Abstracts from American
Heart Association Scientific Sessions 2003,
Orlando, 9-12 november 2003
Henri-Marc Becana, Pitie-Salpetriere
Hospital, Paris, France; Christophe Meune, Cochin Hospital, Paris, France; Guy
Lerebours, Pitie-Salpetriere Hospital, Paris, France; Jean-Yves Devaux, Denis
Duboc, Cochin Hospital, Paris, France; The French Working Group on Heart
Involvement in Myopathies
Background: Duchenne Muscular disease
(DMD) is an inherited X-linked disease due to the absence of dystrophin and
clinically characterized by progressive muscle weakness and constant myocardial
involvement responsible for sudden death or end-stage heart failure in 40% of
patients between the age of 15 and 30. The aim of this study was to evaluate
the preventive effect of early ACEI treatment on left ventricular (LV)
dysfunction in young patients with DMD and normal LVEF at inclusion. Methods:
In phase I, children with genetically proven DMD and radionuclide ejection
fraction (EF) superior than 55% were enrolled in a multicentric, controlled,
randomized, double blind trial of perindopril 2-4 mg/day versus placebo for 3
years. In phase II, all patients then received open-label perindopril for 24
more months. Radionuclide LVEF was performed at 0, 36 and 60 months. Student's
t-tests and chi-square analysis were used for comparisons. Results: 60
children asymptomatic for heart disease were included in the phase I study
(10.6±1.2 years, EF 65.0±5.4%)(31 in the perindropil group and 29 in the
placebo group) and 46 in the phase II study (n=23 in both groups). No adverse
effect related to treatment was documented. At the end of phase I, LVEF
remained stable in both groups (from 64.8±5.3% to 59.6±8.5% in the perindopril
group and 65.5±5.4% to 64.5±9.9% in the placebo group, p=0.114). However, at 60
months, 6 patients in the control group (26%) had a LVEF of less than 45,
versus one in the perindopril group (4%)(p=0.0319). Conclusions:
Early treatment with ACEI perindopril is well tolerated in these young patients
and delay onset of LVEF deterioration. This preventive efficacy of ACEI has to
be evaluated in other groups of patients genetically exposed to develop LV
dysfunction.
Impaired
Strain Rate Measurements in Patients With Duchenne Muscular Dystrophy
Nickolaos Giatrakos, Cardiology dept, Hammersmith
Hospital, NHLI, ICSM, London, United Kingdom; Maria Kinali, Dubowitz
Neuromuscular Centre, Department of Paediatrics, Hammersmith Hospital, ICSM,
London, UK, London, United Kingdom; George Koutroulis, Cardiology dept,
Hammersmith Hospital, NHLI, ICSM, London, United Kingdom; Francesco Muntoni,
Dubowitz Neuromuscular Centre, Department of Paediatrics, Hammersmith Hospital,
ICSM, London, UK, London, United Kingdom; Petros Nihoyannopoulos; Cardiology
dept, Hammersmith Hospital, NHLI, ICSM, London, United Kingdom
Background: Patients with Duchenne muscular
dystrophy (DMD) develop dilated cardiomyopathy at the later stages of the
disease. Strain rate (SR) has been used to study myocardial function in
ischeamia and cardiomyopathies. The aim of this study was to investigate the
usefulness of SR for the early detection of cardiac involvement in young,
asymptomatic patients with DMD.
Methods: We studied 53 patients with genetically confirmed diagnosis of DMD
(mean age 8.7±2.8 years) without clinical symptoms from the cardiovascular
system and normal conventional echocardiographic studies, and compared with 22
normal controls matched for age (mean age 8.5±2.5 years). We used the HDI 5000
(Philips Medical Systems) to acquire from the parasternal long axis the colour
M-mode tissue Doppler (TDI) of the posterior wall of the LV. Images were
digitally stored for offline analysis with dedicated software HDI-lab (Philips
Medical Systems). We calculated the SR using the formula SR=Ua-Ub/d where U the
velocities of the endocardium a and epicardium b, and d the distance of a and b
at each time point.
Results: There was no significant difference for the parameters from
conventional echocardiographic studies between the two groups. The velocities
derived from the TDI, mean velocity at systole (26,99±7,12mm/sec vs.
33,4±7,3mm/sec, p<0,000), at early diastole (-45,79±13,93mm/sec
vs.-60,46±7,58mm/sec, p<0,000) and late diastole (-10,93±3,41mm/sec
vs.-13,32±6,4mm/sec, p<0,02) were significantly different in patients with
DMD when compared with controls. SR was found to be significantly lower at
systole (1,78±0,75s-1 vs. 2,82±0,5s-1, p<0,000) and
early diastole (-5,17±1,98s-1 vs.-9,02±1,25s-1,
p<0,000) but not at late diastole (-1,52±0,84s-1vs.-1.6±0,46s-1,
p<0,568) in patients with DMD.
Conclusions: Estimation of SR of the posterior wall of the LV showed systolic
and diastolic dysfunction at early stages in asymptomatic patients with DMD and
when the conventional echocardiography is still normal. Estimation of SR could
be a sensitive method to investigate the pathophysiology of the disease and
identify early impairment of the cardiac function.