Congenital Heart Disease ffor Post graduates

Dr. Md.Toufiqur Rahman MBBS, FCPS, MD, FACC, FESC, FRCPE, FSCAI,

FAPSC, FAPSIC, FAHA, FCCP, FRCPG

Associate Professor of CardiologyNational Institute of Cardiovascular Diseases(NICVD),

Sher-e-Bangla Nagar, Dhaka-1207

Consultant, Medinova, Malibagh branch

Honorary Consultant, Apollo Hospitals, Dhaka and

STS Life Care Centre, Dhanmondi drtoufiq19711@yahoo.com

CRT 2014Washington DC, USA

USA - 5-8 Per 10,000 live births

Also a major cause of Abortion and Still birth

About 1500-2000 (stipulated) children born with congenital Heart disease every year in Bangladesh.

Incidence

Distribution of CHD

CYANOTIC21%

OTHERS8%

ACYANOTIC71%

ACYANOTICCYANOTICOTHERS

Relative Frequency of CHD

0

5

10

15

20

25

30

35

ACYANOTIC

Relative Frequency of CHD

0

1

2

3

4

5

6

TOF SV/TA TAPVD

CYANOTIC

MULTIFACTORIAL

GENETIC - 90%

ENVIRONMENTAL - 10%

PREDISPOSING FACTORS

- Drugs / abortificient

! Infection

! Radiation

! Systemic disease (maternal)

Etiology

EMBRYOLOGY

ORIGIN

Mesodermal cells Ectodermal/ Neural crest cells

Inter atrial Septum Endocordial cushion

Interventricular septum Dysmorphogenesis

Spiral septum

Abnormal Apoptosis

CLASSIFICATION

ACYANOTIC

(a) 3 holes (L-R) shunt

Ventricular Septal Defect(VSD)

Atrial septal Defect(ASD)

Persistent Ductus Arteriosus(PDA)

(b) 3 tube Defect (Increase Pressure load)

Aortic Stenosis(AS)

Pulmonary Stenosis(PS)

Coarctation of Aorta(CoAo)

( c) Others

CYANOTIC

DECREASED PBF INCREASED PBF

TOF TGA

Tricuspid Atresia TAPVD

Pulmonary atresia TRUNCUS ARTERIOSUS(TA)

DORV SINGLE VENTRICLE(SV)

Ebstein’s Anomaly(E.A) HYPOPLASTIC LEFT HEART SYNDROME

OTHERS :-

Abnormal position of heart and heterotaxy syndrome

DIAGNOSIS OF CHD

S MAJOR COMPONENTS

History & Physical Examination

X-ray chest

E.C.G

Echo cardiography

Cardiac cathetarization

CLUES TO CHD Feeding difficulties Respiratory distress/Tachypnea Cyanosis/clubbing/Cynotic spell Apneic spell Persistent tachycardia Excessive sweating Frequent RTI Exercise intolerance/orthopnea Syncope/seizure/Headache/ vomiting Failure to thrive/Growth Retardation Developmental Delay

CHD usually identified during routine clinical exm. Or with a emergency e.g. CCF or with complains of exertional dyspnea, or with FTT.

PHYSICAL EXAMINATION

Appearance

Anaemia, Cyanosis, clabbing, oedema

Pulse/Heart rate - in quiet state, RR Apex beat, heart sound,

Murmur - innocent/organic(Thrill), Timing - systolic/diastolic,

location- where more intense ?

Hepatomegaly, bilateral basal creps

But always be sure before leveling a case of CHD

X-Ray Chest Lungs - Normal Acyanotic - VSD,PDA,ASD.AS & CoAo(if CCF)

Plethoric - Cyanotic - TGA, TAPVD,TA,HLHS, Oligaemic Acyanotic - PS

Cyanotic - TOF. Tricuspid Atresia, Pul. Atresia DORV WITH PS. EA.

Heart - Normal/ Cardiomegaly - if CCF (VSD,PDA,AS CoAo, TGA, TA. Characteristic cardiac shadow * Boot shape - TOF * EGG on side - TGA

* Box shape - E.A. * Square shape - Tricuspid Atresia

* Snowman - TAPVD (Supracardiac)Bony skeleton - Rib notching - CoAo.

ECG* QRS axis - Lead - I Lead - II

Normal Positive Positive

LAD Positive Negative

RAD Negative -----------

* Ventricular Hypertrophy

LVH S in V1 + R in V5/V6 > 40 mm ( > 1 yr)

RVH R>S in V1 > 30 mm (< 1 yr)

BVH R + S in V3 / V4 > 50 mm any age

VSD

An opening in the interventricular septum.

Type - Perimembranous ©, Out let, inlet, Muscular.

L-R Shunt - size, small/restrictive, Large / Non restrictive

Magnitude on QP : QS.

