ACUTE RHEUMATIC FEVER

Aetiology :
Pharyngeal infection with Lancefield Group A
Streptococci
Diagnoi :
The !one Criteria "#$%%& :
-
E'idence of a (receeding )eta*hae+olytic
Streptococcus infection
-
Two Ma,or Criteria- or
-
.ne +a,or and two +inor criteria
Re'ied !one Criteria for Rhe/+atic Fe'er :
Ma,or Minor in addition
Carditi Fe'er Recent tre(t0 Infect0
Arthriti Pre'io/ Rh fe'er Hitory of carlet fe'er
1ydenha+2 chorea Raied E1R or CRP Poti'e throat wa)
Erythe+a +arginat/+ Arthralgia Raied A1. titre
1/)c/taneo/ nod/le Long PR inter'al Raied anti * D3ae 4
Titre
Clinical feat/re :
-
Carditi
-
Arthriti
-
3od/le
-
Erythe+a +arginat/+
-
1ydenha+2 chorea (St Vitus dance)
In'etigation :
-
3o (ecific tet0
-
CRP 5 - anti*tre(tolyin .
-
Anti*D3ae 5 - E1R 5
-
Anti*hyal/rodinae anti)ody " (re'io/ 1tre(t inf &
1
-
Echocardiogra(hy "early change of tretching of the
anterior +itral chordae&
Treat+ent :
-
1alicylate ra(idly red/ce fe'er and arthriti0 Doe
#66+g78g7day in children0 1er/+ le'el ho/ld )e #9*
:6+g7dl
-
1teroid are /ed rather than 1alicylae for (t with
definite carditi : Predniolone ; < +g78g7day in
de'ided doe for : wee8 ta(ering off =/ic8ly0 If
y+(to+ rec/r the co/re i retarted0
-
Dia>e(a+ i /ed for 1ydenha+2 chorea0
-
Penicilline0 I++ediate treat+ent with 4en>athine
)en>yl(enicillin #0: +illion /nit i0+0 Pre'ent f/rther
atta8 )y /ing Penicillin V0 :96 +g )0d "/( to the age
<6 yr&0 For Penicillin hy(ereniti'ity /e 1/lfadia>ine
966 +g )0d
Mitral 1tenoi0
Aetiology0
Val'ar0
-
Rhe/+atic : al+ot all cae0
-
Congenital : iolated leion or aociated with A1D
"L/te+)acher2 yndro+e&0 1o+e Rhe/+atic M1 (l/
PF.0
-
M/co(olyaccharidoe: H/rler2 yndro+e0 ?lyco*
(rotein de(oition on the +itral leaflet0
-
Endocardial fi)roelatoi (reading on to the 'al'e0
-
Prothetic 'al'e : //ally only in +echanical 'al'e0
"e0g 1tar*Edward- 4,or8*1hiley&
-
Malignant carcinoid0
Inflow o)tr/ction
Condition which +i+ic M1- e0g
-
Left atrial +y@o+a
-
Left atrial )all 'al'e thro+)/
2
-
Hy(erthro(hic .)tr/cti'e Cardio+yo(athy
-
Cor Triatriat/+ "1tenoi of a co++on P/l+onary V0&
Pathogenei
?ro/( A "//ally ty(e #:& 1tre(tococci ha'e cell wall antigen
that cro*react with tr/ct/ral glyco(rotein of the heart
'al'e0 1tenoi occ/r at three le'el :
-
Co++i/re : thee f/e with the 'al'e c/( till
+o)ile
-
C/( : the 'al'e leaflet )eco+e thic8 and e'ent/ally
calcified0
-
Chordae : thee f/e- horten and thic8en0
A co+)ination of all three re/lt in a Afih +o/thB
)/ttonhole orifice0
Patho(hyiology and y+(to+0
#0 Dy(noe on effort : ortho(noea and P3D0 A raiing
LA (re/re i tran+itted to (/l+onary 'ein0 A
econdary PA hy(ertenion re/lt0 P/l+onary oede+a
+ay )e (reci(itated )y :
- De'elo(+ent of /ncontrolled AF
* Pregnancy
* E@ercie
* Chet infection
* E+otional tre
* Anaetheia
:0 Fatig/e : d/e to low C.P in +oderate to e'ere
tenoi0 E@ercie tolerance i claified into % clae
3CHA0
<0 Hae+o(tyi0 May )e d/e to :
* 4ronchial 'ein r/(t/re : A(/l+onary a(o(le@yB-
large hae+orrhage )/t not //ally life threatening0
* Al'eolar ca(illary r/(t/re : (in8 frothy (/t/+ in
(/l+onary oede+a0
* P/l+onary infarction : in low*o/t(/t tate and
3
i++o)ile (t0
* 4lood*tained (/t/+ : in chronic )ronchiti D dy(0
%0 1yte+ic e+)oli : in :6*<6E0 Thro+)/ de'elo( in
Ftagnant2 LA and Atrial a((endage- +ainly in (t with
AF- low o/t(/t and large atria0 It +ay )e (reenting
y+(to+0 Meenteric- addle and iliofe+oral e+)oli
are co++on0 4all 'el'e thro+)/ +ay occ/re in LA0
90 Chronic )ronchiti: co++on in M10 D/e to oede+a*
to/ )ronchial +/coa0
G0 Chet (ain : li8e angina0 In (t with RVH econdary to
(/l+onary hy(ertenion- e'en with nor+al coronarie0
Coronary e+)oli+ +ay occ/r0
H0 Pal(itation : (aro@y+al AF with fat 'entric/lar
re(one0
I0 1y+(to+ of Rigt Heart Fail/re : PH and (oi)le
f/nctional TR- he(atic (ain on effort "he(atic angina&-
acite- an8le and leg oede+a0
$0 1y+(to+ of LA enlarge+ent co+(reing other
tr/ct/re :
* Left rec/rrent laryngeal ner'e-hoarene ".rtner2
yndro+e&0
* .eo(hag/- dy(hagia ")eware (otai/+ re(lace*
+ent ta)let ca/ing oeo(hageal /lceration&
* Left +ain )ronch/- 'ery rarely ca/ing left l/ng
colla(e0
#6- Infecti'e endocarditi : rare in (/re M10
Phyical ign 0
#0 1# lo/d0 Indicate +o)ile leaflet
:0 A: * .1 inter'al horten with increaing e'erity of
tenoi0 LA (re/re Fcli+)2 /( LV (re/re c/r'e
a((roaching in ti+e aortic 'al'e clo/re0
<0 The length of the diatolic +/r+/r i an indication of
the e'erity of tenoi0
4
Differential Diagnoi
#0 Ca/e of inflow o)tr/ction "HCM-LA My@o+a- )all*'al'e
thro+)/&
:0 Ca/e of r/+)ling +itral or tric/(id diatolic +/r+/r :
-
aortic reg/rgitation
-
flow +/r+/r in A1D J thi +ay )e conf/ing
-
tric/(id tenoi- diatolic +/r+/r accent/ated )y
in(iration0
<0 Ca/e of early diatolic o/nd ree+)ling o(ening na(
"/dden ceation of early ra(id 'entric/lar filling& :
