congenital heart disease, PDA, TOF, VSD, ASD , Management

1. Congenital Heart Disease
Dr. Md.ToufiqurDr. Md.Toufiqur
RahmanRahman
MBBS,MBBS, FCPSFCPS, MD,, MD, FACCFACC, FESC,, FESC, FRCPEFRCPE, FSCAI,, FSCAI,
FAPSC,FAPSC, FAPSICFAPSIC, FAHA,, FAHA, FCCP,FCCP, FRCPGFRCPG
Associate Professor of CardiologyAssociate Professor of Cardiology
National Institute of Cardiovascular Diseases(NICVD),National Institute of Cardiovascular Diseases(NICVD),
Sher-e-Bangla Nagar, Dhaka-1207Sher-e-Bangla Nagar, Dhaka-1207
Consultant, Medinova, Malibagh branchConsultant, Medinova, Malibagh branch
Honorary Consultant, Apollo Hospitals, Dhaka andHonorary Consultant, Apollo Hospitals, Dhaka and
STS Life Care Centre, DhanmondiSTS Life Care Centre, Dhanmondi
CRT 2014
Washing
ton DC,
USA

2. Introduction
 Congenital Heart Disease (CHD) areCongenital Heart Disease (CHD) are
diseases a child is born with, that is CHDdiseases a child is born with, that is CHD
are due to defects a child has since birth.are due to defects a child has since birth.
 A congenital abnormality of the heart isA congenital abnormality of the heart is
present in nearly 1% live birth.present in nearly 1% live birth.
 Most form of CHD are amenable to surgery,Most form of CHD are amenable to surgery,
in most cases this lead to cure.in most cases this lead to cure.

3. General Comments
 The appropriate management of the CHDThe appropriate management of the CHD
is depends on the precise identification ofis depends on the precise identification of
the anatomy & pathophysiology of thethe anatomy & pathophysiology of the
patients abnormality. This is achieved bypatients abnormality. This is achieved by
1.1. Clinical AssessmentClinical Assessment
2.2. CXRCXR
3.3. ECGECG
4.4. Echocardiography + Color DopplerEchocardiography + Color Doppler
5.5. Cardiac Catheterization in some casesCardiac Catheterization in some cases

4.  No epidemiological data .No epidemiological data .
 Prevalence of CHD is 1%. As suchPrevalence of CHD is 1%. As such
total CHD patient in Bangladesh istotal CHD patient in Bangladesh is
nearly 1400000.nearly 1400000.
 NICVD is the only centre that hasNICVD is the only centre that has
infrastructure for treating suchinfrastructure for treating such
patientspatients..
CHD in Bangladesh

5. Spectrum of Problem
 Huge number of untreated casesHuge number of untreated cases
 Late presentationLate presentation
 Limited resourcesLimited resources
 IlliteracyIlliteracy
 Poor socio-economic conditionPoor socio-economic condition
 Social and cultural tabooSocial and cultural taboo

6.  Before going on to the details of CHD, letBefore going on to the details of CHD, let
us recapitulate some of the basic anatomyus recapitulate some of the basic anatomy
of the heart and the cardiovascular systemof the heart and the cardiovascular system
as a whole.as a whole.
 Then we shall discuss the classification, in aThen we shall discuss the classification, in a
simplified form.simplified form.
 Then Some of the common CHD is someThen Some of the common CHD is some
details.details.

7. Normal Anatomy of the Heart

10. Classification of CHD
 Classification of CHD can be done in fourClassification of CHD can be done in four
groups, as follows:groups, as follows:
1.1. Pure Obstructive lesionsPure Obstructive lesions
2.2. Simple Left-Right shunt (acyanotic)Simple Left-Right shunt (acyanotic)
3.3. Right –Left shunts (cyanotic)Right –Left shunts (cyanotic)
4.4. Complex congenital defects (mixingComplex congenital defects (mixing
defect)defect)

11.  Pulmonary stenosisPulmonary stenosis
 Mitral stenosisMitral stenosis
 Aortic stenosisAortic stenosis
 Coarctation of aortaCoarctation of aorta
 Interrupted Aortic archInterrupted Aortic arch
Pure Obstructive lesions (congenital)

