Publish in this journal
Journal Information
Vol. 69. Issue 9.
Pages 872-874 (September 2016)
Scientific letter
DOI: 10.1016/j.rec.2016.04.037
Full text access
Clinical Profile of Arrhythmogenic Right Ventricular Cardiomyopathy With Left Ventricular Involvement
Perfil clínico de la miocardiopatía arritmogénica del ventrículo derecho con afección asociada del izquierdo
Visits
...
Erika López-Moreno
Corresponding author
lopezmorenoerika@gmail.com

Corresponding author:
, Juan Jiménez-Jáimez, Rosa Macías-Ruiz, Pablo J. Sánchez-Millán, Miguel Álvarez-López, Luis Tercedor-Sánchez
Servicio de Cardiología, Complejo Hospitalario Universitario de Granada, Granada, Spain
This item has received
...
Visits
(Daily data update)
Article information
Full Text
Bibliography
Download PDF
Statistics
Tables (2)
Table 1. Statistical Comparison of Baseline Clinical Variables Between the Groups With Isolated RV Involvement and Biventricular Involvement
Table 2. Statistical Comparison of Echocardiographic Data Between the Groups With Isolated RV Involvement and Biventricular Involvement
Show moreShow less
Full Text
To the Editor,

The clinical profile of arrhythmogenic cardiomyopathy (ACM) with left ventricular (LV) involvement appears to be distinct from ACM isolated to the right ventricle (RV)1,2, with a higher incidence of ventricular arrhythmias and sudden cardiac death (SCD) shown in some studies.3 However, in Spain, there are few published series correlating LV involvement with an increased risk of arrhythmia and SCD.

Our aim was to analyze the differentiating clinical and morphological characteristics of biventricular ACM compared with ACM isolated to the RV in our series. This was a cross-sectional study that included 30 patients with ACM from 20 families; 17 were probands (56.7%) and 13 were relatives. All met the Task Force criteria for ACM. Participants were divided into 2 groups according to whether they had involvement of the RV alone or biventricular involvement (the LV was considered involved when the ejection fraction on echocardiography was < 50%).

Data were collected on age and sex, as well as clinical information on functional class, syncope, ventricular arrhythmias, atrial fibrillation, implantable cardioverter-defibrillator (ICD) shocks, heart transplantation, and death due to end-stage heart failure. All participants were studied with 12-lead ECG, echocardiography, and where possible, cardiac magnetic resonance (MR). Genetic study was performed in 27 patients (90%).

The statistical analysis was performed by comparing these variables in relation to the presence of isolated RV involvement or biventricular involvement, and nonparametric tests (Mann-Whitney U test) were used for the study of the mean.

Biventricular involvement was predominant in the probands and relatives. Of a total of 55 relatives studied, 18.2% received a new diagnosis of ACM. Follow-up time from diagnosis was similar in both groups (Table 1). In addition, in the group with biventricular involvement, 89% of the patients already had LV involvement at the time of diagnosis. No significant differences were found in the presence of epsilon waves and bundle branch block on ECG, but there was a trend (P = .07) toward an increased presence of inverted T waves in the precordial leads in the group with biventricular involvement.

Table 1.

Statistical Comparison of Baseline Clinical Variables Between the Groups With Isolated RV Involvement and Biventricular Involvement

Clinical variables  Isolated RV involvement  Biventricular involvement  P 
Patients  12 (40)  18 (60)   
Men  8 (66.7)  10 (55.6)  .63 
Age, y  50.3 ± 18.6  55.3 ± 14.9  .49 
Follow-up since diagnosis, y  9.7 ± 7.1  7.8 ± 6.6  .46 
Reason for investigation       
VF  0 (0)  1 (5.6)  .12 
Ventricular arrhythmias  5 (41.7)  3 (16.7)   
Syncope  3 (25)  3 (16.7)   
Dyspnea  0 (0)  2 (11.1)   
Family history  3 (25)  7 (38.9)   
ECG abnormalities  1 (8.3)  1 (5.6)   
Incidental finding  0 (0)  1 (5.6)   
Relatives who died of SCD  0.4 ± 0.7  1.1 ± 0.8  .04 
Family history of SCD (yes), %  33.3  67.7  .13 
NYHA functional class  1.1 ± 0.3  2.7 ± 0.8  < .01 
11 (91.7)  0 (0)   
II  1 (8.3)  9 (50)   
III  0 (0)  5 (27.8)   
IV  0 (0)  4 (22.2)   
Heart transplantation  0 (0)  1 (5.6)  .81 
Syncope  4 (33.3)  4 (22.2)  .63 
Sustained ventricular arrhythmias at follow-up  7 (58.3)  10 (55.6)  .92 
AF  2 (16.7)  3 (16.7) 
ICD implant  4 (33.3)  15 (83.3)  .02 
Ablation  4 (33.3)  2 (11.1)  .33 
Death due to end-stage heart failure  0 (0)  2 (11.1)  .63 

AF, atrial fibrillation; ECG, electrocardiogram; ICD, implantable cardioverter-defibrillator; NYHA, New York Heart Association; RV, right ventricle; SCD, sudden cardiac death; VF, ventricular fibrillation.

Unless otherwise stated, values are expressed as no. (%) or mean ± standard deviation.

