Paediatric Cardiology & Congenital Heart Disease Nursing

GCC Nursing Exam Preparation Guide DHAMOHSCFHSQCHP

Congenital Heart Disease (CHD) affects 8–9 per 1,000 live births globally. Rates are higher in GCC populations due to consanguineous marriages. CHD is the most common congenital anomaly worldwide.

Acyanotic CHD (Left-to-Right Shunt)

Oxygenated blood recirculates through the lungs — child appears pink. Pulmonary overcirculation leads to failure to thrive and recurrent chest infections.

Common Lesions

  • VSD (Ventricular Septal Defect) — Commonest CHD (~30%). Hole between ventricles.
  • ASD (Atrial Septal Defect) — Hole between atria; often asymptomatic in childhood.
  • PDA (Patent Ductus Arteriosus) — Duct between aorta and pulmonary artery remains open.
  • AVSD (Atrioventricular Septal Defect) — Common in Down syndrome; combined ASD+VSD.
Key: Murmur, pulmonary plethora on CXR, bounding pulses (PDA), wide fixed split S2 (ASD).

Cyanotic CHD (Right-to-Left Shunt)

Deoxygenated blood enters systemic circulation — child appears blue/dusky, especially peripherally or centrally.

The 5 Ts Mnemonic

  • Tetralogy of Fallot (ToF) — Commonest cyanotic CHD after neonatal period
  • Transposition of Great Arteries (TGA) — Commonest cyanotic CHD presenting on Day 1
  • Truncus Arteriosus — Single vessel from heart
  • Total Anomalous Pulmonary Venous Drainage (TAPVD) — Pulmonary veins drain to wrong side
  • Tricuspid Atresia — Absent tricuspid valve
Key: Central cyanosis, clubbing (chronic), polycythaemia, SpO2 <95% on room air.

Eisenmenger Syndrome

Long-standing large left-to-right shunt (e.g., large VSD) causes progressive pulmonary hypertension. When pulmonary vascular resistance exceeds systemic resistance, the shunt reverses to right-to-left, causing cyanosis.

Critical Point: Once Eisenmenger syndrome develops, surgical repair of the original defect is contraindicated — the defect now acts as a "pop-off valve." Mortality risk from closure is very high.
  • Signs: Central cyanosis, clubbing, polycythaemia, haemoptysis, syncope
  • Management: Pulmonary vasodilators (bosentan, sildenafil), heart-lung transplant (definitive)
  • Pregnancy is contraindicated — 50% maternal mortality

Prenatal Diagnosis

Fetal Echocardiography

  • Offered at 18–22 weeks gestation (anomaly scan detects ~50% of major CHD)
  • Dedicated fetal echo at 20 weeks for high-risk pregnancies
  • High-risk: family history CHD, maternal diabetes, rubella, teratogens, chromosomal anomaly, consanguinity
  • Views: 4-chamber view, outflow tracts, 3-vessel view
GCC context: consanguinity & higher maternal age increase fetal cardiac anomaly rates. Fetal echo services expanding in Saudi Arabia, Qatar, UAE.

Newborn Pulse Oximetry Screening

Purpose & Protocol

  • Detects critical duct-dependent lesions before collapse
  • Performed at 24–48 hours of life (before PDA closes)
  • Measure SpO2 in right hand (pre-ductal) and either foot (post-ductal)
  • PASS: Both ≥95% AND difference <3%
  • REFER: Any reading <95%, or difference ≥3% (repeat in 1h; if repeated fail → echo)
Duct-dependent lesions (HLHS, pulmonary atresia, coarctation, TGA) may present with cardiovascular collapse when PDA closes at 24–72h.

GCC-Specific Epidemiology

FactorImpact on CHDNursing Implication
Consanguineous marriage (first-cousin ~25–50% in some GCC regions)Increases autosomal recessive cardiac conditions; higher prevalence of CHD overallGenetic counselling referral; family screening
High birth rateLarge absolute numbers of CHD cases per yearAdequate PICU & paediatric cardiac surgical capacity needed
Congenital Rubella SyndromePDA, pulmonary stenosis, ASD — now rare with vaccinationEnsure maternal vaccination records; MMR in vaccine schedule
Maternal GDM (high in GCC)Hypertrophic cardiomyopathy, TGA, VSDFetal echo in diabetic mothers; glucose control in pregnancy

INTERACTIVE TOOL: Paediatric Tet Spell Emergency Guide

Select all features present in the patient then click the button to assess for hypercyanotic (Tet) spell.

