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Malignant Ventricular Arrhythmia in a Case of Adult Onset of Spinal Muscular Atrophy (Kugelberg–Welander Disease)
Author(s) -
ROOS MARKUS,
SARKOZY ANDREA,
CHIERCHIA GIAN BATTISTA,
DE WILDE PHILIPPE,
SCHMEDDING ERIC,
BRUGADA PEDRO
Publication year - 2009
Publication title -
journal of cardiovascular electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.193
H-Index - 138
eISSN - 1540-8167
pISSN - 1045-3873
DOI - 10.1111/j.1540-8167.2008.01327.x
Subject(s) - medicine , cardiology , spinal muscular atrophy , ventricular tachycardia , sma* , disease , mathematics , combinatorics
Adult‐Onset KWD and Arrhythmia. We present a case of a 43‐year‐old male patient with adult onset of spinal muscular atrophy (SMA). The patient first came to our attention with atrioventricular (AV) block. A dual‐chamber pacemaker (DDD‐PM) was implanted. Four years later, the PM data log showed occurrence of frequent episodes of nonsustained ventricular tachycardia (NSVT). The episodes progressed in duration and frequency. An electrophysiological study revealed prolonged His‐ventricular (HV) interval duration and induction of sustained ventricular tachycardia. The patient was successfully upgraded to a prophylactic dual‐chamber cardioverter defibrillator. Our case is the first description of a patient with adult‐onset SMA (Kugelberg–Welander disease [KWD]) and malignant ventricular arrhythmias. (J Cardiovasc Electrophysiol, Vol. 20, pp. 342‐344, March 2009)