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An explorative study into the possible relationship between electrical and structural abnormalities in subclinical and clinical PKP2-pathogenic mutation carriers by body surface potential mapping and echocardiographic deformation imaging : "Towards early detection and risk stratification of arrhythmogenic cardiomyopathy”

Kloosterman, M. (2021) An explorative study into the possible relationship between electrical and structural abnormalities in subclinical and clinical PKP2-pathogenic mutation carriers by body surface potential mapping and echocardiographic deformation imaging : "Towards early detection and risk stratification of arrhythmogenic cardiomyopathy”.

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Abstract:Arrhythmogenic cardiomyopathy (ACM) is a progressive inherited heart disease. Early detection of disease onset and risk stratification are challenging due to the heterogenous disease expression. In previous studies, abnormal RV deformation was observed in asymptomatic pathogenic mutation carriers (PMC) without any signs of disease on the 12-lead ECG. But, electrical abnormalities are expected to precede structural abnormalities. 67-lead body surface maps (BSM) were obtained in 25 controls and 35 PKP2-PMC of whom 24 without definite diagnosis (TFC<4). Subject specific CT/MRI based heart/torso models were created. Isopotential maps were created every 2ms (QRS) and 5ms (STT). Echocardiographic RV deformation patterns were obtained in 20 controls and 35 PKP2-PMC. Compared to controls, BSM identified three depolarization patterns and two repolarization patterns in 24/35 PMC, even in 15/24 PMC with TFC<4. The most common observation (29%) was a maximum during the second half of depolarization on the right side of the chest. This depolarization pattern was observed among different disease stages of ACM, even in 4/10 PMC with normal RV deformation. BSM reveals distinct de- and repolarization patterns in PKP2-PMC and may identify early electrical signs of disease in asymptomatic PMC without detectable structural abnormalities. Thus, BSM may aid subject specific risk stratification.
Item Type:Essay (Master)
Faculty:TNW: Science and Technology
Subject:44 medicine, 50 technical science in general
Programme:Technical Medicine MSc (60033)
Link to this item:https://purl.utwente.nl/essays/89166
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