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C a r d i ova s c l n t e r ve n t R a d i o l ( 1984 ) 7 : 115 - 120 CardioVascularandlnterventional9 Springer-Verlag 1984

Diagnosis of Complex Congeni tal Heart Disease:M orphologic-Anatomic M ethod and Term inology*R i c h a r d V a n P r a a g hD e pa r t m e n t s o f P a t ho l ogy a nd C a r d i o l ogy , C h i l d r e n ' s H o s p i t a l a nd H a r va r d M e d i c a l S c hoo l , B os t on , M a s s a c hus e t ts , U S A

Abstract. A s u m m a r y a n d b r i e f i l lu s t ra t io n o f th em o r p h o l o g i c - a n a t o m i c m e t h o d o f d ia g n o si s o fc o n g e n i t a l h e a r t d i s e a se i s p r e s e n t e d . T h e p r i n c i-p l e s o f s c i en t if ic n e o l o g i z i n g a r e c o n s i d e r e d a n de x e m p l i f i e d . S c i e n t i f i c f r e e d o m o f s p e e c h a n d e x -p r e s s io n i s c o m m e n t e d u p o n . I t is s u g g e st e d t h a tu n n e c e s s a r y r e n a m i n g o f n u m e r o u s c a r d i a c an -a t o m i c s t r u c t u re s a n d m a n y f o r m s o f c o n g e n it a lh e a r t d i s e a s e b e d i s c o n t i n u e d , a n d t h a t t e r m i n o l o -g y b e d e - e m p h a s i z e d .K e y w o r d s : M o r p h o l o g i c a n a t o m y , d i a g n os i s, c la s -s i fi c a ti o n , t e r m i n o l o g y - N e o l o g i s m s - C o n g e n i t a la n o m a l i e s , c l a s s if i c a ti o n o f

F o r t h o s e w h o w i s h t o u n d e r s t a n d t h e f a s c i n a t i n gc o m p l e x i t ie s o f c o n g e n i t a l h e a r t d i s ea s e , F i g u r e 1a n d i t s l e g e n d w i l l r e p a y c a r e f u l s t u d y .T h e a c c o m p a n y i n g t e x t s u m m a r i z e s t h e mor -phologic-anatomic approach [ 1 - 8 ] t o t h e d i a g n o s i so f c o n g e n i t a l h e a r t d is e a s e . T h i s m e t h o d a n d i t st e r m i n o l o g y h a v e s t o o d t h e t e s t o f t i m e , a p p l y c o n -v e n i e n t l y i n a ll s i tu a t i o n s ( n o m a t t e r h o w c o m -p l ex ) , a n d h a v e n o t r e q u i r e d a n y s i g n i f ic a n t c h a n g -e s o v e r t h e p a s t d e c a d e o r m o r e a t t h e C h i l d r e n ' sH o s p i t a l i n B o s t o n .

R e f e r e nc e s t o t h e a n a t o m y a r e m a d e a s f o ll o w s :Cardiac Positions(1 ) levocardia, l e f t - s i d e d h e a r t ;(2 ) dextrocardia, r i g h t -s i d e d h e a r t ;* S uppor t e d i n pa r t by a g r a n t f r om t he M a be l L ou i s e R i l e yF o u n d a t i o n , B o s to n , M AA ddr es s r epr in t r eques t s t o : R i c h a r d v a n P r a ag h , M . D . , D e p a r t -m e n t s o f P a t h o l o g y a n d C a r d i o l o g y , C h i ld r e n 's H o s p i t a l M e d i -c a l C e n t e r , 300 L ongw ood A v e nue , B os t on , M A 02115 , U S A

(3 ) mesocardia, m i d l i n e h e a rt ; a n d(4 ) ectopia cordis, p a r t i a ll y o r c o m p l e t e l y e x t ra -t h o r a c i c h e a r t .M o r p h o l o g i c -A n a t o m i c A p p r o a c h t o D i a g n o s i s( F i g . 1 )T h e m o r p h o l o g i c - a n a t o m i c a p p r o a c h [ 3- 8] to t hed i a g n o s i s o f c o n g e n i t a l h e a r t d i se a s e ( C H D ) i sb a s e d o n t h e s i t u s , a l i g n m e n t s , c o n n e c t i o n s , a n da s s o c ia t e d m a l f o r m a t i o n s o f th e c a r d i a c s e g m e n t s( a n a to m i c a n d d e v e l o p m e n t a l c o m p o n e n t s w h i c ht o g e t h e r m a k e u p a l l h u m a n h e a rt s) .

The main cardiac segments ( F i g . 1 ) a r e : { a t r i a ,v e n t r i c l e s , g r e a t a r te r i e s } . T h e b r a c e s { } i n d i c a t et h a t t h e th r e e m a i n c a r d i a c s e g m e n t s m a y b ev i e w e d a s th e m e m b e r s o f a s et (i n th e m a t h e m a t i -c a l s e n s e o f s e t t h e o r y ) . T h e s e g m e n t a l a p p r o a c ht o d i a g n o s i s i s s e q u e n t i a l , i . e . , t h e m a i n c a r d i a cs e g m e n t s a r e o r g a n i z e d i n a l o g i c a l v e n o - a r t e r i a ls e q u e n c e : { a t r ia , v e n t r i c l e s , g r e a t a r te r i e s } ( F i g . 1 ).The connecting cardiac segments , n o t s h o w n i nF i g u r e 1 , c o n n e c t t h e m a i n c a r d i a c s e g m e n t s . T h ea t r io v e n t r i c u la r ( A V ) c a n a l o r j u n c t i o n c o n n e c t st h e a t r ia a n d t h e v e n t ri c le s , w h i le t h e i n f u n d i b u l u mo r c o n u s c o n n e c t s t h e v e n t r i c l e s a n d t h e g r e a t a r -t e r i e s .I n o u r n o t a t i o n ( F i g . 1 ), t h e s i t u s o f t h e t h r e em a i n c a r d i a c s e g m e n t s i s i n d i c a t e d w i t h i n t h eb r a c e s : ( a t r i a l s i t u s , v e n t r i c u l a r s i t u s , g r e a t a r t e r i a ls i t u s } . S i g n i f i c a n t a b n o r m a l v e n t r i c u l o a r t e r i a la l i g n m e n t s , s u c h a s t r a n s p o s i t i o n o f th e g r e a t a r -t e ri e s ( T G A ) , a r e i n d i c a t e d b e f o r e t h e b r a c e s : T G A{ - , - , - } . I m p o r t a n t a b n o r m a l s e g m e n t a l c o n n ec -t i o n s a n d o t h e r a s s o c i a t e d c a r d i o v a s c u l a r a n o m a -l i e s c u s t o m a r i l y a r e l i s t e d a f t e r t h e b r a c e s : { - , - , - }w i t h s t r a d d l i n g t r i c u s p i d v a l v e a n d v e n t r i c u l a r s e p -t a l d e f e c t o f th e A V c a n a l t y p e .

