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Prof. Gaetano Thiene,
This was an excellent clinical seminar on news in diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy (ARVC/D), with outstanding Faculty and chaired by Gaetano Thiene from Padua, Italy, and Ernst van der Wall from Leiden, The Netherlands.
Bill Mc Kenna from London - U.K., covered the topic of clinical presentation and genetic findings. By illustrating the pathologic basis and natural course of the disease, he stressed the heterogeneity in terms of penetrance, age-related expression, arrhythmias/risk of sudden death (SD), left ventricular involvement and progression to heart failure. There is a concealed phase with subtle signs/symptoms and SD as first manifestation; an overt phase with arrhythmias due to diffuse structural changes at risk of SD, and an advanced phase with heart failure. Unfortunately, there is no single test for diagnosis, which requires the merging of multiple investigations. McKenna, who in 1994 chaired a Task Force establishing the diagnostic criteria, pointed to the need to change these criteria now, especially in family members, giving more emphasis to ECG abnormalities (inverted T wave in right precordial lead, epsilon wave, number of premature ventricular beats at Holter recording) and imaging, with special reference to magnetic resonance imaging (MRI) with LV involvement. Moreover, the discovery of gene mutations allows genetic screening in first degree relatives of the proband, which is of great help in identifying asymptomatic carriers in the concealed phase of the disease. Nikos Protonotarios and Adalena Tsatsopoulous from Naxos – Greece, dealt with the role of non invasive cardiac imaging by echocardiography, first focusing on gross pathologic features of the disease (“triangle of dysplasia”) and emphasizing the occurrence of phenotype expression during adolescence. They presented original data on inflow and outflow tract dilatation of the RV in genotyped ARVC patients (normal up to 39 and 32 mm, respectively).
RVOT >32 mm Specificity : 97%, 56%
RVIM >39 mm, Sensitivity : 97% 42%
According to their data, RV segmental wall abnormalities present a sensitivity and specificity >95%. Excessive trabeculation and hyper-reflectivity of the moderated band may also contribute to the diagnosis. LV involvement, in the absence of aneurysms, may not be seen at echo and misdiagnosed.
Finally, they drew attention to sarcoidosis, which may mimic ARVC/D and can be differentiated only through endomyocardial biopsy, a feature that was emphasized also by Dr. Fontaine during the ensuing discussion.
Dr. Lima from Johns Hopkins Baltimore – U.S.A., presented the role of MRI, which has the potential of investigate abnormal morphology and function as well as tissue characterization through abnormal signal intensity and late-enhancement. The MRI features should be added among the official diagnostic criteria, since this tool may give crucial information on RV enlargement, regional motion abnormalities/aneurysms and presence of fat and fibrosis. Investigating the LV may be informative and diagnostic, especially for fat infiltration and fibrosis, considering that LV involvement is present in at least 2/3 of cases.
Thomas Wichter from Munster – Germany, dealt with the risk factors for SD in ARVC/D (history of syncope-cardiac arrest, family history of SD-ARVC/D, extensive RV dysfunction-LV involvement, polymorphic VT, refractory anti-arrhythmic drug therapy, epsilon wave, late potentials, QRS dispersion). He focused the attention particularly on the role of defibrillator (ICD) on SD prevention, stressing either the complications (lead dislocation, fracture, perforation, undersensing, infections) and the success (24% survival benefit at 48 months).
According to recent, still unpublished investigation by the Padua Group on primary prevention by ICD, patients with ICD implantation on the basis of family history of SD had no event as well as those with asymptomatic VT. Thus, the major indication remains previous episodes of syncope, cardiac arrest, VF and symptomatic VT.
Advances in imaging and management of arrhythmogenic right ventricular cardiomyopathy
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