congenital diorders of heart, Cyanotic and Acyanotic

1. Cardiac disordersCardiac disorders
- Dr Sharad S J- Dr Sharad S J MSMS

3. Development of the heart andDevelopment of the heart and
fetal circulationfetal circulation
 By 12 weeks of fetal life, the primitiveBy 12 weeks of fetal life, the primitive
vascular tube is fully developedvascular tube is fully developed
 Right and left ventricles pump blood inRight and left ventricles pump blood in
parallel rather than in seriesparallel rather than in series
 Presence of three structural shunts:Presence of three structural shunts:

the ductus venosus,the ductus venosus,

the foramen ovale andthe foramen ovale and

the ductus arteriosusthe ductus arteriosus

5.  Soon after birth, pulmonary vascularSoon after birth, pulmonary vascular
resistance falls resulting pulmonaryresistance falls resulting pulmonary
vasodilatation.vasodilatation.
 In addition, within 30 minutes of delivery,In addition, within 30 minutes of delivery,
the ductus arteriosus constricts inthe ductus arteriosus constricts in
response to an increase in blood oxygenresponse to an increase in blood oxygen
levels.levels.
 Reversal of the pulmonary–systemicReversal of the pulmonary–systemic
pressure gradientpressure gradient

6.  The abolition of venous return from theThe abolition of venous return from the
placenta also causes the ductus venosusplacenta also causes the ductus venosus
to close.to close.
 In 20 per cent of adults, the structuralIn 20 per cent of adults, the structural
closure of the foramen ovale remainsclosure of the foramen ovale remains
incomplete, but is of no cardiovascularincomplete, but is of no cardiovascular
significance.significance.

7.  After birth, the act of cutting and tying theAfter birth, the act of cutting and tying the
umbilical cord stops venous blood flowumbilical cord stops venous blood flow
from the placentafrom the placenta
 lowers the pressurelowers the pressure in the inferior venain the inferior vena
cavacava
 right atrial pressure fallsright atrial pressure falls
 Closure of the foramen ovaleClosure of the foramen ovale

8.  Abnormalities of cardiac structure mayAbnormalities of cardiac structure may
arise from thearise from the
 Persistence of normal fetal channels –Persistence of normal fetal channels –

PDA,PDA,

Patent foramen ovalePatent foramen ovale
 Failure of septation-Failure of septation-

atrial septal defect,atrial septal defect,

ventricular septal defect,ventricular septal defect,

tetralogy of Fallottetralogy of Fallot

9.  StenosisStenosis

intracardiac–supravalvular, valvular,intracardiac–supravalvular, valvular,

extracardiac–coarctation of the aortaextracardiac–coarctation of the aorta
 atresia or abnormal connectionsatresia or abnormal connections

transposition of the great arteries,transposition of the great arteries,

total anomalous venous drainagetotal anomalous venous drainage

10. CONGENITAL HEARTCONGENITAL HEART
DISEASESDISEASES
 Congenital heart diseases areCongenital heart diseases are
abnormalities of cardiac structure that areabnormalities of cardiac structure that are
present from birthpresent from birth

11.  Left to right shuntLeft to right shunt—It leads into—It leads into
overloading of the pulmonary circulation.overloading of the pulmonary circulation.
Usually it is acyanotic.Usually it is acyanotic.

PDAPDA——Patent ductus arteriosusPatent ductus arteriosus

ASDASD——Atrial septal defectAtrial septal defect

VSDVSD——Ventricular septal defect.Ventricular septal defect.

12.  Right to Left shuntRight to Left shunt—Blood from the—Blood from the
systemic veins enter the systemic arteriessystemic veins enter the systemic arteries
bypassing the lungs allowing severebypassing the lungs allowing severe
hypoxic blood to enter the systemichypoxic blood to enter the systemic
circulation. It will cause cyanosis.circulation. It will cause cyanosis.
Examples areExamples are

Tetralogy of Fallot andTetralogy of Fallot and

Transposition of great vesselsTransposition of great vessels

13.  Conditions without shuntsConditions without shunts——

Coarctation of aorta, aorticCoarctation of aorta, aortic

stenosis, pulmonary stenosis.stenosis, pulmonary stenosis.