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1 16 R . V a n P r a a g h : D i a g n o s i s o f C o m p l e x C o n g e n i t a l H e a r t D i s ea s eF ig . 1 . Morphologic-anatomic diagnosisof congenital heart disease. H e a r t d i a -g r a m s v i e w e d f r o m b e l o w , t o c o r r e l a t ew i t h f r o n t a l a n g i o c a r d i o g r a p h y a n ds u b x i p h o i d t w o - d i m e n s i o n a l e c h o c a r -d i o g r a p h y . A n t = a n t e r i o r ( v e n t r a l ) ;P o s t = p o s t e r i o r ( d o r s a l ) ; R = - r i g h t ;L = left.R A = m o r p h o l o g i c a l l y r i g h t a t r i -u m ; L A = m o r p h o l o g i c a ll y le f t a t r i u m ;R V = m o r p h o l o g i c a l l y r i g h t v e n t r i c l e ;L V = m o r p h o l o g i c a l l y l e ft v e n t r i c l e ;a o r t i c v a l v e , i n d i c a t e d b y c o r o n a r y o s -t i a ; p u l m o n a r y v a lv e , d i s t in g u i s h e d b ya b s e n c e o f c o r o n a r y o s t i a ; b r o k e nl i n e s d e l i n e a t e t y p e s o f h e a r t n o t d e -s c r i b e d w h e n t h i s d i a g r a m w a s m a d e .Braces { } indicate a set, i n t h em a t h e m a t i c a l s e n s e ; e a c h s et h a s t h r e em e m b e r s , { 1, 2 , 3 } ; m e m b e r 1 = a t r i a ls i t u s ; m e m b e r 2 = v e n t r i c u l a r s i t u s ;m e m b e r 3 = g r e a t a r t e ri a l s i t u s ; a t r i als i t u s s o l i t u s = { S , - , - } ; a t r i a l s i t u s i n -v e r s u s = { I , - , - } ; a t r i a l s i t u s a m b i -g u u s = {A , , - } ( n o t s h o w n ) ; v e n t r ic u -l a r s i t u s s o l i t u s o r D - l o o p = { - , D , - } ;v e n t r i c u l a r s i t u s i n v e r s u s o r L - l o o p ={ - , L , - } ; v e n t r i c u l a r s i tu s a m b i g u u s o rX - l o o p = { - , X , - } ( n o t sh o w n ) ; so l it u sn o r m a l l y r e l a t e d g r e a t a r t e r i e s = { - , - ,S } ; i n v e r t e d n o r m a l l y r e l a t e d g r e a t a r -t e r i es = { - , - , I } ; s o l i t u s o r D - t r a n s p o s i -t i o n , o r D - m a l p o s i t i o n o f t h e g r e a t a r -t e r i e s = { - , - , D } ; i n v e r t e d o r L - t r a n s -p o s i t i o n , o r L-malposi t ion o f t h e g r e a ta r t e r i e s = { - , - , L } ; a m b i g u u s o r a n -t e r o - t r a n s p o s i t i o n , o r A - t r a n s p o s i t i o n ,o r A - m a l p o s i t i o n o f t he g r e a t a r t e r -i e s = { , - , A } ( n o t s h o w n ) .D i a g r a m i s o r g a n i z e d i n f i v e c o l -u r n s f r o m l e f t t o r i g h t , 1 - 5 , a c c o r d i n gt o s e g m e n t a l a l i g n m e n t s : c o l u m n 1n a m e s t h e v a r i o u s t y p e s o f v e n t r i c u -l o a r te r i a l ( V A ) a l i g n m e n t s a n d c o n n e c -t i o n s ; c o l u m n s 2 a n d 3 h a v e v i s c e ro -a t r i a l s i t u s s o l i t u s ; c o l u m n s 4 a n d 5

N O R M A L( , ~ A n t

t S , D , S I H o r i z o n t a l P l a n e [ I . L , I I1 V i e w e d f r o m B e l o w 2

O ,TEOV E N T R I C U L A R [ L V ] R V i L R V I L V tD I S C O R D A N C E [ R A I L A I ~ I . A I R A "i S l L . S l I I , O , [ I1 2

I S O L A T E D %A T R I A L [ L V [ R V IN O N I N V E R S I O N [ R A [ L A 1I S , L , I I

T R A N S P O S I T I O N ~ ) ) ~ ~ t ~l .v l v l l v l .v l C v , .v , E . v . v ,G R E A T R A L A R A L A [ L A ] R A J L I - A ] R A ]A R T E R I E S i S ,D ,D I { S . L ~ . I I I , L , L I I I , D ,D tt 2 3 4

A N A T O M I C A L L Y ~ % (r!~t,2-,C O R R E C T E D l , v t . v l f . v l , v l . = . v , , v . , .. . . .

o f [ ~ le R A L A R A L A L : - L A - - ~ - - - . . a lA ' I L A I R A ]G R E A T A R T E R I E S ~ S .D .L { S ,L ,D I t | , L , D I I I ,D,L I1 2 3 4

