ﺑﺎﺯﮔﺸﺖ ﺑﻪ ﺻﻔﺤﻪ ﻗﺒﻠﯽ
خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
medimedia.ir

Arrhythmogenic right ventricular cardiomyopathy: Anatomy, histology, and clinical manifestations

Arrhythmogenic right ventricular cardiomyopathy: Anatomy, histology, and clinical manifestations
Author:
William J McKenna, MD
Section Editor:
Hugh Calkins, MD
Deputy Editor:
Todd F Dardas, MD, MS
Literature review current through: Jan 2024.
This topic last updated: Oct 10, 2022.

INTRODUCTION — Arrhythmogenic cardiomyopathy (ACM) is defined by a clinical presentation with documented or symptomatic arrhythmia and myocardial structural and functional abnormalities. Arrhythmogenic right ventricular cardiomyopathy (ARVC), formerly called "arrhythmogenic right ventricular dysplasia" (ARVD), is the best characterized of the ACMs in relation to diagnosis, treatment and outcomes. It is an underrecognized clinical entity characterized by ventricular arrhythmias of right ventricular (RV) origin and a characteristic ventricular pathology [1-3]. Macroscopically, there is a scarred appearance with fibrous or fibro-fatty replacement of myocardium. Historically, multiple reports have characterized these pathologic changes as the "triangle of dysplasia" involving the inflow tract, outflow tract, and/or apex of the RV. However, more recent data have noted involvement of the posterolateral LV with sparing of the RV apex early in the disease [4]. The RV myocardial scarring initially produces typical regional wall motion abnormalities but later may involve the free wall and become global, producing RV dilation. The tissue replacement can also involve areas of the left ventricle (LV) with relative sparing of the septum [5].

The anatomic and histologic findings associated with ARVC fall into several categories. Structurally, the hallmark findings are regional, progressing to global, RV dilation, and myocardial thinning. Most patients also have LV myocyte loss and fibrosis, usually involving the LV lateral and posterior walls. Histologically, fibrofatty infiltration of the myocardium is the most common finding. In addition, electron microscopy has shown gap junction abnormalities of desmosomes in the RV myocardium, which is consistent with mutations in genes encoding desmosomal proteins being responsible for ARVC in most patients. (See "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics", section on 'Genetics'.)

Clinical perspectives of ARVC initially arose from experience with patients who present with arrhythmias of RV origin and/or sudden death. However, additional information has been learned regarding the clinical features and clinical course of subjects identified based on electrocardiographic (ECG) abnormalities and by cascade family screening [6]. Presentation is most common between the ages of 10 and 50 years, with a mean age at diagnosis of approximately 30 years [6-9]. The disease is virtually never diagnosed in infants or toddlers and is rarely identified before the age of 10.

The anatomy, histology, and clinical manifestations of ARVC will be reviewed here. The pathogenesis, genetics, diagnostic evaluation, treatment, and prognosis of ARVC are discussed separately. (See "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics" and "Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis" and "Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis".)

PREVALENCE — The prevalence of ARVC in the general adult population is estimated to be approximately 1 in 2000 to 1 in 5000 [10,11]. ARVC is an important cause of sudden cardiac death (SCD) in young adults, accounting for approximately 11 percent of cases overall [12,13]. Although it was once thought that ARVC was more common in Europe that in the United States this is no longer felt to be the case [7].

ARVC is an inherited cardiomyopathy that affects a significant number of first-degree relatives of the proband. In a study of 274 first-degree relatives of 138 ARVC probands, in which all the first-degree relatives underwent a detailed medical history, cardiac imaging, and mutation-specific genetic testing (if a definite mutation had been identified in the family's proband), 96 first-degree relatives (35 percent) were ultimately diagnosed with ARVC [14]. Siblings had a threefold higher risk of being diagnosed with ARVC compared with other first-degree relatives (odds ratio 3.1, 95% CI 1.7-5.9).

