Dr Aditya Bhat, Dr, Amit Misri
Department of Paediatric Cardiology, Medanta-The Medicity, Gurgaon
Background
• A 5 month old previously well infant
who presented with recent onset
severe respiratory distress.
• Physical examination was otherwise
unremarkable [weight-7.2kg, length
62cm] with no developmental delay..
• Xray chest showed cardiomegaly
[cardiothoracic ratio 0.7] and
normal lung shadows.
• ECG revealed sinus tachycardia and
features of Left ventricular [LV]
enlargement.
• ECHO showed grossly dilated LV
with severe dysfunction [LVEF 20
%], global LV hypokinesia and
moderate mitral regurgitation. LV
outflow tract, aortic arch and
coronaries were normal.
• A presumptive diagnosis of dilated
cardiomyopathy [DCMP]/ acute
fulminant myocarditis was
considered and patient started on
decongestive measures, oral digoxin
and inotropic support.
Results
• Lack of inflammatory markers •
sterile blood cultures and negative
viral assays for myocarditis.
• Possibility of non-inflammatory
DCMP
• Serum electrolytes and Vitamin D •
levels normal.
• Cardiac troponins slightly elevated
[Trop I-0.42 ng/ml].
• Carnitine deficiency and organic
acidurias were ruled out by TMS
• Endocrine evaluation showed grossly
elevated TSH levels [461 µIU/ml]
with low free T3 [3.3pg/ml] and
free T4 level [0.63ng/dl].
• On ultrasound, there was absence
of thyroid gland in neck.
•TC99m Pertechnetate scan showed non
visualization of thyroid gland and a small
ectopic lingual thyroid.
Interventions
Started on oral Thyroxin [12.5-
25mcg/day] along with continued
decongestive measures which led to
marked improvement in cardiac
function.
TSH normalized
• At 6 month follow up he was
growing well [weight 8.5 kg, length
73cm],had normal neurodevelopment
with near normal cardiac function
and decrease in LV dilatation
[LVIDd ↓from 38 to 30 mm and
LVIDs from 34 to 25 mm, ]
Conclusions
• Based on clinical findings and a
stepwise approach, diagnosis of
congenital hypothyroidism associated
dilated cardiomyopathy was
established and successfully
treated.
• Despite thyroid hormones having a
direct effect on calcium mediated
cardiac contractility; hypothyroidism
has been rarely seen as a primary
cause of severe LV dysfunction in
children.
• This case highlights the role of
finding a treatable cause in all
paediatric DCMP cases before
labelling them as “idiopathic”
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