14. COARCTATION OfCOARCTATION Of
AORTAAORTA

15. IntroductionIntroduction
 Common congenital malformationCommon congenital malformation
 10 – 15 % CHD10 – 15 % CHD
 M >> FM >> F
 Narrowing of the aorta immediatelyNarrowing of the aorta immediately
beyond the origin of Lt Subclvn artrybeyond the origin of Lt Subclvn artry
 Lies near 2 positn of ligamentumLies near 2 positn of ligamentum
arteriosumarteriosum

16. AETIO-PATHOLOGYAETIO-PATHOLOGY
 Extn of d fibrotic process which converts dExtn of d fibrotic process which converts d
patent ductus into lig. arteriosum.patent ductus into lig. arteriosum.
 2 – 4 cms in length jus blo d origin of Lt2 – 4 cms in length jus blo d origin of Lt
Subcl. artrySubcl. artry
 Aorta distal 2 coarctn – usually dilated –Aorta distal 2 coarctn – usually dilated –
may develop aneurysm (rare -- elderly)may develop aneurysm (rare -- elderly)
 Collateral circln – 2 bypass d constrn –Collateral circln – 2 bypass d constrn –
dilated intercostal arteries, entering ddilated intercostal arteries, entering d
distal aorta – NOTCHING of d ribs.distal aorta – NOTCHING of d ribs.

17. TYPESTYPES
i.i. Post-ductal typePost-ductal type

Usual typeUsual type
i.i. Pre-ductal typePre-ductal type

Coarctn – above d joining of PDACoarctn – above d joining of PDA

Deoxy blood from Pulm artry 2 aorta distal 2Deoxy blood from Pulm artry 2 aorta distal 2
coarctn –coarctn – differential cyanosisdifferential cyanosis

18. CLINICAL FEATURESCLINICAL FEATURES
 Hypertension above d coarctnHypertension above d coarctn

Haedache, dizziness, epistaxisHaedache, dizziness, epistaxis

Throbbing n pulsation of head n neck artrsThrobbing n pulsation of head n neck artrs

Systolic mrmr at apexSystolic mrmr at apex
 Hypotension blo d coactnHypotension blo d coactn

Intermittent claudicationIntermittent claudication

Cold lower extremitiesCold lower extremities

Weak femoral pulseWeak femoral pulse

19. COMPLICATIONSCOMPLICATIONS
 LVFLVF
 Intracranial haemorrhageIntracranial haemorrhage
 Intrathoracic haemorrhageIntrathoracic haemorrhage
 Rupture of AortaRupture of Aorta

20. INVESTIGATIONSINVESTIGATIONS
 CXR-PACXR-PA

Notching of ribsNotching of ribs
 ECGECG

s/o L V Hypertrophys/o L V Hypertrophy
 Cardiac catheterisation & AortographyCardiac catheterisation & Aortography

RoutinelyRoutinely

Exact site n extent of d coarctnExact site n extent of d coarctn

21. TREATMENT -- surgicalTREATMENT -- surgical
 Infants wid CCF –Infants wid CCF – URGENT RxURGENT Rx
 Ideal age – 5 to 7 yrsIdeal age – 5 to 7 yrs
 Coarctation is excised – end 2 end anast.Coarctation is excised – end 2 end anast.

22. PATENT DUCTUSPATENT DUCTUS
ARTERIOSUS (PDA)—10%ARTERIOSUS (PDA)—10%
 Ductus arteriosus is essential in fetal lifeDuctus arteriosus is essential in fetal life
 Blood from pulmonary artery reaches theBlood from pulmonary artery reaches the
aorta through this ductus arteriosusaorta through this ductus arteriosus
 Prostaglandin prevents closureProstaglandin prevents closure
 Indomethacin promotes closureIndomethacin promotes closure
 If it persists in child it is called as PDAIf it persists in child it is called as PDA

24.  In PDA, part of the blood from aorta flowsIn PDA, part of the blood from aorta flows
back to pulmonary arteryback to pulmonary artery
 This causes pulmonary hypertension.This causes pulmonary hypertension.
 Occasionally pressure is so severe thatOccasionally pressure is so severe that
flow may reverse-flow may reverse- Eisenmenger‘sEisenmenger‘s
syndromesyndrome

25. Clinical featuresClinical features
Child presents with dyspnoea,Child presents with dyspnoea,
Chest pain,Chest pain,
Machinery murmurMachinery murmur

