CONGENITAL HEART DISEASE

S4
Dr Eze A.C.
 Introduction
• Congenital=Born with
• CHD are structural heart disease that develops
in utero and the child is born with It.
• It can affect the chambers, partitions(septa),
valves and the great vessels
• The commonest CHD is Bicuspid Aortic valve.
• Most of them present at birth but some won’t
manifest until adulthood even though it had
been there.
 Introduction
• Some are so mild that they require little to no
intervention while others are complex and life
threatening
• Most will require life long follow-up even after
treatment as complications or some sort of
care will be required.
• Complication could be from the defect itself or
the treament.
 INTRODUCTION
• Early detection of CHD is of primary
importance for improvements in the quality of
life and reducing morbidity and mortality of
children
• With advances in both corrective and
palliative surgery, the number of children with
CHD surviving to adulthood has increased
dramatically.
 Epidemiology
• Among the commonest congenital birth defect

• About 1in 100 birth in UK

 Risk factors
• Genetic
• German measles(aka Rubella) in preg
• Smoking in preg
• Alcohol in preg
• Diabetes Mellitus
• Medications eg lithium, statins in preg
• The etiology in about 80% of CHD is
multifactorial and arises through various
combinations of genetic and environmental
contributors
• In about 20% of cases it is attributed to
chromosomal anomalies, Mendelian
syndromes, nonsyndromal single gene
disorders or teratogens.
• All this suggests that there is a lot of room for
the interplay of multifactorial etiologies, such
as the interactions between multiple genes,
environmental factors, and spontaneous
mutations
 Classification
Cyanotic----5Ts –”the T disease”—R-L shunt
• Truncus Arteriosus
• Transposition of great vessels
• Tricuspid valve anomaly
– Tricuspid Atresia
– Tricuspid stenosis
– Tricuspid valve malalignment(Ebstein’s Anomaly)
• Tetralogy of Fallot
• Total Anomolus Pulmonary Venous Connection
• Others
– Pul atresia
– Hypoplastic left heart
 Classification
Acyanotic --As ---L-R shunt
• ASD
• VSD
• PDA
• coA
• Others
– Aortic stenosis
– Bicuspid aortic valve
– AVCD (aka endocardial cushion defect
 Classification
Cyanotic can be further classified as
• Cyanotic with increased pul bld flow
– TGA
– TAPVC
– Truncus Arteriosus
– Hypoplastic left heart
– DORV
• Cyanotic with decreased pul blood flow
– TOF
– Tricuspid Atresia/Stenosis
– PUL atresia
 Classification
CHD can also be clasified into:
• Isolated CHD
• Syndromic CHD
– CHD occuring association with a syndrome eg
• Down syndrome
• DiGeorge syndrome
• Noonan syndrome
• Edward syndrome
• Turner syndrome
Pathophysiology
 PATHOPHYSIOLOGY
Depends on the type of CHD
• Abnormal blood flow
• Poor oxygenation and poor O2 saturation
• Back pressure and subsequent hypertrophy
• Volume overload and heart failure
• Pulmonary congestion
• Poor systemic blood delivery
 Symptoms

May present at birth or later in life

• Easy fatiguability
• Difficulty in breathing
• Fast breathing
• Fainting attacks
• Frequent Squatting
• Blue discoloration around lip/fingertips
• Leg swelling
• Difficulty in feeding/ sucking breast
• Recurrent RTI infection
• Failure to thrieve
 SYMPTOMS
OTHERS
• FEVER
• SEIZURE
• HAEMOPTYSIS
• PREG and Delivary
• Nutrition/feeding
• Developmental hx
• Family hx
 Signs
• General exam including facies
• Central cyanosis
• Oedema
• Signs of resp distress
• Tachycardia
• Hyperactive praecordium
• Displaced apex beat
• Gallop rhythm
• Murmurs
• SPO2—may be low esp in cyanotic
 Signs
• Abdominal swelling/hepatomegally
– 3Ts of heart failure
• Tachycardia
• Tachypnoea
• Tender hepertomegally
• Low weight for age.
• May be asymptomatic
• Look out for VACTERL association
 Signs
Others
• Finger clubbing
• Plethora
• Basal crepitations
 Investigations

