Cardiac magnetic resonance findings in patients with type 1 myotonic dystrophy

Abstract Funding Acknowledgements Type of funding sources: None. Background Heart disease is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), second only to respiratory complications. Cardiac imaging, possibly including cardiac magnetic resonance (CMR), is recommended in patients with DM1. However, limited information is available on CMR findings and their prognostic significance in DM1. Methods We identified all patients with DM1 evaluated from 2009 to 2020 in a CMR laboratory with an established collaboration with a Neuromuscular Disorder Unit. Results Thirty-four patients were retrieved (21 males, aged 45 ± 12). At the time of CMR examination, 97% had neuromuscular symptoms (mean duration 16 ± 13 years), 12 (35%) presented with atrioventricular block (n = 11 1st degree, n = 1 2nd degree type 1), 15 (44%) with intraventricular conduction disturbances (n = 5 left bundle branch block, n = 5 right bundle branch block, n = 3 left anterior fascicular block, n = 2 other non-specific intraventricular conduction delay), 4 (12%) with atrial fibrillation or flutter. No patient had a device. At CMR, 5 (15%) patients had left ventricular (LV) systolic dysfunction (LV ejection fraction [LVEF] <50%) and 5 (15%) a depressed right ventricular (RV) function (RVEF <50%). Compared to age- and sex-specific reference values for our laboratory, 12 (35%) patients showed a decreased LV end-diastolic volume index (LVEDVi), 7 (21%) a decreased LV mass index (LVMi), and 29 (83%) a decreased LVMi/LVEDVi ratio. Nine (26%) patients had mid-wall late gadolinium enhancement (LGE, mean extent 4.5 ± 2.0% of LVM; n = 8 septal, n = 4 inferolateral, n = 2 inferior, n = 1 anterolateral), and 14 (40%) some areas of fatty infiltration (n = 9 involving the LV, n = 13 the RV). Native T1 in the interventricular septum (1,041 ± 53 ms) approached the upper reference limit (1,089 ms), and the extracellular volume was slightly increased (33 ± 2%, reference values <30%). Over a median follow-up of 3.3 years (interquartile interval 1.6-4.7), 2 (6%) patients died, one for infectious and respiratory complications and the other for unknown causes, 5 (15%) patients underwent pacemaker implantation for conduction disturbances, and 4 (12%) had a documentation of high-risk (Lown class ≥4) ventricular ectopic beats (VEBs). Among all CMR variables collected, higher values of LVMi/LVEDVi ratio emerged as univariate predictor of all-cause death (p = 0.044). At logistic regression analysis, anteroseptal wall thickness was associated with the need for pacemaker implantation (p = 0.028), while LGE mass was associated with high-risk VEBs (p = 0.026). Conclusions Patients with DM1 display several structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis and fatty infiltration. The possibility to predict the need for pacemaker implantation, ventricular arrhythmias and all-cause or cardiovascular mortality should be verified in larger cohorts.

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31 Cardiac magnetic resonance findings in patients with Type 1 myotonic dystrophy

European Heart Journal Supplements ◽

10.1093/eurheartj/suab132.030 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Marco Leali ◽

Alberto Aimo ◽

Giulia Ricci ◽

Giuseppe Vergaro ◽

Giancarlo Todiere ◽

...

Keyword(s):

