Current Indications for Right Ventricular Angiography in ARVCD

RV angiography remains an integral imaging modality and reference technique in the diagnosis and evaluation of patients with ARVC D and is complementary to electrocardiographic and other imaging modalities. RV angiography should be performed in all patients with suspected or definite ARVC D who undergo an initial diagnostic evaluation. It may be combined with an electrophysiological study and or RV endomyocardial biopsy as part of the initial assessment or follow-up evaluation of ARVC D. During...

Introduction

The diagnosis of ARVC D is readily apparent when patients have the typical features including ECG changes and morphological abnormalities limited to the right ventricle. Since the right ventricular structure and contractile pattern is asymmetrical it can be difficult to differentiate normal from mild abnormalities of this complex ventricular chamber 1, 2 . This may cause uncertainty in differentiating ARVC D from normal variants or from idiopathic ventricular Table 11.1 Task Force criteria for...

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...

References

Nava A, Rossi L, Thiene G (eds) (1997) Arrhythmogenic right ventricular cardiomyopathy dysplasia. Elsevier, Amsterdam 2. Thiene G, Nava A, Corrado D et al (1988) Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 318 129-133 3. Corrado D, Thiene G, Nava A et al (1990) Sudden death in young competitive athletes Clinicopatholog-ic correlation in 22 cases. Am J Med 89 588-596 4. Towbin JA, Bowles NE (2002) The failing heart. Nature 415 227-233 5. McKoy G, Protonotarios...

In Vivo Tissue Characterization by EMB

A definitive diagnosis of ARVC D relies on histological demonstration of fibro-fatty replacement of ventricular myocardium and it is listed among the major Task Force diagnostic criteria 68 . In vivo, the pathological specimens are obtained through EMB. Right ventricular EMB is a well-established procedure in the diagnosis of heart muscle disease and seems to be particularly helpful in sus pected ARVC D because of the peculiar topographic and histologic features of the disease. The fibro-fat-ty...

Viral Studies

The presence of myocardial inflammation in more than two-thirds of cases has suggested that some pathologic features of ARVC D may be considered a sequela of infective myocarditis 33 . Thus, fibro-fatty infiltration may, in part, be viewed as a healing phenomenon in the setting of chronic myocarditis. Enterovirus was first investigated on the basis of an experimental model in which a BALB C mice infected with Coxsackievirus B3 developed selective right ventricular myocardial cell death, acute...

Impact of Genetic Results on Clinical Evaluation of Patients with ARVCD

Increasing knowledge of the disease from studies of genotype-phenotype correlation should facilitate assessment of the incidence of the disease, modality of disease expression and clinical presentation, the rate of disease progression, the incidence of sudden death and heart failure, as well as evaluation of electrical instability. In addition, it should clarify the role of important diagnostic features as the ECG, echocardio-gram, signal-averaged ECG, and cardiac magnetic resonance. The...

Voltage Mapping Differential Diagnosis Between ARVCD and Idiopathic RVOT Tachycardia

Idiopathic RVOT tachycardia refers to nonfamilial tachycardias, either paroxysmal or repetitive mono-morphic, with a left bundle brunch block and inferior axis QRS pattern that are characteristically triggered by physical exercise or by catecholamine infusion in young individuals without clinically detectable structural heart disease. Although RVOT tachycardia is considered benign and not progressive, it may cause syncope and, rarely, sudden cardiac death 44-46 . These malignant events are most...

Disease Genes

The first identified ARVC D gene in a dominant form was ryanodine receptor-2, involved in ARVD2 25 . In ARVD2, there is fibro-fatty substitution of the myocardial tissue, though much less pronounced than in the typical ARVC D. The distinctive feature of this form is the presence of polymorphic, effort-induced arrhythmias. RYR2 is one of the largest human genes 105 exons , encoding a 565Kda protein located in the membrane of smooth sarcoplasmic reticulum. The homo-tetrameric structure known as...

Intercalated Disc Junctions

Adherens Junctions And Intercalated Disc

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...

