Abstract

Progressive systemic right ventricular (sRV) dysfunction, atrial and ventricular arrhythmias and sudden cardiac death are well-recognized late sequelae of atrial redirection surgery in which the right ventricle is left connected to the systemic circulation. Although cardiac resynchronization therapy (CRT) poses an attractive therapeutic option, little is known about indications, patient selection, and technical aspects of best lead placement. We undertook CRT in a 27-year-old female patient post-Mustard correction for d-transposition (d-TGA) with New York Heart Association (NYHA) grade III disability with QRS duration measuring 130 ms. There was also echocardiographic (TTE) evidence of severe sRV dysfunction. Non-contact mapping (NCM) was used to define sites of late activation within the sRV and the acute intra-arterial blood pressure (BP) response was assessed during implantation of a 4 french (F) lead onto the endocardial surface of the sRV. At 4 weeks post-implant sRV lateral wall motion had improved and the ejection fraction (EF) rose from 23 to 33%. The patient has been successfully anticoagulated and improved to NYHA II status after 6 months. The use of NCM proved safe and effective and provided a qualitative assessment of electrical viability of the sRV complimenting the measurement of mechanical function provided by TTE. The favourable clinical response in the above case justifies a prospective evaluation of this strategy.

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