Cardiac Magnetic Resonance ◽

High Risk ◽

Magnetic Resonance ◽

Myotonic Dystrophy ◽

Fatty Infiltration ◽

Respiratory Complications ◽

Bundle Branch Block ◽

Intraventricular Conduction ◽

Device Implantation

Abstract Aims Heart disease is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), second only to respiratory complications. Cardiac imaging, possibly including cardiac magnetic resonance (CMR), is recommended in patients with DM1. However, limited information is available on CMR findings and their prognostic significance in DM1. Methods and results We identified all patients with DM1 evaluated from 2009 to 2020 in a CMR laboratory with an established collaboration with a Neuromuscular Disorder Unit. Thirty-four patients were retrieved (21 males, aged 45 ± 12). By the time of CMR examination, 90% had neuromuscular symptoms (mean duration 17 ± 13 years), 13 (38%) had previous reports of atrioventricular block (n = 12 1st degree, n = 1 2nd degree type 1), 30 (88%) of intraventricular conduction disturbances (n = 5 left bundle branch block, n = 5 right bundle branch block, n = 3 left anterior fascicular block, n = 17 other non-specific or incomplete intraventricular conduction delay), 4 (12%) of atrial fibrillation or flutter. No patient had a device. At CMR, 5 (15%) patients had left ventricular (LV) systolic dysfunction [LV ejection fraction (LVEF) <50%] and 4 (12%) a depressed right ventricular (RV) function (RVEF <50%). Compared to age- and sex-specific reference values for our laboratory (Figure 1 left), 12 (35%) patients showed a decreased LV end-diastolic volume index (LVEDVi), 7 (21%) a decreased LV mass index (LVMi), and 29 (85%) a decreased LVMi/LVEDVi ratio. Nine (26%) patients had mid-wall late gadolinium enhancement (LGE, mean extent 4.5 ± 2.0% of LVM; n = 8 septal, n = 4 inferolateral, n = 2 inferior, n = 1 anterolateral, see Figure 1 middle), and 14 (41%) some areas of fatty infiltration (n = 9 involving the LV, n = 13 the RV). Native T1 in the interventricular septum (1,041 ± 53 ms) approached the upper reference limit (1089 ms), and the extracellular volume was slightly increased (33 ± 2%, reference values <30%). Over a median follow-up of 2.5 years (interquartile interval: 1.5–4.0), 2 (6%) patients died for infectious and respiratory complications, 5 (15%) underwent device implantation (n = 4 PM; n = 1 ICD), and 4 (12%) had a documentation of high-risk (Lown class ≥4) ventricular ectopic beats (VEBs). Among all CMR variables collected, higher values of LVMi/LVEDVi ratio emerged as univariate predictor of all-cause death (P = 0.044). At logistic regression analysis, anteroseptal wall thickness was associated with the need for device implantation (P = 0.028), while LGE mass was associated with high-risk VEBs (P = 0.026) (Figure 1 right). Conclusions Patients with DM1 display several structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis, and fatty infiltration. The possibility to predict the need for device implantation, ventricular arrhythmias, and all-cause or cardiovascular mortality should be verified in larger cohorts.

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Cardiac Magnetic Resonance Findings and Prognosis in Type 1 Myotonic Dystrophy

10.21203/rs.3.rs-1217399/v1 ◽

2022 ◽

Author(s):

Marco Leali ◽

Alberto Aimo ◽

Giulia Ricci ◽

Francesca Torri ◽

Giancarlo Todiere ◽

...

Keyword(s):

Cardiac Magnetic Resonance ◽

Magnetic Resonance ◽

Ejection Fraction ◽

Myotonic Dystrophy ◽

Fatty Infiltration ◽

Ventricular Ejection ◽

Volume Index ◽

Ventricular Ejection Fraction ◽

Device Implantation

Abstract Purpose Cardiac involvement is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), but limited information is available about myocardial remodelling and tissue changes. Aim of the study was to investigate cardiac magnetic resonance (CMR) findings and their prognostic significance in DM1. Methods We identified all DM1 patients referred from a neurology unit to our CMR laboratory from 2009 to 2020. Results Thirty-four patients were included (aged 45±12, 62% males). At CMR, 5(15%) had a left ventricular ejection fraction (LVEF)<50% and 4(12%) a right ventricular ejection fraction (RVEF)<50%. Compared to age- and sex-specific reference values, 12(35%) had a decreased end-diastolic volume index (LVEDVi), 7(21%) a decreased mass index (LVMi), and 29(85%) a reduced LVMi/LVEDVi. Nine (26%) showed mid-wall late gadolinium enhancement (LGE; 5±2% of LVM), and 14(41%) fatty infiltration. In a subset of 13(38%) patients, native T1 in the interventricular septum (1,041±53 ms) approached the upper reference limit (1,089 ms) and the extracellular volume was slightly increased (33±2%, reference<30%). Over 2.5(1.5-4.0) years, 2(6%) patients died for infectious and respiratory complications, 5(15%) underwent device implantation; 4/21(19%) with Holter developed repetitive ventricular ectopic beats (VEBs). Lower RV volumes (p=0.043), higher anteroseptal wall thickness (p=0.024) and LV fatty infiltration (p=0.029) were associated with device implantation, LGE mass was associated with VEBs (p=0.003) and death (p<0.001). Conclusion DM1 patients display structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis and fatty infiltration. Such changes, as evaluated by CMR, may anticipate the worsening of electrical disturbances.

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Cardiac Magnetic Resonance Predicts Outcome in Patients With Premature Ventricular Complexes of Left Bundle Branch Block Morphology

Journal of the American College of Cardiology ◽

10.1016/j.jacc.2010.03.087 ◽

2010 ◽

Vol 56 (15) ◽

pp. 1235-1243 ◽

Cited By ~ 57

Author(s):

Giovanni Donato Aquaro ◽

Alessandro Pingitore ◽

Elisabetta Strata ◽

Gianluca Di Bella ◽

Sabrina Molinaro ◽

...