26. InvestigationsInvestigations
AortogramAortogram andand
EchocardiographyEchocardiography

27. TreatmentTreatment
 After six months of age, spontaneousAfter six months of age, spontaneous
closure of a PDA is rareclosure of a PDA is rare
 Most should be closed by preschool ageMost should be closed by preschool age
 interventional cardiology using aninterventional cardiology using an
umbrella or coilumbrella or coil
 surgical closure via a left thoracotomy-surgical closure via a left thoracotomy-
ligation or division of the PDAligation or division of the PDA

29. Complications of PDAComplications of PDA
Congestive cardiac failure,Congestive cardiac failure,
bacterial endocarditis.bacterial endocarditis.

30. ATRIAL SEPTAL DEFECTATRIAL SEPTAL DEFECT
(ASD)—7%(ASD)—7%
 Defect in the atrial septum thus causingDefect in the atrial septum thus causing
communication between left and rightcommunication between left and right
atriumatrium
 TypesTypes

Secundum typeSecundum type is the commonest one. It isis the commonest one. It is
elliptical, in the middle of the atrial septumelliptical, in the middle of the atrial septum
and is due to defect in the site of foramenand is due to defect in the site of foramen
ovaleovale

Primum typePrimum type is lower part. It is rareis lower part. It is rare

Sinus venosus typeSinus venosus type is near SVC openingis near SVC opening

33. Clinical FeaturesClinical Features
Shunting of blood from left atrium to rightShunting of blood from left atrium to right
atriumatrium
Left ventricular hypertrophyLeft ventricular hypertrophy
Pulmonary hypertensionPulmonary hypertension
Eventually Eisenmenger’s- cyanosisEventually Eisenmenger’s- cyanosis
DyspnoeaDyspnoea
Fixed split of second heart soundFixed split of second heart sound
Systolic murmur due to increased bloodSystolic murmur due to increased blood
flow through the pulmonary valveflow through the pulmonary valve

34. InvestigationsInvestigations
Echocardiography,Echocardiography,
Cardiac catheterisation andCardiac catheterisation and
angiocardiogramangiocardiogram

35. TreatmentTreatment
Closure is performed during the firstClosure is performed during the first
decade of lifedecade of life
Openheart surgery with CPB and ClosureOpenheart surgery with CPB and Closure
of the defect by direct suturing orof the defect by direct suturing or
Patch either pericardium or DacronPatch either pericardium or Dacron
Closure of small to moderate ASDs usingClosure of small to moderate ASDs using
percutaneous catheterpercutaneous catheter

37. VENTRICULAR SEPTAL DEFECTVENTRICULAR SEPTAL DEFECT
(VSD)—15%(VSD)—15%
 Defect in the ventricular septum causingDefect in the ventricular septum causing
left to right shuntleft to right shunt
 TypesTypes
 Four types of VSD are present dependingFour types of VSD are present depending
on locationon location
 Defect can be small or large.Defect can be small or large.
 may be associated with tetralogy of Fallotmay be associated with tetralogy of Fallot

40. Clinical FeaturesClinical Features
Pansystolic murmur,Pansystolic murmur,
Palpable thrill,Palpable thrill,
Split second sound with pulmonarySplit second sound with pulmonary
accentuation,accentuation,
Recurrent respiratory infections.Recurrent respiratory infections.

41.  TreatmentTreatment
 Surgical closure is indicated for largeSurgical closure is indicated for large
defects when there isdefects when there is

failure to respond to medical therapy forfailure to respond to medical therapy for

left-to-right shunts of >2:1left-to-right shunts of >2:1

Increasing pulmonary vascular resistanceIncreasing pulmonary vascular resistance

presence of complications of VSDpresence of complications of VSD

42. ComplicationsComplications
Aortic regurgitationAortic regurgitation
Infundibular stenosis,Infundibular stenosis,
Infective endocarditisInfective endocarditis

43. CYANOTIC CHDsCYANOTIC CHDs
TOFTOF
TGVTGV

44. FALLOT’s TETRALOGYFALLOT’s TETRALOGY

45. INTRODUCTIONINTRODUCTION
 Commonest of cyanotic CHDsCommonest of cyanotic CHDs
 50% of all cases50% of all cases
 Complex conditionComplex condition

