Adult CHD Irsad 2008

7/26/2019 Adult CHD Irsad 2008

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Irsad Andi ArsoIrsad Andi Arso


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OverviewOverview

US: 1,000,000 adults with congenitalUS: 1,000,000 adults with congenitalheart dzheart dz

20,000 more patients reach20,000 more patients reachadolescents yearlyadolescents yearly

*All figures from ACCSAP V unless otherwise noted


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Adult Congenital Heart DiseaseAdult Congenital Heart Disease

Atrial Septal DeectAtrial Septal Deect !entricular Septal Deect!entricular Septal Deect

"atent Ductus Arteriosus"atent Ductus Arteriosus #oarctation o Aorta#oarctation o Aorta $etralogy o %allot$etralogy o %allot

&'stiens Anomaly&'stiens Anomaly


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((

)ole 'etween the two atria)ole 'etween the two atria *lood lows let to right*lood lows let to right

"%+ "atent oramen o-ale ails to"%+ "atent oramen o-ale ails tocloseclose .ight heart 'ecomes dilated.ight heart 'ecomes dilated

$oo much 'lood to the lungs$oo much 'lood to the lungs


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Three typesThree types

"rimum ASD"rimum ASD Secundum ASDSecundum ASD

Sinus -enosusSinus -enosus A!SD Atrio -entricular septalA!SD Atrio -entricular septal

deectdeect


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Atrial Septal DefectAtrial Septal Defect

11/00 li-e 'irths11/00 li-e 'irths SecundumSecundum

most common A#)D 41056most common A#)D 41056 .AD.AD

"rimum"rimum associated with other endocardial cushion deects cletassociated with other endocardial cushion deects clet

A! -al-es, inlet type !SD6A! -al-es, inlet type !SD6 7AD7AD

Sinus !enosusSinus !enosus

large, associated with anomalous pulmonary -enouslarge, associated with anomalous pulmonary -enousdrainage usually . superior "!6drainage usually . superior "!6 #oronary sinus rare6#oronary sinus rare6

associated with unrooed coronary sinusassociated with unrooed coronary sinus


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ASD Anatomy!PrevalenceASD Anatomy!Prevalence

"Secundum #$%"Primum &$%"Sinus Venosus &'%"Cor Sinus (rare)

raunwauld+s Heart Disease, -thed


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Physiologic Conse.uencesPhysiologic Conse.uences Shunt %lowShunt %low

Size o deectSize o deect

.elati-e compliance o -entricles.elati-e compliance o -entricles

.elati-e resistance o pulmonarysystemic circulation.elati-e resistance o pulmonarysystemic circulation

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. shunting results in diastolic o-erload o .! and. shunting results in diastolic o-erload o .! andincreased pulmonary 'lood lowincreased pulmonary 'lood low

.! dilatationailure and rarely se-ere pulm )$;.! dilatationailure and rarely se-ere pulm )$;

&isenmengerith age, deterioration chiely due to 11

decrease 7! compliance, increased 7decrease 7! compliance, increased 7. shunt. shunt

increase in atrial arrhythmiasincrease in atrial arrhythmias

pulm )$; de-elops, .! -olume ? pressure +7pulm )$; de-elops, .! -olume ? pressure +7

&Perloff, /012 &33$


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Clinical SymptomsClinical Symptoms

+ten asymptomatic until 34(+ten asymptomatic until 34(ththdecadedecadeor moderate4large ASD, may presentor moderate4large ASD, may presentlater in lie or initially smaller ASDlater in lie or initially smaller ASD

%atigue%atigue D+&D+& Atrial arrhythmiasAtrial arrhythmias "arado@ical &m'olus"arado@ical &m'olus .ecurrent "ulmonary inections.ecurrent "ulmonary inections


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Physical SignsPhysical Signs

S2 widei@ed splittingS2 widei@ed splitting .!"A palpa'le impulse i lg deect6.!"A palpa'le impulse i lg deect6

systolic eection murmur 2systolic eection murmur 2ndnd

7 I#S7 I#S mid4diastolic $! rum'lemid4diastolic $! rum'le


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Auscultation in ASDAuscultation in ASD

