Exertional Chest pain and Near Syncope in a Young Adolescent

An adolescent female presented for increasing chest pain (sometime sharp, sometime like someone standing on her chest) and palpitations with exertion for 3 months. It is particularly noticeable with dancing or running, but has also come at rest. On the day she presented to the ED, she was running home, had the onset of CP, then felt dizzy and felt like she was going to pass out. She sat down and the pain slowly went away.  She had never had a full syncopal event.  When she told her mother about the episode, they came to the ED. She denies SOB with her pain. She denied family history of sudden cardiac death at a young age, or for unknown reasons. She is otherwise healthy but has been told that she has a murmur.   On exam, she had a 3/6 systolic murmur that decreased with increasing peripheral vascular resistance.


Here is her ECG:

There is massive voltage (severe LVH) with corresponding repolarization abnormalities.  There is very high voltage R-waves in right precordial leads, highly suggestive of septal hypertrophy.  The ST segments are depressed, discordant to the large R-wave.  This is unlike most LVH, in which the right precordial S-waves are deep, with discordant ST elevation.)  This is highly suspicious for hypertrophic cardiomyopathy with asymmetric septal hypertrophy (HOCM). 

The ECG is almost always abnormal in HOCM, with LVH and repolarization abnormalities, especially if symptomatic.  This is a rather extreme example.

Here is her chest Xray:

Massive cardiomegaly

The emergency physicians performed a bedside echocardiogram: Diagnosis: Hypertrophic Cardiomyopathy. She was transferred to a pediatric referral center.