-
contricti'e (ericarditi
-
retricti'e +yo(athy
%0 Ca/e of lo/d 1# : tachycardia and hy(erdyna+ic tate
" 'al'e till o(en at end diatole and forcef/l )y hy(er*
contractile LV&
E C ?0
Atrial Fi)rilalation "in 1in/ Rhyt+ P +itrale&0 RV hy(er*
Tro(hy0 1+all 'oltage in lead V#0 Progrei'e RAD0
Medical treat+ent0
#0 Digo@in K in AF only0 If fat AF i not lowed )y tandart
doe- either a +all doe of )eta*)loc8ing agent or
'era(a+il ho/ld )e added0 There i no e'idence that
digo@in (re'ent the de'elo(+ent of AF in (t who are till
in in/ rhyt+0
:0 Di/retic are neceary to red/ce (reload and (/l+onary
'eno/ congetion0 They +ay hel( delay the need for
/rgery0
<0 Anticoag/lant are till contro'erial0 They ho/ld )e /ed
in (t who :
-
ha'e had a (re'io/ yte+ic7(/l+onary e+)oli+-
-
ha'e a +etal (rothei "ti/e or +echanical&-
-
ha'e low*o/t(/t tate with right heart fail/re-
5
-
are in AF with +oderate M1 and who ha'e had an
atrial a((endicectoty0
Anticoag/lant are not (ro'en )enefit in in/ rhyt+0
They ho/ld )e a'oided in (regnancy if (oi)le0
Cardio'erion0
May )e atte+(ted if the de'elo(+ent of AF i recent and the
(t i anticoag/lated0 If not- there i a ri8 of yte+ic e+)oli0
* Dr/g cardio'erion0
Thi i +ot li8ely to /cceed with recent*onet AF0
Fleccainide : +g78g i0' o'er #6 +in i the dr/g of
choice )/t ho/ld )e a'oided in (t with (oor LV
f/nction0 Dio(yra+ide 96*#96 +g i0' lowly o'er 9
+in i an alternati'e0 A+iodarone gi'en orally ":66
t0d0 /( to %66 +g t0d0 for # wee8& +ay alo re/lt in
'erion to 1R0 The doe i red/ced after # wee80
A+iodarone i alo 'ery /ef/l: 9 +g78g i0' o'er % hr in
9E de@troe0 Ma@i+/+ i0' doe o'er :% hr in an ad/lt
i #:66 +g0 The (t can )e con'erted to oral
a+iodarone when (ractical0
Mitral 'al'/lo(laty0
The de'elo(+ent of the Ino/e )alloon i a great ad'ance
fro+ the original do/)le*)alloon and two wire techni=/e-and
MV(laty ha now re(laced the o(eration of cloed +itral
'al'/loto+y0 I the techni=/e of choice for (t with (/r M1 )/t
no reg/rgitation or +itral 'al'e calcification0
Val'/lo(laty i a'oided with:
-
LA or LV thro+)/
-
Hitory of yte+ic e+)oli
-
More than ?rade # +itral reg/rgitation
-
Thic8ened rigid +itral leaflet
-
Thic8ened f/ed +itral chordae
-
Moderate or e'ere +itral calcification
6
1/rgery0
#0 Cloed +itral 'al'/loto+y "cloed co++i/roto+y&
:0 .(en +itral 'al'/loto+y "o(en co++i/roto+y&
<0 Mitral 'al'e re(lace+ent0
Mitral Reg/rgitation "M In/fficiency&
Thi +ay )e d/e to a)nor+alitie of the +itral ann/l/- the
+itral leaflet- the chordae or the (a(illary +/cle0 Chordal
or the (a(illary +/cle dyf/nction i /)'al'ar MR0
Aetiology
-
F/nctional +itral reg/rgitation
Pro)a)ly a co+)ination of +itral ann/l/ dilatation
and (a(illary +/cle +alalign+ent0 It occ/r in LV
dilatation fro+ any ca/e- co++only in DCM ang
ichae+ic heart dieae0
-
Ann/l/ calcification
.cc/r in the elderly and i +ore co++on in fe+ale-
dia)etic and (t with Paget2 dieae0The calci/+ +ay
in'ol'e the +itral leaflet- ca/ing MR and e'ent/ally
the cond/cting yte+0 Mitral ann/lar calcification i
an inde(endent ri8 factor for tro8e0
-
Val'ar reg/rgitation
Co++only d/e to RF- infecti'e endocarditi or flo((y
'al'e0 In rhe/+atic ca/e the c/( are thic8ened-with
f/ed co++i/re and often a Ffih +o/th2orifice0Pt
co++only ha'e co+)ined M1 L MR
-
Chordal r/(t/re0
.ften idio(athic. My@o+ato/ degeneration in the
flo((y 'al'e yndro+e +ay alo in'ol'e the chordae-
which tretch and e'ent/ally r/(t/re0 Ichae+ic +ay
ca/e chordal r/(t/re0
7
* Pa(illary +/cle dyf/nction0
Inferior infarction co++only ca/e (oterior
(a(illary +/cle dyf/nction with characteritic
ign0 Anterior (a(illary +/cle dyf/nction i +/ch
rarer and ignifie a large anterior infarct with
(ro)a)le additional right coronary artery dieae0
The Flo((y Val'e
Thi for+ a (ectr/+ of condition fro+ an ay+(to+atic
(t with +idytolic clic8 to one with e'ere MR fro+ chordal
r/(t/re0 " Mitral leaflet (rola(e- +itral clic8 ytolic +/r+/r
yndro+e- 4arlow2 yndro+e- +y@o+ato/ degeneration of
+itral 'al'e- )illowing +itral 'al'e yndro+e&0
It occ/r a the following :
-
an iolated leion often in ay+(to+atic (t0
-
Aociated with other condition e0g0 A1D II- T/rner2
yndro+e- PDA- Marfan2 1yndro+e- oteogenei
i+(erfecta- PM elatic/+- cardio+yo(athy- NPN
yndro+e0
Patho(hyiology and y+(to+0
Mild cae of MR +ay )e ay+(to+atic for +any year0
Mot (t fall into one of two gro/( de(ending on the ti+e*
co/re of e'ent- and the i>e7co+(liance of the LA0
In ac/te MR the +all LA cannot a)or) the reg/rgitant
fraction and the ytolic wa'e i tran+itted to the (/l+onary
'ein- with re/lting ac/te (/l+onary oede+a0
In long tanding MR the LA i large- it can a)or) the
reg/rgitant fraction and the2'2 wa'e tran+itted to the
(/l+onary 'ein i +all0
1y+(to+ are i+ilar to M1 in the chronic tate0
Hae+o(tyi and yte+ic e+)oli are le fre=/ent0