Tetralogy of Fallot (ToF)

4 Components (PROVE mnemonic)

  • Pulmonary stenosis (infundibular/valvar — RV outflow obstruction)
  • Right ventricular hypertrophy
  • Overriding aorta (straddles VSD)
  • Ventricular septal defect (large, non-restrictive)

Clinical Features

  • Cyanosis worsens with crying/feeding/exertion
  • Squatting (older children) — increases SVR, reduces R-to-L shunt
  • Ejection systolic murmur (pulmonary stenosis) — not VSD murmur
  • ECG: RVH, right axis deviation
  • CXR: "Boot-shaped heart" (coeur en sabot), oligaemic lung fields

Surgical Management

  • Palliative: Blalock-Taussig-Thomas (BTT) shunt — subclavian artery to pulmonary artery
  • Definitive: Total surgical repair (VSD patch + RV outflow reconstruction) — usually 3–6 months

Transposition of Great Arteries (TGA)

Pathophysiology

  • Aorta arises from right ventricle, pulmonary artery from left ventricle
  • Two parallel, non-mixing circuits → profound cyanosis from birth
  • Survival depends on mixing (ASD, VSD, PDA)

Emergency Management

1
Prostaglandin E1 (PGE1) infusion — Keeps PDA open; start immediately. Monitor for apnoea (have intubation ready).
2
Rashkind balloon atrial septostomy — creates/enlarges ASD for mixing; done in cardiac catheterisation lab.
3
Arterial Switch Operation (ASO) — definitive repair; performed Day 1–2 of life while LV can handle systemic pressures.
Critical: If ASO delayed >2–3 weeks, the LV "de-conditions" and cannot support systemic circulation post-switch without prior pulmonary artery banding.

Hypoplastic Left Heart Syndrome (HLHS)

Underdevelopment of the entire left side of the heart (mitral valve, left ventricle, aortic valve, ascending aorta). The right ventricle supports both pulmonary and systemic circulations via PDA.

Staged Palliation (Norwood Pathway)

StageOperationAgeGoal
Stage 1Norwood ProcedureDay 1–2 of lifeRV → aorta connection; pulmonary blood flow via BTT shunt or Sano shunt
Stage 2Glenn Procedure (hemi-Fontan)4–6 monthsSuperior vena cava connected to pulmonary artery; removes volume load on RV
Stage 3Fontan Completion2–4 yearsIVC also connected to pulmonary artery; passive pulmonary flow without pumping chamber
Nursing: Interstage (Norwood→Glenn) has highest mortality. Monitor SpO2, weight gain, and watch for sudden desaturation. Home monitoring programmes reduce interstage mortality.

Normal SpO2 Ranges by CHD Type

Condition / AnatomyExpected SpO2Interpretation
Normal biventricular circulation97–100%Normal
ToF (pre-repair)75–85%Normal for lesion
Post-Fontan circulation75–85%Expected — single ventricle
Glenn circulation80–85%Expected
TGA pre-mixing intervention<70%Emergency — needs PGE1/septostomy
Acyanotic CHD (VSD/ASD/PDA)≥95%Should be normal
A Fontan patient with SpO2 of 80% may be completely stable. Always interpret SpO2 in the context of the known cardiac anatomy.
PDA Management Algorithm — By Age & Weight ▶
Patient GroupFirst-lineSecond-lineNotes
Premature <28 wksIndomethacin IV (COX inhibitor) 0.1–0.25 mg/kg q12–24h × 3 dosesIbuprofen IV; paracetamol IV 15mg/kg q6h × 3–7 daysCheck renal function, platelets; avoid if NEC, renal failure, bleeding
Premature 28–32 wksIbuprofen IV or paracetamol IV (fewer renal/pulmonary side effects than indomethacin)Surgical ligation if medical failsFluid restriction + diuretics adjuncts
Term neonate/infantWatchful waiting if small; prostaglandin infusion if duct-dependent lesionTranscatheter device closure (Amplatzer Duct Occluder) when ≥6 months & ≥5 kgSurgical ligation if symptomatic and too small for catheter
Child >1 yearTranscatheter device closure—Coil closure for small PDA; ADO device for moderate/large
Signs of significant PDA: bounding pulses, wide pulse pressure, continuous "machinery" murmur (under left clavicle), pulmonary oedema, poor weight gain.