ANATOMY AND HISTOLOGY

Anatomy — Localized or generalized dilatation of the RV and myocardial thinning in the regions of dilation are the typical anatomic findings in ARVC. Historically, sites of involvement have been characterized as the so-called "triangle of dysplasia," which included the RV inflow tract, the RV outflow tract (RVOT), and posterolateral LV [4]. Although initial studies included the RV apex in this triangle, it is now recognized that the RV apex is typically involved in severe and late-stage disease [4]. The RV myocardium is typically replaced by fibrous tissue and fat, with scattered residual myocardial cells (picture 1). The extent of replacement is variable.

More recent data have noted involvement of the posterolateral LV with sparing of the RV apex early in the disease [4]. In a nationwide autopsy study of 5205 consecutive cases of SCD referred for pathologic examination between 1994 and 2018, 202 cases (4 percent) were identified as having "arrhythmogenic cardiomyopathy" based on histopathologic examination [15]. Of these patients, 13 percent have isolated RV involvement, 17 percent had isolated LV involvement, and 70 percent had biventricular histopathologic findings.

Left ventricular abnormalities and disease progression — Although less prominent than RV disease, LV involvement is common in patients with ARVC [16-20]. In a report of 42 patients with ARVC diagnosed at autopsy or heart transplantation, 76 percent had fibrofatty involvement of the left ventricle [17]. The likelihood of this occurring increases with age, suggesting that ARVC is a progressive disease [16,17]. The degree of involvement of the LV is related to genotype and is least common with a PKP2 mutation, which is the most common mutation identified in North America [21]. (See 'Left ventricular involvement' below.)

Because of the frequency with which LV involvement is observed, particularly in patients with mutations in specific genes (eg, desmoplakin), the field is slowly moving towards replacing the term "arrhythmogenic right ventricular cardiomyopathy" with "arrhythmogenic cardiomyopathy" [19]. The reluctance to move rapidly in this direction is due in part to the fact the disease in patients with a PKP2 mutation, by far the most common mutation in the United States and certain parts of Europe, is a right dominant disease, with left sided involvement seen only with advanced disease. With increasing use of implantable cardioverter-defibrillators (ICDs) and prevention of SCD, the development of right- and/or left-sided heart failure and related outcomes is an important consideration in the management of ARVC, and especially for those with left dominant or biventricular disease. (See 'Clinical manifestations' below.)

When considering ARVC, it is important to recognize that this is a progressive disease. Affected individuals do not have evidence of the disease at birth, and typically the disease starts to manifest clinically at approximately 12 or 13 years of age, with the mean age at diagnosis in the early 30s. In one study that examined cardiac function with repeat echocardiography in 85 patients with ARVC after a mean follow-up of six years, RVOT dimension increased from 35 to 37 mm, RV-FAC decreased from 39 to 34 percent, and LVEF decreased from 55 percent to 54 percent, providing evidence that ARVC is associated with deterioration of cardiac function over time [22]. These findings, coupled with the findings of other studies that have shown that development of heart failure and need for cardiac transplantation is more common in competitive athletes, support the current recommendations for exercise restriction in patients with ARVC, as well as those at risk of development of ARVC [22-24]. (See 'Ventricular arrhythmias during exercise' below and "Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis", section on 'Activity restriction'.)

Histology — Histologic examination of patients who have died suddenly often reveals an active inflammatory process consisting of patchy mononuclear infiltrates surrounding degenerated and/or necrotic myocytes. This may represent the acute or "hot" phase of the disease in which disrupted intercellular connections result in an inflammatory process and myocyte cell death [25-27].

It was initially suggested that there were two distinct types of ARVC with differing histologic features [28-30]. In a series of 30 hearts from patients with presumed ARVC, 18 had the fibrolipomatous (fibrofatty) pattern and 12 had the lipomatous (fatty) pattern [30].

The fibrolipomatous (fibrofatty) pattern is characterized by myocardial atrophy and thinning with replacement of myocardium by both fibrous and fatty tissue, as well as patchy inflammatory infiltrates. RV aneurysms and LV involvement are each found in approximately three-quarters of patients with fibrofatty disease [30].

The lipomatous (fatty) pattern is characterized by normal or increased myocardial thickness with exclusively fatty replacement and infrequent inflammation. In the autopsy series of 12 hearts with the fatty pattern, patchy inflammatory infiltrates were found in only two, RV aneurysms in one, and LV involvement in none [30].

