cardiac Death. Medical Trens, SL. 1998: 34-50.

12. Priori SG, Schwartz PJ, Napolitano C, et al: Risk stratification in the long-QT Syndrome. N Engl J Med. 2003; 348: 1866-1874.

TABLE 1

In this table we can see the differences among cardiac entities with alteration in length of its intervals and its risk for suffer sudden cardiac death.