Ventricular Septal Defect (VSD)

Types

  • Perimembranous — Commonest (80%)
  • Muscular — May close spontaneously
  • Inlet/AVSD type — Associated with Down syndrome
  • Outlet/Supracristal — Risk of aortic regurgitation

Management by Size

  • Small VSD: Watchful waiting; most close spontaneously by age 2–4 years. Regular echo follow-up.
  • Moderate VSD: Medical management (diuretics, ACE inhibitors); repair if failure to thrive.
  • Large VSD: Symptoms — failure to thrive, recurrent LRTI, tachypnoea, diaphoresis with feeds. Surgical or transcatheter closure.
Failure to thrive in an infant with VSD: high calorie feeds (24–30 kcal/oz), NG tube if needed, surgical planning.

Atrial Septal Defect (ASD)

Types

  • Ostium secundum — Commonest (70%); centre of atrial septum
  • Ostium primum — Lower septum; associated with AVSD
  • Sinus venosus — Near SVC/IVC; associated with partial APVD
  • Coronary sinus ASD — Rare

Management

  • Small ASDs may close spontaneously in infancy
  • Transcatheter closure (Amplatzer Septal Occluder) — preferred if ≥4mm rim (adequate tissue around defect)
  • Surgical closure — for large defects, sinus venosus type, or inadequate rim for device
  • Fixed, widely split S2 — classic auscultatory finding

Coarctation of the Aorta (CoA)

Clinical Features

  • Narrowing of descending aorta, usually at ductus arteriosus junction
  • Weak/absent femoral pulses — cardinal sign
  • Upper limb hypertension with lower BP in legs (gradient >20 mmHg)
  • Radio-femoral delay
  • Associated with Turner syndrome (45,X)
  • Bicuspid aortic valve in ~50%

Management

  • Neonatal critical CoA: PGE1 → surgical resection (end-to-end anastomosis)
  • Older child/adolescent: Balloon angioplasty with stenting
  • Long-term: Hypertension monitoring, surveillance for re-coarctation

Pulmonary Stenosis (PS)

Classification

  • Mild: Gradient <40 mmHg — watchful waiting
  • Moderate: Gradient 40–70 mmHg — elective treatment
  • Severe: Gradient >70 mmHg — urgent treatment
  • Critical neonatal PS — duct-dependent → PGE1 emergency

Treatment

  • Balloon pulmonary valvuloplasty — first-line for valvar PS; excellent results
  • Surgical valvotomy — for subvalvar/supravalvar or dysplastic valve (Noonan syndrome)
  • Post-procedure: Watch for pulmonary regurgitation long-term
Noonan syndrome: short stature, webbed neck, pulmonary stenosis (commonest cardiac lesion), hypertelorism — similar phenotype to Turner but normal karyotype.

PICU Post-Cardiac Surgery Monitoring

Invasive Monitoring Lines

  • Arterial line — Continuous BP, frequent ABGs
  • Central venous line (CVP) — Assess filling pressures, inotrope infusions
  • Left atrial (LA) line — Direct LA pressure; guides fluid management after complex repairs
  • Temporary epicardial pacemaker wires — Atrial & ventricular; exit through chest wall
  • Urinary catheter — Hourly urine output (target ≥0.5–1 ml/kg/h)