O U T L E T LV LV R V L V R V R V L VV E N T R I C L E r t S . L .L ~ I I , L . L I I I . D , D1 2 3 4

O U T L E T R V L V . . . . _R V _! L V _ R _ V ~ r - - - - Z ; " *"- 4" ~ . . . .L . . . . "e - - - 4L E F T R A L A ' R A ' L A ', ~, L A I R A . . j , L A ] R A ',VENTRICLE ~ S . D . O ; ! S . L . L ; t I . L . L I I I , D . D I1 2 3 4

h a v e v i s c e r o a t r i a l s i t u s i n v e r s u s ; v i s c e r o - a t r ia l s i tu s a m b i g u u s i s n o t s h o w n ; c o l u m n s 2 a n d 4 h a v e c o n c o r d a n t a t r i o v e n t r i c u l a r( A V ) a l i g n m e n t s ; c o l u m n s 3 a n d 5 h a v e d i s c o r d a n t A V a l i g n m e n t s .D i a g r a m i s o r g a n i z e d i n s e v e n r o w s , 1 - 7 , f r o m t o p t o b o t t o m , a c c o r d i n g t o t h e v a r i o u s t y p e s o f v e n t r i c u l o a r t e r i a l ( V A )a l i g n m e n t s a n d c o n n e c t i o n s . R o w s 1 - 3 h a v e n o r m a l l y r e la t e d g r e a t a r te r i e s . R o w 1 , t h e t w o n o r m a l h e a r t s : s o l i t u s n o r m a l h e a r t = {S ,D , S } ; a n d i n v e r t e d " n o r m a l " h e a r t { I, L , I} . R o w 2 sh o w s i s o l a t e d v e n t r i c u l a r d i s c o r d a n c e : i s o l a t e d v e n t r i c u l a r i n v e r s i o n = { S ,L , S } ; a n d i s o la t e d v e n t r i c u l ar n o n i n v e r s i o n - { I , D , ] }. R o w 3 s h ow s i s o l at e d a t r i a l n o n i n v e r s i o n = { S, L , I } , al s o k n o w n a sv i s c e r o a t r i a l s i t u s s o l it u s , w i t h v e n t r i c u l a r i n v e r s i o n , a n d i n v e r t e d n o r m a l l y r e l a t e d g r e a t a r t e r i es . R o w 4 s h o w s t h e v a r i o u s t y p e so f t r a n s p o s i t io n o f t h e g r e at a r t e ri e s ( T G A ) : p h y s i o lo g i c a ll y u n c o r r e c te d o r c o m p l e t e T G A i n s i tu s s o l i t u s = T G A { S, D , D } ;p h y s i o lo g i c a ll y c o r re c t ed o r i n v e r t e d T G A i n s i tu s s o l i t u s = T G A { S, L , L } ; p h y s i ol o g i ca l l y u n c o r r e c t e d o r c o m p l e t e T G A i ns i tu s i n v e r s u s = T G A { I, L , L } ; p h y s i o lo g i c a ll y c o r re c t e d o r n o n i n v e r t e d T G A i n s it u s i n v e r s u s = T G A { I , D , D } ; T G A i n s it u sa m b i g u u s , u s u a ll y w i t h a s p le n i a o r p o l y s p l e n i a = T G A { A , D , D } a n d T G A { A , L , L } a r e n o t s h o w n . N o t i l l us t r a te d is T G Aw i t h d i r e c t l y a n t e r i o r a o r t i c v a l v e ( A o V ) , a n d w i t h l e f t w a r d A o V , i n v i s c e r o a t r i a l s i t u s s o l i t u s = T G A { S , D , A } , a n d T G A{ S , D , L } ; a n d T G A w i t h d i r e c t l y a n t e r i o r a o r t i c v a l v e , a n d w i t h r i g h t w a r d a o r t i c v a l v e , i n v i s c e r o a t r i a l s i t u s i n v e r s u s = T G A{ I, L , A } , a n d T G A { I, L , D } a l s o a re n o t s h o w n . S i m i l a r l y , R o w s 5 - 7 d e p i c t s o m e o f t h e t y p e s o f a n a t o m i c a l l y c o r r e c t e dm a l p o s i t i o n o f t h e g r e a t a r t e ri e s ( A C M ) , d o u b l e - o u t l e t r i g h t v e n t r i c le ( D O R V ) , a n d d o u b l e - o u t l e t le f t v e n tr i c l e ( D O L V ) . T h ec o m m o n e s t f o r m : o f A C M i s A C M { S, D , L } [2 ]; o f D O R V i s D O R V { S, D , D } [2 2] ; a n d o f D O L V , b u t n o w n o t t h e o n l yf o r m k n o w n , i s D O L V { S , D , D } [ 2 3 ].W i t h i n t h e b r a c e s is t h e s e g m e n t a l s i t u s s e t , e . g. , {S , D , D } o r { S, L , L} . B e f or e t h e b r a c e s th e V A a l i g n m e n t = T G A { - , - , - }o r D O R V { - , - , - } i s s h o w n ; w h i l e a f t e r th e b r a c e s c o m e t h e a t r i o v e n t r i c u l a r ( A V ) m a l a l i g n m e n t s a n d t h e a s s o c i a t e d c a r d i o v a s c u l a rm a l f o r m a t i o n s , e .g . , { , , - } w i t h s t r a d d l i n g t r i c u s p i d v a l v e , v e n t r i c u l a r s e p ta l d e f e ct o f t h e A V c a n a l t y p e , a n d s m a l l - c h a m b e r e dR V . ( R e p r o d u c e d b y p e r m i s s i o n o f t h e A m e r i c a n H e a r t A s s o c i a t i o n , I n c ., f r o m V a n P r a a g h [ 4] .)

