chapter 11 STRENGTHS AND WEAKNESSES OF THE TASK FORCE CRITERIA
CHAPTER 12 IDIOPATHIC RIGHT VENTRICULAR OUTFLOW TRACT 105 Gianfranco Buja, Barbara Ignatiuk,Thomas Wichter,Domenico Corrado CHAPTER 13 ELECTROCARDIOGRAPHIC MANIFESTATIONS 121 Wojciech Zareba, Katarzyna Piotrowicz, Pietro Turrini Danita Yoerger-Sanborn,Michael H.Picard,Barbara Bauce Harikrishna Tandri,Hugh Calkins, David A. Bluemke Thomas Wichter,Julia Indik,Luciano Daliento CHAPTER 17 ELECTROPHYSIOLOGIC STUDY INCLUDING ELECTROANATOMIC 159 Domenico Corrado,James Daubert, Cristina...
Pure Fatty Infiltration of the Right Ventricle vs ARVCD
At present, considerable importance is given to the finding of fatty infiltration of the myocardium, since cardiac magnetic resonance imaging has the ability to identify adipose tissue in vivo with consequent diagnostic and therapeutic implications. It is still a matter of debate whether fatty infiltration of the right ventricle per se should be considered a morphologic hallmark of ARVC D 19 . We must recognize that the original distinction in two histologic variants, i.e., fatty and...
Management of Asymptomatic Patients and Family Members
Asymptomatic patients with ARVC D do not require specific antiarrhythmic or other cardiac treatment. However, they should be followed by regular noninvasive cardiac investigations for early recognition of ventricular arrhythmias and potential progression of the disease with worsening of global or regional my-ocardial dysfunction. These follow-up visits should include a detailed interview concerning the interim occurrence of arrhythmic symptoms or events, ECG at rest, exercise tests, Holter...
ARVCD vs Idiopathic RVOT Ventricular Tachycardia Differential Diagnosis
VT associated with ARVC D may be localized in the outflow tract mimicking idiopathic RVOT tachycardia. Discrimination between the two entities is mandatory for prognostic and therapeutic reasons as well as for genetic implication 80, 81 . Standardized diagnostic criteria for the diagnosis of ARVC D have been proposed and are known as the Task Force criteria 32 . According to these Task Force guidelines, the diagnosis of ARVC D is based on the presence of major and minor criteria including ECG,...
Clinical Diagnosis of ARVCD
The most common clinical manifestations of ARVC D consist of ECG depolarization repolarization changes mostly localized in right precordial leads, ventricular arrhythmias of RV origin, and global and or regional dysfunction and structural alterations of the RV. Clinical diagnosis may be difficult because of several problems related with specificity of ECG abnormalities, different potential etiology of right ventricular arrhythmias, and assessment of the RV structure and function by current...
Acknowledgements
This work has been supported by grant N 99B0691 from the Minist re de l'Education, de la Recherche et de la Technologie. We would like to extend our gratitude to the team of colleagues who have performed the ablation procedures J. Tonet, G. Jauvert, F. Halimi, Ph. Aouate, G. Lascault, F. Poulain, and N. Johnson with special mention for our anesthesiologist Y. Gallais, C. Himbert, V. Bors, cardiac surgeon are also acknowledged. We also appreciate the work of Mrs. Samia Riguet, and Mrs. Deanna...
Angiographic Features of Wall Structure and Wall Motion in ARVCD
A variety of morphological and structural RV an-giographic features have been reported to be suggestive of ARVC D. These include global and regional dilatation, dilatation of the outflow tract, localized akinetic or dyskinetic bulges and outpouchings, polycyclic contours cauliflower aspect , and tra-becular hypertrophy and or disarray with deep horizontal fissures pile d'assiettes as well as dye persistence due to delayed contrast evacuation. In the first publications on RV angiography in ARVC...
Abnormal SAECG
The presence of late potentials and delayed activation of ventricular myocardium detected in the SAECG is considered to represent slowed ventricular conduction, which is a substrate for reentrant tachyarrhyth mias Fig. 13.4 . In the first reported series of patients with this disease, late potentials were recorded in 81 of patients by Marcus, Fontaine et al. 1 . In the 151 patients described by Wichter et al. 17 ,late potentials were found in 41 . Kinoshita et al. 25 analyzed the SAECG in 28...
Ventricular Tachyarrhythmias in Normal Hearts Overview and Classification
VT is usually associated with structural cardiac disease. The most common anatomic substrate of ventricular arrhythmias is chronic ischemic heart disease. Other structural disorders associated with VT are valvular and congenital heart disease, cardiac tumors, myocarditis, and cardiomyopathies. Only approximately 10 of arrhythmias occur in the absence of structural heart disease assessed by clinical examination and imaging studies. Some of them may be caused by inherited cardiac diseases such as...
Acute Efficacy of Antiarrhythmic Drugs in ARVCD
Criteria to evaluate the acute efficacy of antiarrhyth-mic therapy are difficult to define because they may vary with specific clinical situation, arrhythmia characteristics, and appropriateness of test modalities to assess treatment effect. The largest experience on acute and long-term efficacy of antiarrhythmic drug therapy in ARVC D was first published in 1992 by Wichter et al. 37 and included 191 patients with 608 drug tests in their latest updated series 25 . To assess the acute efficacy...
Intercalated Disc Junctions
The ID is the area of end-to-end connections between cardiomyocytes, and consists of three junc-tional complexes desmosomes, fascia adherens, and gap junction also called the nexus . The desmosome is composed of a cytoplasmic electron-dense plaque of intracellular filaments containing desmoplakin, plakoglobin, plakophilin, and transmembrane calcium-sensitive proteins, i.e., desmoglein and desmo-collin. In addition to cell-to-cell adhesion, this type of junction provides a structural connection...
Cardiomyocyte Dedifferentiation
Some cardiomyocytes in ARVC D are characterized by cellular hypertrophy, disruption of sarcomeres, depletion of myofibrils, and T-tubular membrane invaginations of the sarcolemma and sarcoplasmic reticulum. There are also oval nuclei with homoge-nously dispersed chromatin resembling nuclei of fetal cardiomyocytes. These nuclei contain mitochondria of various size and shape with normal cristae Fig. 6.5a , and often abundant glycogen. The above- Fig. 6.5 Features of cardiomyocytes...