Impaired myocardial strain in early stage of Duchenne muscular dystrophy: its relation with age and motor performance

Lilia Oreto 1,2, Gian Luca Vita 3, Giuseppe Mandraffino 1,4, Scipione Carerj 2, Maria Pia Calabrò 5, Roberta Manganaro 2, Maurizio Cusmà-Piccione 2, Maria Chiara Todaro 2,6, Maria Sframeli 3, Maria Cinquegrani 4, Antonio Toscano 7, Giuseppe Vita 3,7, Sonia Messina 3,7, Concetta Zito 2

1 Mediterranean Pediatric Cardiologic Centre, S. Vincenzo Hospital, Taormina – “Bambin Gesù”, Rome, Italy; 2 Cardiology Unit, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy; 3 Nemo Sud Clinical Centre for Neuromuscular Disorders, Messina University Hospital, Messina, Italy; 4 Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Messina, Italy; 5 Pediatric Unit, Department of Human Pathology in Adult and Developmental Age, University of Messina, Italy; 6 Cardiology Unit, Papardo Hospital, Messina, Italy; 7 Unit of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Italy L.O. and G.L.V. are both first authors S.M. and C.Z. are both senior authors

Duchenne muscular dystrophy (DMD) is complicated by an early and progressive left ventricular (LV) dysfunction. Despite the reduction of ejection fraction (EF) usually manifests in the second decade, subtle alterations in LV mechanics can be detected earlier. Longitudinal and circumferential LV deformation, evaluated by speckle tracking echocardiography (STE), are considered sensitive markers of early dysfunction. We retrospectively examined clinical and echocardiographic data of 32 DMD children with preserved LV function. According to the median age, patients were then divided into younger and older than 9 years, and compared to 24 age-matched healthy subjects. Six-minute-walk test (6MWT), North Star Ambulatory Assessment (NSAA), and a comprehensive cardiac evaluation were performed. Although EF was within the normal range, DMD patients had significantly lower values than healthy controls, and the same occurred for the remaining conventional systolic and diastolic indices. Global longitudinal strain (GLS) was reduced in all patients (older and younger, both p < 0.001). Global circumferential strain (GCS) was reduced only in older patients (< 0.001). Both GLS and GCS worsened with age in DMD patients (GLS p = 0.005; GCS p = 0.024). GLS was significantly worse in the apical segments and in the postero-lateral wall. GCS in the antero-septal, anterior and antero-lateral segments was significantly reduced in older patients, with a prevalent involvement of the sole septal wall in the younger boys. 6MWT appeared to be correlated inversely to GLS and directly to EF. A longitudinal evaluation should be scheduled in DMD boys to assess the global cardiac performance over time and to evaluate the impact of therapies.

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