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R . Va n P r a a g h : D i a g n o s i s o f C o m p l e x C o n g e n i t a l He a r t D i s e a s e 1 17T y pe s o f V i sc e roa t r ia l S i tu s (F ig . 1 , Co lu m n s 2 -5 )

(1 ) s i tus sol i tus (S) , i. e ., no rm a l o r non inver te dp a t t e r n o f a n a t o m i c o r g a n i z a ti o n , w i t h s o l it u sm o rpho lo g ica l ly r igh t a t r ium (RA ) [6] to the r igh t ,and so l itu s m orpho log ica l ly l e ft a t r ium (L A) [6]to the lef t ;(2 ) s i tus inversus ( I ) , i . e . , inver ted or mirror-i m a g e p a t t e r n o f a n a t o m i c o r g a n i z a t i o n , w i t h i n -v e r t e d R A t o t h e l e ft a n d i n v e r t e d L A t o t h e r i g h t;a n d(3 ) s i tu s ambiguus (A) , w i th an ana tom ica l lyuncer ta in type o f v i s ce ro -a t r i a l s i tu s (o f ten s eenwi th the asp len ia and po lysp len ia syndrom es , ra r e -l y w i t h n o r m a l l y f o r m e d s p l e e n , n o t s h o w n i nFig. 1).

T y pe s o f Ve n t r i c u lar S i tu s (Fig. 1)(1 ) sol i tus or D- loop ventr ic les (D), i .e. , solitusm orpho log ica l ly r igh t ven t r i c le (RV) typ ica l ly tothe r igh t o f the so l i tu s m orpho log ica l ly l e f t ven t r i -c le (LV) [6-8] ;(2 ) i nv e r t e d or L - loop v e n t r i c l e s (L), i .e. , in-ver ted RV typ ica l ly to the l e f t and inver ted LVto the r igh t [6 -8 ]; an d(3 ) ambiguous or X- loop v e n t r i c l e s ( X - - u n -kno wn ) (no t sho wn in F ig . 1 ) [2] .T y p e s o f A r t er i a l S i t u s (F ig . 1)

(1 ) Si tus sol i tus . Sol i tu s no rm al ly r e la ted g rea ta r t e r i es (S ) and D- t r anspos i t ions and D-m alpos i -t ions o f the g rea t a r t e r i es (D) a re in s i tu s so l i tu s[81. (2 ) Si tus inversus . I n v e r t e d n o r m a l l y r e l a t e dgrea t a r t e r i es ( I ) and L- t r anspos i t ions and L- rea l -pos i t ions o f the g rea t a r t e r i es (L) a r e in s i tu s inver -s u s [ 8 ] .(3 ) S i tus ambiguus . A-t ranspos i t ion and A- rea l -pos i t ion o f the g rea t a r t e r i es (A) a re in s i tu s am bi -guus (nei ther sol i tus , nor inversus) [8] .A V A l i g n m e n t s a n d C o n n e c ti o n s

(1 ) c onc ordan t [ 2 ], R A o p e n i n g i n t o R V a n dLA in to LV (F ig . 1) ;(2 ) d i sc ordan t [ 2 ], R A o p e n i n g i n t o L V , a n dLA in to RV (F ig . 1 ) ;(3 ) t r icuspid a tres ia ;(4 ) s traddl ing tr icuspid valve (TV) ;(5 ) doub le - in l e t L V;(6 ) mi t ra l a t re s ia ;(7 ) s t radd l ing mi t ra l v a lve ( M V ) ;(8 ) doub le - in l e t RV;

(9 ) doub le - ou t l e t RA [10]; and(10) c om mon A V canal .A n i m p o r t a n t d i s t in c t i o n e x is ts b e t w e e n A V al ign-m e n t s a n d A V eonnee t ions [8 ] . Accura te ly speak-ing , the a t r i a do no t connec t w i th the ven t r i c les(excep t a t the bun d le o f H is ) because o f the in te r -p o s i ti o n s o f t h e A V c a n a l o r j u n c t i o n . O n e o f t h em o s t i m p o r t a n t f u n c t i o n s o f t h e A V c a n a l i s t ose para te the a t r i a f rom the ven t r i c les , the reby a f -fo rd ing e lec t r i ca l in su la t ion o f the a t r i a f rom theven t r i c les , excep t a t the H is bund le . The a t r i a arec onne c te d with the ven t r i c les , by the AV junc t ion .(No te tha t the pas s ive vo ice , are c onne c te d , is ana-tom ica l ly accu ra te , whereas the ac t ive vo ice , con-nec t , i s a n a t o m i c a l l y e r r o n e o u s .) T h e A V c o n n e c -t ions pe r se , i .e . , the TV and the M V, a re v i r tua l lya lways concordan t r e la t ive to the i r ven t r i c les o fe n t r y . I n o t h e r w o r d s , t h e A V c o n n e c t i o n s t h e m -se lves ( the TV and the MV) a re es s en t ia l ly neverd i s co rdan t r e la t ive to the ven t r i c les , because thes i tu s o f the AV va lves and o f the ven t r i c les a r etyp ica l ly the s am e . In the in te res t s o f ana to m ic ac -cu racy , the re fo re , i t i s im p or ta n t to d i s t ingu i sh be -t w e e n A V a l i g n m e n t s a n d A V c o n n e c t i o n s , w h i c hare ve ry d i f f e ren t th ings .T h e s i t u s o f t h e A V c o n n e c t i n g s e g m e n t( m a i n l y t h e M V a n d t h e T V ) i s a d e p e n d e n t v a r i-ab le [8 ] , i .e . , dep ende n t on , and the s am e as , thes i tu s o f the ven t r i c les ; whereas the s i tu s o f theo t h e r f o u r d i a g n o s t i c a l l y i m p o r t a n t s e g m e n t s(a t r ia , ven t r i c les , in fund ibu lum , and g rea t a r t e r i es )a re indep ende n t va r iab les [8 ].V e n t r i c u l a r s i t u s ( D - l o o p / L - l o o p ) s h o u l d b ed iagnosed spec i f i ca l ly , because i t canno t be in -f e r re d w i t h c o n s i s t an t a c c u r a c y f r o m t h e A V a l ig n -m en ts ( concordan t /d i s concordan t ) [7 , 8 ] .V i l A l i g n m e n t s a n d C o n n e c t i o n sVent r icu loa r te r i a l (VA) a l ignm en ts m a y be (F ig . 1 ,c o l u m n 1 ):(1 ) normal - so l i t u s n o r m a l , o r i n v e r t e d n o r m a l ;(2 ) t ranspos it i on o f t he gre a t a r t e r i es ( T G A ) - D -T G A , L - T G A , o r A - T G A ( n o t s h o w n i n F ig . 1);(3 ) ana tom ic a l l y c orre c te d ma lpos i t i on o f thegre a t a r t e r ie s ( A C M ) ;(4 ) double -out le t r ight ventr ic le ( D O R V ) ; a n d(5 ) double -out le t le f t ventr ic le ( D O L V ) .A g a i n , t h e r e i s a n i m p o r t a n t d i s t i n c t i o n b e t w e e nV A a l ignme n t s ( as above) and VA c onne c t ions [8].To desc r ibe the ana tom y accura te ly , the ven t r i c lesd o n o t c o n n e c t w i t h t h e g r e a t a r t e r i e s b e c a u s e o ft h e i n t e r p o s i t i o n o f t h e i n f u n d i b u l u m o r c o n u s ;r a the r , the ven t r i c les and the g rea t a r t e r i es are con-ne c te d in va r ious ways by the conus ; o r tha t the

