10.5 Neonatology & Congenital Heart Disease
Key Takeaways
Newborn life support starts with drying, warming and assessment, then 5 inflation breaths; chest compressions at 3:1 begin only if the heart rate stays below 60 after effective ventilation.
Babies have a newborn and infant physical examination within 72 hours and at 6–8 weeks, covering the eyes, heart, hips and testes, plus blood spot screening on day 5.
Suspected early-onset neonatal sepsis is treated with IV benzylpenicillin and gentamicin within 1 hour (NICE NG195).
A collapsed, cyanosed or acidotic neonate in the first days of life may have a duct-dependent heart lesion and needs a prostaglandin infusion to keep the ductus arteriosus open.
Tetralogy of Fallot comprises a VSD, overriding aorta, pulmonary stenosis and right ventricular hypertrophy, and causes cyanotic spells relieved by squatting.
Neonatology & Congenital Heart Disease
Newborn Life Support (Resuscitation Council UK)
- Dry, warm and stimulate the baby; keep warm (plastic bag for very preterm babies); delay cord clamping for at least 60 seconds if the baby does not need immediate resuscitation.
- Assess tone, breathing and heart rate (the most important indicator of success).
- Airway: neutral head position, jaw thrust if needed.
- Breathing: if not breathing adequately, give 5 inflation breaths (each held for 2–3 seconds; about 30 cmH2O for term babies, 25 cmH2O for preterm), starting in air for term babies.
- Reassess the heart rate. If it does not rise, check chest movement and the airway before anything else; then give ventilation breaths.
- Chest compressions if the heart rate stays below 60 per minute despite 30 seconds of effective ventilation: 3 compressions to 1 breath, increasing oxygen to 100%. Consider drugs (adrenaline) and vascular access if there is still no response.
Newborn Screening & Examination
- Newborn and Infant Physical Examination (NIPE) within 72 hours and again at 6–8 weeks: eyes (absent red reflex suggests cataract or retinoblastoma), heart (murmurs, femoral pulses), hips (Barlow and Ortolani tests for developmental dysplasia) and testes (undescended testes).
- Newborn blood spot test (heel prick, ideally on day 5): sickle cell disease, cystic fibrosis, congenital hypothyroidism and six inherited metabolic diseases including phenylketonuria and MCADD deficiency.
- Newborn hearing screen (automated otoacoustic emissions) before discharge or within the first weeks.
Common Neonatal Problems
Neonatal Hypoglycaemia
Babies at risk include those of diabetic mothers, preterm or small for gestational age babies, and babies with hypothermia or sepsis. A blood glucose below 2.6 mmol/L is treated: encourage feeding, consider buccal dextrose gel, and give IV 10% glucose if severe, symptomatic (jitteriness, poor feeding, seizures) or persistent.
Respiratory Distress in the Newborn
| Condition | Clues |
|---|---|
| Transient tachypnoea of the newborn | Term baby after caesarean section; delayed clearance of lung fluid; resolves within 24–48 hours |
| Respiratory distress syndrome | Preterm; surfactant deficiency; ground-glass chest X-ray; prevented by antenatal steroids, treated with CPAP and surfactant |
| Meconium aspiration | Post-term or distressed baby; meconium-stained liquor; patchy X-ray changes |
| Congenital pneumonia / sepsis | Maternal fever, prolonged rupture of membranes, GBS carriage |
| Congenital diaphragmatic hernia | Scaphoid abdomen, bowel sounds in the chest; avoid bag-mask ventilation (intubate) |
Early-Onset Neonatal Infection (NICE NG195)
Risk factors include maternal GBS, intrapartum fever or chorioamnionitis, prolonged rupture of membranes, preterm birth and infection in a twin. Red flags include seizures, signs of shock, need for mechanical ventilation and confirmed or suspected infection in a twin. Take blood cultures and give IV benzylpenicillin and gentamicin within 1 hour of deciding to treat; review at 36 hours with cultures and CRP.
