Dataset Information

Radial and Circumferential CMR-Based RV Strain Predicts Low R Wave Amplitude after ICD Implantation in Patients with Arrhythmogenic Cardiomyopathy.

ABSTRACT: Inadequate R wave amplitude (RWA) after implantable cardiac defibrillator (ICD) implantation in patients with arrhythmogenic cardiomyopathy (ACM) was suspected to relate to right ventricle impairment. However, little data-based evidence was provided to quantify the association. We retrospectively enrolled ACM patients receiving CMR examinations before transvenous ICD implantation from Fuwai Hospital. The RWA was obtained within 24 h and at 2-6-month follow-up after the operation. Structural, functional, as well as tissue characterization of the left ventricle (LV) and right ventricle (RV), were analyzed in relation to RWA. Among the 87 ACM patients (median RWA: 8.0 mV), 19 (21.8%) patients were found with low initial RWA (<5 mV) despite attempts in multiple positions. RV end diastolic diameter (RVEDD), (r = -0.44), RV ejection fraction (RVEF, r = 0.43), RV end diastolic volume index (RVEDVi, r = -0.49), RV end systolic volume index (RVESVi, r = -0.53), RV global circumferential (RVGCS, r = -0.64), and radial strain (RVGRS, r = 0.61, all p < 0.001) rather than LV metrics correlated strongly with initial RWA. RVGCS, RVESVi, and RVGRS were decent predictors of low RWA (areas under the curve AUC: 0.814, 0.769, 0.757, respectively) early after implantation and during 2-6-month follow-up. To summarize, low RWA of ICD lead in ACM patients was associated with RV abnormalities. The RVGCS, RVGRS, and RVESVi can be valuable predictors for identifying low RWA prior to ICD implantation.

SUBMITTER: Chen Z

PROVIDER: S-EPMC9917584 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature

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Radial and Circumferential CMR-Based RV Strain Predicts Low R Wave Amplitude after ICD Implantation in Patients with Arrhythmogenic Cardiomyopathy.

Chen Zhongli Z Song Yanyan Y Chen Liang L Ma Xuan X Dai Yan Y Zhao Shihua S Chen Keping K Zhang Shu S

Journal of clinical medicine 20230122 3

Inadequate R wave amplitude (RWA) after implantable cardiac defibrillator (ICD) implantation in patients with arrhythmogenic cardiomyopathy (ACM) was suspected to relate to right ventricle impairment. However, little data-based evidence was provided to quantify the association. We retrospectively enrolled ACM patients receiving CMR examinations before transvenous ICD implantation from Fuwai Hospital. The RWA was obtained within 24 h and at 2-6-month follow-up after the operation. Structural, fun ...[more]

PMID: 36769534

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Project description:AimsTo investigate the right ventricular (RV) strain in pectus excavatum (PE) patients using cardiac magnetic resonance tissue tracking (CMR TT).Materials and methodsFifty consecutive pectus excavatum patients, 10 to 32 years of age (mean age 15 ± 4 years), underwent routine cardiac magnetic resonance imaging (CMR) including standard measures of chest geometry and cardiac size and function. The control group consisted of 20 healthy patients with a mean age of 17 ± 5 years. RV longitudinal and circumferential strain magnitude was assessed by a dedicated RV tissue tracking software.ResultsFifty patients with images of sufficient quality were included in the analysis. The mean right and left ventricular ejection fractions were 55 ± 5% and 59 ± 4%. The RV global longitudinal strain was -21.88 ± 4.63%. The RV circumferential strain at base, mid-cavity and apex were -13.66 ± 3.09%, -11.31 ± 2.79%, -20.73 ± 3.45%, respectively. There was no statistically significant decrease in right ventricular or left ventricular ejection fraction between patients and controls (p > 0.05 for each). There was no significant difference in RV global longitudinal strain between two groups (-21.88 ± 4.63 versus -21.99 ± 3.58; p = 0.93). However, there was significant decrease in mid-cavity circumferential strain magnitude in pectus patients compared with controls (-11.31 ± 2.79 versus -16.19 ± 2.86; p < 0.001). PE patients had a significantly higher basal circumferential strain (-13.66 ± 3.09% versus -9.76 ± 1.79; p < 0.001) as well as apical circumferential strain (-20.73 ± 3.45% versus -12.07 ± 3.38) than control group.ConclusionMid-cavity circumferential strain but not longitudinal strain is reduced in pectus excavatum patients. Basal circumferential strain as well as apical circumferential strain were increased as compensatory mechanism for reduced mid-cavity circumferential strain. Further studies are needed to establish clinical significance of this finding.