Altered Expression and Distribution of Cell Cell Junction Proteins in Human Cardiomyopathies

To test the hypothesis that defects in the adhesion junction-cytoskeleton network disrupt gap junctions, we analyzed ventricular tissues from patients with Naxos disease, a cardiocutaneous syndrome consisting of the clinical triad of woolly hair, palmoplantar keratoderma, and ARVC D 4 . Patients with Naxos disease are readily identified early in life because of the distinctive cutaneous features the disease is 100 penetrant 5, 6 . Naxos disease is associated with a particularly high incidence...

Mouse Models of Human Cardiomyopathies

Delineation of structural and molecular pathology in human tissues is essential to understanding the cell-cell junction cardiomyopathies yet to elucidate mechanisms of disease, we have turned to analysis of mouse models. The first mouse line we characterized was a model of human desmin-related cardiomyopathy created by X.J. Wang in the laboratory of Jeffrey Robbins 11 . We were attracted to this model because we anticipated that it might exhibit altered desmin-desmo-some interactions and,...

Pure Fatty Infiltration of the Right Ventricle vsARVCD

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...

Preparticipation Screening and Prevention of Sudden Death

For more than 20 years systematic preparticipation screening, based on 12-lead ECG in addition to history and physical examination, has been the practice in Italy 6, 41 . This screening strategy has proven to be effective in the identification of athletes with previously undiagnosed hypertrophic cardiomyopathy, due to the high sensitivity up to 95 of 12-lead ECG for suspicion detection of this condition in otherwise asymptomatic athletes. Moreover, during long-term follow-up no deaths were...

Clinical Manifestations

Ventricular Arrhythmias Dogs

Affected boxer dogs may die suddenly and unexpectedly during vigorous exercise, leisurely walking, or while sleeping. Persistently high sympathetic tone was not found to be a consistent feature of boxer dogs with ARVC D 45 . Syncope is common and has been recorded in approximately half of severely affected dogs, including up to two-thirds of those that subsequently died suddenly, but there was no significant difference in the mode of death, sudden vs. nonsud-den, in dogs with this symptom....

Voltage Mapping Methods and Equipment

Three-dimensional electroanatomic voltage mapping technique is performed using the CARTO system Biosense-Webster 20-26 . In brief, the magnetic mapping system includes a magnetic sensor in the catheter tip that can be localized in 3D using ultralow magnetic field generators placed under the fluoro-scopic table. A 7F Navi-Star catheter, with a 4 mm distal tip electrode and a 2 mm ring electrode with an interelectrode distance of 1mm, is introduced into the RV under fluoroscopic guidance and used...

Naxos Disease History

How Control Ventricular Skin Diseases

In 1986, an autosomal recessive cardiocutaneous syndrome of ARVC D associated with woolly hair and palmoplantar keratoderma was first described in families from the Greek island of Naxos 8 , from which the sydrome took the name of 9 . The autosomal recessive ARVC D in Naxos disease is similar to autosomal dominant ARVC D with respect to age and mode of clinical presentation, distribution of right ventricular and left ventricular involvement, electrocardiographic features, natural history, and...

Gross and Histologic Diagnosis

Myocardium Fatty Histology

The pathologic diagnosis of ARVC D in autopsy hearts or those explanted at the time of cardiac transplantation has been traditionally based upon gross and histologic evidence of transmural fatty or fibrofatty myocardial replacement of the right ventricular free wall. This could be considered a myocardial dystrophy, extending from the epicardium towards the endocardium, sparing only the trabecular myocardium 3-8 . Table 4.1 summarizes the main pathologic features as observed in the original...

Left Ventricular Involvement

Depolarization Left Ventricle Scar

ARVC D has been classically defined as a selective disorder predominantly affecting the right ventricle 1 . Since the early description in the 1980s, left ventricular involvement has been recognized clinically with greater and greater frequency 2,43-45 . From the pathological point of view, several case reports of ARVC D with histologic abnormalities of the left ventricle have been published 46-53 , even in the absence of heart failure. In the Padua series, Basso et al. 4 indicated that left...

