Congenital Heart Disease

Dr Vanessa HolmeConsultant Paediatrician

Aims

� Review Cardiac Anatomy� Fetal Circulation� Common defects� Presentations� Investigations� Management

Normal Heart Anatomy

Congenital Heart Defects

� 6-9/100 live births� 8 defects make up 80% CHD� Most Isolated, some assoc with Syndromes

� Acyanotic (Pink)� Isolated left to right shunt

� Cyanotic (Blue)� Right to left shunt

Pink or Blue?

Ventricular Septal Defect (VSD)Persistent Arterial Duct (PDA)Atrial Septal Defect (ASD)Pulmonary StenosisAortic StenosisCoarctation of the AortaTetralogy of FallotTransposition of Great Arteries

Case Presentation 1

� Baby boy� Full Term NVD

� Mum – Gravida 1� Previous child fit and well� Discharged after a normal 6hr check

Presents to A&E at 24hrs old

� Initial assessment:� A – crying� B – RR = 50/min, no recession

cyanosis to tongue and lipssaturations 78% in air

� C – cap refill 2 secs, HR = 140/minno murmur

� D – alert, PERL� E – no rash, slightly mottled

What do you do?

� Give oxygen� sats still 80% in 10 litres

� IV access� bloods and IV antibiotics

� CXR, ECG ? Echo� Treatment

� Prostin� Transfer to specialist centre

Transposition of Great Arteries

� Commonest cyanotic lesion to present at birth

� Aorta & Pulmonary Arteries arise from incorrect ventricles� Survival dependent on connection b/w

2 circuits� Cyanosis from first few hrs of life

� Gradually worsening over next few days

Fetal Circulation

Management

� Prostin� Balloon Septostomy� “Arterial Switch” Procedure� If Isolated defect then do very well

post-op

Case Presentation 2

� 3 month old baby girl

� Parents first child� Normal antenatal scans� Normal neonatal check

GP 6 week check

� Murmur 3/6

� Femoral pulses present� Child growing along 25th centile

� Bottle feeding well

� Referred to paediatrics

Attended A&E at 3/12

� Initial Assessment� A – crying at times� B – RR = 70/min, mild recession,

sats 93% in air� C – HR 200/min; cap refill < 2 secs

� D – Alert and interactive

Initial Management?

1. Oxygen via face mask2. Call for Help3. Take history from parents & examine

baby4. IV access

Further History

� For last month slower to feed� Occasionally getting SOB and sweaty

when feeding� Last 24 to 48 hrs

� Coughing� Breathing faster� Feeding half normal amounts

Examination

� Baby looks pale and sweaty� HS 2/6 systolic murmur

Active praecordium� Pulses all palpable� BS: bilateral crackles� Liver 3 cm below costal margin

Investigations

� CXR� ECG� Bloods� Echocardiogram?

Chest X-ray

ECG

Treatment

� IV frusemide� Discuss with specialist centre� Eventually will need surgery

� Dependent on growth� Degree of failure� Risk of pulmonary hypertension

Ventricular Septal Defect

� Commonest Congenital Heart Defect

� Very variable in size� Small often asymptomatic with loud

murmur� Large present in failure but often with

softer murmurs

VSD

Progress

� Small VSD often reduce in size leading to spontaneous closure

� Larger ones with failure will require surgical closure

� Failure of closure can lead to Eisenmengers Syndrome.

Eisenmengers Syndrome

Case Presentation 3

� 8 month old boy� Murmur noted at 6/52 check.� Clinically felt to be small VSD

� Thriving� Feeding well� No signs of heart failure

� Awaiting Cardiology Review

Presented to A&E

� Episodes of distress first thing in the morning

� Parents feel he goes blue & sweaty� Settles to sleep on cuddling� Wakes up and appears normal

Examination

� Weight 8.5kg� Slightly cyanosed� Early finger clubbing� RR = 40/min, sats 85% in air� HR = 140/min. normal pulses� Murmur high pitched systolic murmur� No thrill

Rest of examination

� No palpable liver� No resp distress

� No signs of cardiac failure

� Investigations?

