scholarly journals Left ventricular fibro-fatty replacement in arrhythmogenic right ventricular dysplasia/cardiomyopathy: prevalence, patterns, and association with arrhythmias

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Tarek Zghaib ◽  
Anneline S. J. M. Te Riele ◽  
Cynthia A. James ◽  
Neda Rastegar ◽  
Brittney Murray ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Wall Motion ◽  
Task Force ◽  
Fatty Infiltration ◽  
Regional Wall Motion ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Genetic Mutation ◽  
Left Ventricular ◽  
Fatty Replacement ◽  
Structural Disease

Abstract Background Left ventricular (LV) fibrofatty infiltration in arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) has been reported, however, detailed cardiovascular magnetic resonance (CMR) characteristics and association with outcomes are uncertain. We aim to describe LV findings on CMR in ARVD/C patients and their relationship with arrhythmic outcomes. Methods CMR of 73 subjects with ARVD/C according to the 2010 Task Force Criteria (TFC) were analyzed for LV involvement, defined as ≥ 1 of the following features: LV wall motion abnormality, LV late gadolinium enhancement (LGE), LV fat infiltration, or LV ejection fraction (LVEF) < 50%. Ventricular volumes and function, regional wall motion abnormalities, and the presence of ventricular fat or fibrosis were recorded. Findings on CMR were correlated with arrhythmic outcomes. Results Of the 73 subjects, 50.7% had CMR evidence for LV involvement. Proband status and advanced RV dysfunction were independently associated with LV abnormalities. The most common pattern of LV involvement was focal fatty infiltration in the sub-epicardium of the apicolateral LV with a “bite-like” pattern. LGE in the LV was found in the same distribution and most often had a linear appearance. LV involvement was more common with non-PKP2 genetic mutation variants, regardless of proband status. Only RV structural disease on CMR (HR 3.47, 95% CI 1.13–10.70) and prior arrhythmia (HR 2.85, 95% CI 1.33–6.10) were independently associated with arrhythmic events. Conclusion Among patients with 2010 TFC for ARVD/C, CMR evidence for LV abnormalities are seen in half of patients and typically manifest as fibrofatty infiltration in the subepicardium of the apicolateral wall and are not associated with arrhythmic outcomes.

scholarly journals Characteristics of Patients With Arrhythmogenic Left Ventricular Cardiomyopathy

2020 ◽  
Vol 13 (12) ◽  
Author(s):  
Michela Casella ◽  
Alessio Gasperetti ◽  
Rita Sicuso ◽  
Edoardo Conte ◽  
Valentina Catto ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Genetic Variants ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
Voltage Mapping ◽  
Fatty Replacement

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce. Methods: Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV. Results: Twenty-five patients ALVC (53 [48–59] years, 60% male) were enrolled. T wave inversion in infero-lateral and left precordial leads were the most common ECG abnormalities. Overall arrhythmic burden at study inclusion was 56%. Cardiac magnetic resonance showed LV late gadolinium enhancement in the LV lateral and posterior basal segments in all patients. In 72% of the patients an invasive evaluation was performed, in which electroanatomical voltage mapping and electroanatomical voltage mapping-guided endomyocardial biopsy showed low endocardial voltages and fibro-fatty replacement in areas of late gadolinium enhancement presence. Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were identified in 12/25 of the cohort presenting pathogenic/likely pathogenic variants. A definite/borderline 2010 International Task Force Criteria arrhythmogenic right ventricular cardiomyopathy diagnosis was reached only in 11/25 patients. Conclusions: ALVC presents with a preferential involvement of the lateral and postero-lateral basal LV and is associated mostly with variants in desmoplakin and desmoglein-2 genes. An amendment to the current International Task Force Criteria is reasonable to better diagnose patients with ALVC.

scholarly journals Heart MRI Images Analysis In Case Of Arrythmogenic Right Ventricular Cardiomyopathy (ARVC)

2018 ◽  
Vol 9 (2) ◽  
pp. 135-142
Author(s):  
Sriyatun Sriyatun ◽  
Gando Sari ◽  
Nursama Heru Apriantoro
Keyword(s):  
Right Ventricular ◽  
Diagnostic Criteria ◽  
Cardiac Mri ◽  
Wall Motion ◽  
Task Force ◽  
Fatty Infiltration ◽  
Black Blood ◽  
Ventricular Cardiomyopathy ◽  
Mri Examination ◽  
Wall Motion Abnormalities

