What is the likely cause of her syncope?
a. Electrolyte disturbance
b. Atrial fibrillation with rapid ventricular response
c. Sinus pauses
d. Ventricular arrhythmia

Suggested Answer - c

In Figure 1, three events strips are shown. The first one shows junctional escape of 30 bpm. The second strip shows sinus/atrial rhythm of 30 bpm. The third strip shows atrial fibrillation with a ventricular rate of ~78 bpm.

With chronic use of Indapamide for blood pressure control, she is prone to electrolyte disturbance, in particular, hyponatremia and hypokalemia. Hypokalemia causes progressive electrocardiographic changes including flattening of T wave, prominent U wave, increase in amplitude and duration of QRS and ST segment depression. Hypokalemia increases excitability and predisposes the myocardium to catecholamine induced arrhythmia or ventricular fibrillation.

Ventricular arrhythmias, whether primary or secondary to electrolyte disturbance, drugs and ischaemia, impair consciousness within seconds. Recurrent syncope as a result of self-limiting ventricular fibrillation is uncommon and sustained ventricular arrhythmias are usually lethal.

Atrial fibrillation with rapid ventricular response frequently causes lightheadedness and dizzy spells but rarely syncope, except in low cardiac output states, e.g. severe left ventricular dysfunction, outflow tract obstruction, etc.

Sinus bradycardia and pauses are manifestations of tachy-brady syndrome. Figure 2 shows stripes with remarkable sinus pauses with very slow atrial and junctional escape. One could pass out with such prolonged asystole.

Sinus node dysfunction with documented symptomatic bradycardia, including frequent sinus pauses that produce symptoms, is a class I indication for pacemaker implantation. In some patients, bradycardia is iatrogenic and will occur as a consequence of essential long-term drug therapy of a type and dose for which there was no acceptable alternatives. Pacemaker implantation is also indicated.

What are the diagnosis and differential diagnoses?

The diagnosis is infantile haemangioma, superficial type (also called capillary haemangioma or strawberry haemangioma). Differential diagnoses include vascular malformations such as port-wine stain.

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What infants are more likely to have this kind of birthmark?

Infantile haemangioma is more prevalent among premature infants, Caucasians and girls. The prevalence is around 1-2% among Caucasian neonates.

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What is the course of disease?

The lesion will proliferate in the initial 8-12 months (proliferating phase), then starts to regress at 1-5 years (involuting phase) and continues to improve until 6-12 years when it finally reaches the involuted phase. Usually the residual skin changes are barely detectable after its involution. Occasionally, skin atrophy and depigmentation may result.

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What are the possible complications arising from this kind of birthmark?

The possible complications include ulceration/bleeding, obstruction of the airway (for deep haemangioma in submental area), gastrointestinal/genitourinary tracts, and blocking the development of facial structures. For haemangioma overlying the lumbrosacral spine, underlying spinal abnormalities such as tethered cord should be ruled out. In cases of multiple haemangiomas, the child should be evaluated for any internal involvement of the central nervous system, gastrointestinal tract and liver.

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What should be the management?

In uncomplicated conditions, simple protection of the haemangioma, reassurance and regular surveillance will suffice. In complicated cases, they can be treated with intralesional steroids, systemic steroids, pulsed-dye laser, cryotherapy, alphainterferon, surgery or embolization.

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