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1 18 R . Va n P r a a g h : D i a g n o s i s o f C o m p l e x C o n g e n i t a l He a r t D i s e a s ec o n u s c o n n e c t s t h e v e n t r i c le s a n d t h e g r e a t a r t e r ie si n v a r i a b l e w a y s ( a s a b o v e ) .

D i s c u s s i o nT h e a d v a n t a g es o f t h e m o r p h o l o g i c - a n a t o m i c a p -p r o a c h [ 1- 8 ] t o t h e d i a g n o s i s o f c o n g e n i t a l h e a r td i se a se , w i t h i t s m o r p h o l o g i c s e g m e n t a l t e r m i n o l o -g y ( F ig . 1 ), a r e m a n y . T h i s s e g m e n t - b y - s e g m e n t ,a l i g n m e n t - b y - a l i g n m e n t , a n d c o n n e c t i o n - b y - c o n -n e c t i o n m e t h o d h a s b e e n c a l l e d t h e segmental ap-proach [ 1 - 8 ] t o d i a gnos i s . I t ha s a l so be e n de s ig -n a t e d t h e sequential approach [ 9 ] be c a use i t p r o -c e e d s i n a l o g i c a l v e n o a r t e r i a l s e q u e n c e f r o m t h ea t r i a , t o t he ve n t r i c l e s , t o t he g r e a t a r t e r i e s . Th i sd i a g n o s t i c m e t h o d h a s a l so b e e n c a l l ed t h e system-atic approach [ 1 1 ] t o d i f f e r e n t i a l d ia g n o s i s . W i t hm i n o r v a r i a t i o n s i n e m p h a s i s , t h i s m o r p h o l o g i c -a n a t o m i c a p p r o a c h t o t h e d i ag n o s i s o f c o n g e n i t a lh e a r t d i s e a s e - w h i c h is s e g m e n t a l [ 1- 8 ], a n d s e-q u e n t i a l [ 9 ], a n d s y s t e m a t i c [ i 1 ] - h a s w o n w o r l d -w i d e a p p r o v a l [9 , 11 - 16 ] . S i n c e t h e t e r m i n o l o g yo f t h e m o r p h o l o g i c - a n a t o m i c a p p r o a c h is la r g e lyb a s e d o n L a t i n , i t r e a d i l y t r a n s c e n d s m a n y l a n -g u a g e b a r r i e r s a n d is e s se n t i a ll y i n t e r n a t i o n a l . T h eL a t i n - b a s e d s e g m e n t a l s y m b o l s a r e w i d e l y u n d e r -s t o o d ( F i g . 1 ) , w h e r e a s w o r d s a n d l o n g e r d e s c r i p -t iv e p h r a s es m a y n o t b e .

N e o l o g i s m sS i n c e l a n g u a g e i n g e n e r a l , a n d s c i e n ti f ic t e r m i n o l o -g y in p a r t i c u l a r , a r e c o n s t a n t l y e v o l v i n g , t h e r e c a nb e n o l a s t i n g status quo. A s E r n e s t W e e k l e y p u ti t, " S t a b i l i t y i n l a n g u a g e i s s y n o n y m o u s w i t h rigormortis." I n t e r m i n o l o g y , t h e r e f o r e , t h e o n l y c o n -s t a n t w e c a n e v e r k n o w i s c h a n g e . B u t s o m e c h a n g -e s a r e b e t t e r t h a n o t h e r s . C o n s e q u e n t l y , i t is im p o r -t a n t t h a t p h y s i c ia n s i n a c a d e m i c m e d i c i n e b e c o m ec o m p e t e n t a n d r e s p o n s ib l e n e o l o g is t s.C h a n g e s i n t e r m i n o l o g y a re o p e r a t i o n s o n t h et a x o n o m i c b o d y o f o u r s p e ci a li ty . C h a n g e s i n n o m -e n c l a tu r e , b o t h g o o d a n d b a d , c o n f u s e p r a c t it i o n -e r s. U n n e c e s s a r y c h a n g e s i n t e r m i n o l o g y , li k e u n -n e c e s s a r y s u r g e r y , s h o u l d t h e r e f o r e b e a v o i d e d . Asummary of suggestions fo r neologizing f o l l o w s :1 . D o n o t i n t r o d u c e a n y n e w t e r m u n l e s s i t i s a b s o -lu t e ly ne c e s sa r y , i . e . , un l e s s t he r e i s no e x t a n tt e r m , o r u n l e s s t h e e x is t i n g n o m e n c l a t u r e i s v e r yi n a c c u r a t e . T h i s i s t h e principle of necessity.2 . A v o i d v e r b o s i ty , w h e n f l a g r a n t k n o w n a s g o b -b l e d i g o o k [ 1 7] . T h i s i s t h e principle o f economy.3 . N e w t e r m s , i n a d d i t i o n t o b e i n g e s s e n t ia l a n db r i e f , m u s t a l s o b e s p e c i f i c , m u t u a l l y e x c l u s i v e ,