Symptomatic - at 4 - 6 wks

Clinical Presentation

* Small (<0.5 cm) - asymptomatic,develops normally. * Moderate to Large VSD with Large L-R shunt-

Symotomatic in early infancy, Dyspnea at rest or during feeding, sweating, C.C.F. failure to thrive frequent

RTI. * Large VSD with PHT-With above features, cyanosis usually absent but intermittent with crying / exertion

(if R-L shunt)

On Examination

Normal pulse, S1 masked by murmur, S2 Accentuated if PH, murmur - Pansystolic in LLSE, Apical mid-diastolic (if large shunt), ESM in ULSE

On Investigation

X-ray - normal/Cardiomegly, Pul. Plethra ECG - normal/LVH or RVH & LVHECHO - diagnostic, anatomical defect size location pressure gradient & assoc. problems.CATH - Diagnostic, determine PVR, QP : QS.

ASDASD

An opening in inter atrial septum.

Type - O.Secundum (c), O.Primum, S.Venosus.

C/F - Secundum type- Usually asymptomatic. With large L-R

shunt- recurent RTI, Easy fatigue, PHT in older.

Primum type- asymptomatic but with large L-R shunt-

Dyspnea, RTI & C.C.F may occur.

O/E - Normal pulse, S2 wide and fixed splitting. ESM in

ULSE, Apical mid-diastolic if large shunt.

On investigation- X-Ray chest normal/Cardiomegaly, Pul.Plethora.

ECG-rsR’in V1.ECHO-Diagnostic. Cath-QP:QS,

PVR.

ASD

PDA

* Persistent communication by ductus between PA & AO. Neonate - pre-term/term. Infant & children.

* Small-no symptom, large -Growth retardation, easy fatigue

exertional dyspnea, heart failure.

* Collapsing pulse, S1 S2 masked by continuos machinery murmur in ULSE to left clavicle.* X-Ray chest - Normal/cardiomegaly. Pul. Plethora. * ECG - Normal or LVH.* ECHO - Diagnostic.* CATH - Diagnostic & Therapeutic.* Complication - Infective endarteritis, PAHT.

* Type - Valvar, Infravalvar, Supravalvar.

* C/F - Mild to Modarate - No symtom. Severe stenosis- Exertional Dyspnea & C.C.F. Critical stenosis - Neonatal cyanosis with C.C.F.

* O/E - Normal pulse, Click if pliable valve, ESM in ULSE.* Chest X-Ray - Normal/cardiomegaly.* ECG - RVH with strain pattern.* ECHO - Diagnostic, determine peak systolic gradient across pulmonary valve & indicate the requirement of intervention.* CATH - Diagnostic and therapeutic for valvuloplasty.

PUlMONARY STENOSISPUlMONARY STENOSIS

AORTIC STENOSIS

* Type - Valvar, Supravalvar, Infravalvar, IHSS.

* C/F - Mild from-asyptomatic. Moderate- dizziness, easy fatigue, sweating, exertional dyspnea, syncope, angina. Severe- C.C.F. sudden death, FTT.

* O/E - Pulse reduced in volume, click if pliable valve, ESM in

URSE, O/I* X-Ray Chest - Normal/Cardiomegali.* ECG - Normal / LVH with strain pattern.* ECHO - Diagnostic. Thickend/Doomed AV. Peak systolic gradient classify the degree of stenosis.

AORTIC STENOSISNarrowing at LVOT

COARCTATION OF AORTA

Discrete narrowing of Aorta from Arch to Iliac bifurcation

Type - Infantile & Adult. Preductal/Postductal.

C/F - Severe from - Headache, dyspnea on exertion, hypertension, C.C.F. differential cyanosis & differential blood presser. Less severe from - well during infancy but hypertension in later life. Pulse -Decreased or delayed femorals. Click if bicuspid aortic valve. Mid-systolic murmur in

Apex and back. O/IX-Ray chest - Normal/ cardiomegaly. Figure of “3” sign. Notching of 4-8 ribs.ECG - Normal or LVH.ECHO - Diagnostic. Pressure gradient classify the degree of coarctation.CATH - Diagnostic and Therapeutic or Balloon angioplasty.

TGA

D-TGA

AO arises from RV and PA arises from LV with or without shunt (ASD, VSD, PDA)C/F - Without shunt: Early Neonatal severe cyanosis, Hypoxemia, acidosis, Heart failure, Sudden Death if not urgently treated. With shunt: Late neonatal Presentation of cyanosis, acidosis, Failure to thrive & CCFO/EHeart sound - S2 LoudMurmur - Absent or systolic (if shunt)O/IChest radiology - “Egg on side” Heart Shadow with narrow base, pulmonary plethora. E.C.G - RAD RVH, RVH+LVH, may be normal Echo - Aorta arises from RV & PA from LV also confirms other associate. Cath - Diagnostic & Therapeutic for atrial septostomy

TOF

1.RVOT obstruction, 2. Large VSD, 3. Overriding of AO to RV, 4. RV hypertrophy. Degree of cyanosis depends on Severity of RVOT obstruction and systemic versus pul. resistance. C/F - Neonatal cyanosis (if severe)

Infancy - Hypercyanotic spell, clubbing, Failure to thrive, convulsion, Hemiplegia Cerebral infarction (<2yrs.) or cerebral Abscess (>2yrs.)

O/E - Heart Sound - S2 single , Murmur- Ejection Systolic in left sternal edge. Less the intensity of murmur more severe the disease.

