Dilated cardiomyopathy (DCM) is a relatively common diagnosis encountered in the pediatric cardiac outpatient clinic (PCOPD).
We present a case where a presumed viral myocarditis leading to DCM revealed itself to be aorto-arteritis (AA). The purpose of this submission is to highlight AA as an important differential in all cases of dilated cardiomyopathy.
An 8yr old girl presented to our PCOPD with a dyspnea on exertion. 2 months prior she had been diagnosed with DCM of unknown etiology. At that time, her echocardiogram showed a severely dilated and poorly contractile left ventricle. This was confirmed in our clinic. She was placed on routine decongestive and cardiosupportive medications. Subsequently she was seen in 2 other PCOPDs in the country where intravenous immunoglobulins were given after endomyocardial biopsy. At her next visit with us, she was hypertensive and had weight loss. A CT scan revealed isolated coarctation of the lower thoracic aorta. The pulse wave Doppler of the abdominal aorta showed depressed waveforms. Mantoux test was positive; anti Kochs therapy was initiated. Balloon angioplasty was successfully performed. 6months post angioplasty, her hypertension is under control but her DCM has not improved. A diagnosis of AA related primary DCM was made and the patient is currently being co-managed by a rheumatologist.
Non specific inflammation on endomyocardial biopsy is not specific for viral myocarditis. AA should be considered in the differential diagnosis of every child presenting with DCM in a third world country.