A study from Padova revealed difficulty in distinguishing the fatty infiltration seen in ARVC from adipositas cordis seen in obesity from the normal fat of aging [31]. However, at least some cases of fatty infiltration of the RV without fibrosis may represent a distinct pathologic process unrelated to ARVC. This was illustrated in an autopsy study that compared the clinical and pathologic features of 25 hearts with fibrofatty ARVC that included seven cases that only had fatty replacement and 18 control hearts [29]. Twenty-three of the 25 with fibrofatty replacement had died suddenly. Of the seven with only fatty replacement (who had also died suddenly), six had mitigating factors (eg, epilepsy, alcohol-associated liver disease, obesity) and/or other cardiac disease, leaving an open question regarding the significance of the isolated fatty changes. None of the subjects with fatty replacement had a history of arrhythmias or a family history of SCD. In contrast, more than one-half of those with fibrofatty ARVC had these historical features. Epicardial fat infiltration is part of the "normal aging" process of the RV and may confound interpretation of magnetic resonance imaging and autopsy, particularly in adult patients.

Electron microscopy — Most of the gene mutations in ARVC involve proteins that make up desmosomes, which are intracellular adhesion complexes that provide mechanical connections between cardiac myocytes. Support for a pathogenic role of these mutations was provided in an electron microscopy study of RV myocardium from 21 ARVC probands [32]. A variety of abnormalities in desmosomal structure, size, number, or location were noted in up to 75 percent of patients with ARVC, but in none of 10 controls or 10 patients with dilated cardiomyopathy. Similar electron microscopic alterations in gap junction structure were identified in an eight-year-old child from Naxos with the two base pair recessive deletion in plakoglobin who died from acute leukemia. She had significant ECG changes and nonsustained ventricular arrhythmia, and the gap junction abnormalities were seen in the absence of any of the typical histopathological changes [33].

CLINICAL MANIFESTATIONS — Many patients with ARVC remain clinically silent and asymptomatic for decades, making the disease difficult to recognize, especially in sporadic cases with no recognized familial involvement. The clinical presentations of ARVC are variable, including palpitations, syncope, chest pain, dyspnea, and, rarely, SCD. Clinical perspectives of ARVC primarily arise from experience with patients who present with arrhythmia of RV origin and/or sudden death. Presentation is rare before puberty and most common between the ages of 10 and 50 years, with a mean age at diagnosis of approximately 30 years [6-9,19,34]. The disease is virtually never diagnosed in infants or toddlers and uncommonly before the age of 10, while approximately 20 percent of patients are identified after 50 years of age [35]. Male sex has been reported to be associated with more malignant course [6].

ARVC is caused by mutations in a variety of genes, most of which encode desmosomal proteins, with greater than 30 percent of cases being familial in origin [8,36]. Two patterns of inheritance have been described in ARVC:

Autosomal dominant form, which is most common.

Autosomal recessive form (eg, Naxos disease, Carvajal syndrome) in which ARVC is part of a cardiocutaneous syndrome including hyperkeratosis of the palms and soles and woolly hair.

Patients with multiple gene mutations tend to present earlier and with more frequent ventricular tachyarrhythmias, heart failure, and left ventricular dysfunction [21]. Clinical features associated with mutations in each of the associated genes are discussed separately. (See "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics", section on 'Genetics'.)

Symptoms — The majority of patients who are ultimately diagnosed with ARVC are symptomatic on presentation [34]. The principal symptoms associated with ARVC are dizziness, palpitations, and syncope. However, as many as 40 percent of persons diagnosed with ARVC are asymptomatic, coming to attention following screening that was triggered by a definite ARVC diagnosis in a family member.

In one of the largest reported series of 130 patients at a tertiary referral center, the frequency of specific symptoms was as follows [9]:

Palpitations – 67 percent

Syncope – 32 percent

Atypical chest pain – 27 percent

Dyspnea – 11 percent

RV failure – 6 percent

Ventricular arrhythmias — Patients who are diagnosed with ARVC typically present with palpitations or syncope as the manifestation of ventricular arrhythmias. Ventricular arrhythmias can range from frequent ventricular premature beats to sustained ventricular tachycardia (VT) [7,34].