Key Nursing Assessments

  • Continuous ECG monitoring (arrhythmia detection)
  • Near-infrared spectroscopy (NIRS) — cerebral & somatic oxygenation
  • Chest drain output: character, volume, clotting
  • Skin colour, capillary refill time (<2 sec target)
  • Temperature (target normothermia after rewarming)
  • Sedation/analgesia assessment (FLACC/COMFORT scale)

Low Cardiac Output Syndrome (LCOS)

Peak LCOS risk: 6–18 hours post-bypass (myocardial oedema, ischaemia-reperfusion injury)

Diagnostic Features (4 signs)

  • Tachycardia
  • Poor/absent peripheral pulses
  • Prolonged CRT (>3 sec)
  • Metabolic acidosis (raised lactate)
  • Oliguria (<0.5 ml/kg/h)
  • Cold mottled extremities
  • Hypotension (late sign)
  • Decreased conscious level

Inotropic Support

DrugMechanismDoseUse
MilrinonePDE-III inhibitor — inodilator0.25–0.75 mcg/kg/minFirst-line LCOS; reduces afterload and improves contractility
DopamineDA, β1, α1 (dose-dependent)3–10 mcg/kg/minModerate LCOS; low doses renal vasodilation
Adrenaline (Epinephrine)α1, β1, β2 agonist0.05–0.5 mcg/kg/minSevere LCOS/cardiac arrest
Noradrenalineα1 > β10.05–0.5 mcg/kg/minVasoplegic syndrome post-bypass

Junctional Ectopic Tachycardia (JET)

Commonest post-cardiac surgery arrhythmia in children. AV node automaticity — rate typically 170–230 bpm. Particularly dangerous after VSD, ToF, AVSD repair.

ECG Features

  • Narrow complex tachycardia (usually)
  • AV dissociation — atrial rate < ventricular rate
  • Irregular cannon waves in JVP

Management Protocol

1
Cooling — Target temperature 34–35°C (reduces JET rate by slowing automaticity)
2
Amiodarone IV — 5 mg/kg over 20–60 min, then infusion 10–15 mg/kg/day
3
Atrial pacing — Use epicardial wires to pace atria faster than JET rate; restore AV synchrony
4
Electrolyte optimisation — Mg2+ and K+ repletion; correct acidosis
5
Minimise catecholamines — Reduce adrenaline/dopamine doses if haemodynamically possible
Post-Cardiac Surgery Chest Drain Monitoring Guide ▶
FindingSignificanceAction
Bright red blood >3–5 ml/kg/h for 2+ hoursSurgical bleedingUrgent surgical review; blood products; re-exploration
Sudden cessation of output + haemodynamic deteriorationCardiac tamponade (clot in pericardium)Emergency: milk drain; call surgeon; prepare for re-sternotomy
Milky/chylous fluid (especially left thoracic drain)Chylothorax (thoracic duct injury)Low-fat/MCT formula; consider octreotide; TPN if severe
Air bubbles in drainAir leak from lungEnsure underwater seal intact; monitor respiratory status
Serosanguineous (normal post-op)Expected drainageMonitor volume; ensure swinging/bubbling appropriate
Chylothorax management: Milky drain fluid confirms diagnosis (triglycerides >1.1 mmol/L). Start fat-free diet or MCT formula. Octreotide 1–10 mcg/kg/hr reduces chyle flow. TPN if drainage persists. Surgical ligation of thoracic duct if no improvement in 4–6 weeks.

Chylothorax

  • Lymphatic fluid accumulates in pleural space after thoracic duct injury
  • Common after: Norwood, Glenn, Fontan, CoA repair, PA band
  • Fat in chyle absorbs into lymphatics — hence fat-free diet works
  • MCT (medium-chain triglyceride) formula — MCT absorbed directly into portal circulation, bypassing thoracic duct
  • TPN to rest gut if refractory
  • Monitor electrolytes (hyponatraemia, lymphopenia)

Phrenic Nerve Palsy & Chest Closure

Phrenic Nerve Palsy

  • Hemi-diaphragm paralysis from nerve injury during cardiac surgery
  • Right phrenic more commonly injured with right thoracotomy/SVC procedures
  • Presents: respiratory distress, elevated hemi-diaphragm on CXR, difficulty weaning from ventilator
  • Treatment: Diaphragm plication (surgical tightening)

