File1-Congenital Heart Disease

8/18/2019 File1-Congenital Heart Disease

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Congenital heart diseaseCongenital heart disease

** congenital cardiac malformations resulting from defectivecongenital cardiac malformations resulting from defective

embryonicembryonicdevelopment without obvious cause.development without obvious cause.

** Between 3-8 weeks “gestation” all the fetal heart structures areBetween 3-8 weeks “gestation” all the fetal heart structures areformed “organogenesis”formed “organogenesis”

IncidenceIncidence

8-1!1 live births in india8-1!1 live births in india appro"imately #! year appro"imately #! year

$ncidence is more in %-$ncidence is more in %-a-&remature a-&remature

b-abortions b-abortions

c-still births c-still births

$ncidence increased for siblings.$ncidence increased for siblings. #-'()1-1#()#*(#-'()1-1#()#*(

EtiologyEtiology

+ultifactorial inheritance pattern “mostly” % +ultifactorial inheritance pattern “mostly” %

,hromosomal abnormality *-1(.,hromosomal abnormality *-1(.

•• -/risomy #1 *( 0 -2 canal)24)4) others.-/risomy #1 *( 0 -2 canal)24)4) others.

•• -/risomy 18 8(0 24)4)others.-/risomy 18 8(0 24)4)others.

•• -/risomy 13 5(0 24)4)&4)others.-/risomy 13 5(0 24)4)&4)others.

•• -/urner syndrome "o0Bicuspid aortic valve and co-ao-/urner syndrome "o0Bicuspid aortic valve and co-ao-others. -others.

Adverse maternal conditions (enviromential)Adverse maternal conditions (enviromential)

66 MaternalMaternal infectionsinfections 07ubella%&4)&07ubella%&4)&

••

Maternal diseasesMaternal diseases0 &9-24)40 &9-24)4 4+%left septal hypertrophy 4+%left septal hypertrophy


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•• DrugsDrugs0fetal hydntoin syndrome- 240fetal hydntoin syndrome- 24

2alproate effect-co ao left heart hypoplasia2alproate effect-co ao left heart hypoplasia

66 Fetal alcohol syndromeFetal alcohol syndrome0 24)4),:-:.0 24)4),:-:.

-Advance maternal age. -Advance maternal age.

Syndrome complexesSyndrome complexes

-2,/7;eart24)/:=)-2 canal) tresiatresia

choanal)7etardation)?ential);ar abnormalties.choanal)7etardation)?ential);ar abnormalties.

-artagener syndrome 4e"trocardia -artagener syndrome 4e"trocardia

Majority of cases of the congenital heart diseases are unno!n causeMajority of cases of the congenital heart diseases are unno!n cause

ClassifcationClassifcation

,.>.4 with little or no cyanosis “acyanotic”,.>.4 with little or no cyanosis “acyanotic”

a-@ith


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•• Atrial septal defectsAtrial septal defects

•• Atrio-ventricular septal defectsAtrio-ventricular septal defects

•• $atent ductus arteriosus$atent ductus arteriosus

•• %runcus arteriosus%runcus arteriosus

••

Aortic stenosisAortic stenosis•• Mitral stenosis&incompetenceMitral stenosis&incompetence

•• "oarctation of aorta"oarctation of aorta

•• %ricuspid regurgitation%ricuspid regurgitation

"ommon "yanotic 'esions"ommon "yanotic 'esions

Decreased flo!Decreased flo!

1. /etralogy of =allot1. /etralogy of =allot

#. /ricuspid tresia#. /ricuspid tresia

3. evere &ulmonic tenosis3. evere &ulmonic tenosis

5. ;bsteinCs anamoly5. ;bsteinCs anamoly

ncreased Flo!ncreased Flo!*. /ransposition of great vessles*. /ransposition of great vessles

'. 24 with pulmonary atresia '. 24 with pulmonary atresia . /runcus rteriosus. /runcus rteriosus

8. >ypoplastic left heart8. >ypoplastic left heart

D. ingle ventricle D. ingle ventricle

PrevalencePrevalence

"yanotic **+"yanotic **+

Acyanotic ,+Acyanotic ,+

–– 2424 #*(#*(

–– 44 '('(–– &4&4 '('(

–– /:=/:= *(*(

–– &&*(*(


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–– *(*(

DiagnosisDiagnosis

;arly diagnosis of ,.>.4 mean better results.;arly diagnosis of ,.>.4 mean better results.

