Nava-martini-thiene syndrome

Nava-martini-thiene syndrome is a medical syndrome, sometimes causing sudden death from ventricular fibrillation, resembling Brugada syndrome.

Definition

A syndrome is an association between a clinical event and some investigational finding.

The syndrome of right bundle branch block (RBBB), ST-segment elevation and sudden death was first described in 1988 in Italy by Italian doctors from Padua in the Giornale Italiano di Cardiologia1Nava A, Canciani B, Martini B, "et al." La ripolarizzazione precoce nelle precordiali destre. Correlazioni ECG-VCGelettrofisiologia. (abstr) G Ital Cardiol 1988; 18 (Suppl 1): 118.] Martini B, Nava A, Buja GF, "et al." Fibrillazione ventricolare in apparente assenza di cardiopatia. Descrizione di 6 casi. (abstr) G Ital Cardiol 1988; 18 (Suppl 1): 136.] in Mises à Jour CardiologiquesNava A, Canciani B, Schiavinato ML, Martini B. La repolarisation precoce dans le precordiales droites: trouble de la conduction intraventriculaire droite? Correlations de l’electrocardiographie- vectorcardiographie avec l’electrophysiologie. Mises a Jour Cardiologiques 1988; 17: 157-9.] and in the "American Heart Journal"Martini B, Nava A, Thiene G, "et al." Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J 1989; 118: 1203-9. ] . This syndrome is now known worldwide as the “Brugada syndrome”, reflecting the name of those who described later the same entity in 1992.Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 1992; 20: 1391-6. ] At present, up to 1000 papers on this syndrome are reported in the Internet site http://www.brugada.crtia.be . In more than 20% of cases some form of organic heart disease has been recognized, mainly of the right ventricle, while in the vast majority of patients a structural abnormality has not been identified. This may be because the investigation was incomplete. This has led to two different theories on the pathophysiology of the syndrome: one relates the precordial ECG to a depolarization abnormality or to an organic heart disease whereas the second ascribed the syndrome to a functional abnormality of repolarization. [http://digilander.libero.it/martini_syndrome]

Historical notes

In 1953, Osher and Wolff [Osher HL, Wolff L. Electrocardiographic pattern simulating acute myocardial injury. Am Med Sci 1953; 226: 541-5.] reported a dynamic ECG abnormality (Osher-Wolff ECG), simulating an acute myocardial infarction, in a healthy man. It is of interestFact|date=April 2008 that they wrote: “This is apparently due to prolongation of the depolarization process by right bundle block or possibly focal block with delayed activation of a portion of the right ventricle: unusually early onset of repolarization may also play a role”.

A similar ECG pattern, this time associated with an aborted sudden death and occurring in a 42-year-old male while talking with a post officer on the 2nd of October 1984, was seen in Padua, Italy. In the following years incomplete details were given on similar patients. [Nava A, Scognamiglio R, Buja GF, "et al." Arrhythmogenic right ventricle. Clinical spectrum and familiarity. New Trends in Arrhythmias 1986; 2: 13-9.] [Nava A, Canciani B, Scognamiglio R, "et al." La tachicardia e la fibrillazione ventricolare nel ventricolo destro aritmogeno. G Ital Cardiol 1986; 16: 741-9.] A new “syndrome” characterized by a clinical event (sudden or aborted sudden death) associated with the abnormal ECG findings, was first presented at the National Congress of Italian Cardiologists, held in Florence, by Nava, Martini, Thiene and colleagues, working in Padua with Professor Sergio Dalla Volta in 1988. Shortly after, Nava "et al." published the first ECG characteristic of the syndrome in 1988, which is considered worldwide as the typical ECG of the syndrome, and which is now called the “Brugada sign”.

One year later, a full description of the syndrome was published in the "American Heart Journal". It is noteworthy that in ourWho|date=April 2008 paper we re-published not only “the typical” 9, but most of the ECG variations of the syndrome, namely dynamic or isolated Stsegment abnormalities, incomplete or com-plete RBBB, sometimes associated with an atrioventricular and fascicular conduction impairment, and a prolonged HV interval. A prolonged PR interval, left axis deviation, some incomplete RBBB and minor ST-segment elevation were present in patient 4 of our paper.

The same patient was re-published by Corrado "et al.", [Corrado D, Basso C, Buja GF, Nava A, Rossi L, Thiene G. Right bundle branch block, ST-segment elevation, and sudden death in young people. Circulation 2001; 103: 710-7.] and that ECG is an excellent example of the dynamic pattern sometimes seen in this syndrome. Despite the typical functional ECG pattern, this patient had anatomical evidence of a right ventricular cardiomyopathy. A complete RBBB morphology was present in patient 1, with only a slight ST-segment elevation in V1 and V2. Isolated slight ST/T anomalies/elevation as seen in patients 2 and 5 of our paper4, may very well have been a potential marker of the syndrome. Drug testing was not performed at that time. The presence of late potentials, corresponding to ST-segment elevation, was proven both by intracavitary recordings and by signal-averaged ECG [Nava A, Canciani B, Buja GF, Martini B. El electrocardiograma y el vectorcardiograma en la dysplasia arritmogénica del ventriculo derecho. Rev Lat Cardiol 1992; 15: 276-83. ] . The second description of the syndrome was presented by Aihara "et al." [Aihara N, Ohe T, Kamakura S, "et al." Clinical and electrophysiologic characteristics of idiopathic ventricular fibrillation. Shinzo 1990; 22 (Suppl 2): 80-6.] in patients without apparent heart disease, and the third by the Brugada brothers, FIVE years after the initial Italian description.

References

* Martini B Nava A. Point of view 1988-2003. Fifteen years after the first Italian description by Nava-Martini-Thiene and colleagues of a new syndrome (different from the Brugada syndrome?) in the Giornale Italiano di Cardiologia: do we really know everything on this entity? (Ital Heart J 2004; 5 (1): 53-60)

External links

* [http://digilander.libero.it/martini_syndrome/ martini syndrome]
* [http://www.italheartj.org/pdf_files/Martini.pdf Martini.pdf] Citation broken|date=April 2008
* [http://digilander.libero.it/martini_syndrome/downloadpage.htm Download historical articles]