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a cause of sudden cardiac death in otherwise healthy young adults. (ARVD/C) is an inherited cardiomyopathy characterized by life-threatening ventricular arrhythmias and slowly progressive ventricular dysfunction. To diagnose ARVC is indicated by the Task Force of ARVC criteria in 1994 and then revised in 2010 set by the WHO/ISFC. To identify clinical characteristics of ARVC using CMR. The design of this study was qualitative descriptive, which was done by the observational method using MRI 1.5 Tesla. The subject was an adult patient who did Cardiac MRI examination in National Cardiovascular Centre Harapan Kita in March 2018. The collected data were diagnostic images of CMR sequence. Then, it compared with the diagnostic criteria of ARVC which was indicated by Task Force. The result of this study of several ARVC diagnostic criteria in Cardiac MRI examination with T1 Black Blood sequence is clear and accurate in indicating the presence of fat infiltration, CINE sequence is clear in visualizing the dilatation in the right ventricle, wall motion abnormalities, accordion sign, bulging, and LGE clearly shows fibrosis. The detection of right ventricular enlargement, fatty infiltration, fibrosis, and wall motion abnormalities in CMR is useful in the diagnosis of ARVC.

scholarly journals Magnetic Resonance Detects Structural Heart Disease in Patients with Frequent Ventricular Ectopy and Normal Echocardiographic Findings

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1505
Author(s):  
Raffaele Scorza ◽  
Anders Jansson ◽  
Peder Sörensson ◽  
Mårten Rosenqvist ◽  
Viveka Frykman
Keyword(s):  
Magnetic Resonance ◽  
Right Ventricular ◽  
Wall Motion ◽  
Regional Wall Motion ◽  
Left Ventricular ◽  
Ventricular Ectopy ◽  
Premature Ventricular Contractions ◽  
Regional Wall ◽  
Regional Wall Motion Abnormalities ◽  
Wall Motion Abnormalities

The prognosis of patients with ventricular ectopy and a normal heart, as evaluated by echocardiography, is virtually unknown. Cardiac magnetic resonance (CMR) can detect focal ventricular anomalies that could act as a possible site of origin for premature ventricular contractions (PVCs). The aim of this study was to investigate the presence of cardiac anomalies in patients with normal findings at echocardiogram. Methods: Fifty-one consecutive patients (23 women, 28 men, mean age 59 years) with very high PVC burden (>10,000 PVC/day) and normal findings at standard echocardiography and exercise test were examined with CMR. The outcome was pathologic findings, defined as impaired ejection fraction, regional wall motion abnormalities, abnormal ventricular volume, myocardial edema and fibrosis. Results: Sixteen out of 51 patients (32%) had structural ventricular abnormalities at CMR. In five patients CMR showed impairment of the left ventricular and/or right ventricular systolic function, and six patients had a dilated left and/or right ventricle. Regional wall motion abnormalities were seen in six patients and fibrosis in four. No patient had CMR signs of edema or met CMR criteria for arrhythmogenic right ventricular cardiomyopathy. Five patients had extra-ventricular findings (enlarged atria in three cases, enlarged thoracic aorta in one case and pericardial effusion in one case). Conclusions: In this study 16 out of 51 patients with a high PVC burden and normal findings at echocardiography showed signs of pathology in the ventricles with CMR. These findings indicate that CMR should be considered in evaluating patients with a high PVC burden and a normal standard investigation.

Assessment of interventricular septal motion by cross-sectional echocardiography in patients with isolated atrial septal defects within the oval fossa

1992 ◽  
Vol 2 (1) ◽  
pp. 30-34
Author(s):  
Michael Vogel ◽  
Karin Schulze ◽  
Konrad Bühlmeyer
Keyword(s):  
Right Ventricular ◽  
Atrial Septal Defect ◽  
Wall Motion ◽  
Septal Defect ◽  
Regional Wall Motion ◽  
Ventricular Volume ◽  
Left Ventricular ◽  
Right Ventricular Volume ◽  
Regional Wall ◽  
Floating System

SummaryTo assess whether paradoxical (systolic anterior) motion of the interventricular septum is a true abnormality of septal contraction caused by right ventricular volume overload, we examined the regional pattern of left ventricular contraction in 20 patients with an atrial septal defect within the oval fossa using a fixed and floating reference system of analysis of wall motion. The patients, with a median age of 6.8 (3.5–13.4) years, had a Qp/Qs ratio of 3.5:1 (1.2:1–8:1). They were studied by cross-sectional echocardiography four days (3–5) before and 14.5 (3–67) days after surgical closure of the atrial septal defect. The postoperative Qp/Qs ratio was 1.1:1 (0.9:1–2:1). Regional wall motion of the left ventricle was analyzed in the parasternal short axis view at the level of the mitral valve and papillary muscles as well as in the apical four-chamber view. The endocardium was digitized manually in end-systolic and end-diastolic frames. The center of mass of this figure was calculated and connected to an outside reference point in the right ventricle. With the floating system, both centers of mass and the reference lines were superimposed, thus correcting for movement of the heart inside the thorax. With the fixed system,both end-diastolic and end-systolic frames were measured separately without correcting for movement of the heart. The left ventricle was divided in eight segments in a clockwise fashion and regional change in area was measured and compared to 40 normal age matched controls. With the floating system, left ventricular regional wall motion was normal in all patients before and after closure of the atrial septal defect and, thus, was not influenced by the change in right ventricular volume load. With the fixed system, both before and after surgical closure of the atrial septal defect, left ventricular regional wall motion was reduced in the two segments representing the basilar and middle portion of the interventricular septum. The floating system of analysis of left ventricular regional wall motion has the ability to correct for movement of the heart, which makes the supposed “abnormal” systolic anterior motion in right ventricular volume overload, a condition prevalent in patients with atrial septal defect, disappear. Thus, we conclude that the so-called paradoxical septal motion in atrial defect is an artifact. This may be caused by an increased motion of the heart but is unrelated to the volume load of the right ventricle.

