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Pulsed Doppler tissue imaging in dystrophinopathic cardiomyopathy
Angelo Agretto1, Luisa Politano, Eduardo Bossone
1Medical Surgical Institute of Cardiology, II University of Naples, Parco Grifeo 28, 80121 Naples, Italy. a.agretto@irm.it
Insights
Becker muscular dystrophy (BMD) patients and carriers show cardiac dysfunction. Pulsed Doppler tissue imaging (DTI) effectively detects early systolic and diastolic alterations, even with normal LVEF in these individuals.
Area of Science:
- Cardiology
- Genetics
- Biomedical Engineering
Background:
- Cardiac dysfunction is a significant concern in Becker muscular dystrophy (BMD) and its female carriers.
- Early detection of cardiac involvement is crucial for managing dystrophinopathic cardiomyopathy.
Purpose of the Study:
- To evaluate cardiac function in BMD patients and female carriers using conventional echocardiography and pulsed Doppler tissue imaging (DTI).
- To assess the utility of pulsed DTI in identifying early diastolic and systolic alterations in individuals with BMD and carriers, particularly those with normal LVEF.
Main Methods:
- Conducted echocardiography and pulsed DTI on 28 BMD patients, 20 female carriers, and 38 controls.
- Analyzed left ventricular ejection fraction (LVEF), diastolic parameters (early filling peak, isovolumic relaxation time), and systolic parameters (peak systolic wave, time-velocity integral of S wave).
- Subgrouped BMD patients based on LVEF (A1: >=55%, A2: <55%).
Main Results:
- BMD patients and carriers exhibited lower LVEF compared to controls.
- Diastolic alterations, including prolonged isovolumic relaxation time, were observed in BMD patients and carriers.
- Pulsed DTI revealed significant early diastolic and systolic alterations in various cardiac segments in BMD patients and carriers, often preceding LVEF reduction.
Conclusions:
- Pulsed DTI is a valuable tool for assessing diastolic dysfunction in dystrophinopathic cardiomyopathy.
- Pulsed DTI shows promise in detecting early regional systolic alterations in BMD patients and carriers, even when LVEF is normal.
Abstract:
Cardiac dysfunction is a primary feature in patients and female carriers of Becker muscular dystrophy (BMD). Conventional echocardiography and pulsed Doppler tissue imaging (DTI) were performed in 28 patients with BMD, in 20 female carriers, and in 38 control participants (20 men and 18 women). Left ventricular ejection fraction (LVEF) was lower in BMD patients (P <.02) and carriers (P <.02) than in normal participants. Two subgroups of BMD patients were identified: A1 = LVEF > or = 55% (n = 20) and A2 = LVEF < 55% (n = 8). The carriers displayed LVEF > or = 55%. Compared with control participants the diastolic alterations by conventional echocardiography were lower early filling peak in the subgroup A1 (P <.05) and prolonged isovolumic relaxation time in A1, A2, and in carriers (at least P <.02). Furthermore, pulsed DTI showed lower early diastolic wave peak at basal septum in A2 (P <.05) and in carriers (P <.0001); at lateral mitral annulus and at basal inferior wall in A1, A2, and in carriers (at least P <.05); lower early/late diastolic wave ratio at basal septum and lateral mitral annulus (P <.05) in carriers; and prolonged isovolumic relaxation time in A1, A2, and in carriers (at least P <.02), except at lateral mitral annulus of carriers. Systolic parameters investigated by pulsed DTI detected lower peak systolic wave at basal septum in A1, A2, and carriers (P <.02); at lateral mitral annulus in carriers (P <.02); at basal inferior wall in A1, A2 (P <.02), and in carriers (P <.0001); and lower time-velocity integral of S wave at each segment in A1, A2, and in carriers. In dystrophinopathic cardiomyopathy, pulsed DTI may be a useful technique to assess diastolic dysfunction and appears to be a promising tool in identifying early regional systolic alterations in patients and carriers with normal LVEF.