4ncreased flow across the pulmonaryvalve produces a systolic e5ection murmur

and fi6ed splitting of the second heart sound7

8i6ed splitting of S9 may in part :e due to

delayed right :undle conduction7

4ncreased flow across the TV produces a

diastolic rum:le at the mid to lower right

sternal :order7

"Older pt loses pulm e5ection

murmur as shunt :ecomes

:idirectional"signs of pulm HT/! CH8 may

predominate


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0C;0C;


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1(1(

0CHO0CHO

Su'costal

-iew o

Intraatrial

Septum

#olor

%low

#ontrast

Bood orsecundum

, primum


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TreatmentTreatment

Cedical : diuretics, A#&I, AldactoneCedical : diuretics, A#&I, Aldactone .epair.epair

#onsider when s@s, p:sE1F/#onsider when s@s, p:sE1F/ Inter-entional "ercutaneous #losure6Inter-entional "ercutaneous #losure6 +nly or secundum deects+nly or secundum deects (45 success Amplatzer6(45 success Amplatzer6 adeGuate superiorinerior rim around ASDadeGuate superiorinerior rim around ASD no .47 shuntingno .47 shunting


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Surgical #losureSurgical #losure

Bood prognosis:Bood prognosis: closure age H 2/, "A pressure H(0closure age H 2/, "A pressure H(0 I E2/ or "AE(0, decreased sur-i-al due toI E2/ or "AE(0, decreased sur-i-al due to

#)%, stroe, and ai'#)%, stroe, and ai'

Treatment


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)ole 'etween the two -entricles)ole 'etween the two -entricles

7et to right shunt maority7et to right shunt maority Dilated right heart too much 'loodDilated right heart too much 'lood

to lungs increase in pulmonaryto lungs increase in pulmonary

pressurepressure Smaller deects can closeSmaller deects can close

spontaneouslyspontaneously


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Three typesThree types

"erimem'ranous !SD most common"erimem'ranous !SD most common Cuscular !SD can 'e multipleCuscular !SD can 'e multiple

Apical !SD usually smallApical !SD usually small !aria'le in size!aria'le in size


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Ventricular Septal DefectVentricular Septal Defect

Cay 'e anywhere inCay 'e anywhere inintra4-entricular septum4intra4-entricular septum4clinical course dependsclinical course dependson the shunt size andon the shunt size and

in-ol-ement oin-ol-ement opulmonary -ascular 'edFpulmonary -ascular 'edF

Appro@ J o all !SDs areAppro@ J o all !SDs aresmall, and more than Jsmall, and more than Jclose spontaneouslyFclose spontaneouslyF )ighest closure rates in the)ighest closure rates in the

irst decade o lieFirst decade o lieF


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PATHOPHYSIOLOGYPATHOPHYSIOLOGY

primarily depends on sizeKstatus o pulmF -ascular 'ed ratherprimarily depends on sizeKstatus o pulmF -ascular 'ed ratherthan locationthan location

Small communication less than 0F/cmL6 !SD is restricti-e KSmall communication less than 0F/cmL6 !SD is restricti-e KrtF-entricular pressure is normal does not cause signiicantrtF-entricular pressure is normal does not cause signiicanthemodynamic derangementp:sM1F8/:1F06hemodynamic derangementp:sM1F8/:1F06

Coderately restricti-e !SD with a moderate shuntp:sM1F/4Coderately restricti-e !SD with a moderate shuntp:sM1F/42F/:1F06 Kposes hemodynamic 'urden on 7!2F/:1F06 Kposes hemodynamic 'urden on 7!

7arge nonrestricti-e !SDsmore than 1F0cmL6 .tK7t7arge nonrestricti-e !SDsmore than 1F0cmL6 .tK7t-entricular pressure are eGualisedp:s is more than 2:16-entricular pressure are eGualisedp:s is more than 2:16

7arge !SDs at 'irth ,"!. may remain higher than normal and7arge !SDs at 'irth ,"!. may remain higher than normal and7t to .t shunt may intially limited in-olution o media o small7t to .t shunt may intially limited in-olution o media o small

pulmFarterioles,"!. decreasesNlarge 7t to .t shunt ensuespulmFarterioles,"!. decreasesNlarge 7t to .t shunt ensues In some inants large !SDs ,pulmF arteriolar thicness ne-erIn some inants large !SDs ,pulmF arteriolar thicness ne-er

decreases pulmFo'structi-e disease de-elops Fwhendecreases pulmFo'structi-e disease de-elops Fwhenp:sM1:1 shunt 'ecomes 'idirectional,signs o heart ailurep:sM1:1 shunt 'ecomes 'idirectional,signs o heart ailurea'ate KptF 'ecomes cyanoticF &isenmenger syndrome6a'ate KptF 'ecomes cyanoticF &isenmenger syndrome6