?enerally- (/l+onary hy(ertenion and right heart
y+(to+ are not a fre=/ent in MR a in (/re M10 Howe'er-
infecti'e endocarditi i +ore co++on in MR0
8
Phyical ign in the flo((y 'al'e yndro+e0
Thee 'ary with the degree of MR0 Nith +ild or
+oderate degree of MR- with e'ere reg/rgitation (hyical
ign are le (ecific0
A(e@ )eat0
A do/)le a(e@ +ay )e noted in o+e (t with flo((y
'al'e0 Tening of the chordae in +id ytole +ay ca/e thi
+idytolic di(0 It i )et felt when the (t i lying on hi or her
left ide0
M/r+/r0
A LV 'ol/+e di+inihe in +id ytole the flo((y 'al'e
tart to (rola(e and a +idytolic clic8 "tening of chordae&
often (recede the +/r+/r of MR "there +ay )e +ore than
one clic8& 0 In 'ery +ild cae a +idytolic clic8 with no
+/r+/r i co++on0
Differential Diagnoi0
#0 Aortic 'al'e tenoi :the flo((y 'al'e ha a nor+al or
lightly colla(ing (/le0 The +idytolic clic8 occ/r
after the carotid /(tro8e0
:0 HCM : thi i +ore diffic/lt )eca/e )oth +ay ha'e
i+ilar (/le- do/)le a(e@ )eat and +/r+/r getting
lo/der on a+yl nitrat inhalation0 HCM doe not ha'e a
+idytolic clic80
<0 V1D : here the +/r+/r i //ally (anytolic with a
thrill- )oth +a@i+/+ at L140
%0 Pa(illary +/cle dyf/nction : claically (otinferior
infarct0 The +/r+/r +ay )e late ytolic )/t witho/t a
clic80 In +or e'ere cae the +/r+/r i (anytolic0
90 Tric/(id reg/rgitation : an Fin(iratory2 +/r+/r lo/det
at L140 4et ign i (ro+inent ytolic wa'e in the !VP0
Phyical ign in chronic 'al'ar MR0
9
#0 1/dden (re+at/re 'entric/lar e+(tying d/e to MR
re/lt in early aortic 'al'e clo/re0 The +/r+/r +ay
contin/e thro/gh A:01: i th/ +ore than nor+ally
(lit and P: +ay )e lo/d if additional PH i (reent0
:0 Feat/re to /gget chordal r/(t/re a o((oed to
'al'ar reg/rgitation :
* 1in/ rhyt+
* a(ical thrill in ytole
* +/r+/r i +ore e,ection in =/ality and o+eti+e
+id to late ytolic0
<0 In (oterior chordal r/(t/re- the ,et i directed to the
anterior wall of the LA0 The +/r+/r i often lo/det
at L140 In anterior chordal r/(t/re- the ,et i directed
(oteriorly and the +/r+/r +ay )e lo/det in the
)ac80
EC?0
#0 AF in chronic dieae0 If in 1R : LA O0
:0 LVH
<0 A few cae how right 'entric/lar hy(ertro(hy in
addition0
Echocardiogra(hy0
#0 1how LA i>e with ytolic e@(anion
:0 May how flail +itral leaflet with chaotic +o'e+ent0
<0 May how (oterior MLP7MVP J late or (anytolic:
'egetation on +itral al'e- +itral ann/l/ calcification0
%0 Dilated LV with ra(id filling0 Di+enion relate to
(rognoi0
90 Ra(id diatolic +itral clo/re rate0 "tee( E*F lo(e&
d/e to ra(id filling0
G0 Mean Velocity of circ/+ferential fi)re hortening
"VCF& often increaed with good LV f/nction0
H0 Poi)ly additional flo((y tric/(id or aortic 'al'e0
10
I0 Do((ler eta)lihe i>e and ite of reg/rgitant ,et0
$0 TEE gi'e the clearet 'iew of the +itral leaflet or of a
+alf/nctioning (rothetic 'al'e0
Chet M*ray0
-
LV dilatation enlarging the 'entric/lar +a and left
heart )order0
-
LA dilatation in chronic cae0 Rarely giant LA +ay
occ/r with calcified wall0
-
Mitral 'al'e calcification- ign of (/l+onary 'eno/
congetion- Perley 4 line a in M10
Medical treat+ent0
-
A in M1 fat AF i treated with Digo@in or 4eta*
)loc8er0
-
Anticoag/lant are not indicated /nle there i a
hitory of yte+ic e+)oli+K a (rothetic MV- either
@enograft or +echanicalK additional M1 with low
o/t(/t0
-
Di/retic are needed to red/ced (/l+onary 'eno/
congetion and LV (reload0
-
Afterload red/ction with IV 3itrate or 3itro(r/ide
i indicated in Ac/te MR0 ACE inhi)itor are /ed
e+(irically )/t with little e'idence of their long ter+
)enefit0
-
In ac/te MR with chordal r/(t/re and (/l+onary
oede+a- artificial 'entilation and f/ll +onitoring a in
cardiogenic hoc8 +ay )e neceary0
-
Infecti'e endocarditi ho/ld )e conidered0
Prognoi0
A in Chronic AR- chronic MR i relati'ely well*tolerated
leion if LV f/nction i (reer'ed0 A((ro@i+ately G6E of (t
with chronic MR are ali'e #6 yr later0
Poorer (rognotic feat/re are :
11
-
y+(to+atic hitory O # year
-
atrial fi)rillation
-
(atient aged O G6 yr
-
angiogra(hic EF Q 96E
-
angiogra(hic LVEDV O #667+
:
and LVE1V O G67+
:
-
echocardiogra(hic di+enion of LV : end*ytolic
di+enion O 9 c+- end*diatolic di+enion O H c+0
1/rgery0
All (t with (/re MR ho/ld ha'e TEE (rior to
theo(eration to hel( deter+ine which (t are /ita)le for
+itral 'al'e re(air0 In e'ere ac/te MR with (/l+onary
oede+a thi will need to )e done on a 'entilated (t0 TEE i
re(eated intra o(erati'ely i++ediately after the re(air to
chec8 for reid/al 'al'e reg/rgitation0 1o+e reco'ery of LV
f/nction after /rgery i (oi)le )/t thi ta8e +any +onth0
#0 Ac/te MR with chordal r/(t/re : /rgery i neceary in
+ot cae )eca/e +edical treat+ent alone carrie a
(oor (rognoi0 ?enerally re(air i reer'ed for ingle
(rola(ing leaflet- with 'al'e re(lace+ent (referred if
)oth leaflet are in'ol'ed0
