Diazoxide‐associated pulmonary hypertension in a patient with noncompaction cardiomyopathy

Abstract

Development of pulmonary hypertension after initiation of diazoxide for treatment of neonatal hyperinsulinemic hypoglycemia is a rare, but previously described association. Risk factors for development of diazoxide associated pulmonary hypertension include lower gestational age and congenital heart disease. This novel case report describes an infant with noncompaction cardiomyopathy who developed pulmonary hypertension shortly after initiation of diazoxide for hyperinsulinemic hypoglycemia which resolved upon cessation of the drug. This case highlights the benefit of having pre-treatment knowledge of underlying cardiac anatomy and makes a case for routine echocardiographic screening for neonates initiating diazoxide treatment.

Pulmonary hypertension associated with diazoxide: the SUR1 paradox

“…We identified 31 individual case reports and three series that reported a total of 30 additional cases, resulting in 61 cases in total ( supplementary table S1 ) [ 2 – 5 , 37 – 47 ]. All cases occurred in infants aged <6 months treated with diazoxide for neonatal hypoglycaemia or hypoglycaemia complicating other diseases, including genetic diseases.…”

Congenital Hyperinsulinaemic Hypoglycaemia—A Review and Case Presentation

“…Fluid restriction is therefore advisable, along with the administration of chlorothiazide (which also has a synergistic action over the K ATP channels [ 6 , 27 , 35 ]), or furosemide with spironolactone to prevent fluid overload [ 31 ]. In extreme preterm infants and children with lung disease and congenital heart defects, diazoxide can induce reversible pulmonary hypertension (caused probably by direct cardiac toxicity [ 36 ]), reopening of the ductus arteriosus, sepsis syndromes, heart failure, neutropenia, thrombocytopenia, hyperuricemia, and hyperosmolar coma [ 24 , 26 , 27 , 35 , 37 , 38 ].…”