scholarly journals 423 Prevalence and prognostic significance of atrial arrhythmias in arrhythmogenic cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Antonio De Luca ◽  
Chiara Cappelletto ◽  
Simona Romani ◽  
Maria Perotto ◽  
Caterina Gregorio ◽  
...  
Keyword(s):  
Cumulative Incidence ◽  
Task Force ◽  
Function Analysis ◽  
Nyha Class ◽  
Prognostic Significance ◽  
Muscle Disease ◽  
Atrial Arrhythmias ◽  
Arrhythmogenic Cardiomyopathy ◽  
Long Term Study

Abstract Aims Limited data are available concerning atrial arrhythmias (AA) role in arrhythmogenic cardiomyopathy (AC). The aim of the present study was to assess the prevalence and incidence of AA in a large cohort of AC patients and to evaluate its association with clinical outcomes. Methods and results We retrospectively analysed 115 patients with definite diagnosis of ARVC, according to 2010 Task Force Criteria, enrolled in the Trieste Heart Muscle Disease Registry. Subjects were further classified into two phenotypic variants, based on ventricular involvement: right-dominant and biventricular form. Uni- and multivariable, extended Kaplan-Meier and cumulative incidence function analysis were performed, as appropriate, for the primary composite endpoint of death and heart transplant (HTx) and for the two distinct secondary endpoints of: (i) death, HTx and first heart failure (HF) hospitalization; (ii) first major ventricular arrhythmias (MVA). Mean age of patients at the time of enrollment was 39 ± 16 years and 80% were male. 73 patients (63%) had a right-dominant form, while 42 (37%) presented a biventricular involvement. AA occurred in the 26% of our study population at baseline or during a median follow-up of 214 months (IQR: 105–311), with a non-significant trend in higher cumulative incidence of AA in patients with biventricular form. At baseline, patients experiencing AA were older (44 ± 18 vs. 37 ± 15 years, P = 0.044) and had larger atrial dimensions, in particular of the right atrium (RA) (23, IQR: 19–27 vs. 18, IQR: 15–25 cm2, P < 0.007). AA emerged as independently associated to death/HTx (HR 2.68, 95% CI: 1.02–7.05, P = 0.046) along with NYHA class >2 (HR: 7.08, 95% CI: 2.50–20.1, P < 0.001) and RA area (HR: 1.05, 95% CI: 1.01–1.09, P = 0.011). Consistently, AA were also independently associated with the secondary endpoint of death/HTx/HF hospitalization (HR: 2.50, 95% CI: 1.06–5.88, P = 0.036), together with NYHA >2, the presence of biventricular involvement, higher RA and left atrium area. Finally, AA did not emerge as independently correlated to MVA during follow-up. Conclusions This observational long-term study suggests that AA were common in patients with AC and were independently associated with poor outcomes, mostly related to HF events. A prompt identification throughout the follow-up of AA appears relevant in improving the clinical management of AC patients.

scholarly journals Arrhythmogenic Cardiomyopathy—Further Insight into the Clinical Spectrum of Desmoplakin Disease

2021 ◽  
Vol 11 (4) ◽  
pp. 219-229
Author(s):  
Joanne Simpson ◽  
Joan Anusas ◽  
Denise Oxnard ◽  
Sylvia Wright ◽  
Ruth McGowan ◽  
...  
Keyword(s):  
Ethical Issues ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Clinical Spectrum ◽  
Dna Testing ◽  
Arrhythmogenic Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Insight Into

Arrhythmogenic cardiomyopathy is a familial heart muscle disease characterized by structural, electrical, and pathological abnormalities. Recognition of left ventricular (LV) involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC) has led to the newer term of arrhythmogenic cardiomyopathy (ACM). We report on a family with autosomal dominant desmoplakin (DSP) related ACM to illustrate the broad clinical spectrum of disease. The importance of evaluation of relatives with cardiac magnetic resonance imaging and consideration of genetic testing in the absence of Task Force diagnostic criteria is discussed. The practical and ethical issues of access to the Guthrie collection for deoxyribonucleic acid (DNA) testing are considered.

The prognostic significance of lymphovascular tumor invasion in localized high-grade osteosarcoma: Outcomes of a single institution over ten years.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e22013-e22013
Author(s):  
Charles Gusho ◽  
Ira Miller ◽  
Bishir Clayton ◽  
Matthew W. Colman ◽  
Steven Gitelis ◽  
...  
Keyword(s):  
Cumulative Incidence ◽  
Tumor Invasion ◽  
Proportional Hazards ◽  
Prognostic Significance ◽  
Cox Proportional Hazards ◽  
High Grade ◽  
Dismal Prognosis ◽  
Confounding Variables ◽  
High Grade Osteosarcoma