:0 Chronic MR : MVR ho/ld )e (erfor+ed )efore LV
f/nction deteriorate irre'eri)ly0 1/rgery i indicated
for y+(to+ of increaing fatig/e and dy(noea0 "3CHA
?rade < and %&- and alo in (t <ith ?rade : y+(to+
who ha'e enlarging heart on CMR and increaing
dy(noea0 Ann/lo(laty i generally not a 'ery
atifactory (roced/re - altho/gh o+eti+e /ef/l in (t
with a groly dilated ann/l/ "a in Dilated CM&0
<0 Pot infarct MR : (a(illary +/cle infarction or r/(t/re
//ally re=/ire /rgent MVR witho/t delay0 Inteni'e
'aodilator thera(y or IA4P +ay hold the it/ation for a
few ho/r )/t no /)tit/te for /rgery0
12
Aortic 1tenoi 0
Le'el of Aortic 1tenoi0
A1 +ay occ/r at three le'el and the three are not +/t/ally
e@cl/i'e0
#0 Val'ar aortic tenoi
:0 1/(ra'al'ar tenoi
<0 1/)'al'/lar aortic tenoi0 Thi +ay d/e to :
-
dicrete fi)ro+/c/lar ring
-
HCM
-
T/nnel /)aortic tenoi
-
Ano+alo/ attach+ent of anterior +itral leaflet- e0g in
AV canal- or (arach/te defor+ity of +itral 'al'e with
f/ed (a(illary +/cle0
Val'ar Aortic 1tenoi 0
The co++onet ca/e of A10 It doe not ha'e a ingle
aetiology0
#0 Congenital 'al'ar a)nor+ality : The co++onet ca/e of
iolated A1- H:E in one erie0 More fre=/ent in +ale
"%:#&0
-
4ic/(id 'al'e " #E of the (o(/lation&0 The
co++onet for+ of congenital heart dieae0 4oth
ty(e )eco+e increaingly fi)rotic and calcified with
age0 "Age %6 * R G6 yr&
-
.ther degree of co++i/ral f/ion : /nico++i/ral
with eccentric hole- or e'en dia(hrag+ "three f/ion
c/(& with central orifice0 It often (reent a (art of
the hy(o(latic left heart yndro+e0
:0 1enile calcification of a nor+al 'al'e : .cc/r in the age
gro/( O G6 yr0 The 'al'e i tric/(id0 The co++i/re
are not f/ed )/t the c/( are i++o)ili>ed )y hea'y
calcification0 Thi often ca/e an e,ection ytolic
+/r+/r0
<0 Infla+atory 'al'/liti0 RF re/lt in co++i/ral f/ion of
a tric/(id 'al'e0 The 'al'e i //ally reg/rgitant0
13
%0 Atherocleroi0 1e'ere hy(ercholeterolae+ia in
ho+o>ygo/ ty(e II hy(erli(o(roteinae+ia0 ?ro
athero+a in'ol'e aortic wall- +a,or arterie - aortic 'al'e
and coronary arterie0
Dieae (rogreion0
Val'ar o)tr/ction grad/ally increae e'en in children who
+ay )e ay+(to+atic0 Progrei'e 'al'e calcification occ/r-
and +ay )e 'ii)le on CMR fro+ a)o/t the age of %6 yr
onward0 The e'erity of calcification correlate ro/ghly with
the degree of tenoi0
Patho(hyiology and y+(to+0
#0 Co+(enated : good LV f/nction with 'al'e area O # c+
:
0
May )e ay+(to+atic 0 Children +ay )e ay+(to+atic
with e'ere dieae0 Ad/lt +ay not (reent /ntil age O G6
yr0
:0 Angina : occ/r with nor+al coronary arterie0 D/e to
i+)alance of +yocardial o@ygen /((ly and de+and0
Increaed de+and Decreaed /((ly
5 Cardiac wor8 Prolonged ytole with horter diatole
5 M/cle +a fro+* Re'ered coronary flow in ytole fro+
Hy(ertro(hy 'ent/ri effect of narrow 'al'e orifice
5 Nall tre fro+ J High intra+/ral (re/re in ytole *
High intraca'ity* (re'enting ytolic coronary flow0
(re/re: )oth in* Low aortic (erf/ion (re/re in dia*
ytole and diatole0 tole with high LVEDP0
Rarely calcification e@tending to coro*
nary otia0
14
<0 Dy(noea: occ/r d/e to high diatolic (re/re in the LV
increaing with e@ercie0 A LV f/nction deteriorate "or
AF occ/r&- ortho(noea or P3D /(er'ene0
%0 ?iddine or 1ynco(e on efford0 Poi)le reaon are :
-
High intra+/ral (re/re on e@ercie- firing )arore*
ce(tor to (rod/ce refle@ )radycardia and 'aodilatati*
on0
-
18eletal +/cle 'aodilatation on e@ercie with no
increae in C.P or additional rhyth+ dit/r)ance0
-
De'elo(+ent of co+(lete AV )loc8 with aortic ring
calci/+ e@tending into the /((er 'entric/lar e(t/+0
90 1yte+ic e+)oli : often retinal or cere)ral0 A+a/roi
f/ga@ +ay )e the (reenting y+(to+- e(ecially when
'al'e i calcified0 1+all fle8 of calci/+ and7or (latelet
e+)oli +ay )e een wedged in retinal arteriole on
o(hthal+oco(y0
G0 1/dden death : +ay occ/re in H-9 E of cae- e'en )efore
e'ere EC? de'elo(- e0g in children0
H0 Infecti'e endocarditi0
I0 Congeti'e heart fail/re : e'ere A1 +ay (reent for the
firt ti+e a CHF with a large heart- 'ery low (/le
'ol/+e and oft +/r+/r- or no a/di)le +/r+/r at all0
Phyical ign:
-
!VP 1+all Fa2 wa'e 'ii)le in e'ere cae
-
Low (/le (re/re0 P/l/ alternan in e'ere cae
-
4ic/(id : * 1oft 1#- E,ection o/nd
* E,ection ytolic +/r+/r heard at )ae-
L14- aortic area and carotid0
-
Rhe/+atic : * Early diatolic +/r+/r or AR7AI
-
LV hea'e
15
Coe@iting leion0
In addition to the fact that an aortic 'al'e a)nor+ality
+ay coe@it with /)'al'ar tenoi- )oth leion +ay occ/r
with certain other congenital cardio'ac/lar defect- /ch a K
-
aortic 'al'e tenoi ")ic/(id 'al'e& D coarctation of
the aorta "e0g T/rner2 yndro+e&-
-
aortic 'al'e tenoi D coarctation D PDA
-
V1D P1
-
A (art of hy(o(latic left heart yndro+e
-
Corrected T?A
-
1/(ra'al'ar tenoi with (/l+onary artery )ranch
tenoi0
E C ?
