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Pioneers in cardiology: Dr Guy Fontaine

Guy Fontaine and arrhythmogenic right

ventricular dysplasia

Cardiomyopathies are the Focus of this EHJ issue. Guy Fontaine

an electrical engineer, then a physician, played a pivotal role

in the discovery of ARVD

T h e cu r i o u s a n d ve r y d i s t u rb i n g ‘ su d d e n d e a th ’ o f h i t h e r t o

a p p a re n t l y h e a l t h y yo u n g a th l e t e s w i t h n o p r i o r e v i d e n ce o f

ca rd iac p rob lems has in i t i a ted much con jec tu re and resea rch ,

certa in ly s ince the midd le o f the 20th century. One o f the p ioneers

i n t h i s f i e l d o f ca rd i o l o g y i s t h e r e n o w n e d F re n ch ca rd i o l o g i s t

D r Gu y Fon ta ine MD , PhD, HDR .

Fonta ine was born in Corbe i l Essonnes in France. He a t tended

secondary schoo l a t the Lycee Montesqu ieu in Bordeaux before

mov ing to Pa r is . In add i t ion to h is medica l deg ree , he had a lso

trained in electrical engineering which was of great benefit in his

p ionee r ing wo rk in e lec t roca rd iog raphy and e le c t rophys io log y .

The beg inn ing o f h is med ica l ca ree r in the 1960s co r responded

with a rapid development in medica l e lectronics; Fontaine in fact

des igned and bu i l t d i f fe ren t e lec t ron ic dev ices fo r the t rea tmen t

o f pat ien ts present ing wi th e lectr ica l d isorders inc lud ing a tr ia l

f ib r i l la t ion . He a lso used h is e lectr ica l eng ineer ing knowledge for

t h e a d va n c e m e n t o f p a ce m a ke r t e c h n o l o g y . F o r h i s m e d i ca l

t hes i s , he p re sen ted one de v ice to s tud y the ca rd iac pa c ing

t h re sh o l d o f p a t i e n t s a w a i t i n g p a ce m a ke r i m p l a n t s . H e w a s

a w a rd e d h i s d o c to ra l d e g re e i n 1 9 6 6 f o r a n e x ce l l e n t t h e s i s

en t i t l ed ‘Con t r ibu t ions to E lec t r i ca l S t imu la t ion o f the Human

H e a r t ’ . I n 1 9 6 7 , F o n t a i n e w a s t h e f i r s t p h y s i c i a n i n P a r i s t o

implant permanent pacemakers using intracardiac leads; his exper -

t ise in this f ie ld led to the referral of many patients with diff icult

a r r h y thm ia s f r o m bo th Eu rope a nd f u r th e r a f i e ld . I n 196 8 , he

jo ined the card iac department a t the Hôpi ta l de la Sa lpêtr iè re in

Paris led by Professor Jean Facquet.

Rather than accept any o f the severa l o f fe rs o f academic pos -

i t ions he rece ived, Fonta ine cont inued to pursue h is deep in terest

in research into cardiac pacing. Influenced by his mentor Professor

J.J. Welt i and after witnessing mult ip le autopsies displaying ‘hearts

t o o g ood t o d i e ’ , Fon ta ine f e l t ce r t a in t ha t i t w ou ld be po ss i b le

to eradicate ‘sudden death’ l inked to a tr ioventr icular conduct ion

d is tu rbances by h is new app roaches to so lve re levan t te chn ica l

and b io techn ica l p rob lems.

