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Patient
Young man 27 years of age, athletic, referred in the department for detection of frequent ventricular extrasystoles;
Trace
Sinus rhythm; low amplitude P wave; first degree atrioventricular block (fixed and prolonged PR interval of 300 ms); narrow QRS with normal axis; poor R wave progression from V1 to V3; 2 isolated ventricular extrasystoles with left conduction delay but early transition (in V3), clearly positive in the inferior leads and negative in leads I and aVL (extrasystole arising from the left ventricular outflow tract);
Exergue
In a patient with dilated cardiomyopathy, various elements should lead to the diagnosis of laminopathy:
1) a familial context of neuromuscular disease, conduction disorder, sudden death or dilated cardiomyopathy;
2) peripheral muscle damage;
3) the occurrence in a young patient of supraventricular rhythm disturbances or conductive disorders (mainly first degree AV block).
A genetic confirmation is hence essential and the systematic indication of defibrillator implantation should be discussed, given the major risk of sudden death.
The association of conduction disorder (first degree AV block) and ventricular irritability in this young patient should evoke the diagnosis of laminopathy which was confirmed by genetic testing.