Necrotising Enterocolitis (NEC)
Mainly preterm babies after feeds are started: feed intolerance, bilious aspirates, abdominal distension, bloody stools and systemic instability. The abdominal X-ray shows pneumatosis intestinalis (gas in the bowel wall) or free air. Management: stop feeds, nasogastric decompression, IV antibiotics, parenteral nutrition and surgery for perforation.
Neonatal Abstinence Syndrome
Withdrawal in babies exposed to opioids in pregnancy: irritability, high-pitched cry, tremor, poor feeding, diarrhoea and seizures. Use a structured scoring tool, supportive care and, if severe, oral morphine. It also needs safeguarding assessment.
Congenital Heart Disease (CHD)
Acyanotic Lesions
| Lesion | Murmur & Signs | Key Points |
|---|---|---|
| Ventricular septal defect | Pansystolic murmur at the lower left sternal edge | Most common CHD; small defects often close; large defects cause heart failure |
| Atrial septal defect | Ejection systolic murmur in the pulmonary area, fixed split second heart sound | Often found later; risk of paradoxical embolism |
| Patent ductus arteriosus | Continuous "machinery" murmur below the left clavicle, bounding pulses | Common in preterm babies; NSAIDs (ibuprofen, indometacin) can close it |
| Coarctation of the aorta | Weak or delayed femoral pulses, hypertension in the arms | Associated with Turner syndrome and bicuspid aortic valve; severe forms collapse when the duct closes |
| Atrioventricular septal defect | Murmur may be absent in the newborn | Strongly associated with Down's syndrome (all babies with Down's syndrome have an echocardiogram) |
Cyanotic Lesions
- Tetralogy of Fallot: VSD, overriding aorta, pulmonary (right ventricular outflow) stenosis and right ventricular hypertrophy. Cyanotic "tet spells" with irritability and deepening cyanosis are relieved by squatting or knee-chest position (which raises systemic resistance); chest X-ray shows a boot-shaped heart. Surgical repair in infancy.
- Transposition of the great arteries: profound cyanosis within the first day; "egg on a string" X-ray; survival depends on mixing through the duct or foramen ovale until the arterial switch operation.
Caution
Duct-Dependent Circulation: A baby who becomes cyanosed, shocked or acidotic in the first days of life as the ductus arteriosus closes (for example with transposition, critical pulmonary stenosis, severe coarctation or hypoplastic left heart) needs an IV prostaglandin infusion to reopen the duct, plus urgent discussion with a paediatric cardiology centre. Watch for apnoea as a side effect.
Innocent Murmurs
Common in healthy children, especially with fever. Typical features: soft, systolic, short, symptom-free, left sternal edge, varying with posture, with normal pulses and no thrill or added sounds. Refer if there are symptoms (poor feeding, breathlessness, faltering growth, cyanosis), a diastolic or loud murmur, a thrill, or abnormal pulses.
A baby is born at 39 weeks and is floppy with no breathing after being dried and stimulated. Five inflation breaths are given with good chest movement, followed by 30 seconds of ventilation breaths. The heart rate is now 50 beats/min. What is the next step?
Stop resuscitation, as the heart rate shows no response
Continue ventilation breaths alone and reassess in 2 minutes
Start chest compressions at a ratio of 3 compressions to 1 breath
Give IV adrenaline before any compressions
A 3-day-old baby boy becomes grey, breathless and poorly perfused. His femoral pulses are impalpable, and blood gas shows a metabolic acidosis. Pre-ductal and post-ductal saturations differ. What is the most important immediate treatment?
Oral propranolol
IV ibuprofen to close the duct
IV furosemide
IV prostaglandin infusion
A baby is born to a mother whose labour was complicated by fever of 38.6°C and membranes ruptured 30 hours before birth. The baby is grunting at 2 hours of age. Blood cultures are taken. What is the most appropriate empirical antibiotic regimen?
IV ceftriaxone alone
IV benzylpenicillin and gentamicin
Oral amoxicillin and observation for 24 hours
IV vancomycin and meropenem
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