a n d m o r p h o l o g i c . T h i s is the principle o f specific-ity.N e w u n d e r s t a n d i n g ( b e i t a n a t o m i c , e m b r y o -l o g i c , o r e t i o l o g i c ) s e l d o m i s a v a l i d r e a s o n f o rc h a n g i n g d i a g n o s t i c t e r m i n o l o g y . N e w u n d e r -s t a n d i n g s h o u l d b e u s e d t o e n r i c h t h e o l d t e r m s ,n o t t o d e s t r o y t h e m . F o r e x a m p l e , j u s t b e c a u s ew e n o w t h i n k t h a t t r a n s p o s i t i o n o f t h e g r e a t a rt e r -i e s ( T G A ) i s d u e t o i n f u n d i b u l o - a r t e r i a l s i t u s d i s -c o r d a n c e [ 8 ], w e d o n o t t h i n k t h a t t h i s is a v a li dr e a so n f o r p r o p o s i n g t h a t T G A b e r e n a m e d . T h e r ei s n o t h i n g w r o n g w i t h t h e o l d t e r m " T G A " .H e n c e , t o c h a n g e t h i s t e r m w o u l d b e t o v i o l a t et h e p r i n c i p l e o f n e c e s s i ty . T h e p r i c e o f e a c h n e wi n s ig h t s h o u l d n o t b e c h a n g e s i n d i a g n o s t i c te r m i -n o l o g y . O t h e r w i s e o u r t e r m i n o l o g i c " i m p r o v e -m e n t s " w i l l s o o n r e s u l t i n a t o w e r o f B a b e l.L e t u s b r i e f l y c o n s i d e r o b j e c t i v e l y s o m e r e c e n tn e o l o g is m s . R e f e r e n c e s a re i n t e n t io n a l l y o m i t t e db e c a u s e i t i s n o t m y i n t e n t t o s u g g e s t p r a i s e o rb l a m e i n a n y p e r s o n a l s e n s e . W e a r e c o n s i d e r i n gt h r e e p r i n c i p l e s ( n e c e s s i ty , e c o n o m y , a n d s p e c if i ci -t y ) t h a t c o n s t i t u t e the bas is of scientific neologizing,a n d t h a t a p p l y t o a l l o f u s :( 1) " c r i s s c r o s s A V r e l a t i o n s , " a s u c c es s b e c a u s ei t i s n e c e s s a r y a n d g r a p h i c ;( 2 ) " p r i m i t i v e v e n t r i c l e , " a f a i l u r e - n o t b e c a u s eo f i ts e m b r y o l o g i c o r i g i n s ( m a n y s u c c e s s fu l cl i n ic a ld i a g n o s t i c te r m s a r e o f e m b r y o l o g i c p r o v e n a n c e- s e c u n d u m a t r i a l s e p t a l d e f e c t , p r i m u m a t r i a l s e p -t a l d e f e c t , s i n u s v e n o s u s a t r i a l s e p t a l d e f e c t , c o m -m o n A V c a n a l , t r u n c u s a r t e r i o s u s c o m m u n i s , p a -t e n t d u c t u s a r t e r i o s u s ) - b u t b e c a u s e " p r i m i t i v ev e n t r i c l e " h a s n o s p ec i fi c m o r p h o l o g i c a n a t o m i cm e a n i n g ( a v i o l a t i o n o f t h e p r i n c i p le o f s p e c i f ic i t y );(3 ) " m a i n c h a m b e r , " " a c c e s s o r y c h a m b e r , "" o u t l e t c h a m b e r , " a n d " t r a b e c u l a t e d p o u c h " a ls oh a v e n o s p e c i f i c m o r p h o l o g i c a n a t o m i c m e a n i n g s( a d d i t i o n a l v i o l a t i o n s o f t h e p r i n c i p l e o f s p ec i fi c i-t y : i n te r m s o f m o r p h o l o g i c a n a t o m y , t h e r e a r eo n l y th r e e c h a m b e r s b e l o w t h e a t r i a - L V , R V , a n di n f u n d i b u l u m ) ;( 4) " u n i v e n t r i c u l a r h e a r t o f l e ft v e n t r i c u la rt y p e ," a n u n n e c e s s ar y a n d w o r d y s y n o n y m f o r" s i n g l e L V " ( v i o l a t io n s o f t h e p r i n c i p l es o f n e c es s i -t y a n d e c o n o m y ) ; " u n i v e n t r i c u l a r h e a r t o f le f t v e n -t r i c u l a r t y p e w i t h a b s e n t r i g h t a t r i o - v e n t r i c u l a rc o n n e c t i o n , " j a r g o n f o r " t r ic u s p i d a t r e s i a " ( ig -n o r e s t h e p r i n c ip l e s o f n e c e s si t y a n d e c o n o m y ) ;a n d (5 ) " a n t e r i o r c o m m u n i c a t i n g A V n o d e , " n e c es -s a r y a n d c o n c i s e , h e n c e a s u c c e s s . T h u s g o o d n e o -l o g i s m s a r e n e c e s s a r y , b ri e f , a n d s p e c i f i c ; b a d o n e sa r e n o t .W e s h o u l d a d h e r e t o morphologic-anatomic ter-

