13th International Congress
THE "NEW FRONTIERS"
OF ARRHYTHMIAS 1998

January 24-31, 1998
Marilleva, Trento, Italy

RT-244

Further observations of the Brugada-Martini syndrome. Familial distribution of the incomplete bundle branch block, STT. Elevation and sudden death syndrome. A clinical-morphologic and genetic study of nine families

Franco Naccarella, Angelo Rolli, Angelo Carboni, Angelo Finardi, Antonello Zoni, Giovannina Lepera, Gaetano Barbato*, Massimo Palmieri*, Pietro Ticci**, Luigi Padeletti**, Andrea Nava°, Bortolo Martini°, Alessandra Rampazzo°°, Gianantonio Danieli°°, Paola Bertaccini^, Rossella Fattori^, Giampaolo Gavelli^.
Dipartimento di Cardiologia, Azienda Ospedaliera di Parma, *Dipartimento di Cardiologia, Ospedale Maggiore, Bologna, °Cattedra di Cardiologia, Universita di Padova,
°°Dipartimento di Genetica Medica, Universita di Padova, ^Dipartimento di Radiologia Medica III e NMR, Universita di Bologna, **Dipartimento di Medicina Interna e Cardiologia, Universita di Firenze, Italy

The incomplete right bundle branch block, ST elevation and sudden death syndrome (SDS), described separately by Brugada-Brugada and Nava and Thiene, has been differently commented as a primary electrical disease or an initial form of the right ventricular dysplasia cardiomyopathy syndrome (RVDC)1-19. We observed 7 different subjects with SDS, 6 of whom suffered from VF and cardiac arrest (CA) and 1 had VT, as presenting arrhythmia. We screened, so far, 9 families, 5 in Bologna, 3 in Florence and 1 in Parma. We screened 59 members (MEM) of the 9 families, with ECG, ECHO and NMR was performed in 23 possibly affected subjects.
Preliminary results are reported in the following table I.

TABLE 1 - Analysis of the different families

 

ICD

DST

MEM

ECG PA

SD/CA

NMR AS

1st family

+

+++

6

3/6

1

1/3

2st family

+

-++

7

3/7

2

1/2

3rd family

++

++

4

2/4

1 (ICD)

0/2

4th family

+

++

6

2/6

1

1/2

5th family

+

+++

18

5/18

2

1/4

6th family

+++

++

7

4/7

1 (ICD)

0/0

7th family

+

+++

4

2/4

1 (ICD)

0/0

8th family

+

+

5

2/5

0

0/0

9th family

++

++

2

2/3

0

0/1

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