e22013 Background: Lymphovascular tumor invasion (LVI) has shown evidence of an association with worse survival in high-grade osteosarcoma. The purpose of this investigation was to prognosticate LVI as a predictor of survival in these patients. Methods: This study was a retrospective review of high-grade, localized osteosarcoma patients diagnosed over a consecutive ten-year period. Cox proportional hazards regression was used to assess the prognostic significance of LVI on overall survival (OS). Results: A total of 42 cases met inclusion criteria with a median follow-up of 64 months (range, 6-158 months). LVI was present in 21.4% (n = 9) cases. The five and ten-year probabilities of OS in LVI (+) were 40% and 20%, respectively, compared to LVI (-) with five and ten-year estimates of 93% and 81%, respectively (p < 0.001). After controlling for confounding variables, advanced age at diagnosis (HR, 1.134; 95% CI, 1-1.2; p = 0.01) and LVI (HR, 21.768; 95% CI, 3-135; p = 0.001) were found to be significantly negative predictors of OS. Using a competing risk analysis and Gray's test of equality, LVI (+) and LVI (-) were not statistically different with respect to cumulative incidence of recurrence (p = 0.8118), though were highly significant for cumulative incidence of mortality over time (p = 0.0029). Conclusions: The presence of LVI in the setting of high-grade, localized osteosarcoma is associated with greater rates of mortality and tumor recurrence and portends a dismal prognosis.

scholarly journals High penetrance and similar disease progression in probands and in family members with arrhythmogenic cardiomyopathy

2019 ◽  
Vol 41 (14) ◽  
pp. 1401-1410 ◽  
Author(s):  
Monica Chivulescu ◽  
Øyvind H Lie ◽  
Bogdan A Popescu ◽  
Helge Skulstad ◽  
Thor Edvardsen ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Mixed Model ◽  
Task Force ◽  
Family Members ◽  
Genetic Diagnosis ◽  
Right Ventricular Outflow Tract ◽  
Progression Rate ◽  
Arrhythmogenic Cardiomyopathy ◽  
Structural Progression

Abstract Aims We aimed to assess structural progression in arrhythmogenic cardiomyopathy (AC) patients and mutation-positive family members and its impact on arrhythmic outcome in a longitudinal cohort study. Methods and results Structural progression was defined as the development of new Task Force imaging criteria from inclusion to follow-up and progression rates as annual changes in imaging parameters. We included 144 AC patients and family members (48% female, 47% probands, 40 ± 16 years old). At genetic diagnosis and inclusion, 58% of family members had penetrant AC disease. During 7.0 [inter-quartile range (IQR) 4.5–9.4] years of follow-up, 47% of family members without AC at inclusion developed AC criteria, resulting in a yearly new AC penetrance of 8%. Probands and family members had a similar progression rate of right ventricular outflow tract diameter (0.5 mm/year vs. 0.6 mm/year, P = 0.28) by mixed model analysis of 598 echocardiographic examinations. Right ventricular fractional area change progression rate was even higher in family members (−0.6%/year vs. −0.8%/year, P &lt; 0.01). Among 86 patients without overt structural disease or arrhythmic history at inclusion, a first severe ventricular arrhythmic event occurred in 8 (9%), of which 7 (88%) had concomitant structural progression. Structural progression was associated with higher incidence of severe ventricular arrhythmic events adjusted for age, sex, and proband status (HR 21.24, 95% CI 2.47–182.81, P &lt; 0.01). Conclusion More than half of family members had AC criteria at genetic diagnosis and yearly AC penetrance was 8%. Structural progression was similar in probands and family members and was associated with higher incidence of severe ventricular arrhythmic events.

Potential role of imaging markers in predicting future disease expression of arrhythmogenic cardiomyopathy

2020 ◽  
Author(s):  
Emanuele Monda ◽  
Giulia Frisso ◽  
Marta Rubino ◽  
Martina Caiazza ◽  
Augusto Esposito ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Potential Role ◽  
Predictive Accuracy ◽  
Task Force ◽  
Arrhythmogenic Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
Risk Of Disease ◽  
Imaging Markers

Aim: To evaluate the predictive accuracy of trabecular hypertrophy/derangement and late gadolinium enhancement in predicting diagnosis of arrhythmogenic cardiomyopathy (AC). Materials & methods: Fifty-nine suspected AC patients were evaluated. To evaluate the ability of these markers to detect patients fulfilling definite diagnosis at 4-year follow-up, sensitivity (Se), specificity (Sp), positive and negative predictive value (PPV and NPV) and predictive accuracy (PA) were calculated. Results: Presence of trabecular hypertrophy/derangement showed high NPV, while late gadolinium enhancement at cardiac MRI high Sp, NPV and PA. Their combination with symptoms and/or revised Task Force Criteria showed a high Sp, NPV and PA for definitive diagnosis during follow-up. Conclusion: In suspected AC patients, the absence of these markers allows to identify those with lower risk of disease progression.

Theoretical and Methodological Problems of a 10-Year Follow-Up Program Evaluation Study

2002 ◽  
Vol 18 (3) ◽  
pp. 229-241 ◽  
Author(s):  
Kurt A. Heller ◽  
Ralph Reimann
Keyword(s):  
Program Evaluation ◽  
Evaluation Studies ◽  
Evaluation Study ◽  
Control Groups ◽  
Cross Sectional ◽  
Long Term Study ◽  
Partial Inclusion ◽  
Methodological Problems

Summary In this paper, conceptual and methodological problems of school program evaluation are discussed. The data were collected in conjunction with a 10 year cross-sectional/longitudinal investigation with partial inclusion of control groups. The experiences and conclusions resulting from this long-term study are revealing not only from the vantage point of the scientific evaluation of new scholastic models, but are also valuable for program evaluation studies in general, particularly in the field of gifted education.

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