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...

Qualitative Echocardiographic Features of ARVCD

There are several structural abnormalities that have been noted with increased frequency in individuals with ARVC D. Morphologic abnormalities on echocardiography including a hyperreflective moderator band, trabecular prominence and derangement, and focal aneurysms or sacculations have been described 2, 3 . In a study comparing ARVC D probands from the North American Registry to matched controls, trabecular derangement was the most frequently noted abnormality, occurring in 54 of affected...

How to Perform Right Ventricular Angiography in ARVCD

Angiography Calibration

The following recommendations have been proposed by Wichter et al. 12 for the core laboratory of RV angiography within the NIH-funded North American Multidisciplinary Study of ARVD 13 and the EU-funded European Registry of ARVC D 14 . The protocol was designed to perform RV an-giograms of best quality to assess structural and functional RV abnormalities in ARVC D and to allow quantitative measurements of RV volumes, ejection fraction, and regional contraction and relaxation. After local...

Negative T Waves

Negative Precordial Waves

A recent literature review by Marcus 11 showed that negative T waves in leads V2-V5 are infrequent over the age of 12 years Fig. 13.2 . In adolescents 12-18 years of age, T wave inversion in V1 and V2 are present in 10 -20 and in up to 5 of adolescents in V3. This juvenile pattern of inverted T waves in leads V1-V3 is observed in 1 -3 of healthy adults aged 19 to 45 years 12 . The location of the precordial recording electrodes can influence ECG pattern and misplaced positioning of lead V2 and...

Pathogenesis Virus and Myocyte Death

Arvc Myocytes Apoptose

Over the past two decades, the use of molecular-based studies have enabled viral genomes to be analyzed in the hearts of human subjects with cardiac dysfunction. Bowles and colleagues were the first to identify coxsackievirus in myocardial specimens of patients with myocarditis and dilated cardiomyopathy DCM using molecular techniques 42,43 . Towbin and colleagues later showed the high incidence of adenovirus in myocarditis and DCM 44-48 as well as par- Fig. 9.2 Viral genomes frequency in 36...

Safety of ICD Implantation in ARVCD

Concern has been expressed about the potential for risks and complications of transvenous lead implantation in diseased RV myocardium. These relate not only to an increased risk of perforation, but also to suboptimal sensing, pacing, and defibrillation efficacy. Although procedure-related deaths were extremely rare in all series following patients with ARVC D after ICD implantation, both procedural complications and long-term performance complications of the leads were not uncommonly...

Epsilon Waves

Ecg Epsilon Waves

The epsilon wave consists of high-frequency notches located on the ST segment immediately after the QRS complex, usually recorded in the right precordial leads on the standard ECG Fig. 13.3 . This ECG wave was first described in patients with ARVC D 2, 20 . It is believed that epsilon waves represent delayed activation of affected areas of right ventricle. Late potentials in the signal average ECG SAECG are another manifestation of the same phenomenon. It is recommended that the ECG should be...

Acute Efficacy of Antiarrhythmic Drugs in ARVCD

Antiarrhythmic Drugs

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...

Device Selection

A conservative approach to the complexity of the lead system used with the implanted defibrillator appears prudent. While dual-chamber systems seem attractive since they may reduce inappropriate ICD therapies because of improved ability to discriminate between supraventricular and ventricular arrhythmias, a reduction in the number of implanted leads may be a favorable approach in this relatively young patient group, precisely because of the incidence of lead-related complications over long-term...

Risk Stratification

The proportion of ARVC D as the underlying disease in cases of sudden and unexpected cardiac death is unknown but has been estimated to be 15 -25 in patients below 35 years of age 17 , provided there is a detailed postmortem investigation of the right ventricle performed by an experienced pathologist. These data correspond well with the natural history of ARVC D with mortality rates of up to 25 after 10 years or 2.5 per year on empiric uncontrolled antiarrhythmic drug therapy 18-21 . However,...