The most common ventricular arrhythmia is sustained or nonsustained monomorphic VT that originates in the RV and therefore has a left bundle branch block (LBBB) pattern [7,8]. As an example, among a cohort of 100 patients with ARVC, 51 of the 69 patients diagnosed antemortem had documented VT with LBBB morphology [7]. VT may originate from the RV inflow tract, RV apex, or RV outflow tract. When VT arises from the RV outflow tract, it may be difficult to distinguish ARVC from idiopathic RV outflow tract tachycardia (waveform 1). (See "Ventricular tachycardia in the absence of apparent structural heart disease", section on 'Repetitive monomorphic VT'.)

The frequency of various ventricular arrhythmias in ARVC differs along with the severity of the disease [8,37].

Out of 439 index patients presenting to two major ARVC referral centers in the Netherlands and US, 268 (61 percent) had experienced ventricular arrhythmia, the majority (95 percent) of which were symptomatic [6].

In a series of 151 patients, ventricular arrhythmias were present in all patients with severe disease, 82 percent with moderate disease, and 23 percent with mild disease as defined by echocardiographic markers of disease severity [8].

Among a cohort of 137 patients with ARVC, including 108 with an ICD, who were prospectively followed for an average of 3.3 years, 22 patients (20 percent) with an ICD experienced a life-threatening ventricular arrhythmia [37]. These data are consistent with ICD therapy rates from ARVC cohorts reported from other major centers, but disproportionately higher than expected sudden death rates. Potential explanations include:

Under-recognition of ARVC as a cause of sudden death

Assignation of greater lethality to the documented VT than actually occurs

An adverse effect of the ICD leads causing disease progression

The incidence of ventricular premature beats in patients with Naxos disease is as high as 92 percent, and patients with Naxos disease have a higher incidence of sustained ventricular arrhythmias and SCD [38,39]. (See "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics", section on 'Autosomal recessive disease and Naxos disease'.)

Although most of these arrhythmias appear to arise from the RV, histopathologic abnormalities (fibrosis and/or adipose tissue) in the LV and in the His bundle and its branches were commonly observed in an autopsy series of patients with ARVC and SCD [18]. This observation suggests a possible role for conduction abnormalities, though clinically significant conduction disease is an uncommon feature in ARVC.

Sudden cardiac death — SCD occurs in patients with ARVC and can be the first presentation of the disease [7,8,12,13,40,41]. In a study of 100 families with ARVC, 49 of the probands had initial presentation with sudden death; 43 of the 49 were aged 11 to 40 years, though 10 percent were >50 years [42]. In a review from France of forensic autopsies on 1930 cases of unexplained SCD (mean age 34 years, males and females equally represented), 200 (10.4 percent) were associated with ARVC [18]. Three-quarters of episodes occurred during routine daily activities, 10 percent during the perioperative period, and only 3.5 percent while participating in sports. (See 'Ventricular arrhythmias during exercise' below.)

Ventricular arrhythmias during exercise — Patients with ARVC and also those at risk of development of ARVC as a result of a desmosomal mutation should avoid competitive and endurance exercise [34]. Both VT and SCD in patients with ARVC can be exercise-induced, and in selected populations ARVC is a frequent cause of SCD in athletes [8,13,18]. Additionally, endurance exercise is strongly associated with disease development, particularly among patients with a desmosomal mutation. (See "Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis" and "Athletes: Overview of sudden cardiac death risk and sport participation", section on 'Arrhythmogenic (right and/or left) ventricular cardiomyopathy'.)

In a report from northern Italy evaluating the causes of SCD among 269 subjects under 35 years of age, ARVC accounted for 22 percent of deaths among the 49 athletes and 8 percent among the 220 non-athletes [13].

In young athletes in the United States, ARVC appears to be a less common cause of SCD, accounting for only 4 percent of deaths in one series of 286 cases of SCD [40].

The risk of SCD during exercise may be due in part to increased stress on the RV [43]. Data from an animal model of ARVC suggest that exercise may increase RV dilation and thus worsen the manifestations of ARVC [44]. The association with exercise and RV stress and the frequent induction of arrhythmia by isoproterenol infusion suggests a role for catecholamines [45,46]. The sensitivity to catecholamines, which may be acquired, could result from abnormalities in cardiac sympathetic function [47,48]. Consistent with these data, abnormal iobenguane I-131 (therapeutic) SPECT uptake, indicative of impaired catecholamine handling, was documented in 25 of 42 (59 percent) ARVC patients and was associated with a higher incidence of arrhythmic events [49].