#0 1ho/ld )e 1R0 If in AF- /(ect additional +itral 'al'e
dieae or ichae+ic heart dieae0
:0 P +itrale with (ro+inent negati'e P*wa'e co+(onent in
V "d/e to high LVEDP&0
<0 LV hy(ertro(hy
%0 F1train (attern2 in lateral chet lead0 In children- T*wa'e
In'erion in inferior lead often occ/r firt0 1e'ere A1
+ay occ/r with a nor+al EC? in children0
90 LAD "d/e to LAH4&
G0 Poor R*wa'e (rogreion in anterior chet lead0
H0 L444 or co+(lete heart )loc8 with calcified ring " 9E
of cae&0
Following AV re(lace+ent there i often a re'erion of the P*
or T*wa'e change grad/ally o'er the year and a red/ction in
LV 'oltage a the LV +a in red/ced0
Chet M*ray0
May how :
-
LVH- Calcified AV "age R %6 yr&- (ot tenotic
dilataion of acending aorta0
-
P/l+onary 'eno/ congetion and ign of LVE0
16
Echocardiogra(hy0
May how the following0
-
4ic/(id 'al'e "eccentric Fclo/re2 line& with red/ced
'al'e o(ening0
-
Calcified 'al'e "+/lti(le echo*)and&
-
LVH- aea LV f/nction0
-
Diatolic Fl/ttering of anterior +itral leaflet if
additional AR i (reent0
-
Ae+ent of AV gradient fro+ Do((ler echocardio*
gra(hy +ay )e a /)tit/te for cardiac catheteri>ation
in the yo/nger (t0
Rate of (rogreion of A10
Thi i 'ery 'aria)le and y+(to+ +ay occ/r at any tage
of the dieae and any 'al'e area0 ?enerally- an a'erage
ann/al7 change to )e e@(ected wo/ld )e :
-
?radien increae )y 9 J #6 ++Hg "6-GH*#-< 8Pa&
-
Pea8 'elocity on contin/o/*wa'e Do((ler: increae of
6-:*6-< +70
-
Aortic 'al'e area : red/ction of 6-# c+
:
0
Indication for 1/rgery0
#0 In Children- aortic 'al'/loto+y or'al'/lo(laty i
(erfor+ed in y+(to+atic or ay+(to+atic (t with
e'ere A10
:0 In Ad/lt- AVR i reco++ended once y+(to+
de'elo(0 The nat/ral hitory of +edically treated (t
who are y+(to+atic i (oor "a'erage /r'i'al i :*<
yr with angina or ynco(e- #*: yr with cardiac
fail/re&0 Ad/lt who are a)ol/tely ay+(to+atic can
)e +anaged +edically )/t +/t )e 8e(t /nder cloe
o)er'ation in the o/t(atient clinic w'wry G +onth0
1/rgery i neceary a oon a y+(to+ de'elo(0
17
<0 The deciion to o(erate on the elderly (t +/t de(ent
on :
-
ade=/ate he(atic and renal f/nction
-
ade=/ate l/ng f/nction "FEV
#
(refera)ly O 6-I * # L&0
-
reaona)le ad/lt weight "O %6 8g&
-
the e'erity of additional coronary dieae or LV
dyf/nction0
A'erage o(erati'e +ortality for iolated AVR i now
Q 9E0 The need for additional coronary re'ac/lari>ation or
the (reence of (oor LV f/nction increae the ri8 "#6 J :6 E&0
Long ter+ /r'i'al after AVR de(ent on the (reence of :
-
additional CAD and hitory on infarction
-
heart i>e increaing (reo(erati'ely
-
low C.P
-
(/l+onary hy(ertenion0
Aortic 'al'/lo(laty0
Thi techni=/e ha (ro'ed of o+e 'al/e in a 'ery +all
gro/( of elderly (t with e'ere A1 who are conidered
ino(era)le "'ery (oor l/ng f/nction- renal fail/re etc&0
Following 'al'/lo(laty there i a gradient red/ction and
//ally an increae in aortic 'al'e area0 Co+(lication incl/de
(rofo/nd )radycardia- hy(otenion- ta+(onade- yte+ic
e+)oli and death in a few cae0 Aortic 'al'/lo(laty cannot
)e conidered an alternati'e to AVR0

1/(ra'al'ar A10
Thi i ca/ed )y a contricting ridge of fi)ro/ ti/e at
the /((er +argin of the in/e of Vala'a0 The coronary otia
are )ellow the tenoi0 Rarely- the o)tr/ction i a +ore
generali>ed hy(o(laia of acending aorta0
18
Aociated condition
Nillia+2 yndro+e "a/too+al do+inant with 'aria)le
(enetration&0 Children with :
-
elfin facie "large +o/th with (rotr/ding /((er li(-
high forehead- e(icanthic fold- receed naal )ridge-
+ental retardation- tra)i+/- low*et ear&
-
hy(er'ita+inoi D and hy(ercalce+ia
-
other cardiac leion: (eri(heral (/l+onary artery
tenoi- 'al'ar P1- AR7AI0
-
+eenteric artery tenoi- thoracic aortic ane/ry+0
-
R/)ella yndro+e0
Cardiac leion and ign0
1/(ra'al'ar A1 ho/ld alo )e conidered in a child who
ha additional AR- no e,ection o/nd ang )lood (re/re in the
left ar+ lower than the right0 The CMR doe not how
(ottenotic dilatation of acending aorta0
1y+(to+ are thoe of 'al'ar tenoi0 Coronary arterie
are characteritically large )/t tend to ha'e (re+at/re arterial
dieae d/e to the high (re/re )elow the /(ra'al'ar tenoi0
The /(ra'al'ar helf and the adherent aortic c/( +ay
rarely iolate the coronary artery orifice"Fho/e*+artin2 net
a((earance on angiogra+& and AMI or /dden death occ/r0
1/rgery0
Le atifactory than for aortic 'al'e tenori0 It +ay
only )e (oi)le if the acending aorta i of reaona)le i>e0 A
gradient fro+ LV to acending Aorta of O H6 ++Hg "$-<8PA&
wo/ld )e an indication for o(eration0 The aortic wall i often
thic8ened- increaing the diffic/ltie of /rgery0
Dicrete fi)ro+/c/lar /)aortic tenoi0
Thi contit/te a((ro@i+ately #6 E of congenital A10