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CLINICAL FEATURESCLINICAL FEATURES

.ace.ace : no particular racial predilection: no particular racial predilection Se@Se@:no particular se@ preerence:no particular se@ preerence AgeAge::

infantsinfants diicult in postnatal period,although cc diicult in postnatal period,although cc

during irst mths is reGuent,O4rayKB areduring irst mths is reGuent,O4rayKB arenormalFnormalF

childrenchildrenNater irst year -aria'le clinical pictureNater irst year -aria'le clinical pictureemergesFemergesF

small !SD asymptomaticsmall !SD asymptomatic large !SD:large !SD:

4palpitation,dyspnoea on e@ertion,eeding4palpitation,dyspnoea on e@ertion,eedingdiiculties ,poor growthdiiculties ,poor growth

4reGuent chest inections4reGuent chest inections


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2(2(

PHYSICAL FINDINGSPHYSICAL FINDINGS

"ulse pressure is relati-ely wide"ulse pressure is relati-ely wide

"recordium is hyperinetic with a systolic thrill at 7S*"recordium is hyperinetic with a systolic thrill at 7S*

S1KS2 are mased 'y a "SC at 7tFsternal 'orderS1KS2 are mased 'y a "SC at 7tFsternal 'order

,ma@F intensity o the murmur is 'est heard at,ma@F intensity o the murmur is 'est heard at

33rdrd,(,(ththK/K/thth7t interspaceFAlso well heard at the 27t interspaceFAlso well heard at the 2ndnd

space 'ut not conducted 'eyond ape@space 'ut not conducted 'eyond ape@

7tF 27tF 2ndndspace widely split K-aria'le accentuated "2space widely split K-aria'le accentuated "2

Delayed diastolic murmur at the ape@ KS3Delayed diastolic murmur at the ape@ KS3 "resence o mid4diastolic ,low pitched rum'le at the"resence o mid4diastolic ,low pitched rum'le at the

ape@ is caused 'y increased low across the mitralape@ is caused 'y increased low across the mitral

-al-e-al-e


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INVESTIGATIONSINVESTIGATIONS

CHEST RADIOGRAPHYCHEST RADIOGRAPHY

4 normal4 normal

4 'i-entricular hypertrophy4 'i-entricular hypertrophy

4 pulmonary plethora4 pulmonary plethora

ELECTROCARDIOGRAPHYELECTROCARDIOGRAPHY 44small!SD = normal tracingsmall!SD = normal tracing

4modF!SD = 'road,notched " wa-e characteristic o 7tF Atrial4modF!SD = 'road,notched " wa-e characteristic o 7tF Atrial

o-erload as well as 7! o-erload,namely,deep wa-es K tallo-erload as well as 7! o-erload,namely,deep wa-es K tall

. wa-es in leads !/ and ! and oten A%. wa-es in leads !/ and ! and oten A%

4large !SD =.!) with rtF a@is de-iationF >ith urther4large !SD =.!) with rtF a@is de-iationF >ith urther

progression 'i-entricular hypertrophyP" wa-es may 'eprogression 'i-entricular hypertrophyP" wa-es may 'e

notchedpeaedFnotchedpeaedF


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ECHOCARDIOGRAPHYECHOCARDIOGRAPHY

two4dimensional Kdoppler colour lowtwo4dimensional Kdoppler colour low

A;BI+B.A")QA;BI+B.A")Q cardiac catheterization and angiographycardiac catheterization and angiography66

INVESTIGATIONSINVESTIGATIONS..