Data obtained from exercise questionnaires and interviews indicate a strong association of endurance training with disease development and arrhythmic risk, supporting three decades of anecdotal observations that endurance sport is particularly dangerous in ARVC and should be avoided [8,23,50-52]. Studies have also shown that patients with a desmosomal mutation require far less exercise to develop ARVC than patients who are mutation and family history negative [51,52]. Based on this large body of literature, it is now clear that patients with ARVC and also those at risk of development of ARVC as a result of a desmosomal mutation should avoid competitive and endurance exercise. Only low intensity activity (eg, walking, golf, etc) should be advised. Although a specific cut-off for what level of exercise is safe is not well defined, any activity that causes symptoms of palpitations, presyncope, or syncope should be avoided. (See "Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis" and "Athletes: Overview of sudden cardiac death risk and sport participation", section on 'Arrhythmogenic (right and/or left) ventricular cardiomyopathy'.)

Supraventricular tachyarrhythmias — Supraventricular tachyarrhythmia (SVT) is present in up to 25 percent of patients with ARVC referred for treatment of ventricular arrhythmias; less often, SVT may be the only arrhythmia present [53,54]. In decreasing order of frequency, supraventricular arrhythmias in these patients include atrial fibrillation, atrial tachycardia, and atrial flutter. In one cohort of 248 patients with ARVC who were followed for a median of 5.8 years, 35 patients (14 percent) developed one or more SVTs, with the SVT being atrial fibrillation in 80 percent of patients [53]. In a cohort of 66 patients with ARVC without evidence of atrial arrhythmia or heart failure, functional cardiovascular magnetic resonance (CMR) revealed enlarged atria and decreased atrial function as predictors of subsequent atrial arrhythmia, providing supporting evidence that the atria are involved in ARVC [55].

Nonarrhythmic manifestations — A minority of patients with familial ARVC have autosomal recessive disease, which is associated with palmoplantar keratosis and wooly hair. Incomplete cutaneous phenotypes have also been observed in patients with autosomal dominant ARVC. (See "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics", section on 'Autosomal recessive disease and Naxos disease'.)

Intracardiac thrombus within the RV has also been reported [56].

Asymptomatic presentations — ARVC is inherited predominantly in an autosomal dominant pattern with variable penetrance and expressivity. Approximately 40 percent of patients are asymptomatic when ARVC is identified as part of screening of affected family members. ARVC is often suspected due to the presence of nonspecific ECG or echocardiographic abnormalities, or ventricular arrhythmias on Holter or exercise testing in the context of a positive family history [8,9,57]. In a review of 37 families, 151 of 365 family members were classified as affected [8]. An additional 17 subjects were classified as healthy carriers (lacking clinical evidence of ARVC but transmitted ARVC to their progeny) [8].

Left ventricular involvement

Disease patterns — The broader use of CMR, in combination with longer-term follow-up of probands and family members, is allowing for increasingly comprehensive characterization of disease patterns in ARVC. Among the most notable findings is the recognition that involvement of the left ventricle (LV) is more common than previously appreciated. (See "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics", section on 'Desmoplakin gene' and "Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis".)

In a cohort of 200 probands and relatives, all of whom satisfied criteria for familial ARVC (table 1), CMR was performed as part of a comprehensive clinical evaluation [58]. LV involvement was suggested by ECG, rhythm monitoring, and/or CMR findings in >80 percent of patients. Three patterns of disease expression were identified:

Classic – This pattern is defined by isolated RV disease or LV involvement in association with significant RV impairment (78 patients [39 percent], of whom 46 [59 percent] had some LV involvement).

Left dominant – Characterized by early and prominent LV manifestations and relatively mild right-sided disease (10 patients [5 percent]).

Biventricular – Parallel involvement of both ventricles (112 patients [56 percent]).