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R. Van Praagh" Diagnosis of Complex Congenita l Hear t Disease 119m i n o l o g y ( e . g . , R A , L A , R V , L V , i n f u n d i b u l u m ,e t c . ) a s i n F i g u r e 1 . M o r p h o l o g i c - a n a t o m i c t e r m sa r e s t r ic t ly l im i t e d b y m o r p h o l o g i c - a n a t o m i c re a l i-t y. N o n - m o r p h o l o g i c - a n a t o m i c t e r m s - d e s i g n a-t io n s t h a t h a v e n o s p e ci fi c m o r p h o l o g i c a n a t o m i cm e a n i n g - s h o u l d b e a v o i d e d ( e. g. , " m a i nc h a m b e r , " " a c c e s s o r y c h a m b e r , " " o u t l e tc h a m b e r , " " t r a b e c u l a t e d p o u c h , " " p r i m i ti v e v e n-t r i c l e " ) .T h e t e r m i n o l o g y o f c o n g e n i ta l h e a r t d i s e a ses h o u l d n o t v i o l a t e t h e pr inc ip l e s o f l og ic . F o r e x a m -ple , one v ar iab le c anno t be use d to de f ine ano the rvariable . F o r i n s t a n c e , v e n t r i c u l a r s i tu s i s o n e v a r i -a b l e . A V a l i g n m e n t ( e . g . , c o n c o r d a n t o r d i s c o r -d a n t ) is a n o t h e r ( a n d a d i f f e r e n t ) v a r i a b le . C o n s e -q u e n t l y , o n e c a n n o t d i a g n o s e t h e v e n t r i c u l a r s i t u s( D - l o o p o r L - l o o p ) w i t h c o n s i s t e n t a c c u r a c y b ym e a n s o f th e A V a l i g n m e n t s ( c o n c o r d a n t o r d is c o r -d a n t ) , b e c a u s e t h e s e a r e t w o d i f f e r e n t v a r i a b l e s[7 , 8 ]. S i m i l a rl y , o n e c a n n o t w i t h c o n f i d e n c e d i a g -n o s e t h e a n a t o m i c s t a t u s o f t h e v e n t r i c le s ( e. g ., t h ep r e s e n c e o r a b s e n c e o f s i n g l e v e n t r i c l e ) b y m e a n so f t h e A V a l i g n m e n t s b e c a u s e , a g a i n , t h e s e a r e t w od i f f e r e n t va r i a b l e s [ 7, 8] . Ea c h va r i a b l e m u s t bed e f i n e d i n t e r m s o f i ts e lf . T h u s , t h e d i a g n o s i s o fs in g le v e n tr i cl e d e p e n d s o n t h e a n a t o m y o f t h e v e n -t r i c u l a r p o r t i o n o f t h e h e a r t , n o t o n t h e s t a t u s o ft h e A V a l i g n m e n t s [ 1 8 ]. E a c h is a n i n d e p e n d e n tv a r i a b l e . F o r e x a m p l e , d o u b l e - i n l e t R V o c c u r sw i t h a n d w i t h o u t s i n g l e R V . S i n g le L V o c c u r s w i t ha n d w i t h o u t d o u b l e - i n le t L u [ 18 , 1 9] . I n d e p e n d e n tv a r ia b l e s s h o u l d b e d i a g n o s e d i n d e p e n d e n t l y ; o n ec a n n o t b e u s e d t o i n f e r t h e o t h e r .F r e e d o m o f s p e ec h - i n c l u d i n g t h e r i g h t t o u s ew h a t e v e r t e r m s o n e h a p p e n s t o p r e f e r - is t h e l if e 'sb l o o d o f a ll f o r m s o f s c ie n c e , i n c l u d i n g c a r d i o l o g y .E n f o r c e d t e r m i n o l o g i c c o n f o r m i t y , f o r a n y " r e a -s o n " w h a t s o e v e r , m u s t b e av o i d e d . T e r m i n o l o g ie sa n d c o d i n g s y s t e m s s h o u l d b e descr ipt ive ( b a s e do n t h e t e r m s t h a t a r e a c t u a l l y b e i n g u s e d ) , n o t pre -scr ip t ive ( b a s e d o n t h e t e r m s t h a t " s h o u l d " b eu s e d ) . I n a l l f o r m s o f l a n g u a g e , u s a g e i s k i n g .T h e t e r m i n o l o g ic a s p e c t s o f c o n g e n i t a l h e a r td i s e a s e s h o u l d b e de - e mphas i ze d . T e r m i n o l o g y isn o t o n e o f t h e g r e a t p r o b l e m s f a c in g p e d i a t ri c c a r -d i o lo g y . I n d e e d , w e t h i n k t h a t t h e t e r m i n o l o g y o fc o n g e n i t a l h e a r t d i s e a s e is l a rg e l y a s o l v e d p r o b l e m( F i g . 1 ). N o t e r m i n o l o g y is p e r f e c t, h o w e v e r , a n da l l l a n g u a g e m u s t k e e p o n e v o l v i n g , o r d i e . B u tn u m e r o u s u n n e c e s s a r y ch a n g e s a n d s y n o n y m i z i n g( s u c h a s u n i v e n t r i c u l a r h e a r t i n s t e a d o f s i n g le v e n -t r ic l e ) s h o u l d b e a v o i d e d o n p r i n c ip l e .T h e r e a l p ro b l e m s f a c in g t h o s e w h o a r e p r o fe s -s i o n al ly c o n c e r n e d w i t h c o n g e n i t a l h e a r t d i s e a s ei n c l u d e :