T h e n e x t m a j o r t o p i c o f i n t e re s t f o r F o n ta i n e i n 1 9 7 5 w a s

t a ch y c a rd i a s a n d t h e u s e o f p a c e m a ke r s t o t r e a t t h e m . H e

observed many episodes of chronic ventricu lar tachycard ia (VT)

p re ce ded b y e x t r a sys to l e s and t h e se ap pea re d t o be i nd u c ing

the i r own repe t i t ion : th is led h im to use a p rope r ly t imed s t imu -

la t ion o f the ven tr i c le to sho r ten the recove ry pause a f te r the

e x t r a s y s t o l e i n o rd e r t o p re ve n t V T . L a te r , t h i s co n ce p t w a s

u s e d t o o b t a i n t h e s a m e r e s u l t t h r o u g h s t i m u l a t i o n o f t h e

a tr ium ra ther than the ventr i c le and was ach ieved for permanent

pacing with the incorporation of a resistor in the ventricular lead

connec ted to the no rma l a t r ia l l ead and on to the ou tpu t o f the

f i r s t s ing le - chambe r d ig i ta l pacema ke r (V i ta t ron DPG -1 ) . Th i s

design which useful ly employed Fontaine’s knowledge and experi -

ence of electricity and biophysic parameters was seen as an intel -

l e c tua l ‘ tou r de fo rce ’ .

In 1976, Fonta ine co -authored and pub l ished, w i th h is mentors

and col leagues Professors Grosgogeat and Wel ti , the book ‘The

Essent ia ls o f Card iac Pacing ’ . A t tha t t ime, he was a lso fascinated

by the f i rs t a t tempts a t surg ica l ab la t ion o f the accessory bypa ss

t r a c t i n t h e W o l f f – P a r k i n s o n – W h i t e ( W P W ) s y n d r o m e w h i c h

w a s su cce ss fu l l y pe r f o rmed a t D u ke Un i ve r s i t y (US A ) in 1 968 .

I n 197 1 , a f te r 6 mon th s o f r e le van t r e sea rch in t he Ch r i s t i an

Cabrol surgica l laboratory in Paris with his col league Dr Guiraudon,

h e de ve lo ped t he te chn i que o f e p i ca rd ia l map p ing and t he y

performed the f i rst successfu l European surg ica l ab la t ion o f an

accesso ry pa thway in WPW synd rome . He fu r the r re f ined the

techn ique o f mapping by design ing ampl i f ie rs w i th summation,

rect i f ica t ion , and f i l te r ing properties, fo r the s imul taneous record -

ing of signals from three electrodes located at the distal end of a

specia l l y bu i l t curved probe. The purpose o f th is was to record

po ten t ia ls o r ig ina t ing on the d iaph ragm su r face o f the ven t r i c le ,

CardioPulse 1043

thus avo id ing the need to ra ise the bea t ing hea r t du r ing su rge ry. A gr id based on anatomica l landmarks, independent o f heart s ize , was

des igned and subsequen t l y used wo r ldw ide by resea rche rs .

After perfecting the epicard ial mapping technique in animal lab o ra to ry resea rch , the f i rs t Eu ropean pa t ien t was ope ra ted on in 1971 and

the repor t pub l i shed in 1973 . I t was then cons ide red possib le to use the ep icard ia l mapping in o ther l i fe th reaten ing arrhythmias, i .e . VT,

and four pat ien ts w i th VT were successfu l ly treated before the f i rst arrhythmogenic r ight ventr icular (RV) dys p lasia pat ien t ; a 65 -year-o ld

male who had suf fered for some 10 yea rs f r o m re cu r ren t VT un re l a te d t o co rona ry a r t e r y d i sea se . The ECG suggested th is pat ien t ’ s

tachycard ia or ig inated in the RV—an in terest ing f ind ing since in the previous four pat ien ts, the s i te o f o r ig in was the le f t ventr ic le .

T h e o p e r a t i o n t o o k p l a c e o n 3 0 O c t o b e r 1 9 7 3 , a n d o n e xpo su re , t he su rg eon i mm ed ia te l y n o ted mo de ra te d i l a t ion o f the RV

which was hypokinetic and covered by an unusually signi f i can t layer o f fa t . Mapp ing , wh ich took a round 20 m in , conf i rmed the ‘s ite of or igin ’

to be in the mid-RV free wall . A ‘s imple ventr i cu l o to m y ’ w a s p e r fo rmed a lo ng th e an te r io r b o rde r o f t he R V w h i c h s h o w e d a n

a p p a r e n t l y n o r m a l R V c a v i t y . G i v e n t h e pover ty o f the muscu la r wa l l essen t ia l l y rep laced by fa t , an RV b iopsy had been d i f f icu l t.