MR Imaging of ARVCD

Mri Scan Patient With Arvc

Among the current cardiac MR applications in cardiomyopathies, the greatest potential as well as biggest challenges are in the diagnosis of ARVC D. MR imaging allows both qualitative and quantitative analysis of RV function 14 . MR has the ability to demonstrate intramyocardial fat 12 and recently, delayed enhancement MR imaging has been shown to be useful in detecting fibrosis in the RV in ARVC D 15 . The last 10 years have seen significant improvements in MR hardware, with tremendous...

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...

Efficacy of ICDs in ARVCD

A considerable body of data exists about the efficacy of ICD therapy in patients diagnosed with ARVC D. While no prospective randomized trials of ARVC D have compared ICD therapy with antiarrhythmic drug treatment, catheter ablation of VT, or no therapy, the available clinical data supports the use of ICDs in ARVC D. Forty percent of ARVD patients have appropriate ICD therapy if they have spontaneous or induced VT with hemodynamic compromise or unexplained syncope 10 . Despite this high...

The Role of Right Ventricular Angiography in ARVCD

Angiocardiography

The structural abnormalities of the RV myocardium are the basis for the main clinical and diagnostic features of ARVC D. Apart from the ventricular tach-yarrhythmias that are frequently the first clinical manifestation of ARVC D, they also result in characteristic features in the ECG and imaging. However, the clinical diagnosis of ARVC D may be difficult because there is no easily obtained single test or finding that is definitely diagnostic. Therefore, the diagnosis of ARVC D usually requires...

Treatment Options

Pictures For Ventricle Tacycardia

Concerning the treatment of patients with ARVC D, most of the data available to date refer to retrospective analyses in single centers with limited numbers of patients. There is limited data on long-term outcomes with different treatment modalities in patients with ARVC D. Controlled randomized trials have not been performed in ARVC D and international registries 7,8 are currently enrolling patients but have not yet reported results on treatment. It is difficult to compare patient cohorts from...

Abnormal SAECG

Signal Averaged Ecg

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...

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,...

QRS Duration and Morphology

Localized widening of the QRS complex in right precordial leads is one of the typical ECG findings in ARVC D 19,20 . It is likely that conduction delay due to fibrofatty replacement of the right ventricle is a cause of the localized QRS prolongation. Fontaine et al. 2,19,20 reported that the QRS duration gt 110 ms in right precordial leads and differences in QRS dura tion between right and left precordial leads gt 25 ms are characteristic of ARVC D. This localized prolongation of the QRS...

Computed Tomography of ARVCD

Motion Artifact Animated

Computed tomographic CT imaging utilizing the x-ray technology was developed in the early 1970s, and has been used extensively for its ability to provide cross-sectional images of the body 50 . There has been a rapid development of CT technology, particularly in the last 10 years, focused on cardiac imaging. The latest generation CT scanners, namely the multidetector CT MDCT , provide excellent spatial resolution, and allow accurate high-resolution assessment of morphological detail of both...

Quantitative Echocardiographic Features of ARVCD

Arvc Echo Images

RV dilatation is a common finding in individuals with ARVC D 2,4,5 . The echocardiographic study from the North American ARVC D Registry provided potential quantitative parameters to differentiate probands from matched controls 3 . Table 14.2 shows the mean RV dimensions at end-systole and end-diastole in probands and matched controls. An enlarged right ventricular outflow tract RVOT was found in 100 of probands. RVOT long axis dimension of gt 30mm had a sensitivity of 89 and specificity of 84...

Results

During the ablation procedure, VT was monomor-phic in six patients, five patients had two morphologies, four had three, and seven had from four to eight morphologies Fig. 19.3 . Thirteen patients were successfully treated by a single session, four required two sessions, two required three sessions, and one four. However, two patients of the same cohort received eight and eleven sessions respectively, during the same study period Fig. 19.4 . They will be later referred to as Case 1 and Case 2...