The LEOPARD Syndrome

Report:

Sinus tachycardia 122/min

Right axis deviation +150o

Right bundle branch block

Nonspecific ST/T changes

Lead V1 – V3 reversal

Comment:

There are multiple eponyms for this rare autosomal dominant syndrome but the acronym LEOPARD is the most commonly used name. It stands for lentigines, electrocardiographic conduction anomalies, ocular hypertelorism, pulmonic stenosis, abnormal genitals, retarded growth and deafness. There is nothing specific about the ECG abnormalities, commonly 1o AV block, LAHB and IVCD. Their significance is highly variable; sudden death has been reported.

Other cardiac involvement comprises pulmonary and sometimes aortic stenosis, fibroelastosis or HOCM. Overall cardiac involvement in the syndrome is present in 95% of cases.

This ECG could be interpreted as RBBB with LPHB if RVH can be excluded. Not enough is known at present to advise prophylactic pacing (or ICD). This patient had no other evidence of cardiac involvement and no relevant symptoms.

Reader, if the patient did not have the interesting LEOPARD syndrome, his ECG would not be in this Library.

Fig 64. 89 year old man with complex ECG but no cardiac history or symptoms.

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