TOF

Chest radiology - Boot shaped heart with pulmonary concavity & apex tilted up, Oligaemic lungs.

E.C.G - RAD, RVH

Echo - Aorta Large with overriding to RV, RVOT- Narrow PA/PV- Variable in morphology, RV Hypertrophied, Large Nonrestrictive VSD with bi-directional shunt.

TAPVD All four pul. veins open to a common sac which opens to other than LA

Type - Supracardiac (C), Cardiac, infracardiac, Mixed.

C/F - With obstruction: (With/Without ASD) Early/neonatal

cyanosis, Acidosis, Hypoxemia, Heart failure (with small heart in radiology).

Without obstruction:Late cyanosis / Clubbing, RTI, failure to thrive.

TAPVD Heart sound - Normal , or S1-loud S2- single accentuated

Murmur - Absent or Ejection Systolic in pul. area radiating to lungs. Chest radiology - Wide supracardiac shadow as figure of ‘8’ (supra cardiac form), pul. plethora.

E.C.G - Normal or RVH, RAD, Tall-P

Echo - LA, LV smaller, Rt sided volume overload, Identify anomalous pul. venous connections.

Tricuspid Atresia

TV atretic, with ASD and VSD with variable RV and PA

C/F - Early central cyanosis & clubbing later on failure to

thrive, exertional dyspnea and C.C.F.

Heart sound - S1-Normal, S2-Single accentuated

Murmur - Soft systolic in LLSE

Tricuspid Atresia

Chest radiology - Small cardiac shadow Often square

shape, oligaemic lungs.

E.C.G - LAD , RAH, LVH

Echo - Thick atretic TV with ASD may be with

TGA/ without TGA, with variable size of

VSD or PDA.

TRUNCUS ATERIOUSUS.

Ao & PA arises from single great vessel overriding a VSD without right venticular infundibulum

Type - 1,2,3,4 according to position of origin of PA

C/F - At birth-minimal cyanosis, at 2-3 wks-collapsing pulse, breathlessness, RTI, failure to thrive & finally C.C.F.

Heart Sound - S2 loud & single

Murmur - Click, Systolic murmur

Chest radiology - Cardiomegaly, pul. plethora.

TRUNCUS ATERIOUSUS.

E.C.G - Normal in early case, RVH or (RVH +LVH) in older case.

Echo - Diagnostic. Characteristic overriding of a single great artery and determine the position and flow across PA from truncus and bi-directional shunt across VSD. Cath - Diagnostic

EBSTEIN’S ANOMALY

Downward displacement of TV into the RV with a large RA due to atrialized portion of RV which is reduced.

C/F - In Severe from - Neonatal cyanosis, C.C.F. Less servere -Late presentation with palpitation, dyspnea, chest deformity, failure to Thrive, Arrhythmia like RBBB/AF.

Heart Sound - Gallop rhythm or irregular Heart beat. (if CCF/AF)

Murmur - Systolic & Diastolic Murmur with/without click.

EBSTEIN’S ANOMALY Chest radiology - Cardiomegaly with Box-shaped heart with Oligaemic Lungs.

E.C.G - Tall-P increased RP interval, RBBB or AF

Echo - Downward displacement of TV with atrialzed RV and also any associated lesion.

Cath - Confirms Diagnosis.

PULMONARY ARTESIA Absence of PV with no or very little communication between RV and MPA usually with ASD, VSD or PDA.

Type I - With a small tricuspid and small RV TypeII - With tricuspid vale insufficiency and a large RV C/F - Without shunt -Central cyanosis at birth. Hypoxia acidosis & Right Heart failure. With shunt - Variable cyanosis, Clubbing, FTT, CCF

PULMONARY ARTESIA

Heart Sound - S2 Single

Murmur - Absent or may be systolic (If shunt)

Chest radioloy - Mild cardiomegaly, Oligaemic Lungs.

E.C.G - Normal or Tall-P (RAH)

Echo - PV not visualized or very thick with a little or no blood flow. Small RV, variable TV

Congenital Heart Disease (CHD)

Look forcentral cyanosis

Acyanotic Cyanotic

Auscultate Murmur

PSM in LLSE ESMin ULSE

ESMin URSE

ContinuousMachinery in ULSE & LICLA

BSM in Left Axilla & Radio-femoral delay

CoAoPDA

As RSCLA

PS/ASDVSD

S2 Split & wide S2 Absent

ASD PS Contd...

Cyanotic

Look for Age

Early (<6M)TGA, TAPVD (obs)Sevre TOF, Tr. Ar.Tricuspid Atresia (TA) with small shuntpulmonary atresia (PA)(without shunt)

Late (>6M)TOFTAPVD (without obs)TA (with large shuntEbstein’s Anomaly (EA)S.V, A-V canal defect

Chest Radiography

Plethoric Lungs

TGA, TAPVDTr. Ar.

TOF, PA,EA, TA

Oligaemic Lungs

CharactprostocCardiac shadow

Egg shape Figure of 8 Square shape Boot shape Box shape Large RAcardiomegaly

TGA TAPVD Tr, Ar TOF EAPA/TA

Thank Youdrtoufiq19711@yahoo.com

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