Project description:Arrhythmogenic cardiomyopathy (AC) is a heart muscle disease clinically characterized by life-threatening ventricular arrhythmias and pathologically by an acquired and progressive dystrophy of the ventricular myocardium with fibro-fatty replacement. Due to an estimated prevalence of 1:2000-1:5000, AC is listed among rare diseases. A familial background consistent with an autosomal-dominant trait of inheritance is present in most of AC patients; recessive variants have also been reported, either or not associated with palmoplantar keratoderma and woolly hair. AC-causing genes mostly encode major components of the cardiac desmosome and up to 50% of AC probands harbor mutations in one of them. Mutations in non-desmosomal genes have been also described in a minority of AC patients, predisposing to the same or an overlapping disease phenotype. Compound/digenic heterozygosity was identified in up to 25% of AC-causing desmosomal gene mutation carriers, in part explaining the phenotypic variability. Abnormal trafficking of intercellular proteins to the intercalated discs of cardiomyocytes and Wnt/beta catenin and Hippo signaling pathways have been implicated in disease pathogenesis.AC is a major cause of sudden death in the young and in athletes. The clinical picture may include a sub-clinical phase; an overt electrical disorder; and right ventricular or biventricular pump failure. Ventricular fibrillation can occur at any stage. Genotype-phenotype correlation studies led to identify biventricular and dominant left ventricular variants, thus supporting the use of the broader term AC.Since there is no "gold standard" to reach the diagnosis of AC, multiple categories of diagnostic information have been combined and the criteria recently updated, to improve diagnostic sensitivity while maintaining specificity. Among diagnostic tools, contrast enhanced cardiac magnetic resonance is playing a major role in detecting left dominant forms of AC, even preceding morpho-functional abnormalities. The main differential diagnoses are idiopathic right ventricular outflow tract tachycardia, myocarditis, sarcoidosis, dilated cardiomyopathy, right ventricular infarction, congenital heart diseases with right ventricular overload and athlete heart. A positive genetic test in the affected AC proband allows early identification of asymptomatic carriers by cascade genetic screening of family members. Risk stratification remains a major clinical challenge and antiarrhythmic drugs, catheter ablation and implantable cardioverter defibrillator are the currently available therapeutic tools. Sport disqualification is life-saving, since effort is a major trigger not only of electrical instability but also of disease onset and progression. We review the current knowledge of this rare cardiomyopathy, suggesting a flowchart for primary care clinicians and geneticists.

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Project description:Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder characterised by progressive fibrosis predominantly of the right ventricular (RV) myocardium, resulting in life-threatening arrhythmias and heart failure. The diagnosis is challenging due to a wide spectrum of clinical symptoms. The important role of ECG was covered in the current diagnostic criteria. The role of the epsilon wave (EW) is still under discussion. Aim: The aim of the study was to examine a potential association between the EW and late ventricular potentials (LPs) in ARVC patients (pts). The correlation between RV dilatation or dysfunction and LPs/EW was also analysed. Methods: The ARVC group consisted of 81 pts (53 men, aged 20-78 years) fulfilling 2010 International Task Force Criteria. 12-lead ECG, LPs, Holter, and ECHO were performed in all pts. The presence of EW was analysed in ECG by 3 investigators. LPs were detected by signal-averaged ECG (SAECG). SAECG was considered positive for LPs when at least two of the three following criteria were met: (1) the filtered QRS duration (fQRS) ≥ 114 msec; (2) the duration of the final QRS fragment in which low-amplitude signals lower than 40 μV are recorded (LAS-40 > 38 msec); and (3) the root mean square amplitude of the last 40 milliseconds of the fQRS complex (RMS-40 < 20 μV). The results were compared with a reference group consisting of 53 patients with RV damage in the course of atrial septum defect (ASD) or Ebstein's Anomaly (EA). Results: In the ARVC group, a significant relationship was observed between the occurrence of EW and the presence of LPs. EW was more common in the LP+ than in the LP- patients (48.1% vs. 6.9%, p < 0001; OR 12.5; 95% CI [2.691-58.063]). In ARVC pts, RVOT > 36 mm, RVIT > 41 mm, and RV S' < 9 cm/s were observed significantly more often in the LPs+ than in the LPs- group (OR [95% CI]: 8.3 [2.9-1.5], 6.4 [2.2-19.0] and 3.6 [1.1-12.2], respectively). In the ARVC group, any of fQRS > 114 ms, LAS > 38 ms, and RMS < 20 μV were significantly more frequent in EW+ pts. In multivariate analysis, the independent factors of the EW were LAS-40 and RV S'. In the LPs- subgroup, RVOT > 36 mm was more frequent in ASD/EA than in ARVC (70.4% vs. 25%, p = 0.002). Similarly, in the LPs- subgroup, RVIT > 41 mm was encountered more frequently in ASD/EA than in ARVC (85.2% vs. 48.3%, p = 0.004). Conclusions: In ARVC, there is an association between EW and LPs, with both probably resulting from the same process of fibrofatty substitution of the RV myocardium. Although RV dilatation is common in ASD and EA, it does not correlate with LPs.

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Dataset Information

Radial and Circumferential CMR-Based RV Strain Predicts Low R Wave Amplitude after ICD Implantation in Patients with Arrhythmogenic Cardiomyopathy.

Publications

Radial and Circumferential CMR-Based RV Strain Predicts Low R Wave Amplitude after ICD Implantation in Patients with Arrhythmogenic Cardiomyopathy.

Similar Datasets

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