( 1 ) H o w c a n w e i m p r o v e d i a g n o s t i c a c c u r a c y ,b o t h p r e n a t a l l y a n d p o s t n a t a l ly ?( 2 ) H o w c a n w e d e li v e r m o r e e f f e c t iv e t h e r a p y ,b o t h m e d i c a l a n d s u r g ic a l ?( 3) H o w c a n w e a c q u i r e a b e t te r u n d e r s t a n d i n go f e t io l o g y a n d m o r p h o g e n e s i s , i n o r d e r t o f ac il i-t a te t h e p r e d i c ti o n a n d p r e v e n t i o n o f c o n g e n i ta lh e a r t d i s e a s e ?I t i s a s o b e r i n g f a c t t h a t d e s p i t e o u r e x c e l l e n td i a g n o s t i c a n d t h e r a p e u t i c f a c i l i t i e s , c o n g e n i t a lh e a r t d i s e a s e re m a i n s a l e a d i n g c a u s e o f d e a t h a to u r h o s p i t a l [ 2 0 ] a n d a t o t h e r c h i l d r e n ' s h o s p i t a l sn a t i o n w i d e . H e n c e , I w o u l d g e n t l y s u g g es t t h a t t h er e n a m i n g o f c o n g e n it a l h e a r t d i se a s e s h o u l d b eq u i e t l y d i s c o n t i n u e d , n o t o n l y b e c a u s e i t i s u n n e c -e s s a r y , b u t a l s o b e c a u s e w e h a v e m a n y m o r e i m -p o r t a n t t h i n g s to d o .Acknowledgements. My thanks to Gloria Gaskill for clericalassistance and to Terrence Wrightson and his colleagues forphotography.Reference s

1. Van Praagh R, Ongley PA, Swan HJC (1964) Anatomictypes of single or common ventricle in man, morphologicand geometric aspects of sixty autopsied cases. Am J Car-diol 13 : 367-3862. Van Praagh R, Van Praagh S, Vlad P, Kei th JD (1964)Anatomic types of congenital dextrocardia, diagnostic andembryologic implications. Am J Cardiol 13 : 510-5313. Van Praagh R (1972) The segmental approach to diagnosisin congential heart disease. The cardiovascular system(Original Article Series). Birth Defects 8:4.234. Van Praagh R (1977) Terminology of congential heart dis-ease, glossary and commentary. Circulation 56:139-1435. Van Praagh R, Weinberg PM, Van Praagh S (1977) Malpo-sition of the heart. In: Moss AJ, Adams FH, Emmanoui-lides GC (eds) Heart disease in infants, children and adoles-cents, 2nd edn. Williams and Wilkins, Baltimore, pp 394-4176. Van Praagh R, Vlad P (1978) Dextrocardia, mesocardia,and levocardia: The segmental approach to diagnosis incongenital heart disease. In: Kei th JD, Rowe RD, VladP (1978) Heart disease in infancy and childhood. Macmil-lan, New York, pp 638-6957. Van Praagh R, David I, Gordon D, Wright GB, Van PraaghS (1981) Ventricular diagnosis and designation. In: G odmanMJ (ed) Paediatric Cardiology, World Congress, Lond on

1980, vol.4, Churchill Livingstone, Edinburgh, pp 153-1688. Van Praagh R, Van Praagh S (1982) Embryology and anat -omy : Keys to the understanding of complex and congenitalheart disease. Coeur 13 : 315-3369. Shinebourne EA, Macartney FJ, Anderson RH (1976) Se-quential chamber localization - logical approach to diagno-sis in congenital heart disease. Br Heart J 38 : 327-34010. Biichler JR, Rabelo R, Marino R, David I, Van PraaghR (1980) Double outlet right at rium: Autopsied case ofnewly recognized entity . World Congress of Paediatric Car-diology, London, (abstr 233) 198011. Rao PS (1981) Systematic approach to differential diagno-sis. Am Heart J 102:389-40312. Otero Coto E, Quero Jimenez M (1977) Aproximacion seg-mentaria al diagnostico y clasificacion de las cardiopatias

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120 R. Van Praagh: Diagnosis of Complex Congenital Heart Diseasecongenitas. Fundamentos y utilidad. Rev Esp Cardiol30:557-56613. Stanger P, Rudolph AM, Edwards JE (1977) Cardiac mal-positions, an overview based on study of sixty-five necropsyspecimens. Circulation 56:159-17214. Tyn an MJ, Becker AE, Macartney F J, Quero Jimenez M,Shinebourne FA, Anderson RH (1979) Nomenclature andclassification of congenital heart disease. Br Heart J41:544-55315. Calcaterra G, Anderson RH, Lau KC, Shinebourne EA(1979) Dextrocardia - value of segmental analysis in itscategorization. Br Heart J 42:497-50716. Ando M, Santomi G, Takao A (1980) Atresia of tricuspidor mitral orifice: Anatomic spectrum and morphogenetichypothesis. In : Van Praagh R, Takao A (eds) Etiology andmorphogenesis of congenital heart disease. Futura, Mr.Kisco, New York, pp 421-48717. Pei M (1965) The story of language. Language and politicalinstitutions. Mentor Books, New American Library, NewYork, pp 263-26418. Van Praagh R, Plet t JA, Van Praagh S (1979) Single ventri-cle: Pathology, embryology, terminology, and classification.Herz 4:113-150

19. Van Praagh R, Wise JR, Dahl BA, Van Praagh S (1980)Single left ventricle with infundibu lar outlet chamber andtricuspid valve opening only into outlet chamber in a44-year-old man with thoracoabdomina l ectopia cordiswithout diaphragmatic or pericardial defect: Importance ofmyocardial morphologic method o f chamber identificationin congenital heart disease. In: Van Praagh R, Takao A(eds). Etiology and morphogenesis of congenital heart dis-ease, Futura, Mr. Kisco, New York, pp 379-42020. Van Praagh R, Visner MS (1976) The postoperative pathol-ogy of congenital heart disease. Am J Cardiol 38:225-23021. Van Praagh R, Durnin RE, Jockin H, Wagner HR, KornsM, Garabedian H, Ando M, Calder AL (1975) Anatomi-cally corrected malposition of the great arteries {S,D, L}.Circulation 51:20-3122. Van Praagh S, Davidoff A, Chin A, Shiel FS, ReynoldsJ, Van Praagh R (1982) Double-outlet right ventricle: Ana-tomic types and developmental implications based on astudy of 101 autopsied cases. Coeur 13:389-439

23. Van Praagh R, Weinberg PM (1977)Double-out let left ven-tricle. In: Moss A J, Adams FH, Emmanouilides GC (eds)Heart disease in infants, children and adolescents. Wil liamsand Wilkins, Baltimore, pp 367-380