Left-dominant arrhythmogenic cardiomyopathy — The pathologic process in classic ARVC predominantly involves the RV and sometimes extends to the LV [16,17,58-60]. In contrast, patients with left-dominant arrhythmogenic cardiomyopathy (LDAC, also known as left-sided ARVC or arrhythmogenic left ventricular cardiomyopathy) have pathological changes that predominantly involve the LV [61]. (See 'Left ventricular abnormalities and disease progression' above.)

Patients with LDAC present with palpitations and chest pain in a similar fashion to those with classic ARVC. However, syncope appears to be a less frequent clinical manifestation in LDAC. The clinical characteristics of left-dominant arrhythmogenic cardiomyopathy were described in a 42-patient cohort of individuals with unexplained (infero) lateral T-wave inversion, arrhythmia of LV origin, and/or proven LDAC [61].

Patients presented with arrhythmia or chest pain but not heart failure at ages ranging from adolescence to over 80 years.

By CMR, 12 of 41 patients (29 percent) had an LVEF <50 percent. CMR findings included late gadolinium enhancement (LGE) of the LV in a subepicardial/midwall distribution.

Fifty percent had previously been misdiagnosed with viral myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, or idiopathic VT.

Arrhythmic events included presentation with ventricular fibrillation in one patient and SCD on follow-up of two patients.

Possible left-sided ARVC should be considered in patients who present with sustained ventricular tachyarrhythmia or SCD as the initial manifestation of apparent DCM. (See "Determining the etiology and severity of heart failure or cardiomyopathy".)

DIFFERENTIAL DIAGNOSIS FOR ARVC — The differential diagnosis for the most common presenting symptoms in patients with ARVC (palpitations, syncope, and chest pain) is extensive, as these symptoms, which are common, are neither sensitive nor specific for ARVC. The differential diagnosis for these common symptoms is discussed in detail separately. (See "Evaluation of palpitations in adults" and "Syncope in adults: Clinical manifestations and initial diagnostic evaluation".)

The differential diagnosis for causes of generalized or localized cardiomyopathy with involvement of the RV with associated ventricular arrhythmias is more limited. The ECG characteristics of the VT and the imaging features reflecting localized wall motion abnormalities, with or without inflammation, are often difficult to distinguish from cardiac sarcoidosis and/or myocarditis [62]. In addition, right ventricular enlargement and distorted right ventricular anatomy can occur in a number of other conditions, such as congenital heart disease, pulmonary hypertension, and chest wall deformities [63]. The ECG characteristics of VT seen in ARVC may be indistinguishable from an idiopathic RV tachycardia, which arises from the RV outflow tract and is seen in patients with a normal heart.

Uhl anomaly — Uhl anomaly (also called parchment right ventricle) is a rare disorder characterized by deficiency of RV myocardium. It is a separate entity from ARVC with differing pathologic features and clinical presentation (table 2) [64,65]. The RV is paper-thin and almost devoid of muscle fibers. The finding that a portion of the RV that is completely devoid of ventricular musculature, with apposition of endocardium and epicardium, should be classified as Uhl anomaly rather than partial ARVC.

Cardiac sarcoidosis — Any localized, inflammatory process in the right ventricle, most commonly myocarditis or sarcoidosis, may exhibit clinical features similar to ARVC. Positive findings on endomyocardial biopsy may be helpful in distinguishing these entities, but negative findings are more likely due to sampling error [66,67]. The recognition of LV involvement in the majority of patients with ARVC, however, has removed LV systolic dysfunction as a potential distinguishing feature [67]. (See 'Left ventricular involvement' above and "Clinical manifestations and diagnosis of cardiac sarcoidosis" and "Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis".)

One distinguishing feature is conduction disease, which is a common early manifestation of cardiac sarcoidosis, but uncommon in ARVC. In a comparison of 15 patients with cardiac sarcoidosis (who had definite ARVC based on 2010 diagnostic criteria but were misdiagnosed) and 42 patients with desmosomal mutations and definite ARVC, patients with cardiac sarcoidosis had significant conduction system disease which was not present among ARVC patients, including PR interval prolongation (53 percent versus 0 percent) and high-grade atrioventricular block (46 percent versus 0 percent) [68].