Abstract 2838: Sequential Changes of Left Ventricular Systolic and Diastolic Synchronicity in Acute ST-Elevation Myocardial Infarction after Primary Coronary Intervention

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Seong-Mi Park ◽  
Yong-Hyun Kim ◽  
Soon-Jun Hong ◽  
Do-Sun Lim ◽  
Wan-Joo Shim
Keyword(s):  
Myocardial Infarction ◽  
Wall Motion ◽  
Chronic Phase ◽  
Regional Wall Motion ◽  
Coronary Intervention ◽  
Left Ventricular ◽  
Doppler Imaging ◽  
Regional Wall ◽  
St Elevation

The aims of this study were to assess the sequential changes of left ventricular (LV) systolic and diastolic synchronicity in patients with acute myocardial infarction (AMI) and to assess their relation with LV recovery and remodeling. Forty-patients with acute ST-elevation MI were examined within 2days, 6weeks and 6months after primary coronary intervention. Fifteen-age matched subjects were enrolled for normal control. The time from the onset of QRS complex to peak systolic velocity (Ts) and to peak early diastolic velocity (Te) were measured on color-coded tissue Doppler imaging. To assess LV synchronicity, SDs of Ts (Ts-SD) and Te (Te-SD) of all 12 segments were calculated (within 2days, at 6weeks and 6months; SD1, SD2 and SD3, respectively). LV recovery was defined as the improvement of wall motion at least more than two segments at 6 weeks. In all AMI patients, the wall motion score index was 1.72±0.27 and LV ejection fraction was 45.9±9.9%. The Ts-SD1 was higher in AMI patients than in controls (45.4±13.5 vs 29.4±13.3ms, p<0.05), but Te-SD1 was not different (18.7±6.9 vs 16.2±10.0). Twenty-two patients (group1) showed a recovery and 18 patients (group2) showed no recovery. The Ts-SD1 was smaller in group1 than in group2 (43.4±12.6 vs 47.9±11.7 ms, p<0.05). In group1, Ts-SD were much decreased as follow up (Ts-SD2, 3; 36.6±14.0 and 31.1±9.5, respectively, p<0.05). In contrast, in group2, Ts-SD was not significantly changed (Ts-SD2,3; 46.7±13.2 and 43.7±8.8, respectively) but Te-SD was increased as follow up (Te-SD1,2,3; 17.8±5.5, 20.4±4.3 and 25.0±3.8, respectively, p<0.05). The LV end-diastolic and systolic volume were increased and the deceleration time of early diastolic mitral inflow velocity was shortened in group2 (p<0.05). This clinical study shows: 1) in acute phase, the regional wall motion abnormalities of AMI had an impact on LV systolic synchronicity; 2) the AMI patients with LV recovery showed better LV systolic synchronicity; 3) the LV systolic synchronicity became better as regional wall motion was improved; and 4) in chronic phase, the LV diastolic synchronicity became worse in AMI patients with no recovery, which might be related to LV remodeling and worsening of LV diastolic function.

scholarly journals Takotsubo Cardiomyopathy in a Squash Player

2009 ◽  
Vol 2009 ◽  
pp. 1-4 ◽  
Author(s):  
Mathieu Berry ◽  
Jerome Roncalli ◽  
Olivier Lairez ◽  
Meyer Elbaz ◽  
Didier Carrié ◽  
...  
Keyword(s):  
Takotsubo Cardiomyopathy ◽  
Wall Motion ◽  
Ventricular Dysfunction ◽  
Apical Ballooning ◽  
Regional Wall Motion ◽  
Left Ventricular ◽  
The Body ◽  
Coronary Syndrome ◽  
Regional Wall Motion Abnormalities ◽  
Wall Motion Abnormalities

Takotsubo cardiomyopathy is usually described following acute emotional stress. We report here the case of a 48-year-old woman admitted for acute coronary syndrome after an intensive squash match. Diagnosis of Takotsubo cardiomyopathy due to acute physical stress was suspected in presence of normal coronary arteries and transitory left ventricular dysfunction with typical apical ballooning. Cardiac magnetic resonance imaging confirmed regional wall-motion abnormalities and was helpful in excluding myocardial infarction diagnosis. During squash the body is subject to sudden and vigorous demands inducing a prolonged and severe workload on the myocardium.

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