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COMPLICATIONSCOMPLICATIONS

#ongesti-e cardiac ailure#ongesti-e cardiac ailure Inecti-e endocarditis on rtF-entricular sideInecti-e endocarditis on rtF-entricular side Aortic insuiciencyAortic insuiciency #omplete heart 'loc#omplete heart 'loc Delayed growth K de-elopment %$$6 inDelayed growth K de-elopment %$$6 in

inancyinancy

Damage to electrical conduction systemDamage to electrical conduction systemduring surgerycausing arrythmias6during surgerycausing arrythmias6

"ulmonary hypertension"ulmonary hypertension

INTERVENTION


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INTERVENTIONINTERVENTION

3 CAR+. $Q"&S3 CAR+. $Q"&S SMALL (less than 3mmSMALL (less than 3mmdiameterdiameter

4 hemodynamically4 hemodynamically

insigniicantinsigniicant

4 'w 9049/5 o all !SDs4 'w 9049/5 o all !SDs

4 all close spontaneously4 all close spontaneously

/05 'y 2yrs /05 'y 2yrs

05 'y yrs 05 'y yrs 105 during school yrs 105 during school yrs

4 muscular close sooner4 muscular close soonerthan mem'ranousthan mem'ranous


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MODERATE !SDsMODERATE !SDs

34/mm diameter 34/mm diameter least common group o children34/56 least common group o children34/56

wo e-idence o cc pulmFhtn can 'e wo e-idence o cc pulmFhtn can 'eollowed until spontaneous closureollowed until spontaneous closure

occursF occursF LARGE !SDs "ITH #ORMAL P!RLARGE !SDs "ITH #ORMAL P!R

410mm in diameter 410mm in diameter

usually reGuires surgery usually reGuires surgery #onser-ati-e#onser-ati-etreatmenttreatment


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D084/4T4O/D084/4T4O/

Patent ductus arteriosusPatent ductus arteriosus

(PDA) is a heart problem(PDA) is a heart problemthat is usually noted inthat is usually noted in

the first few weeks orthe first few weeks or

months after birth. It ismonths after birth. It is

characterized by acharacterized by aconnection between theconnection between the

aorta and the pulmonaryaorta and the pulmonary

artery, which allowsartery, which allows

oxyen!rich (red) bloodoxyen!rich (red) bloodthat should o to thethat should o to the

body to re!circulatebody to re!circulate

throuh the lunsthrouh the luns


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4/ D0PTH4/ D0PTH


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4/ ;=OSS4/ ;=OSS


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3(3(

H02OD>/A24CSH02OD>/A24CS

7$ $+ .$ S)U;$7$ $+ .$ S)U;$+##U.S *+$)+##U.S *+$)DU.I;B SQS$+7&DU.I;B SQS$+7&

K DIAS$+7&K DIAS$+7& 7A.B& AC$ +%7A.B& AC$ +%

*7++D "ASS&S*7++D "ASS&S$).U "U7C A.$47$$).U "U7C A.$47$

A$.IUC CI$.A7A$.IUC CI$.A7!A7!&!A7!&

#+;$I;+US#+;$I;+USCU.CU.CU.CU.

A##&;$UA$&D S1A##&;$UA$&D S1 CI$.A7 D&7AQ&DCI$.A7 D&7AQ&D

DIAS$+7I#DIAS$+7I#CU.CU.CU.CU.


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H02OD>/A24CSH02OD>/A24CS


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P=0S0/TAT4O/P=0S0/TAT4O/

%reGuent chest inections%reGuent chest inections atigueatigue sweatingsweating rapid 'reathingrapid 'reathing hea-y 'reathinghea-y 'reathing congested 'reathingcongested 'reathing

disinterest in eeding, or tiring while eedingdisinterest in eeding, or tiring while eeding poor weight gainpoor weight gain


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O/ 0?A24/AT4O/O/ 0?A24/AT4O/

I;S"$I+;I;S"$I+; #A.+$ID#A.+$ID

"U7SA$I+;S"U7SA$I+;S

)Q"&.TI;&$I# K 7$)Q"&.TI;&$I# K 7$!&;$.I#U7A. $Q"&!&;$.I#U7A. $Q"&+% A"I#A7 IC"U7S&+% A"I#A7 IC"U7S&

"A7"A$I+;"A7"A$I+; SQS$+7I# +.SQS$+7I# +.