Delayed Sternal Closure

  • Chest left open post-CPB if: myocardial oedema, haemodynamic instability, bleeding
  • Covered with sterile patch/skin; closed in PICU when stable (24–72h)
  • Strict sterile precautions; immobility of upper limbs; high infection risk

Heart Failure in Children — Causes by Age

Age GroupCommon CausesKey Features
Neonates (0–28 days)Duct-dependent CHD (HLHS, critical CoA, pulmonary atresia); metabolic cardiomyopathyCollapse when PDA closes; acidosis; grey/mottled; SpO2 differential
Infants (1–12 months)Large VSD, AVSD, cardiomyopathy (dilated, HCM), anomalous coronary (ALCAPA)Failure to thrive, diaphoresis with feeding, tachypnoea, hepatomegaly
Children (1–5 yrs)Dilated cardiomyopathy (DCM), myocarditis (viral), unrepaired CHDExercise intolerance, periorbital oedema, hepatomegaly
Older children (>5 yrs)Myocarditis, Kawasaki disease (coronary aneurysm), rheumatic heart disease (GCC), DCMClassic adult HF symptoms; NYHA-equivalent

Ross Heart Failure Classification (Paediatric)

Modified NYHA scale adapted for infants and children

  • Class I: No symptoms; no limitation of ordinary activity
  • Class II: Mild symptoms with ordinary activity; comfortable at rest; mild feeding difficulties in infants
  • Class III: Marked limitation; symptoms with minimal exertion; infants: prolonged feeding time, diaphoresis, growth failure
  • Class IV: Symptoms at rest; bedridden; infants: tachypnoea/diaphoresis at rest

Heart Failure Pharmacotherapy

First-Line

  • Furosemide (loop diuretic) — 1–2 mg/kg BD–TDS; monitor K+
  • Captopril / Enalapril (ACE inhibitor) — reduces afterload; monitor BP, renal function
  • Spironolactone — potassium-sparing diuretic; add to furosemide

Advanced Therapy

  • Carvedilol (non-selective β-blocker) — improves survival in chronic HF; start low, titrate
  • Digoxin — improves symptoms in DCM; monitor levels, ECG
  • Cardiac resynchronisation therapy (CRT) — biventricular pacing in LBBB/dyssynchrony
  • VAD / Heart transplantation — end-stage; available in KFSH, HMC Doha
Kawasaki Disease — Diagnostic Criteria & IVIG Protocol ▶
Kawasaki Disease is the leading cause of acquired heart disease in children in developed countries & increasingly recognised in GCC Arab children. Aetiology unknown — likely infectious trigger in genetically susceptible individuals.

Classic Diagnostic Criteria

Fever ≥5 days PLUS ≥4 of the following 5 features:

#FeatureDetails
1Conjunctival injectionBilateral, non-exudative bulbar conjunctival injection (no discharge)
2Oral changesErythema/cracking lips, strawberry tongue, erythema of oral/pharyngeal mucosa
3RashPolymorphous exanthem (not vesicular); often in nappy area in infants
4Extremity changesAcute: erythema/oedema of palms/soles; Sub-acute: periungual desquamation (10–14 days)
5Cervical lymphadenopathyNode ≥1.5 cm, usually unilateral anterior cervical
Incomplete Kawasaki: Fever ≥5 days + 2–3 features + elevated CRP/ESR + echo changes → treat as Kawasaki. Common in infants <12 months — highest risk of coronary aneurysm.

IVIG Protocol

  • IVIG 2 g/kg as single infusion over 10–12 hours — most important treatment; reduces coronary aneurysm risk from 25% to <5%
  • Give within first 10 days of fever (ideally day 5–7)
  • Aspirin — high dose (30–50 mg/kg/day in 4 doses) during acute phase; low dose (3–5 mg/kg/day) after fever resolves for 6–8 weeks (or until echo normal)
  • Repeat IVIG if fever persists >36h after first dose (IVIG-resistant — 10–20% cases)
  • Infliximab — second-line for IVIG-resistant Kawasaki

Echo Monitoring Schedule

  • At diagnosis (baseline)
  • At 2 weeks (peak aneurysm formation)
  • At 6–8 weeks (resolution assessment)
  • Annual if persistent aneurysms
Live vaccines (MMR, varicella) must be deferred for 11 months after IVIG (IVIG may interfere with vaccine response).