The fi)ro+/c/lar ring o)tr/ct the LV.T i++ediately
)eneath the aortic 'al'e0
19
Ditinction fro+ 'al'ar A10
Thi +ay )e 'ery diffic/lt0 Dicrete fi)ro+/c/lar
/)aortic tenoi i a (oi)ility if :
-
there i AR "thic8ening of 'al'e d/e to high 'elocity ,et
thro/gh o)tr/ction or e'en attach+ent to the right
coronary c/(&0
-
a)ent e,ection o/nd
-
no 'al'e calcification
Pot tenotic dilatation of acending aorta +ay or +aynot
relia)le diagnoticall0
Echocardiogra(hy
In'al/a)le in eta)lihing the diagnoi0 .n M*+ode it
+ay how the following :
-
'ery early ytolic clo/re of aortic 'al'e "right
coronary c/( e(ecially& and ytolic fl/ttering of
aortic leaflet0
-
Cl/ter of /)aortic echoe a)o'e anterior +itral
leaflet0
Cardiac catheteri>ation
Confir+ /)aortic o)tr/ction0 The ring i 'i/ali>ed on
LV angiogra(hy0 The degree of o)tr/ction can )e +ea/red
and additional AR aeed0
1/rgery0
E@ciion of the fi)ro+/c/lar ring i (oi)le- )/t often
reid/al a)nor+al LV +/cle re+ain "'ery i+ilar to HCM&0
The ring i e@cied thro/gh the aortic 'al'e0 There i //ally a
+all reid/al gradient and o+eti+e +ild AR0
Follow /( with re(eat cardiac catheteri>ation i neceary
to e@cl/de rec/rrent o)tr/ction0 .ccaionally AVR i re=/ired
later for AR0
20
Aortic Reg/rgitation 0
Thi +ay )e d/e to (ri+ary dieae of AV or to aortic
root dieae with dilatation and tretching of the 'al'e ring0
The reg/rgitation +ay )e thro/gh the 'al'e or rarely- down a
channel ad,acent to the 'al'e ring "e0g r/(t/red in/ of
Vala'a ane/ry+- aorta*left 'entric/lar t/nnel&
Patho(hyiology
.ften +oderate AR i tolerated with no y+(to+ :
-
AR re/lt in an increae in LVEDV and LVE1V
-
1V i high in co+(enated cae0
-
LV +a i raied with LV hy(ertro(hy
-
Co+(enatory tachycardia red/ce the reg/rgitant
flow (er )eat )y hortening diatole- and allow an
increae in C.P0
A the reg/rgitation increae and LV f/nction deteriorate :
-
LVEDP rie and +ay e'ent/ally e=/al aoertic diatoli
(re/re
-
Pre+at/re MV clo/re occ/r- (re'enting diatolic
forward flow thro/gh the MV
-
LVEDV rie f/rther )/t 1V fall0
Aetiology0

Congenital Ac=/ired
Valve disease
4ic/(id 'al'e Rhe/+atic fe'er
1/(ra'al'ar tenoi Infecti'e endocarditi
Dicrete /)'al'ar fi)ro+/c/lar Rhe/+atoid arthriti
ring "'al'e nod/le&
1/(racrital V1D with (rola(e* 1LE
.f right coronary c/(0 PME
H/rler2 yndro+e
21
Aortic root disease
R/(t/red in/ of Vala'a* Diection "Ty(e A&
Ane/ry+ Hy(ertenion
Cytic +edial necroi e0g
Marfan2 yndro+e
.teogenei i+(erfecta
?iant cell aortiti
1y(hili
Tra/+a
1y+(to+
A in A1- )/t angina and ynco(e are +/ch le co++on0
Unli8e A1- AR i a well*tolerated leion if grad/al co+(en*
atory +echani+ can occ/r0 E'en +oderate AR +ay )e
tolerated for year0 Howe'er- ac/te 'al'ar AR or r/(t/red
in/ of Vala'a i (oorly tolerated and =/ic8ly (rod/ce LVF
or CHF0 Inteni'e +edical thera(y followed )y in'etigation
and /rgery i often neceary0
E(ony+ aociated with AR0
#0 Austin Flint murmur0 D/e to 'i)ration in diatole of
anterior +itral leaflet- ocillating )etween reg/rgitant ,et
and antegrade )lood flow fr+ LA0 Very i+ilar to M1-
)/t 1
#
i =/iet and there i no o(ening na(0
:0 Duroziezs sign: to*and*fro +/r+/r a/di)le o'er fe+oral
arterie0
<0 Quinckes pulse: ca(illary (/lation in finger ti( or
+/co/ +e+)rane0
%0 Traubes sign : F(itol*hot2 o/nd a/di)le o'er fe+oral
arterie0
90 De ussets sign : head )o))ing d/e to colla(ing (/le0
Differential diagnoi0
-
PR- PDA- V1D D AR- R/(t/red in/ of Vala'a- AP
window0
22
E C ?0
LVH with diatolic o'erload (attern"(ro+inent S wa'e
in anterolateral lead&- 1T de(reion and T*wa'e in'erion
occ/r a the condition deteriorate0
Echocardiogra(hy
May how the following :
-
LV f/nction and di+enion0
-
Aortic 'al'e thic8ening0 Poi)le F'egetation2 on AV0
-
Diatolic fl/ttering of anterior +itral leaflet "+ay )e
a/di)le a the A/tin Flint +/r+/r&
-
Pre+at/r MV clo/re0 .ccaionally only the Fa2 wa'e
o(en the MV at all in e'ere cae0
-
Flail aortic leaflet (rola(ing into LV.T0
Chet M*ray0
May how :
-
AV calcification /nco++on in (/re AR
-
Large LV
-
Acending aorta +ay )e 'ery (ro+inent "e0g
diection& or ane/ry+al "e0g Marfan2 yndro+e-
y(hili&
-
Calcification of acending aorta "y(hilitic AR&
-
1ign of (/l+onary 'eno/ congetion or (/l+onary
oede+a0
Cardiac catheteri>ation
3eceary to doc/+ent the following :
#0 The e'erity of AR
:0 The anato+y of the aortic root-and to chec8 the
rg/rgitation i 'al'ar and not r/(t/red in/ of Vala'a- to
e@cl/de diection and to chec8 for rarer congenital defect
+i+ic8ing AR0
<0 To ae LV f/nction0 Nith e'ere AR the LVEDP0
23
%0 To chec8 coronary arterie and coronary otia0
90 Additional 'al'e dieae0
./t(atient follow*/(0
All (t need Anti)iotic for dental or /rgical (roed/re0 The