Table 1 shows the clinical characteristics of the groups with isolated RV involvement and with biventricular involvement. The functional class was clearly more advanced in the biventricular group. Although the presence of syncope and ventricular arrhythmias was similar, the burden of family history of SCD was higher in patients with biventricular involvement (P = .04). However, on dichotomous analysis (yes/no) of this family history, a trend was found; this did not reach statistical significance (P = .13). More ICDs were implanted in the biventricular group, mainly as primary prevention (66.7% of implants); none of the patients with isolated RV involvement received an ICD for this indication. The distribution of ICD therapies differed according to the indication (P < .01): there was a higher number of appropriate shocks in the group with an ICD as secondary prevention (88.9%), while a minority of the patients with an ICD as primary prevention had received appropriate ICD therapies at follow-up (10%). These findings were similar to those observed in other registries.4

Investigation of ventricular function showed an increased LV end-diastolic diameter and a higher degree of RV and LV dysfunction in patients with biventricular involvement (Table 2). A limitation of this study is the insufficient availability of additional data from MR.

Table 2.

Statistical Comparison of Echocardiographic Data Between the Groups With Isolated RV Involvement and Biventricular Involvement

Echocardiographic variables  Isolated RV involvement  Biventricular involvement  P 
Qualitative LVEF      < .01 
Normal  12 (100)  0 (0)   
Mild LVD  6 (33.3)   
Moderate LVD  6 (33.3)   
Severe LVD  6 (33.3)   
LVEF, %  63.1 ± 4  37.2 ± 8.9  < .01 
LVEDD, mm  47.2 ± 7.6  52.3 ± 4.5  .03 
Qualitative RVEF      < .01 
Normal  5 (41.7)  0 (0)   
Mild RVD  5 (41.7)  4 (22.2)   
Moderate RVD  1 (8.3)  5 (27.8)   
Severe RVD  1 (8.3)  9 (50)   

LVD, left ventricular dysfunction; LVEDD, left ventricular end-diastolic diameter; LVEF, left ventricular ejection fraction; RV, right ventricle; RVD, right ventricular dysfunction; RVEF, right ventricular ejection fraction.

Unless otherwise stated, values are expressed as no. (%) or mean ± standard deviation.

The results confirm that biventricular involvement represents a more advanced stage of isolated RV disease. Our study corroborates the association between LV involvement and a more advanced functional class. Certainly, previous studies have established the relationship between LV involvement and an increased mortality rate due to heart failure.1,5,6 However, although the analysis of our series does not demonstrate a significant association, it does shows a trend: the 2 patients who died from heart failure and the single patient who received a transplant were all from the biventricular involvement group. The lack of significance is probably due to an insufficient sample size. Regarding the risk of arrhythmia, of the group with isolated RV involvement, 4 patients had ICDs as secondary prevention compared with only 2 in the biventricular group. In contrast, all ICDs implanted as primary prevention were in patients with biventricular involvement, because their risk profile was higher based on the presence of significant LV dysfunction3 (Table 1). These differences could explain why the incidence of arrhythmic events was similar in both groups at follow-up, even though a relationship with the presence of LV dysfunction would have been expected.

We can conclude that LV dysfunction is associated with greater RV dysfunction, worse functional class, and an increased tendency to events due to heart failure. No clear relationship was found between LV involvement and an increased rate of arrhythmic events, although there was an association with an increased burden of family history of SCD.

References
[1]
B. Pinamonti, G. Sinagra, A. Salvi, A. Di Lenarda, T. Morgera, F. Silvestri, et al.
Left ventricular involvement in right ventricular dysplasia.
Am Heart J., 123 (1992), pp. 711-724
[2]
S. Sen-Chowdhry, P. Syrris, S.K. Prasad, S.E. Hughes, R. Merrifield, D. Ward, et al.
Left-dominant arrhythmogenic cardiomyopathy: an under-recognized clinical entity.
J Am Coll Cardiol., 52 (2008), pp. 2175-2187
[3]
D. Corrado, L. Leoni, M.S. Link, P. Della Bella, F. Gaita, A. Curnis, et al.
Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia.
Circulation., 108 (2003), pp. 3084-3091
[4]
J. Alzueta, A. Asso, A. Quesada.
Registro Español de Desfibrilador Automático Implantable. XI Informe Oficial de la Sección de Electrofisiología y Arritmias de la Sociedad Española de Cardiología (2014).
Rev Esp Cardiol., 68 (2015), pp. 996-1007
[5]
H.Y. Wang, L. Liu, L. Li, L.L. Wang, F.Y. Lü, H. Zhao, et al.
Pathologic features of arrhythmogenic right ventricular cardiomyopathy with severe heart failure.
Zhonghua Bing Li Xue Za Zhi., 37 (2008), pp. 169-173
[6]
K. Lemola, C. Brunckhorst, U. Helfenstein, E. Oechslin, R. Jenni, F. Duru.
Predictors of adverse outcome in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy: long term experience of a tertiary care centre.
Heart., 91 (2005), pp. 1167-1172
Copyright © 2016. Sociedad Española de Cardiología
Idiomas
Revista Española de Cardiología (English Edition)

Subscribe to our newsletter

Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

es en
Política de cookies Cookies policy
Utilizamos cookies propias y de terceros para mejorar nuestros servicios y mostrarle publicidad relacionada con sus preferencias mediante el análisis de sus hábitos de navegación. Si continua navegando, consideramos que acepta su uso. Puede cambiar la configuración u obtener más información aquí. To improve our services and products, we use "cookies" (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here.