CXR

ECG

Tetralogy of Fallot

� VSD� Pulmonary Stenosis� Right Ventricular Hypertrophy� Overriding Aorta

� Important are VSD & pulm stenosis

Clinical Features

� Cyanosis appears late in infancy� Systolic murmur along LLSE, pulm

area and radiates thro to back� Hypoxic Spells

� Marked pallor or cyanois with dyspnoea and distress

� Reduced exercise tolerance with age

Management

� Manage spells with beta blockers

� Definitive repair if possible� May need a temporary shunt between

aorta and pulm artery

Case Presentation 4

� 3 year old boy� Cough and cold for 3 days� Febrile above 39oC� Worsening resp distress

� GP has noticed a murmur and referred to COAU for assessment.

Assessment

� A – audible wheezetalking in short sentences

� B – RR = 45/min, sc recession,tracheal tug, sats 92% in air

� C – HR 130/min, cap refill < 2 secsBP 100/45HS soft systolic murmur

� D- Alert,

Management

� Given salbutamol inhaler via spacer� Improved RR =30/min� Better AE and reduced wheeze

� On discharge recommended see GP for review of murmur

Referred by GP to OPD

� Well grown child� Soft mid systolic murmur 2/6� Left sternal edge & apex� Murmur softer on standing� Louder on lying flat� Normal BP, pulses

Management

� Reassured� Innocent flow murmur� No need for further investigation� Explain murmur is not a medical

problem

Innocent Murmurs

� Can be heard in up to 50% of school age children

� 4 types of innocent murmur� Stills Murmur� Pulmonary Flow Murmur� Carotid Bruit� Venous Hum

Assessment

� Concerning Features� Unwell child� Diastolic

� Grade 3 or louder� Loudest over pulmonary area

� Heart sounds not separate� Any concerns about child

Other Common Defects

� Other Shunt lesions

Persistent Ductus Arteriosus

� Failure of Duct to Close� Commonest in Prematurity

� Premature babies can close after weeks/months

� Symptomatic may need treatment

PDA

� Clinical� May be asymptomatic� Continuous murmur at upper left

sternal border

� Larger• Collapsing pulses• Signs of failure

PDA

� Treatment� Drug – Indomethacin/Ibuprofen

� Surgical – coil or other devices

PDA Closure

Atrial Septal Defect

� Usually in region of foramen ovale� Small ASD can go undetected

� Clinically� Ejection systolic murmur� Second Heart sound split

� Soft diastolic murmur

ASD

� Investigations� CXR - increase in cardiac diameter &

pulm plethora� ECG – Partial RBBB, Rt axis deviation

� Treatment� Closure if significant shunt or when

older

ECG in ASD

AVSD

� 3% of all defects� Commonly seen in Trisomy 21

� Similar to large VSD� Symptomatic within first few months of

life

Obstructive Lesions

� Pulmonary Stenosis� Usually involves valve itself� Asymptomatic in Infancy & Childhood

� Ejection systolic murmur heard in pulm area and radiating to back

� Severe can lead to angina and heart failure with exercise intolerance

Aortic Stenosis

� Abnormal valve� Symptom free in Childhood� Ejection Systolic Murmur

� Right of sternum� Radiates to carotids

� Progressive� Dizziness and Syncope on exertion� Angina, Effort Intolerance, Sudden Death

Coarctation of Aorta

� Stricture of Aortic Arch close to duct� Often assoc with other defects� Varied presentation

� CCF in neonatal period� Absent femoral pulses� Radio-femoral delay

� Hypertension in upper limbs

CXR

Treatment

� Newborn� Prostin� Treat Heart Failure

� Early Repair

� Older children� If hypertensive or in second decade

Case Presentation 5

� 4 year old girl� Previously fit and well� Occasionally looks pale and sits down

for few mins

� Otherwise well and active

Brought to A&E

� Looked pale and sweaty� c/o heart feels funny� Mum noticed pulse in neck� Examination

� Alert, RR = 25/min, no recession� HR = 240/min, BP = 95/50

� Cap refill < 2 secs

ECG

Supraventricular Tachycardia

� Commonest paediatric arrythmia� Sudden onset of Tachycardia

� Infants look pale poor feeding

� Older children will c/o funny sensation

� Treatment?� Valsalva manouvre etc

� Adenosine

Delta wave

Long QT syndrome

� Rare cause of collapse and sudden death� Often familial

� Any collapse should calculate QTc� Normal < 0.44

� If concerning features then exercise ECG/cardiology referral

Conclusions

� Congenital Heart Disease significant morbidity and mortality

� Variable presentation� Newborn period often need urgent

treatment� Other defects develop over time

Conclusions

� Heart murmurs often benign� Important to know when to investigate

� Commonest arrythmia is SVT

Any Questions