5( of ,.>.4 diagnosed at 15( of ,.>.4 diagnosed at 1stst w of life.w of life.

*-' ( diagnosed at 1*-' ( diagnosed at 1stst two months .two months .

:thers are usually later during routine medical e"amination.:thers are usually later during routine medical e"amination.

4iagnosis depend on good clinical history E good medical4iagnosis depend on good clinical history E good medicale"amination and investigations.e"amination and investigations.

istoryistory

ge of the patient.ge of the patient.

sk forsk for-=eeding difficulties-=eeding difficulties

-2omiting-2omiting

-ypoactivity

-=././-=././

ll may be manifestation of congestive heart failure.ll may be manifestation of congestive heart failure.

->istory of dysnea on e"ertion.->istory of dysnea on e"ertion.

-hortness of breathing.-hortness of breathing.

-:rthopnea.-:rthopnea.

-


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-,onvulsion.-,onvulsion.

-ll may be manifest of congestive heart failure.-ll may be manifest of congestive heart failure.

Physical examinationPhysical examination

$nspection$ns pection look for%look for%

-,yanosis-,yanosis

-4igital clubbing-4igital clubbing

-/achypnea-/achypnea

-&rominence of the precordium cardiomegaly)7t.heart enlargement -&rominence of the precordium cardiomegaly)7t.heart enlargement

-Fugular veins engorgement “older children”-Fugular veins engorgement “older children”

-ny associated defects or findings down syndrome) 4igorge syndromeGetc-ny associated defects or findings down syndrome) 4igorge syndromeGetc

00 AuscultationAuscultation

=irst heart sound -2 valves closure=irst heart sound -2 valves closure

“Best heard at the


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InvestigationInvestigation ,B,---- polycythemia) anemiaG.etc,B,---- polycythemia) anemiaG.etc

,H7----heart siIe and shape,H7----heart siIe and shape

;,?--->7)a"is;,?--->7)a"is

www.hearta"is.comwww.hearta"is.com

)rythm)rythm

)72>)B2>)BBB.)72>)B2>)BBB.

;chocardiography;chocardiography

+7$+7$

,ardiac catheteriIation,ardiac catheteriIation

"yanosis is it a cardiac cause or lung cause"yanosis is it a cardiac cause or lung cause

>ypero"ia test>ypero"ia test

–– Jeonates with cyanotic congenital heart disease Jeonates with cyanotic congenital heart diseaseusually do not have significantly raised arterial &ao#usually do not have significantly raised arterial &ao#

during administration of 1( o"ygen.during administration of 1( o"ygen.

#entricular Defect#entricular Defect

Small #SDSmall #SD

–– symptomaticsymptomatic

–– loud) harsh) or blowing holosystolic murmur. loud) harsh) or blowing holosystolic murmur. 'arge #SD'arge #SD

–– dyspnea) feeding difficulties) poor growth) profusedyspnea) feeding difficulties) poor growth) profuse perspiration) recurrent pulmonary infections) and perspiration) recurrent pulmonary infections) and

cardiac failure in early infancy.cardiac failure in early infancy.

2entricular eptal 4efect 242entricular eptal 4efect 24

#entricular Septal defects#entricular Septal defects

3K*( of small defects close spontaneously) most freLuently during the 1st #3K*( of small defects close spontaneously) most freLuently during the 1st #

yr of life.yr of life.

mall muscular 24s are more likely to close up to 8( than membranousmall muscular 24s are more likely to close up to 8( than membranous

24s are up to 3*(.24s are up to 3*(.

infants with large defects have repeated episodes of respiratory infection andinfants with large defects have repeated episodes of respiratory infection and

heart failure despite optimal medical management.heart failure despite optimal medical management.

urgical repair prior to development of an irreversible increase in pulmonaryurgical repair prior to development of an irreversible increase in pulmonaryvascular resistance usually prior to the patientMs second birthday.vascular resistance usually prior to the patientMs second birthday.


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Atrial Septal Defects secundumAtrial Septal Defects secundum

+ost common form of 4 fossa ovalis+ost common form of 4 fossa ovalis

$n large defects) a considerable shunt of o"ygenated blood flows from the left to$n large defects) a considerable shunt of o"ygenated blood flows from the left tothe right atrium.the right atrium.

+ostly asymptomatic+ostly asymptomatic

/he #nd heart sound is characteristically widely split and fi"ed./he #nd heart sound is characteristically widely split and fi"ed.

