BACKGROUND: Left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC) is a common finding in autopsy studies. In clinical studies using myocardial scintigraphy, MRI and echocardiography, contradictory results have been reported. In this study, we therefore investigated a group of 15 patients with ARVC using thallium-201 (Tl) single-photon emission tomography (SPECT) and echocardiography including assessment of mitral annular motion with M-mode and pulsed tissue Doppler. METHODS: Exercise and rest Tl-201 SPECT were performed in 15 patients with ARVC. The time from diagnosis of the disease varied from less than 1-16 years. All patients fulfilled the established diagnostic criteria for ARVC. An echocardiographic examination, including assessment of left and right ventricular motion and measurements of the mitral annulus motion with M-mode and pulsed tissue Doppler was performed in the patients and in 25 normal subjects. RESULTS: Tl-201 uptake defects in the left ventricular myocardium were present in all except one patient (93%). The uptake defects were predominantly located to the anteroseptal and basal posterior segments. Wall motion abnormalities were seen in the same segments, and in addition to this, in the septal area. In line with this, the total amplitude and the peak systolic velocity of mitral annular motion at the septal point were significantly decreased in the patients compared with the control group. CONCLUSIONS: Our data show that left ventricular involvement is common in ARVC. Tl-201 SPECT and echocardiographic abnormalities were seen not only in patients with long-lasting symptoms but also in asymptomatic patients and in those with short duration of symptoms.
BACKGROUND:Left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC) is a common finding in autopsy studies. In clinical studies using myocardial scintigraphy, MRI and echocardiography, contradictory results have been reported. In this study, we therefore investigated a group of 15 patients with ARVC using thallium-201 (Tl) single-photon emission tomography (SPECT) and echocardiography including assessment of mitral annular motion with M-mode and pulsed tissue Doppler. METHODS: Exercise and rest Tl-201 SPECT were performed in 15 patients with ARVC. The time from diagnosis of the disease varied from less than 1-16 years. All patients fulfilled the established diagnostic criteria for ARVC. An echocardiographic examination, including assessment of left and right ventricular motion and measurements of the mitral annulus motion with M-mode and pulsed tissue Doppler was performed in the patients and in 25 normal subjects. RESULTS: Tl-201 uptake defects in the left ventricular myocardium were present in all except one patient (93%). The uptake defects were predominantly located to the anteroseptal and basal posterior segments. Wall motion abnormalities were seen in the same segments, and in addition to this, in the septal area. In line with this, the total amplitude and the peak systolic velocity of mitral annular motion at the septal point were significantly decreased in the patients compared with the control group. CONCLUSIONS: Our data show that left ventricular involvement is common in ARVC. Tl-201 SPECT and echocardiographic abnormalities were seen not only in patients with long-lasting symptoms but also in asymptomatic patients and in those with short duration of symptoms.
Authors: Nancy D Merner; Kathy A Hodgkinson; Annika F M Haywood; Sean Connors; Vanessa M French; Jörg-Detlef Drenckhahn; Christine Kupprion; Kalina Ramadanova; Ludwig Thierfelder; William McKenna; Barry Gallagher; Lynn Morris-Larkin; Anne S Bassett; Patrick S Parfrey; Terry-Lynn Young Journal: Am J Hum Genet Date: 2008-02-28 Impact factor: 11.025