#+;$I;+US $).I77#+;$I;+US $).I77A$ 2A$ 2;D;D7$7$I;$&.S"A#&I;$&.S"A#&


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A@SC@TAT4O/A@SC@TAT4O/

A##&;$UA$&D S1A##&;$UA$&D S1 ;A..+>7Q +. "A.AD+OI#A77Q S"7I$;A..+>7Q +. "A.AD+OI#A77Q S"7I$

S2S2 7+UD "27+UD "2 #+;$I;+USBI*S+;S$.AI;4I;4#+;$I;+USBI*S+;S$.AI;4I;4

$U;;&7 CU.CU. *&S$ )&A.D I;$U;;&7 CU.CU. *&S$ )&A.D I;

I;%.A#7A!I#U7A. .&BI+;I;%.A#7A!I#U7A. .&BI+; CI$.A7 D&7AQ&D DIAS$+7I# CU.CU.CI$.A7 D&7AQ&D DIAS$+7I# CU.CU.


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C?= 84/D4/;SC?= 84/D4/;S

#A.DI+C&BA7Q#A.DI+C&BA7Q

7A &;7A.B&C&;$7A &;7A.B&C&;$ 7! &;7A.B&C&;$7! &;7A.B&C&;$ ".+CI;&;$".+CI;&;$

A+.$I# T;U#T7&A+.$I# T;U#T7& "U7C "7&$)+.A"U7C "7&$)+.A


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(0(0

0C; CHA/;0S0C; CHA/;0S

;+.CA7 AOIS >I$) 7$;+.CA7 AOIS >I$) 7$!&;$.I#U7A. )Q"&.$.+")Q!&;$.I#U7A. )Q"&.$.+")Q

0CHO P4CT@=0S0CHO P4CT@=0S


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(1(1

0CHO P4CT@=0S0CHO P4CT@=0S


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(2(2

2A/A;020/T2A/A;020/T

C&DI#A7C&DI#A7

I;D+C&$)A#I; 0F1I;D+C&$)A#I; 0F1mggdose,orally,'d or three dosesmggdose,orally,'d or three doses

Digo@in or increasing woring capacityDigo@in or increasing woring capacityo hearto heart

Diuretics to reduce preload on heartDiuretics to reduce preload on heart


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(3(3

S@=;4CA =0PA4= BD0V4C0SS@=;4CA =0PA4= BD0V4C0S


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(/(/

Coarctation of the AortaCoarctation of the Aorta

Cales twice as reGuently as emalesFCales twice as reGuently as emalesF 95 o all coarctations at segment o95 o all coarctations at segment o

aorta adacent to ductus arteriosusFaorta adacent to ductus arteriosusF "roduced 'y 'oth an e@ternal narrowing"roduced 'y 'oth an e@ternal narrowing

and an intraluminal mem'raneFand an intraluminal mem'raneF *lood low to the lower 'ody maintained*lood low to the lower 'ody maintained

through collateral -esselsFthrough collateral -esselsF


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;arrowing in;arrowing in

pro@imal descendingpro@imal descendingaortaaorta

Cay 'e longtu'ularCay 'e longtu'ular'ut most commonly'ut most commonly

discrete ridgediscrete ridge ;atural h@: poor;atural h@: poor

prognosis iprognosis iunrepairedunrepaired

AorticAorticAneurysmdissectionAneurysmdissection

#)%#)%

"remature #ADz"remature #ADz


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(9(9

C4/4CAC4/4CA

A'sent or wea emoral pulsesFA'sent or wea emoral pulsesF Systolic pressure higher in upperSystolic pressure higher in upper

e@tremities than in lowere@tremities than in lowere@tremitiesP diastolic pressures aree@tremitiesP diastolic pressures aresimilarFsimilarF

)arsh systolic murmur heard in the)arsh systolic murmur heard in the

'acF'acF .i' notching on #O. pathognomonic.i' notching on #O. pathognomonic


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=i: notching=i: notching


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/0/0

TreatmentTreatment

Despite surgery, patients still ha-e signiicantDespite surgery, patients still ha-e signiicant

mor'iditymortality with a-erage age 39mor'iditymortality with a-erage age 39

Up to 805 o repaired patients still go on toUp to 805 o repaired patients still go on to

de-elop )$;, pathology not well understoodde-elop )$;, pathology not well understood

.ecurrence in 94/(5 o repairs, can undergo.ecurrence in 94/(5 o repairs, can undergo

repeat surgery or 'alloon angioplastyrepeat surgery or 'alloon angioplasty

Aortic Aneurysmruputure may occur despiteAortic Aneurysmruputure may occur despite

successul repair and correction o )$; reGsuccessul repair and correction o )$; reGaround anastomosis site on patch repair around anastomosis site on patch repair

305 in one study6305 in one study6


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/1/1


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/2/2

Tetralogy of 8allotTetralogy of 8allot

( eatures( eatures Calalignment !SDCalalignment !SD

+-erriding Aorta+-erriding Aorta

"ulmonic Stenosis"ulmonic Stenosis .!).!)

