Risk factors for pulmonary hypertension in diazoxide-treated hyperinsulinaemic hypoglycaemia

About 7 percent of diazoxide‐treated paediatric patients with hyperinsulinaemic hypoglycaemia develop pulmonary hypertension (PH), with risk factors including congenital heart disease and fluid overload, as shown in a retrospective study.

The study population comprised 177 hyperinsulinaemic hypoglycaemia patients treated with diazoxide in four regional centres in UK.  PH was diagnosed in 13 patients, resulting in an incidence rate of 7 percent or one in every 14 cases.

Patients who did vs did not develop PH were older at the time of hyperinsulinaemic hypoglycaemia diagnosis (median age, 9 vs 1 day; p<0.001) and were more likely to have an underlying congenital heart disease (62 percent vs 23 percent; p=0.008).

In the PH group, hyperinsulinaemic hypoglycaemia was diagnosed at a median of 9 days, with diazoxide initiated 4 days after diagnosis and reached a maximum dose of 7 mg/kg/d. Most patients (eight of 13) developed PH within 2 weeks of treatment.

PH resolved at a median of 41 days following complete diazoxide discontinuation, but not dose reduction. PH persisted beyond 12 months in three patients.

Significant factors associated with the development of PH included the presence of congenital heart disease (p=0.008) and total fluid volume exceeding 130 mL/kg/d in the immediate 24 hours preceding diazoxide (p=0.019).

Based on our findings, researchers recommended the inclusion of echocardiography and fluid restriction to 130 mL/kg/d prior to diazoxide treatment and immediate discontinuation of diazoxide if PH develops.