46. COMPONENTSCOMPONENTS
 Features –Features –
i.i. Pulm stenosis (infundibular/ valvular/ both)Pulm stenosis (infundibular/ valvular/ both)
 Rt Ventricular Outflow obstructionRt Ventricular Outflow obstruction
i.i. VSDVSD
 Large defect 2 -3 cmsLarge defect 2 -3 cms
i.i. Overriding of aortaOverriding of aorta
 Recieves blood from both ventriclesRecieves blood from both ventricles
i.i. Rt Vent. HypertrophyRt Vent. Hypertrophy

48. PATHOLOGYPATHOLOGY
 Obstruction to RV outflow + VSD – venousObstruction to RV outflow + VSD – venous
blood in RV shunted directly 2 Aorta –blood in RV shunted directly 2 Aorta –
cyanosiscyanosis
 Pulmonary blood flow decreased – lowPulmonary blood flow decreased – low
oxygen saturation – exercise intolerance.oxygen saturation – exercise intolerance.
 Very low saturation – cerebral anoxia –Very low saturation – cerebral anoxia –
unconsciousness.unconsciousness.

49. CLINICAL FEATURESCLINICAL FEATURES
 SYMPTOMSSYMPTOMS

Usually asymptomaticUsually asymptomatic

Cyanosis as pulmonary stenosis progresses,Cyanosis as pulmonary stenosis progresses,
typically develops within the first yeartypically develops within the first year

Dyspnoea on exertionDyspnoea on exertion

Lethargy and tirednessLethargy and tiredness

Walking 4 short distances, interrupted byWalking 4 short distances, interrupted by
squatting – pathognomonic.squatting – pathognomonic.

50.  Squatting :Squatting :
Systemic vascular resistance increases andSystemic vascular resistance increases and
the venous return to the heart is divertedthe venous return to the heart is diverted
into the pulmonary circulation withinto the pulmonary circulation with
increased oxygenationincreased oxygenation

51. C/F -- SignsC/F -- Signs
 CyanosisCyanosis
 ClubbingClubbing
 Normal heart sizeNormal heart size
 Systolic murmer a/w thrill in 50%Systolic murmer a/w thrill in 50%
 P2 weak/absentP2 weak/absent
 A2 increasedA2 increased

52. INVESTIGATIONSINVESTIGATIONS
 CXR-PACXR-PA

‘‘boot-shapedboot-shaped’ heart’ heart

Decreased vascularity—lung fieldsDecreased vascularity—lung fields
 ECGECG

Rt axis deviation, RVHRt axis deviation, RVH
 Cardiac CatheterizationCardiac Catheterization

R – L shuntR – L shunt

pulmonary blood flow and pressure decreasedpulmonary blood flow and pressure decreased
 Selective AngiocardiographySelective Angiocardiography

to plan the surgeryto plan the surgery

simultaneous opacification of Aorta + Pulmonarysimultaneous opacification of Aorta + Pulmonary
Artery when dye is injected into the RV.Artery when dye is injected into the RV.

53.  Surgery to correct the tetralogy can beSurgery to correct the tetralogy can be
performed early as a single completeperformed early as a single complete
primary repair orprimary repair or
 Later following an initial palliative shunt,Later following an initial palliative shunt,
which diverts systemic blood into thewhich diverts systemic blood into the
pulmonary circulation and may be used topulmonary circulation and may be used to
improve oxygenationimprove oxygenation

54. TREATMENT – surgeryTREATMENT – surgery
 LIFE SAVING ANASTOMOTICLIFE SAVING ANASTOMOTIC
PROCEDUREPROCEDURE

Infants with cyanosis + unconsciousnessInfants with cyanosis + unconsciousness

shunt procedure –shunt procedure – BLALOCK’s SubclavianBLALOCK’s Subclavian
Pulmonary AnastamosisPulmonary Anastamosis

other anastomotic proceduresother anastomotic procedures
• side 2 side anastomosis between ascending aortaside 2 side anastomosis between ascending aorta
and right pulmonary arteryand right pulmonary artery
• Potts anastamosis between descending aorta andPotts anastamosis between descending aorta and
left pulmonary arteryleft pulmonary artery
• Glenn anastamosis between SVC + Rt Pulm artry.Glenn anastamosis between SVC + Rt Pulm artry.