!aria'ility correlates!aria'ility correlates

with degree o .!+$with degree o .!+$o'struction ando'struction andsizeanatomy o "Asizeanatomy o "A


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/3/3


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To8B Surgical TreatmentTo8B Surgical Treatment

Systemic "ulmonary ShuntSystemic "ulmonary Shunt *laloc4$aussig*laloc4$aussig

>aterston ."A6>aterston ."A6

"otts 7"A6"otts 7"A6 #omplete .epair#omplete .epair

taedown o prior shunttaedown o prior shunt

patch !SDpatch !SD

resection o su'pulmonic o'structionresection o su'pulmonic o'struction

transannular patch around pulm -al-e annulustransannular patch around pulm -al-e annulususually leads to se-ere "I6usually leads to se-ere "I6

0:steins Anomaly0:steins Anomaly


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0:steins Anomaly0:steins Anomaly

Atrialization o .!, sail4lieAtrialization o .!, sail4lie

$!, $.$!, $. /05 ASD"%+/05 ASD"%+ /05 B e-idence o/05 B e-idence o

>">>"> Age at presentation -ariesAge at presentation -aries

rom childhoodrom childhoodadulthoodadulthoodand depends on actorsand depends on actorssuch as se-erity o $.,such as se-erity o $.,

"ulm !ascular resistance in"ulm !ascular resistance innew'orn, and associatednew'orn, and associateda'normalities such as ASDa'normalities such as ASD

www7ucch7org


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Massive cardiomegaly,mainly due to RAE


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0:steinsB Clinical Presentation0:steinsB Clinical Presentation

"ediatric"ediatric murmurmurmur

Adult unrepaired with ASD6Adult unrepaired with ASD6

atrial arrhythmiasatrial arrhythmias murmurmurmur

cyanosiscyanosis ..7 shunt ;+$ due to "ulm)$; 'ut $. et directed7 shunt ;+$ due to "ulm)$; 'ut $. et directed

across ASDacross ASD e@ercise intolerancee@ercise intolerance

Surgery in pts with signiicant $.s@sSurgery in pts with signiicant $.s@s


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0isenmenger+s Syndrome0isenmenger+s Syndrome

%inal common pathway or all%inal common pathway or allsigniicant 7signiicant 7. shunting in which. shunting in whichunrestricted pulmonary 'lood lowunrestricted pulmonary 'lood low

leads to pulmonary -aso4occlusi-eleads to pulmonary -aso4occlusi-edisease "!+D6P .disease "!+D6P .77shuntingcyanosis de-leopsshuntingcyanosis de-leops

Benerally need p:s E2:1Benerally need p:s E2:1


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0isenmenger Complications0isenmenger Complications

#oagulopathyplatelet consumption#oagulopathyplatelet consumption *rain a'cesses*rain a'cesses #ere'ral microem'oli#ere'ral microem'oli Airway hemorrhageAirway hemorrhage

especially mo-ing rom lowerespecially mo-ing rom lowerhigherhigheraltitudes air tra-el, mountains6altitudes air tra-el, mountains6

0i T t t0i T t t


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0isenmengerB Treatment0isenmengerB Treatment

S@s ?polycythemiaS@s ?polycythemia phle'otomyphle'otomy

#areul i microcytosis, strongest predictor o#areul i microcytosis, strongest predictor o

cere'ro-ascular e-entscere'ro-ascular e-ents

.U7& +U$ #+..$A*7& DIS&AS&.U7& +U$ #+..$A*7& DIS&AS&

+nce diagnosis esta'lished, a-oid+nce diagnosis esta'lished, a-oid

aggressi-e testing as many patients dieaggressi-e testing as many patients die

during cardio-ascular proceduresduring cardio-ascular procedures

Diuretics prn, o@ygenDiuretics prn, o@ygen Deiniti-e: )eart 7ung transplantDeiniti-e: )eart 7ung transplant

"rostacyclin therapy may delay, e@pensi-e"rostacyclin therapy may delay, e@pensi-e


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Documents

Adult CHD Irsad 2008