Cardiomyopathy with RV involvement — In classic ARVC, the clinical presentation is with right ventricular arrhythmia and electrocardiographic changes, usually in association with structural and functional changes in the right ventricle that are demonstrable on imaging studies. With disease progression, there may be left ventricular involvement, and in an autopsy series, the majority of hearts had significant left ventricular involvement [17]. This correlated with clinical markers of progression, such as T wave changes extending to V4-V6, ventricular arrhythmia of right bundle branch block as well as left bundle branch block morphology, and decreased left ventricular ejection fraction (LVEF). (See 'Left ventricular involvement' above.)

The distinction between ARVC and dilated cardiomyopathy (DCM) with predominant RV dysfunction may be difficult if the LVEF is less than 50 percent. However, it is rare in ARVC to have marked and progressive left ventricular dilatation. Conversely, it is uncommon to have sustained ventricular tachyarrhythmia or sudden cardiac arrest as the initial presentation of DCM. Therefore, when a patient presents with sustained VT or sudden cardiac arrest, an arrhythmogenic cardiomyopathy should be suspected. (See "Causes of dilated cardiomyopathy".)

Some patients who fulfill the diagnostic criteria for ARVC but have no family history of this disorder may have myocarditis. In a study of 31 patients with a clinical diagnosis of ARVC who underwent electroanatomic mapping, 20 patients had regions of low voltage electrograms and 11 did not [69]. The group with abnormal electrograms had significantly higher rates of myocyte loss and fibrofatty replacement on endomyocardial biopsy, and 65 percent had a family history of ARVC. In contrast, the patients with normal electrograms had inflammatory abnormalities on biopsy specimens, and none had a family history of ARVC. The patients with normal electrograms also had better arrhythmic outcomes. (See "Clinical manifestations and diagnosis of myocarditis in adults".)

Brugada syndrome — The Brugada syndrome, which is manifested by a specific ECG pattern consisting of a pseudo-right bundle branch block and persistent ST segment elevation in leads V1 to V3 (waveform 2), may resemble ARVC. This was illustrated in a report of 96 Italian victims of SCD who were ≤35 years of age and had a baseline ECG available [70]. Right precordial ST segment elevation with or without right bundle branch block was present in 13 (14 percent); at autopsy, all but one had ARVC, though the typical coved Brugada type I pattern is rare in ARVC and should suggest the possibility of two diagnoses. (See "Brugada syndrome: Clinical presentation, diagnosis, and evaluation".)

RVOT tachycardia — VT with LBBB morphology and an inferior axis may originate from the RV outflow tract in patients without structural heart disease. This idiopathic VT, which is a form of repetitive monomorphic VT, generally has a much more benign prognosis than ARVC and can be successfully treated with radiofrequency ablation [71]. In addition, it is not genetically determined, and thus other family members are not at risk. (See "Ventricular tachycardia in the absence of apparent structural heart disease", section on 'RV outflow tract'.)

It is important to distinguish RVOT tachycardia from VT due to ARVC. A potential source of confusion is that RVOT tachycardia, like ARVC, may be associated with right ventricular outflow dilatation. (See "Ventricular tachycardia in the absence of apparent structural heart disease".)

The following tests may be helpful:

ECG – The resting ECG in sinus rhythm is typically normal in patients with RVOT tachycardia. The ECG is also normal in 40 to 50 percent of patients with ARVC at presentation, but in almost no patient after six years [72]. In a cohort of 16 patients with ARVC compared with 42 patients with RVOT tachycardia, patients with ARVC had significantly longer QRS duration in lead V1 (150 versus 123 milliseconds), were more likely to have precordial transition in lead V6 (18 versus 0 percent), and more commonly had at least one lead with QRS notching (65 versus 21 percent) [73]. (See "Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis", section on '12-lead ECG'.)

Electrophysiologic study (EPS) – The response during EPS testing is also helpful. In a study of 56 patients with right VT, including 41 with RVOT tachycardia and 15 with ARVC, who underwent EPS, programmed premature stimulation induced VT in 14 of 15 patients with ARVC compared with only 2 of 41 patients with RVOT tachycardia (93 versus 3 percent) [74]. The induction of VTs with different QRS morphologies was seen only in ARVC (73 versus 0 percent).

If an EPS is performed, most electrophysiologists will administer a 45 mcg/min isoproterenol infusion. This simple test has been shown to be of value in the diagnosis of ARVC. (See "Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis", section on 'ECG with isoproterenol infusion'.)

