MLA Domain 5 · Presentation
Cyanosis
19 conditions in Finalist present this way. Work down the list and ask what would separate each one from the next — that discriminator is the exam question.
classic presentation · 5
Established Eisenmenger CONTRAINDICATES shunt closure (the shunt is now offloading the right heart) — the window was mis
Persistent pulmonary hypertension (severe hypoxia, pre/post-ductal saturation difference) → intensive care (± nitric oxi
Hypercyanotic 'tet' spell → knee-chest position, oxygen, morphine, IV fluids, beta-blocker/phenylephrine — can be life-t
Obstructed (infradiaphragmatic) TAPVR → emergency surgery (prostaglandin may not help; obstruction is the problem)
Profound day-1 cyanosis → emergency: PROSTAGLANDIN (keep the duct open) ± balloon atrial septostomy
recognised feature · 14
A SILENT chest, exhaustion, normal or rising CO2, or SpO2 <92% = life-threatening → seek ICU
Exhaustion, cyanosis, falling GCS, silent chest = peri-arrest airway → immediate senior airway support
Life-threatening features (silent chest, SpO2 <92%, exhaustion, normal/rising PaCO2) → ITU/anaesthetic involvement — a n
Heart failure + early pulmonary hypertension (complete AVSD) → timely surgical repair
Critical neonatal coarctation presenting as collapse when the duct closes → PROSTAGLANDIN to reopen/maintain the duct +
Eisenmenger physiology → avoid pregnancy and systemic vasodilators; refer to specialist adult congenital heart disease (
Severe neonatal Ebstein (cyanosis + heart failure) → intensive care ± prostaglandin
Critical neonatal PS → prostaglandin (duct-dependent) + intervention
Digital ulceration/critical ischaemia → urgent treatment (secondary Raynaud's)
Worsening distress → pneumothorax (sudden deterioration), need for escalating support
Exhaustion, rising CO2 with acidosis, or peri-arrest → urgent ventilatory support (NIV/intubation) and critical care
Stridor/airway compromise, cerebral oedema (severe headache, confusion) → emergency treatment
Duct closure → worsening cyanosis → prostaglandin
Early heart failure and pulmonary hypertension → timely surgery