acti'ity of (t with +ild reg/rgitation need not )e retricted0
Pt with +oderate or e'ere reg/rgitation ho/l a'oid
io+etric e@ercie and co+(etiti'e (ort and need G*+onthly
follow*/(0 Digo@in i not )enefit /nle the (t i in AF0
Indication for /rgery0
The AV +/t )e re(laced )efore irre'eri)le LV dyf/nction
de'elo( and thi +ay occ/r in the ay+(to+atic (t0
Conider AVR if the following a((ly :
#0 1y+(to+ increaing dy(noe and LVF0
:0 In the (t with no y+(to+ or HF :
-
enlarging heart on CMR "O #H c+ on PA fil+& or
increaing LV di+enion on Echocardiogra(hy:
LVEDD O H6 ++ "<9++7+
:
& and LVE1D O 96 ++ ":9
++7+
:
&
-
(/le (re/re O #66++Hg "#<0< 8Pa& e(ecially if
diatolic Q %6 ++Hg "90< 8Pa&
-
EC? deterioration with T*wa'e in'erion in lateral
chet lead0
.f (t with all three of thee criteria- G9E will either die
or de'elo( CHF within < yr0
<0 R/(t/red in/ of Vala'a ane/ry+0
%0 Infecti'e endocarditi not re(onding ti +edical
treat+ent0
24
P/l+onary 1tenoi0
.)tr/ction to RV o/tflow +ay )e at e'eral le'el- a in
A10
!erip"eral pulmonar# arter# stenosis 0
1tenoi of +ain tr/n8 of PA or +ore dital tenoi0
Thee tenoi +ay )e locali>ed or diff/e0 Co++only
aociated with /(ra'al'ar A1 and infantile hy(ercalce+ia0
Alo (art of the R/)ella 1yndro+e aociated with PDA0
!ulmonar# valve stenosis 0
A co++on iolated leion "HE of congenital heart
leion&0 Alo occ/r a (art of 3oonan2 yndro+e- Fallot2
tetralogy and R/)ella yndro+e
!ulmonar# in$undibular stenosis 0
Rare a an iolated leion0 U/ally aociated with a V1D-
or a (art of Fallot2 Tetralogy or ,/t in aociation with
P/l+onary 'al'e tenoi0
Subin$undibular stenosis 0
Thi rare for+ ha )een decri)ed0 It +ay occ/r a (art
of right*ided HCM0
Patho(hyiology and y+(to+0
The effect of P1 de(ent on it e'erity and the tr/ct/re
and f/nction of the ret of the right heart- i0e RV f/nction
"ytolic and diatolic&- co+(etence of the tric/(id 'al'e-
(reence or a)ence of a V1D- (reence or a)ence of an
A1D7PF.- +aintenance of 1R0
25
Nith good RV f/nction and co+(etent tric/(id 'al'e
(l/ 1R- +oderate P1 can )e tolerated with no y+(to+0 Very
e'ere F(inhole2 P1 i 'irt/al (/l+onary atreia and +ay lead
to early infant death- e(ecially if the d/ct cloe0
The additional (reence of an A1D or PF. +ay lead to
right*to*left h/nting "e0g on effort&- with cyanoi0
RV fail/re i the co++onet ca/e of death- with gro
cardiac enlarge+ent0
Co++on y+(to+ are th/ :
-
dy(noea and fatig/e "low C.P&- not ortho(noea or
P3D&
-
cyanoi "if A1D or PF.&
-
RV fail/re with acite- leg oede+a- ,a/ndice- etc0
-
Retarded growth in children0
1y+(to+ which are /nco++on "/nli8e A1& are angina-
ynco(e on effort and y+(to+ fro+ infecti'e endocarditi0
Pt +ay )e aware of (/lation in the nec8 fro+ giant Fa2 wa'e
in the !VP0
Phyical ign to note0
Characteritic facie +ay )e :
-
ro/nded (l/+( face with iolated (/l+onary 'al'e
tenoi0
-
3oonan2 yndro+e "F+ale T/rner2&
-
Nillia+2 yndro+e "hy(ercalce+ia D /(ra'al'ar A1
D P/l+onary artery tenoi&- elf*li8e facie- !VP :
(ro+inent or giant Fa2 wa'e- RV hy(ertro(hy- (al(a)le
RV.T thrill0
Val'e tenoi0
Mild 'al'e tenoi : there i an e,ection o/nd- E1M and A
:
and P
:
clearly heart and widely (lit0 A the tenoi )eco+e
+ore e'ere- the +/r+/r i longer and o)c/re A
:
0 P
:
i
delayed till f/rther and i ofter0 Nith e'ere tenoi P
:
)eco+e a/di)le and the e,ection o/nd dia((ear a the 'al'e
26
calcifie0 The +/r+/r radiate toward the left ho/lder and
o'er the left l/ng (oteriorly0
Nith inf/ndi)/lar tenoi there i no e,ection o/nd and
the +/r+/r +ay )e +ore (ro+inent at L140
Differential Diagnoi0
DD: Aortic 'al'e or /)'al'ar tenoi- V1D- E)tein2
ano+aly- A1D and innocent RV.T +/r+/r in children0
E C ?0
RAD- RA hy(ertro(hy- FP (/l+onale2 - RVH- inco+(lete oe
co+(lete R4440
Chet M*ray0
Pot tenotic dilatation of the PA )/t l/ng field are oligae+ic-
in contrat to A1D0 RVH ca/e o+e cardiac enlarge+ent-
with the a(e@ lifted off the left he+idia(hrag+0
Nith e'ere long*tanding P1 the heart +ay )e 'ery
large- with an enor+o/ RA " the wall*to*wall heart &0
Thi a((earance i een :
-
e'ere P1 in the ad/lt
-
E)tein2 ano+aly
-
large (ericardial eff/ion "chronic&
-
M1 with giant atria
-
Dilated CM
-
Uhl2 ano+aly "RV hy(o(laia&
Cardiac Catheteri>ation0
3eceary to doc/+ent the gradient and ite of the tenoi-
and the i>e of the PA and (oi)le additional tenoi in the+0
RV f/nction are i+(ortant0 The (oition and co+(arati'e i>e
of the great 'eel are i+(ortant in +ore co+(le@ leion "e0g
Fallot2 tetralogy- D.RV with P1- T?A with V1D and P10
P/l+onary 'al'/lo(laty0
27
P/l+onary 'al'/lo(laty i now an acce(ta)le alternati'e to
/rgery0 ?ood red/ction of (/l+onary 'al'e gradient i
o)tained- long*ter+ re/lt are good and RVH on the EC?
