Raffaele Falsaperla1, Giovanna Vitaliti1, Ausilia Desiree Collotta1, Chiara Fiorillo2, Alfredo Pulvirenti3, Salvatore Alaimo3, Catia Romano1, Martino Ruggieri4. 1. 1 General Pediatrics and Pediatric Acute and Emergency Complex Unit, Policlinico-Vittorio-Emanuele University Hospital, University of Catania, Italy. 2. 2 Unit of Paediatric Neurology and Muscle Diseases, G. Gaslini Institute, Genoa, Italy. 3. 3 Department of Clinical and Experimental Medicine, Section of Bioinformatics, University of Catania, Italy. 4. 4 Unit of Rare Diseases of the Nervous System in Childhood, Policlinico-Vittorio-Emanuele University Hospital, University of Catania, Italy.
Abstract
BACKGROUND: This study aimed to show the impairment of autonomic cardiac conduction causing bradycardia and/or electrocardiographic alterations in children affected by spinal muscular atrophy type 1 and 2 (SMA 1 and 2). METHODS: We included 25 spinal muscular atrophy patients, admitted from November 2016 to May 2017. All patients underwent an electrocardiographic examination and we studied PR and QRS intervals, P-waves and QRS amplitudes, and heart rate in spinal muscular atrophy patients compared to a control group. RESULTS: In all patients, we found longer PRi and QRSi ( P < .05), lower P-wave and QRS complex amplitudes ( P < .01), and a decreased heart rate ( P < .01) with respect to controls. When we divided our patients into SMA1 and SMA2 subgroups, we found that statistical differences were maintained for P-wave and QRS complex amplitudes and heart rate, but not for PRi and QRSi with respect to controls. CONCLUSION: We suggest the hypothesis of SMN expression on cardiac tissue condition and/or autonomic cardiac conduction.
BACKGROUND: This study aimed to show the impairment of autonomic cardiac conduction causing bradycardia and/or electrocardiographic alterations in children affected by spinal muscular atrophy type 1 and 2 (SMA 1 and 2). METHODS: We included 25 spinal muscular atrophypatients, admitted from November 2016 to May 2017. All patients underwent an electrocardiographic examination and we studied PR and QRS intervals, P-waves and QRS amplitudes, and heart rate in spinal muscular atrophypatients compared to a control group. RESULTS: In all patients, we found longer PRi and QRSi ( P < .05), lower P-wave and QRS complex amplitudes ( P < .01), and a decreased heart rate ( P < .01) with respect to controls. When we divided our patients into SMA1 and SMA2 subgroups, we found that statistical differences were maintained for P-wave and QRS complex amplitudes and heart rate, but not for PRi and QRSi with respect to controls. CONCLUSION: We suggest the hypothesis of SMN expression on cardiac tissue condition and/or autonomic cardiac conduction.
Authors: René Günther; Claudia Diana Wurster; Isabell Cordts; Jan Christoph Koch; Christoph Kamm; Daniel Petzold; Elisa Aust; Marcus Deschauer; Paul Lingor; Albert Christian Ludolph; Andreas Hermann Journal: Front Neurol Date: 2019-11-01 Impact factor: 4.003
Authors: Guzal Khayrullina; Kasey E Moritz; James F Schooley; Naheed Fatima; Coralie Viollet; Nikki M McCormack; Jeremy T Smyth; Martin L Doughty; Clifton L Dalgard; Thomas P Flagg; Barrington G Burnett Journal: Skelet Muscle Date: 2020-05-08 Impact factor: 4.912