Europace Advance Access published online on November 19, 2007
Europace, doi:10.1093/europace/eum245
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Cardiac resynchronization therapy in left ventricular hypertrabeculation/non-compaction and myopathy
1 2. Medizinische Abteilung, Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030 Wien, Österreich; 2 Medizinische Abteilung, Hanusch Krankenhaus, Heinrich-Collin-Straße 30, 1140 Wien, Österreich; 3 Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030 Wien, Österreich
Aims: Little is known how patients with left ventricular hypertrabeculation/non-compaction (LVHT) and heart failure respond to cardiac resynchronization therapy (CRT).
Methods and results: Included in this retrospective study were 8/102 patients (3 female, age range 43–78 years), in whom LVHT was diagnosed and in whom a CRT system was implanted. All eight patients were investigated neurologically and in seven of them a myopathy was found. The mean follow-up after CRT implantation was 39 (4–68) months. All patients improved by one or more New York Heart Association (NYHA) classes, and two by two NYHA classes. The left ventricular end-diastolic diameter decreased by 
5% in 2 patients, by 6–10% in 3 patients, by 12% in 1 patient, and by >30% in 2 patients. The left ventricular systolic function, as assessed by the fractional shortening, did not change in 2 patients, increased by 10% in 2 patients, by 59% in 1 patient, doubled in 2 patients, and showed a five-fold increase in 1 patient. Two patients died during follow-up.
Conclusion: CRT by biventricular pacing in LVHT, heart failure, and myopathy leads to improvement in functional capacity in all patients and improvement of systolic function in half of the patients. The weak response of LVHT patients to CRT may be due to inappropriate selection or comorbidities, in particular, neuromuscular disorders.
Key Words: Cardiomyopathy, Heart failure, Myopathy, Cardiac resynchronization therapy, Biventricular pacemaker
* Corresponding author: Steingasse 31/18, A-1030 Wien, Österreich. Tel/fax: +43 1 713 98 70. E-mail address: claudia.stoellberger{at}chello.at
Manuscript submitted 25 July 2007. Accepted after revision 15 October 2007.
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