regre0
1/rgey0
P/l+onary 'al'/loto+y and7or inf/ndi)/lar reection ho/ld
)e conidered if there i RV fail/re- or if (ea8 ytolic gradient
at 'al'e7/)'al'e le'el i O H6 ++Hg "$0< 8Pa&0 E+ergency
/rgery +ay )e needed in infant0
Tric/(id 'al'e dieae 0
The co++onet tric/(id 'al'e dieae i f/nctional reg/rgi*
tation econdary to PH0 Tric/(id 'al'e detr/ction fro+
infecti'e endocarditi i increaingly een in dr/g addict0
.the for+ of tric/(id 'al'e dieae are /nco++on0
Congenital leion Ac=/ired leion
Tric/(id atreia F/nctional reg/rgitation
Tric/(id hy(o(laia Detr/ction fro+ infecti'e endo*
carditi0
E)tein2 ano+aly Rhe/+atic in'ol'e+ent
Cleft tric/(id 'al'e* Flo((y 'al'e
"AV canal& Endocarditi d/e to he(atic car*
cinoid0
Fenfl/ra+ine- (henter+ine

Tric/(id reg/rgitation
Dilatation of the 'al'e ring with deteriorating RV f/nction i
co++on in(t with PH fro+ any ca/e0 It often occ/r in (t
with Rhe/+atic MV dieae and PH0 The de'elo(+ent of AF in
A1D i aociated wit TR0
28
1y+(to+0
If any- there +ay )e fatig/e- he(atic (ain on efford- (/lation
in the throat and f/llne in the face on effort- acite and an8le
oede+a0
1ign0
1ytolic F2 wa'e in the !VP with ra(id Fy2 decent- if till in
1R "rare& (ro+inent Fa2 wa'e alo- RV hea'e- oft in(iratory
(anytolic +/r+/r at L14- (/latile li'er- an8le oede+a and
(oi)le acite and ,a/ndice-(eri(heral cyanoi00
Treat+ent0
1o+e degree of TR can )e tolerated in the a+)/lant (t )y
con'entional Di/retic thera(y and Digo@in0 1(ironolactone-
a+iloride or an ACE inhi)itor ho/ld )e (art of the regi+e0
1/((ort toc8ing +ay hel( (re'ent tro/)leo+e an8le oede+a
and 'eno/ /lceration0
In +ore e'ere and y+(to+atic (t a (eriod of )ed ret and
IV Di/retic thera(y i needed0 The y+(to+ =/ic8ly rec/r
//ally once the (t i +o)ili>ed0 In thee cae TVR +/t )e
conidered0
Tric/(id 1tenoi0
Thi i rare- al+ot alway rhe/+atic-and aociated with
additional +itral or aortic 'al'e dieae0 1y+(to+ are a in
TR0
1ign0
1low Fy2 decent in !VP- (ro+inent Fa2 wa'e if in 1R- RV
hea'e a)ent- tric/(id diatolic +/r+/r at L14 )et heart on
in(iration and after effort0 At cardiac cathetheri>ation e'en a
gradient of <*% ++Hg "6-%*6-9 8Pa& acro the tric/(id 'al'e
i highly ignificant0 RV angiogra(hy //ally how addiotal
TR0 The only treat+ent i 'al'/lo(laty or 'al'e re(lace+ent0
29
E)tein2 ano+aly0
A tric/(id 'al'e dy(laia with downward di(lace+ent of
the 'al'e into the )ody of the 'entricle0 The tric/(id leaflet
are a)nor+al- they +ay )e f/ed- (erforated or e'en a)ent
and their chordae are a)nor+al0
The clinical (ict/re de(end on the following0
-
1e'erity of TR
-
RV f/nction: the Atriali>ed (ortion of the RV i thin*
walled and f/nction (oorly0
-
Rhyt+ dit/r)ance0 Thee are fre=/ent and incl/de
)oth 1VT and VT0 There i often an a)nor+al
cond/cting yte+ with ty(e 4 "right*ided& NPN
yndro+e0
-
Aociated leion- co++only A1D or PF.- (/l+onary
tenoi- corrected tran(oition0 Le co++only M1-
Fallot2 tetralogy0
Preentation 0
%n$anc#& HF fro+ e'ere TR with chronic L.P0 Cyanoi fro+
right*to*left h/nting at atrial le'el "PF. or A1D&0 Thi +ay
increae when a PDA cloe a (/l+onary flow i red/ced till
f/rther0 Prognoi at thi age i (oor0
'lder c"ild or #oun adult& Thi +ay )e with a +/r+/r or
Paro@y+al 1VT0 Mild for+ +ay )e ay+(to+atic0
Phyical ign0
De(ent on a)o'e leion0 U/ally the child i cyanoed- with
ele'ated !VP and he(ato+egaly0 At L14 liten for (anytolic
+/r+/r "TR&- 1
<
"RV- tric/(id diatolic +/r+/r0
Chet M*ray0
30
1how 'er/ large RA in y+(to+atic cae often with oligae+ic
l/ng field0 Nith large glo)/lar heart conider (ericardial
eff/ion- P1- dilated CM a alternati'e0
E C ?0
1how R444- RAD- RA D "P (/l+onale&- o+eti+e ty(e 4
NPN yndro+e0
Echocardiogra(hy0
Thi i diagnotic0 Here the tric/(id and +itral echoe are
often een well at the a+e ti+e- tric/(id clo/re i delayed
till f/rther0 The e(t/+ nor+ally act a (art of the LV- with
(oterior +o'e+ent in ytole0 1o+e condition re/lt in
(arado@ical e(tal +otion in which the e(tal2 (oterior
+o'e+ent i delayed- it +o'e anteriorly in ytole acting a
(art of the RV0
Cardiac catheteri>ation0
RV angiogra(hy i diagnotic- )/t fre=/ently (rod/ce rhyt+
dit/r)ance that +ay )e diffic/lt to control0
Treat+ent0
I +edical initially to control y+(to+ of RHF and
arrhyt+ia if (reent0 Tric/(id VR (l/ clo/re of an A1D i
(oi)le )/t re/lt are generally not good0
31
4AHA3 PULIAH

1EME1TER * H
Prof Dr0 Reggy L0 Lefrandt0 1(!P"P&0
32