12. Simple L-R Shunts
 Patient Ductus Arteriosus (PDA)Patient Ductus Arteriosus (PDA)
 Atrial Septal Defect (ASD)Atrial Septal Defect (ASD)
 Ventricular Septal Defect (VSD)Ventricular Septal Defect (VSD)
 Endocardial cushion defect (AV canal)Endocardial cushion defect (AV canal)
 Aortopulmonary windowAortopulmonary window

13. R-L Shunts Cyanotic Heart disease
 Tetralogy of Fallot (TOF)Tetralogy of Fallot (TOF)
 Pulmonary atresia with VSDPulmonary atresia with VSD
 Pulmonary atresia with out VSDPulmonary atresia with out VSD
 Tricuspid atresiaTricuspid atresia
 Ebstein’s anomalyEbstein’s anomaly

14. Complex CHD (Mixing defect)
 Double outlet right ventricle (DORV)Double outlet right ventricle (DORV)
 Univentricular heartUniventricular heart
 Transposition of Great Arteries (TGA)Transposition of Great Arteries (TGA)
 Total Anomalous Pulmonary VenousTotal Anomalous Pulmonary Venous
Drainage (TAPVD)Drainage (TAPVD)
 Truncus ArteriosusTruncus Arteriosus
 Hypoplastic Left Heart SyndromeHypoplastic Left Heart Syndrome

15. Spectrum of CHD
AcyanoticAcyanotic
 L>R shuntL>R shunt
ASD .VSD.ASD .VSD.
PDA. AVSDPDA. AVSD
 Obstructive lesionObstructive lesion
CoA. AS. PSCoA. AS. PS
CyanoticCyanotic
 With increasedWith increased
PBFPBF TGATGA.. TAPVC.TAPVC.
TRUNCUSTRUNCUS ARTERIOSUSARTERIOSUS
 With decreasedWith decreased
PBFPBF:: TOF. PA.TOF. PA.
DORVDORV

16. Some myths about CHD
 Complex CHD is not worth treatingComplex CHD is not worth treating
 Most VSD’s close spontaneously andMost VSD’s close spontaneously and
so can be ignored in early childhoodso can be ignored in early childhood
 Child below 10 kg does not with standChild below 10 kg does not with stand
surgerysurgery
 Cyanosis is harmless till the childCyanosis is harmless till the child
grows upgrows up
 Child with CHD cannot digest richChild with CHD cannot digest rich
foodfood

17. CHD - REALITIESCHD - REALITIES
 Simple CHD can become inoperable if notSimple CHD can become inoperable if not
tackled on timetackled on time
 Late presentation has high morbidity and longLate presentation has high morbidity and long
term survival is sub optimal.term survival is sub optimal.
 Timing of surgery is crucial to optimalTiming of surgery is crucial to optimal
survival.survival.
 Child with CHD needs extra calories and isChild with CHD needs extra calories and is
capable of digestingcapable of digesting it.it.

18.  Common & Usually symptomaticCommon & Usually symptomatic
 Diagnosis is made by Clinical +Diagnosis is made by Clinical +
EchoEcho
 Surgery totally cures by “Surgery totally cures by “ DivisionDivision
& Suturing& Suturing ””
PDA

19. PDA…indications
 Surgery whenever diagnosedSurgery whenever diagnosed
 All detectable PDA’s should beAll detectable PDA’s should be
closedclosed
 In pre-term PDA-institutionalIn pre-term PDA-institutional
approachapproach

20. ASD…1
 Usually symptomless in childrenUsually symptomless in children
 Diagnosis is by clinical and echoDiagnosis is by clinical and echo
 Exceptional cases -catheter &Exceptional cases -catheter &
angiographyangiography
 Cures totally by open heartCures totally by open heart
surgerysurgery