The histology of the fragment showed no signs of inf lammation. C losure o f the ventr ic le and wi thdrawal o f extracorporea l c i rcu la tion

fol lowed by burst st imulation did not result in inducible tachy c a r d i a : a v e r y s a t i s f a c t o r y r e s u l t a n d w i t h o u t t h e n e e d f o r anti-arrhythmic

drugs. Fonta ine’s team was convinced that a surg i -ca l t rea tmen t o f res i s tan t VT had been d iscove red . The pa t ien t d ied o f card iac fa i lu re

aged 86 years but w i thout fu r ther ep isodes o f VT . The resu l ts we re p resen ted in 1975 a t an in te rna t io na l meet ing in Amsterdam. 1 t another

international meeting in Liege, Belgium, in 1976, Fontaine found it necessary to propose a new name for this recently d i sco ve red c l i n i ca l

e n t i t y . 2 ‘ A r rh y th moge n i c ’ wa s an ob v i ou s cho ice as was ‘ r igh t ventr icu lar ’ s ince both descr ibed sa lien t fea tures o f or ig ina l re ferra ls. Last ly,

‘dysp lasia ’ was chosen to cover wha t appea red to be a deve lopmen ta l p rob lem. G iven the young age o f pat ien ts, i t was thought that the

d isease star ted ear ly in deve lopmen t , o r as we now know, in embryo —and thus the b i r th of ‘Arrhythmogenic Right Ventricular Dysplasia’ . 2 –

4

Fontaine has written 864 scientif ic articles and at least 195 book chapters, many published in English. He became expert in t he pathology of the

RV to explain the natural history of the disease.5 He has rece ived numerous honours and awards and the French ‘Leg ion d ’Honneu r ’ i s pend ing .

He has been inv i ted to lec tu re wo r ldw ide . He is rightly considered as the ‘epitome ofthe international scientist ’ . His interests and hobb ies

include classic music and the new unique style of 2300 ‘Call igraphic Paintings and Drawings’.

Diana Berry, MA

References

1 . Fon ta i ne G , G u i ra udon G, Frank R , Cou t te R , D rag odann e C . Ep ica rd ia l ma pp i ng a n d s ur g ic a l t re a tm e n t i n 6 c as es o f re s is tan t v e n t r i cu l a r t a ch yc ar d ia n o t re l a ted to coronary

ar tery disease. In: Wellens HJJ, L ie KI , Janse MJ, eds. The Conduction Sys tem o f the Hear t . Le iden : S ten fe r t Kroese Pub ; 1976 . p545 .

2 . Fontaine G, Gui raudon G, Fra nk R , Vede l J , Grosgogea t Y, Cabrol C , Facque t J . St imu la t ion s tud ies and ep ica rd ia l mapp ing in ven t r icu la r tachycard ia : s tudy o f mechanisms and

selection for surgery. In: Kulbertus HE, ed. Reentrant Arrhythmias. Lancas te r : M T P Pu b ; 19 77 . p 334 –3 50 .

3 . Fontaine G, Fontaliran F, Hebert JL, Chemla D, Zenati O, Lecarpentier Y, Frank R. Ar r hy thmo gen ic r igh t ven t r ic u la r dysp las ia . An n Rev Med 199 9 ; 50 : 17–3 5 .

4 . Fontaine GH. La mor t sub i te du jeune e t de l ’a th lè te . SFEM, Par is . 2010 . p89 –97 . ISBN 2 -9521576-6 -9 .

5 . Fontaine G. The multip le facets of right ventricular cardiomyopathies. Eur Heart J 2011 ;32 :do i :10 .1093 /eurhear t j /eh r088 .