Congenital Heart Disease

Question 1

A 2-month old presents with an audible murmur. She gets breathless and sweaty with feeds and takes a long time feeding. There is a history of poor weight gain. discuss your management including long-term prognosis and possible complications.

A 1-month old baby brought in by mum complaining of poor feeding, sweating during breastfeeds and breathless. On examination, the baby has a loud systolic murmur, is tachycardia and has an enlarged liver. How do you assess and manage?

Answer 1

Impression
Most likely congenital heart disease given the constellation clinical symptoms namely poor feeding, sweaty and breathless. FTT is a common consequence.

Congenital heart disease can be classified as either acyanotic or cyanotic;

Acyanotic (L to R shunt)
- PDA - persistent ductus arteriosus
- VSD (can become R to L shunt in Eisenmenger’s syndrome)
- ASD
- Combined atrioventricular septal defect
Other obstructive:
- valvular
- aortic coarctation

Cyanotic
- Tetralogy of fallot
- Tricuspid atresia
- Transposition of great vessels
- Ebstein’s anomaly (increased risk lithium use in pregnancy)
others;
- Truncus arteriosus
- Total anomalous pulmonary venous return

Goals
- Ensure HD stability, then thorough Hx/Ex/Ix
- Echocardiogram to diagnose defect
- Referral to paeds cardiothoracics for definitive management, retrieval if HD unstable/life-threatening

Question 2

CHD - History

Answer 2

History
- sx: sweaty, irritable, breathless, poor feeding, poor growth/FTT, recurrent bronchopulmonary infections, peripheral oedema, skin colour (cyanotic vs acyanotic), breathless/sweaty whilst feeding.
- RISKS: teratogen during birth: valproate, lithium, infections, alcohol, warfarin. genetic abnormalities/ family history, diabetes during pregnancy
- Antenatal history, details of birth, complications
- Paeds hx: growth, development, vaccinations

Question 3

CHD - Examination

Answer 3

Examination
- General appearance + vitals (blue skin)
- Cardio: describe murmur and location, palpate for thrills/heaves
o VSD: holosystolic, @ L lower sternal edge
o ASD: systolic ejection mumur, @ 2 ICS parasternally
o Innocent heart murmur: Still’s = positional,
o PDA - continuous murmur
o Coarctation: radio-radial delay or radio-femoral delay
- Respiratory examination: pulmonary oedema
- Clubbing - seen in cyanotic CHD
- growth parameters and plotting

Question 4

CHD - Investigations

Answer 4

Investigations
- Bedside: ECG, VBG, SP02
- Bloods: FBC, UEC, LFT, ABG,
- Imaging: CXR (looking for pulmonary complications), Echocardiogram - visualise and confirm heart defect

Question 5

CHD - Management

Answer 5

Management
Depends on patient presentation and how large the defect is, and what the anomaly is.

Acute
- diuretics if heart failure
- oxygen therapy if low
- ACEi for heart failure
- beta blockers for heart failure

If cyanotic and HD unstable;
- paeds cardio for management
- NETS retrieval
- PICU/NICU involvement early
- discussion with Paeds cardiothoracics for definitive cardiac surgery
o FTT, large VSD, CCF, pulmonary hypertension - are indications for surgery

Supportive
- SP02 supplemental as required