Myocarditis in Children

  • Inflammatory myocarditis — viral (enterovirus/Coxsackie B commonest, adenovirus, SARS-CoV-2)
  • Presents: acute-onset HF, chest pain, arrhythmia, shock in previously well child
  • Elevated troponin, echo shows global LV dysfunction
  • Management: supportive; milrinone; consider IVIG; mechanical support (ECMO) if cardiogenic shock
  • MRI cardiac: gadolinium enhancement confirms myocardial inflammation
MIS-C (Multisystem Inflammatory Syndrome in Children) post-COVID-19 can mimic Kawasaki disease with myocarditis. Treat with IVIG + aspirin ± steroids.

GCC-Specific Context: CHD & Paediatric Cardiology

Consanguinity & CHD

  • First-cousin marriage: ~25–60% in Gulf countries (Saudi Arabia, Qatar, UAE, Kuwait)
  • Increases autosomal recessive genetic disorders with cardiac involvement
  • Higher prevalence of: syndromic CHD, cardiomyopathies, arrhythmia syndromes (LQTS)
  • Genetic counselling essential in CHD family planning
  • Cascade family screening recommended when CHD confirmed

Congenital Rubella Prevention

  • MMR vaccination significantly reduced CRS in GCC
  • CRS cardiac lesions: PDA, pulmonary stenosis, ASD
  • Check maternal rubella immunity at antenatal booking

Major GCC Paediatric Cardiac Centres

  • KFSH&RC, Riyadh (Saudi Arabia) — Largest cardiac programme; full surgical range including transplant; ECMO; complex CHD
  • Hamad Medical Corporation (HMC), Doha (Qatar) — National paediatric cardiac service; catheterisation & surgery; ECMO; heart transplant
  • Cleveland Clinic Abu Dhabi (CCAD), UAE — Advanced paediatric cardiac programme; complex neonatal surgery
  • King Abdulaziz Medical City, Jeddah — Paediatric cardiac services
  • Sidra Medicine, Doha — Paediatric heart disease centre

Fetal Cardiac Abnormality Counselling — Islamic Perspective

Termination of pregnancy for fetal anomaly presents significant ethical complexity within Islamic jurisprudence.

  • Majority scholarly position: Termination is generally not permitted after ensoulment (120 days / ~17 weeks in some schools; 40 days in others)
  • Some scholars: Permit termination before 120 days for severe lethal anomalies incompatible with life
  • HLHS, Trisomy 18 with CHD: Highly complex ethical discussions; family must involve religious scholars & medical team jointly
  • Nurses must provide non-judgemental support; refer to hospital chaplain/religious affairs and ethics committee
  • GCC hospitals increasingly have Islamic bioethics committees to guide such decisions
Nursing approach: Provide factual information about prognosis; avoid imposing personal values; respect family's religious consultation process; document thoroughly.

GCC Nursing Regulatory Competencies

BodyCountryPaediatric Cardiac Relevance
SCFHS (Saudi Commission for Health Specialties)Saudi ArabiaPaediatric nursing with cardiac focus; specialism registration; CPD requirements
DHA (Dubai Health Authority)Dubai, UAEHAAD/DHA exam includes paediatric cardiology; license renewal CPD
DOH (Department of Health Abu Dhabi)Abu Dhabi, UAECompetency framework includes neonatal cardiac nursing
QCHP (Qatar Council for Healthcare Practitioners)QatarPrometric exam; paediatric cardiology topics in exam blueprint
MOH Kuwait / MOH Bahrain / MOH OmanGCCSimilar licensing exams; Prometric-format MCQs

