Pocket Pediatrics: The Massachusetts General Hospital for Children Handbook of Pediatrics (Pocket Notebook Series), 2 Ed.

CYANOTIC CHILD

Pathophysiology

• Blue to dusky hue in the newborn; 5 g/dL of deoxyhemoglobin produces cyanosis

• Dependent on absolute concentration of reduced hemoglobin

• 2/2 hypoventilation, R to L shunt/intrapulmonary shunting, V–Q mismatch, diffusion impairment

Etiology (Pediatr Clin North Am 2004;51:999)

Clinical Manifestations

• Central cyanosis: Decreased arterial oxygen content

• Periph cyanosis: Nml PaO2; w/ cold exposure, Raynauds, polycythemia, early shock

• Differential cyanosis: Pink upper body, cyanotic lower part (R to L shunt from PDA)

• Reverse diff cyanosis: Upper part of body cyanotic, lower part pink (Transposition w/ pHTN, interrupted aortic arch, critical aortic coarctation)

• Harlequin condition: 1 quadrant or ½ of body cyanotic (vasomotor instability)

Diagnostic Studies

Management

• Management and prognosis depends on the dx, severity, and the time of presentation

• Requires initial stabilization, assuring hemodyn stability w/ IVFs, oxygen administration, and possible referral to a NICU, use Prostaglandin E if failed hyperoxia test