Keyword(s):

Cardiac Magnetic Resonance ◽

Magnetic Resonance ◽

Left Bundle Branch Block ◽

Bundle Branch Block ◽

Premature Ventricular Complexes

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Pacemaker and conduction disturbances in patients with atrial septal defect

Cardiology in the Young ◽

10.1017/s1047951120001365 ◽

2020 ◽

Vol 30 (7) ◽

pp. 980-985 ◽

Cited By ~ 1

Author(s):

Diana H. R. Albæk ◽

Sebastian Udholm ◽

Anne-Sif L. Ovesen ◽

Zarmiga Karunanithi ◽

Camilla Nyboe ◽

...

Keyword(s):

Atrial Septal Defect ◽

Atrioventricular Block ◽

Right Bundle Branch Block ◽

Septal Defect ◽

Pacemaker Implantation ◽

Bundle Branch Block ◽

Conduction Disturbances ◽

Septal Defects ◽

Atrial Septal Defects ◽

Background Population

AbstractObjective:To determine the prevalence of pacemaker and conduction disturbances in patients with atrial septal defects.Design:All patients with an atrial septal defect born before 1994 were identified in the Danish National Patient Registry, and 297 patients were analysed for atrioventricular block, bradycardia, right bundle branch block, left anterior fascicular block, left posterior fascicular block, pacemaker, and mortality. Our results were compared with pre-existing data from a healthy background population. Further, outcomes were compared between patients with open atrial septal defects and atrial septal defects closed by surgery or transcatheter.Results:Most frequent findings were incomplete right bundle branch block (40.1%), left anterior fascicular block (3.7%), atrioventricular block (3.7%), and pacemaker (3.7%). Average age at pacemaker implantation was 32 years. Patients with defects closed surgically or by transcatheter had an increased prevalence of atrioventricular block (p < 0.01), incomplete right bundle branch block (p < 0.01), and left anterior fascicular block (p = 0.02) when compared to patients with unclosed atrial septal defects. At age above 25 years, there was a considerably higher prevalence of atrioventricular block (9.4% versus 0.1%) and complete right bundle branch block (1.9% versus 0.4%) when compared to the background cohorts.Conclusions:Patients with atrial septal defects have a considerably higher prevalence of conduction abnormalities when compared to the background population. Patients with surgically or transcatheter closed atrial septal defects demonstrated a higher demand for pacemaker and a higher prevalence of atrioventricular block, incomplete right bundle branch block, and left anterior fascicular block when compared to patients with unclosed atrial septal defects.

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Late onset of new conduction disturbances requiring permanent pacemaker implantation following TAVI

Heart ◽

10.1136/heartjnl-2019-315967 ◽

2020 ◽

Vol 106 (16) ◽

pp. 1244-1251 ◽

Cited By ~ 2

Author(s):

Nynke H M Kooistra ◽

Martijn S van Mourik ◽

Ramón Rodríguez-Olivares ◽

Alexander H Maass ◽

Vincent J Nijenhuis ◽

...

Keyword(s):

Risk Factors ◽

Late Onset ◽

Pacemaker Implantation ◽

Early Discharge ◽

Permanent Pacemaker ◽

Conduction Delay ◽

Intraventricular Conduction ◽

Conduction Disturbances ◽

Permanent Pacemaker Implantation ◽

Timing Of Onset

BackgroundThe timing of onset and associated predictors of late new conduction disturbances (CDs) leading to permanent pacemaker implantation (PPI) following transcatheter aortic valve implantation (TAVI) are still unknown, however, essential for an early and safe discharge. This study aimed to investigate the timing of onset and associated predictors of late onset CDs in patients requiring PPI (LCP) following TAVI.Methods and resultsWe performed retrospective analysis of prospectively collected data from five large volume centres in Europe. Post-TAVI electrocardiograms and telemetry data were evaluated in patients with a PPI post-TAVI to identify the onset of new advanced CDs. Early onset CDs were defined as within 48 hours after procedure, and late onset CDs as after 48 hours. A total of 2804 patients were included for analysis. The PPI rate was 12%, of which 18% was due to late onset CDs (>48 hours). Independent predictors for LCP were pre-existing non-specific intraventricular conduction delay, pre-existing right bundle branch block, self-expandable valves and predilation. At least one of these risk factors was present in 98% of patients with LCP. Patients with a balloon-expandable valve without predilation did not develop CDs requiring PPI after 48 hours.ConclusionsSafe early discharge might be feasible in patients without CDs in the first 48 hours after TAVI if no risk factors for LCP are present.

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