GCC Exam Prep: DHA / MOH / SCFHS / QCHP Style MCQs

Q1. A 5-month-old infant with known large VSD presents with poor weight gain, tachypnoea, and diaphoresis during feeds. Which of the following is the MOST appropriate initial nursing intervention?
A. Administer oxygen at 10 L/min via non-rebreather mask
B. Provide high-calorie formula feeds and position upright during feeding
C. Restrict all oral feeding and insert a Foley catheter
D. Prepare for immediate surgical closure of VSD
Answer: B. Infants with large VSD and heart failure present with failure to thrive, tachypnoea and diaphoresis (sympathetic activation) with feeds. Initial management includes high-calorie feeds (24–30 kcal/oz), upright positioning, and diuretics (furosemide). Surgical repair is considered when medical management fails or the infant is ≥3–6 months old.
Q2. A 2-year-old child is brought to the ED with fever for 6 days, red cracked lips, strawberry tongue, bilateral conjunctival injection, a polymorphous rash, and swelling of the hands. Which treatment should be initiated FIRST?
A. High-dose amoxicillin IV for 14 days
B. IVIG 2 g/kg as a single infusion over 10–12 hours plus high-dose aspirin
C. Low-dose prednisolone for 7 days
D. IV acyclovir for suspected viral encephalitis
Answer: B. This child meets classic Kawasaki disease criteria (fever ≥5 days + 4/5 features). Standard treatment is IVIG 2 g/kg single infusion + high-dose aspirin (30–50 mg/kg/day). IVIG reduces coronary artery aneurysm risk from 25% to <5%. Echocardiogram should be performed at diagnosis.
Q3. A cyanotic newborn on prostaglandin E1 infusion becomes increasingly apnoeic. What is the MOST likely explanation for the apnoea?
A. Hypoglycaemia from the infusion diluent
B. Known side effect of prostaglandin E1 requiring anticipatory intubation readiness
C. Pulmonary haemorrhage secondary to the CHD
D. Sepsis from the umbilical catheter insertion
Answer: B. Apnoea is a well-recognised side effect of prostaglandin E1 (alprostadil), occurring in ~10–12% of neonates, especially in smaller preterm infants and at higher doses. Before starting PGE1, intubation equipment must be immediately available. This should be anticipated and the team briefed before initiation.
Q4. A 9-month-old with Tetralogy of Fallot suddenly becomes deeply cyanosed and inconsolable during a nappy change. The nurse notes the infant is hyperpnoeic with reduced femoral pulses. What is the CORRECT sequence of initial management?
A. Start O2, check blood glucose, call cardiologist, prepare for echo
B. Place in knee-chest position, apply O2, prepare IV morphine 0.1 mg/kg
C. Perform immediate defibrillation, start CPR, call arrest team
D. Insert NG tube, administer IV glucose, warm the infant
Answer: B. This is a hypercyanotic ("Tet") spell. Immediate management: (1) Knee-chest position — increases SVR and reduces R-to-L shunt, (2) O2 via face mask, (3) IV/IO morphine 0.1 mg/kg — reduces hyperpnoea and anxiety, (4) IV propranolol — reduces RVOT spasm, (5) IV phenylephrine if refractory — further increases SVR. Urgent surgical review for palliative BTT shunt or definitive repair.
Q5. A 6-week-old post-Norwood procedure infant in the PICU develops milky white fluid draining from the left thoracic drain. Triglycerides in the drain fluid are 2.3 mmol/L. What is the MOST appropriate initial management?
A. Administer IV vancomycin for suspected empyema
B. Change to a fat-free or MCT (medium-chain triglyceride) formula/diet and consider octreotide infusion
C. Perform urgent surgical ligation of the thoracic duct immediately
D. Remove the chest drain and observe clinically
Answer: B. Milky drain fluid with elevated triglycerides (>1.1 mmol/L) confirms chylothorax — a known complication of cardiac surgery (especially Norwood/Glenn/Fontan). First-line management: fat-free or MCT formula (MCT absorbed directly into portal blood, bypassing thoracic duct). Octreotide 1–10 mcg/kg/hr can reduce chyle production. TPN if drainage is severe. Surgical thoracic duct ligation is reserved for cases refractory after 4–6 weeks of conservative management.