Dr Aditya Bhat, Dr Rajesh Sharma, Dr Romel Akole, Dr, Amit Misri
Department of Paediatric Cardiology, Medanta-The Medicity, Gurgaon
Background
• 3 month old female infant with rapid
breathing.
• Failure to thrive [weight 2.6 kg and
length 49.5 cm] and poor weight gain
• ECHO showed Complex single ventricle
physiology,Double inlet left ventricle,
l-malposed aorta, 6 mm secundum ASD
and a large bulboventricular foramen.
• Underwent Pulmonary Artery Banding
with significant clinical improvement.
• However ,continued to have episodes
of poor feeding preceeded by lethargy
warranting further evaluation
Initial evaluation
• Obvious causes of dullness were
sequentially ruled out.
• No evidence of septicaemia.
• Cardiac evaluation showed a well-
functioning PA band with no
evidence of cardiac dysfunction or
Low Cardiac output state.
• Intracranial haemorrhage was ruled
out
• Electrolytes were well within normal
range.
Role of metabolic screening
• However metabolic screening showed •
recurrent hypoglycaemia that
persisted despite adequate enteral
feeding.
• In view of recurrent hypoglycaemia
iv infusions with high Glucose
Infusion Rates [GIR] upto •
15mg/kg/hour were re-initiated.
• A short trial of steroids given to
increase glucose levels.
• Despite receiving high volume of •
concentrated dextrose infusions,
patient continued to have daily
hypoglycaemic recordings on serial
monitoring.
• Critical sample testing done at RBS
< 40 mg/dl showed normal Growth
hormone levels and cortisol levels,
with significantly elevated INSULIN
level [2.4 µU/ml].
• Urinary ketones were not detected.
Interventions
In view of elevated insulin, no
evidence of fatty acid oxidation
defects and abnormal glycaemic
response to glucagon a diagnosis of
Hyperinsulinemic Hypoglycaemia of
infancy was established.
Patient was started on subcutaneous
Octreotide 15µg/kg/day, leading to
rapid weaning of iv glucose infusions
and attainment of euglycemic state.
Oral Diazoxide at 5mg/kg/day was
added with tapering and stoppage of
octreotide injections after one week
and a successful discharge on
enteral feeds
Conclusions
• This case highlights the role of
looking beyond the obvious causes
when dealing with an infant with
complex cardiac conditions and
concurrent medical illness.
• Although infrequently described
Hyperinsulinemic Hypoglycaemia of
Infancy can have structural cardiac
defects in up to 10%, and should be
kept in mind while dealing with
patients having recurrent,
persistent hypoglycaemia in PCICU.
Designed & Printed at KL Wig CMET, AIIMS