22. ASD…2
 Infancy ( relatively uncommon)Infancy ( relatively uncommon)
Symptoms of CHF unresponsive to therapySymptoms of CHF unresponsive to therapy
§§ Failure to thriveFailure to thrive
§§ Documented Pulmonary hypertension,Documented Pulmonary hypertension,
Obstructed TAPVCObstructed TAPVC
 Beyond infancyBeyond infancy
Evidence of RV volume over loadEvidence of RV volume over load
§§ Increased Pulmonary Shunt (Qp/Qs >1.8)Increased Pulmonary Shunt (Qp/Qs >1.8)
§§ Presence of PAPVC as Load IncreasesPresence of PAPVC as Load Increases
with agewith age

23. ASD…3
 Contentious issue isContentious issue is
§§ Shunt <1.5Shunt <1.5
§§ Large DefectLarge Defect
§§ Paradoxical EmbolismParadoxical Embolism
 ContraindicationsContraindications
§§ Established PVDEstablished PVD
§§ RV FailureRV Failure

24. VSD’s - FEW MYTHSVSD’s - FEW MYTHS
 MostMost VSDs close sontaneouslyVSDs close sontaneously
 Large VSDs also ClosesLarge VSDs also Closes
SpontaneouslySpontaneously
 Time of surgery: 10 years/10 KgsTime of surgery: 10 years/10 Kgs
 Clinical improvement as ClosureClinical improvement as Closure

25. VSD - FEW FACTSVSD - FEW FACTS
 Highest incidence among CHDHighest incidence among CHD
 Timely surgery: mortality < 1%Timely surgery: mortality < 1%
 Commonest Cause of EisenmengerCommonest Cause of Eisenmenger
ComplexComplex

26. VSD’s - CURRENT FACTSVSD’s - CURRENT FACTS
 Large VSDs & subarterial VSDs doLarge VSDs & subarterial VSDs do
not close spontaneouslynot close spontaneously
 Onset of Eisenmenger complex isOnset of Eisenmenger complex is
also coincident with clinicalalso coincident with clinical
improvementimprovement..

27. Large VSD
 Beyond 3 months of age at presentationBeyond 3 months of age at presentation
§§ CHF control a formidable taskCHF control a formidable task
§§ Poorly tolerate inter current illnessesPoorly tolerate inter current illnesses
§§ Development of PVD particularly rapidDevelopment of PVD particularly rapid
 Prior to 3 monthsPrior to 3 months
§§ Chance at medical stabilization , if failsChance at medical stabilization , if fails
SurgerySurgery

28. Large vsd : How to proceed ?Large vsd : How to proceed ?
 Optimize CHF managementOptimize CHF management
 Hyper Caloric feeds? NG Tube feedsHyper Caloric feeds? NG Tube feeds
 Surgical Intervention :Surgical Intervention :
§§ Poor weight gain despite best effortsPoor weight gain despite best efforts
§§ Evidence of increased PAHEvidence of increased PAH

29. THRUST TOWARDS
EARLY SURGERY- Why ?
 Restores Normal physiologyRestores Normal physiology
 Better preservation of Myocardial functionBetter preservation of Myocardial function
Hyperplasia and angiogenesis Vs.Hyperplasia and angiogenesis Vs.
HypertrophyHypertrophy
Better remodelingBetter remodeling
Reduces potential for electrical instabilityReduces potential for electrical instability
 How early is early ? Realistically 3 monthsHow early is early ? Realistically 3 months
and beyondand beyond

30. MODERATE SIZED VSD
 Operate Electively at 6 monthsOperate Electively at 6 months
 Risk of surgery MinimalRisk of surgery Minimal
 Chances of spontaneous closureChances of spontaneous closure
remoteremote
 Increased possibility of PVDIncreased possibility of PVD
 Earlier if:Earlier if:
- Poor control of CHF- Poor control of CHF
- Failure to thrive- Failure to thrive

31. Small VSD- approach
 Medical follow up.Medical follow up.
 Nutritional counselingNutritional counseling
 IE prophylaxisIE prophylaxis
 SurgerySurgery :(restrictive indications):(restrictive indications)
§§ Sub- arterial VSD with aortic incompetenceSub- arterial VSD with aortic incompetence
(Grade-III/IV; calcification /(Grade-III/IV; calcification /
fibrosis)fibrosis)
§§ IE or LV dilatationIE or LV dilatation