55.  CORRECTIVE OPERATIONCORRECTIVE OPERATION

High vertical ventriculotomy.High vertical ventriculotomy.

VSD closed with a DACRON patch.VSD closed with a DACRON patch.

pulmonary Obstruction widened – DACRONpulmonary Obstruction widened – DACRON
graft.graft.

ventriculotomy closed – DACRON patch.ventriculotomy closed – DACRON patch.

56. TRANSPOSITION OFTRANSPOSITION OF
GREAT VESSELSGREAT VESSELS

57. INTRODUCTIONINTRODUCTION
 30 - 40 %30 - 40 %
 Most common cause of cardiac failure inMost common cause of cardiac failure in
newborn.newborn.
 Aorta arises from RV n pulmonary arteryAorta arises from RV n pulmonary artery
from LVfrom LV
 Often a/w other cong. abnormalitiesOften a/w other cong. abnormalities

59. PATHOLOGYPATHOLOGY
 Aorta – RV, Pulm artry – LVAorta – RV, Pulm artry – LV
 Venous Blood --- aorta.Venous Blood --- aorta.
 Oxy. Blood – lungs – left heart – PulmOxy. Blood – lungs – left heart – Pulm
artry –artry – back 2 lungs.back 2 lungs.
 Deep cyanosis.Deep cyanosis.

60. CLINICAL FEATURIESCLINICAL FEATURIES
 Deep cyanosis at birth (80%), others byDeep cyanosis at birth (80%), others by
the end of 1the end of 1stst
yr.yr.
 Frequent anoxic spells – unconsciousnessFrequent anoxic spells – unconsciousness
 Cardiac failure, Dyspnoea on exertion.Cardiac failure, Dyspnoea on exertion.
 Age > 2yrs – clubbing, polycythemia.Age > 2yrs – clubbing, polycythemia.
 Asso features – pulm congestion.Asso features – pulm congestion.
cardiac enlargement.cardiac enlargement.
hepatomegaly.hepatomegaly.

61.  chest radiograph: pulmonary plethora,chest radiograph: pulmonary plethora,
‘‘egg on its side’egg on its side’
 Cardiac catheterisation andCardiac catheterisation and
 echocardiographyechocardiography

62. TREATMENTTREATMENT
 EMERGENCYEMERGENCY

RRashkind Bashkind Balloon Septostomyalloon Septostomy – Create– Create
communications betn pulm & Sys circulation.communications betn pulm & Sys circulation.

intravenous prostaglandinsintravenous prostaglandins
 CORRECTIVECORRECTIVE

Arterial switch procedure- carried out as aArterial switch procedure- carried out as a
two-stage procedure, whereby the pulmonarytwo-stage procedure, whereby the pulmonary
artery is first banded to ‘tone up’ the leftartery is first banded to ‘tone up’ the left
ventricleventricle

Atrial switchAtrial switch

63. Total anomalous pulmonaryTotal anomalous pulmonary
venous drainagevenous drainage
 l–2 per cent of congenital heart diseasel–2 per cent of congenital heart disease
 Pulmonary venous drainage has becomePulmonary venous drainage has become
disconnected from the left atriumdisconnected from the left atrium
 Drains into the systemic venous circulationDrains into the systemic venous circulation
at some other point- Inferior vena cava,at some other point- Inferior vena cava,
superior vena cava, coronary sinus or rightsuperior vena cava, coronary sinus or right
atrium.atrium.

65.  TAPVD presents after the first week of lifeTAPVD presents after the first week of life
with cyanosiswith cyanosis
 cardiac failure,cardiac failure,
 recurrent chest infections,recurrent chest infections,
 failure to thrive andfailure to thrive and
 feeding difficultiesfeeding difficulties

66.  Echocardiography and cardiacEchocardiography and cardiac
(pulmonary) angiography are necessary to(pulmonary) angiography are necessary to
confirm the diagnosisconfirm the diagnosis
 Surgical principle : Re-establish theSurgical principle : Re-establish the
pulmonary venous drainage into the leftpulmonary venous drainage into the left
atrium.atrium.
 Operative technique depends on theOperative technique depends on the
anatomy and type of TAPVDanatomy and type of TAPVD