Atrial Septal Defects primumAtrial Septal Defects primum

ituated in the lower portion of the atrial septum and overlies the mitral andituated in the lower portion of the atrial septum and overlies the mitral and

tricuspid valves. $n most instances) a cleft in the anterior leaflet of the mitraltricuspid valves. $n most instances) a cleft in the anterior leaflet of the mitralvalve is also noted.valve is also noted.

,ombination of a left-to-right shunt across the atrial defect and mitral,ombination of a left-to-right shunt across the atrial defect and mitral

insufficiencyinsufficiency

,!= similar to that of an ostium secundum 4,!= similar to that of an ostium secundum 4

Atrial Septal DefectAtrial Septal Defect

;nlargement of the right ventricle;nlargement of the right ventricle

;nlargement of atrium;nlargement of atrium


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:stium primum defects are approached surgically:stium primum defects are approached surgically

$atent Ductus Arteriosus$atent Ductus Arteriosus

mall defect no symptoms.mall defect no symptoms.


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7ight ventricular hypertrophy7ight ventricular hypertrophy

““,yanosis) especially in the adult) is the result of a small number of cardiac,yanosis) especially in the adult) is the result of a small number of cardiac

malformations well determinedG. :neGis much more freLuent than themalformations well determinedG. :neGis much more freLuent than theothersG. /his malformation consists of a true anatomopathologic typeothersG. /his malformation consists of a true anatomopathologic type

represented by the following tetralogyrepresented by the following tetralogy%%

11tenosis of the pulmonary arterytenosis of the pulmonary arteryPP

##$nterventricular communication$nterventricular communicationPP

334eviation of the origin of the aorta to the rightP and4eviation of the origin of the aorta to the rightP and

55>ypertrophy) almost always concentric in type) of the right ventricle>ypertrophy) almost always concentric in type) of the right ventricle..

=ailure of obliteration of the foramen ovale may occasionally be added in a wholly=ailure of obliteration of the foramen ovale may occasionally be added in a wholly

accessory manner.accessory manner. tt

+ost common cyanotic lesion to 1( of all ,>4+ost common cyanotic lesion to 1( of all ,>4

66/ypical features/ypical features

11..,yanosis after the neonatal period,yanosis after the neonatal period

##..>ypo"emic spells during infancy>ypo"emic spells during infancy

33..7ight-sided aortic arch in #*( of all patients7ight-sided aortic arch in #*( of all patients

••0epair may 1e staged (modified /% shunt) or0epair may 1e staged (modified /% shunt) or

completecomplete

••%reatment of "..D%reatment of "..D

•• /his is depend on the type of the ,.>.4./his is depend on the type of the ,.>.4.

•• Jo treatment observationEreassurance Jo treatment observationEreassurance

•• +edical treatmentantifailure)antiarythmaic..etc.+edical treatmentantifailure)antiarythmaic..etc.

•• urgical treatment palliative or curative.urgical treatment palliative or curative.

•• ,ardiac transplant or lung heart transplant.,ardiac transplant or lung heart transplant.

••

11-- 2eneral measures2eneral measures

•• pecial positions pecial positions. semisiting )knee chest position. semisiting )knee chest position

•• :#:# most patients need :# and other need little :#.most patients need :# and other need little :#.

•• $$2=2=again depend on type of ,>4 ) some needagain depend on type of ,>4 ) some need $2= as &4 and & )$2= as &4 and & )

:/>;7 need:/>;7 need $2= when ever there are pulmonary congestion or$2= when ever there are pulmonary congestion or

volume overload.volume overload.

•• alt restrictionalt restriction..


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•• ;"ercise restriction;"ercise restriction..

•• 7" of anemia7" of anemia..

**-- 0x of congestive heart failure0x of congestive heart failure

–– 4igo"in4igo"in 4igitaliIation “.5mg!kg”4igitaliIation “.5mg!kg”+aintenance “.1mg!kg”+aintenance “.1mg!kg”

––


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∗∗ void pregnancy above age of 38 year.void pregnancy above age of 38 year.

∗∗ void smoking.void smoking.

∗∗ void alcohol.void alcohol.

∗∗ void e"posure to radiation.void e"posure to radiation.

∗∗

void drugs intake before medical consultation.void drugs intake before medical consultation.∗∗ 2accination of all childbearing women for rubella.2accination of all childbearing women for rubella.

File1-Congenital Heart Disease

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