• Chest X- ray
• ECHOCARDIOGRAPHY
• ECG
• Cardiac catheteriasation
• Cardiac CT –Scan
• MRI
• Cardiac Catheteriastion
• Arterial blood gases
• FBC
• OTHERS-urinalysis SUCER
 CXR
Know the normal
• Pul vasculature
– Congested
– Oligaemia
• Aorta
– Position
– size
– Shape eg fig of 3 sign in coA
• Heart
– Shape
– Size
 CXR
NORMAL ABNORMAL
CXR/ECHOCARDIOGRAPHY
• Boot shaped-TOF
• Figure of 8—TAPVC(TAPVD)
• Egg-on-sting ---d-TGA
• ECG-dominant R-wave in V1 and dominant S-
wave in V6----TOF
• Arrhythmias
 ECHO
• Diagnosis
• Assess if suitable for surg
• For postop follow
• Examples
– VSD or ASD, no of opening and where
– Extent of overide (TOF ) or transposition in TGA
 Cardiac Catheterisation
Indicated only in some patients
• Anatomy eg RVOT and Main PA in TOF
• Pressure
• Site and size of eg VSD
• RV and LV function
• Valve competence
 Treatment
Depends on the type and
• Medications
• Pacemaker/ICD
• Catheter based procedures
• Surgery
– Palliative
– Corrective
• One stage or multistage
• Heart transplant
 MEDICATION
• Maintaining adequate hemoglobin levels in all
CHD patients is useful.
• Cyanotic patients may have a low mean
corpuscular hemoglobin concentration despite
polycythemia.
• Iron deficiency should therefore be looked for
and corrected
 MEDICATION
• Control of heart failure
• Reduction in pul resistance/Improvement in
pul out flow

• CARDIOLOGISTS play great role

 MEDICATIONS
• Vasodilators
• Diuretics
• B-blockers
• O2
• Morphine-calm patient with hypercyanotic
spell
• PGE1-to maintain patency of PDA in TOF
 Hypercyanotic spell
• Knee-chest position
• Calm patient
• O2
• Morphine
 PALLIATIVE
• Blalock-Taussig
– Classical –obsolete
• Sublavian art sacrificed
– Modified
• PTFE(Gortex) from suclavian art to branched PA
• Central
– AA to Main PA
• Waterson
– AA to Right PA
• Pott
– DA to left PA
• Pul art banding –to reduce blood flow to the lung
Modified B-T shunt
 Other palliatives
• Rashkind Atrial septostomy for TGA
• Glenn procedure
 DEFINITVE
• Catheter based Technique
– Amplatzer occluder for VSD, PDA
– Coil occlusion for PDA
Amplatzer occlusion Device
 Definitive
• Ligation of PDA
• Closure of ASD or VSD
• Total correction of TOF
• Ross Procedure
• Others eg Heart transplant
 Complications
• Reccurrent chest infection
• CCF which can attimes be intractable
• Embolism /stroke
• Arrythmias
• Raised Pul vascular resistance
• Eisenmenger syndrome
 From surgery
• Residual shunt or PDA
• Recannalisation of PDA
• Damage to conducting sys –Arrythmia
• Damage to valves eg Aortic or Tricupid leading to
regurgitation
• Damage to phrenic nerve
• Damage to recurrent larengeal nerve in PDA repair
• Embolisation of coil
• Inadvertent occlusion of aorta with Amplatzer
 Prevention
• Modifiable risk factors should be avoided in
child beaing age.
• Good control of DM in pregnancy
• Immunise against rubella if yet to.
 Prognosis
• Depends on type and complexity

• Timely and proper treatment affects it too

• Advances in diagnosis and treatment

modalities has improved the outlook
Thank you
QUESTIONS
 Question
All these are cyanotic heart disease except
• Pulmonary atresia
• TOF
• TGA
• TAPVD
• AVCD
 QUESTION
The commonest CHD IS
• VSD
• ASD
• TOF
• Bicuspid Aortic Valve
• Coarctation of the Aorta
 Question
Boot-shaped heart on CXR is usally seen in
• ASD
• TGA
• VSD
• TOF
• Single ventricle
 Question
Concerning CHD
• All CHD will eventually require surgery
• CXR is usually enough to diagnose the particular
lesion
• Echocardiography is usually enough to diagnose the
particular lesion
• Cardiac catheteriasation is usually indicated in most
of the lesion
• FULL BLOOD COUNT is a useless investigation in CHD