32. TETROLOGY OF FALLOT,
DORV,P.S
 Asymptomatic - Total Correction >6 MonthsAsymptomatic - Total Correction >6 Months
wt>7 Kgwt>7 Kg
 Symptomatic - SpellsSymptomatic - Spells
- Desaturation (Spo2 <70% )- Desaturation (Spo2 <70% )
- Failure To thrive- Failure To thrive
 3 Months - B.T Shunt3 Months - B.T Shunt
 3-6 Months - Good Anatomy - Total3-6 Months - Good Anatomy - Total
CorrectionCorrection

33. TOF : TOTAL CORRECTION

34. IS LOW WEIGHT FOR AGE
DETRIMENTAL
 ↑↑ release of inflammatory markersrelease of inflammatory markers
- IL-6, IL-2, TNF, ICAM- IL-6, IL-2, TNF, ICAM
 Poor immunologic profilePoor immunologic profile
 Poor ventilatory mechanics - CPAP PostPoor ventilatory mechanics - CPAP Post
extubation more a rule than exceptionextubation more a rule than exception
 Re-institution of post-op. feeding poorRe-institution of post-op. feeding poor
 ↑↑ Inodilator requirements (PGE- inhibitors)Inodilator requirements (PGE- inhibitors)
 ↑↑ ICU + Hospital stayICU + Hospital stay

35. OPERATIVE RISK FOR
VARIOUS CHD
 PDA, ASDPDA, ASD
VSD without PHTVSD without PHT
Unobstructed TAPVCUnobstructed TAPVC 0-2 %0-2 %
Coarctation RepairCoarctation Repair
 VSD/P SVSD/P S
Senning RepairSenning Repair
AV Canal RepairAV Canal Repair 3-5 %3-5 %
Conduit RepairConduit Repair

36. OPERATIVE RISK CONTD...
 Arterial switch operationArterial switch operation
Fontan OperationFontan Operation
Obstructed TAPVCObstructed TAPVC
TGA / VSD With hypertensionTGA / VSD With hypertension 10-15%10-15%
VSD With PulmonaryVSD With Pulmonary
hypertensionhypertension

37. RECENT ADVANCES
 In preoperative diagnosis &In preoperative diagnosis &
stabilization.stabilization.
 In Operative Management.In Operative Management.
 In Post operative careIn Post operative care

38. PREOPERATIVE ADVANCES
 Early accurate noninvasiveEarly accurate noninvasive
diagnosisdiagnosis
- 2 D Echocardiography with Color Doppler- 2 D Echocardiography with Color Doppler
- Fetal echocardiography- Fetal echocardiography
 StabilizationStabilization
- Prostaglandin ( PGE1 )- Prostaglandin ( PGE1 )
- Elective Ventilation- Elective Ventilation
- Ionotrope- Ionotrope
 Monitor TransportMonitor Transport

39. INTRAOPERATIVE
ADVANCES
 Improved Cardiopulmonary bypassImproved Cardiopulmonary bypass
CircuitsCircuits
 Improved understanding of DeepImproved understanding of Deep
Hypothermia and circulatory arrest.Hypothermia and circulatory arrest.
 Better myocardial protection.Better myocardial protection.

40. ADVANCES IN POST-ADVANCES IN POST-
OPERATIVE CAREOPERATIVE CARE
Sophisticated haemodynamicSophisticated haemodynamic
monitoringmonitoring
Pharmacological manipulationPharmacological manipulation
Better ventilatorsBetter ventilators
Mechanical support : ECMO /Mechanical support : ECMO /
LVAD/IABPLVAD/IABP
Nitric Oxide therapy.Nitric Oxide therapy.

41. Thank Youdrtoufiq19711@yahoo.com
Asia Pacific Congress of
Hypertension, 2014,
February
Cebu city, Phillipines
Seminar on
Management of
Hypertension,
Gulshan, Dhaka