In addition to the above findings on ECG, surface ECG, and EP testing, a scoring system has been proposed to differentiate VT in patients with ARVC from RVOT tachycardia. In a cohort of 37 patients with ARVC and 49 patients with RVOT tachycardia, the following scoring system was used [75]:

Anterior T-wave inversion in leads V1 to V3 during sinus rhythm – 3 points

QRS duration in lead I ≥120 ms during ventricular arrhythmia – 2 points

QRS notching during ventricular arrhythmia – 2 points

Precordial transition in lead V5 or later during ventricular arrhythmia – 1 point

Patients with a score of 5 or more were found to have ARVC in 93 percent of instances, with a sensitivity and specificity of 84 and 100 percent, respectively.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Cardiomyopathy".)

SUMMARY AND RECOMMENDATIONS

Prevalence – Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a clinical entity characterized by ventricular arrhythmias and a specific ventricular pathology. The incidence of ARVC is unknown, but the prevalence in the general adult population is estimated to be approximately 1 in 2000 to 1 in 5000. (See 'Prevalence' above.)

Anatomy and histology – The anatomic and histologic findings associated with ARVC fall into several categories. Structurally, the hallmark findings are right ventricle (RV) dilation, RV dysfunction, and regional RV wall motion abnormalities, although some patients also have left ventricle (LV) involvement. Histologically, fibrofatty infiltration of the myocardium is the most common finding. (See 'Anatomy and histology' above.)

Clinical manifestations

Symptoms – The principal symptoms of ARVC are dizziness, palpitations, and syncope. Other symptoms include atypical chest pain and dyspnea. As many as 40 percent of patients with ARVC are asymptomatic. (See 'Symptoms' above.)

Ventricular arrhythmias – The frequency of ventricular arrhythmias varies with the severity of ARVC. The most common arrhythmia is monomorphic ventricular tachycardia (VT) (sustained or nonsustained) with a left bundle branch block (LBBB) pattern. Sudden cardiac death (SCD) occurs in patients with ARVC and can be the first presentation of the disease. Although most SCD in ARVC occurs during routine activity, VT and SCD can be exercise-induced. (See 'Ventricular arrhythmias' above.)

Left ventricular involvement – LV involvement is more common in patients with ARVC than initially appreciated. Many patients have similar degrees of involvement of both left and right ventricles, some have predominantly or solely RV involvement, and relatively few have predominantly LV disease. (See 'Left ventricular involvement' above.)

Differential diagnosis – The differential diagnosis for ARVC includes sarcoidosis, myocarditis, and various forms of congenital heart disease, particularly those involving left to right shunts, other cardiomyopathies with RV involvement, Uhl anomaly, and idiopathic RV tachycardia. (See 'Differential diagnosis for ARVC' above.)

  1. Gemayel C, Pelliccia A, Thompson PD. Arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol 2001; 38:1773.
  2. Sen-Chowdhry S, Lowe MD, Sporton SC, McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management. Am J Med 2004; 117:685.
  3. Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm 2018; 15:e190.
  4. Te Riele AS, James CA, Philips B, et al. Mutation-positive arrhythmogenic right ventricular dysplasia/cardiomyopathy: the triangle of dysplasia displaced. J Cardiovasc Electrophysiol 2013; 24:1311.
  5. Richardson P, McKenna W, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation 1996; 93:841.
  6. Groeneweg JA, Bhonsale A, James CA, et al. Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Patients and Family Members. Circ Cardiovasc Genet 2015; 8:437.
  7. Dalal D, Nasir K, Bomma C, et al. Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation 2005; 112:3823.
  8. Nava A, Bauce B, Basso C, et al. Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol 2000; 36:2226.
  9. Hulot JS, Jouven X, Empana JP, et al. Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 2004; 110:1879.
  10. Corrado D, Thiene G. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: clinical impact of molecular genetic studies. Circulation 2006; 113:1634.
  11. McKenna WJ, Judge DP. Epidemiology of the inherited cardiomyopathies. Nat Rev Cardiol 2021; 18:22.
  12. Corrado D, Fontaine G, Marcus FI, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation. Circulation 2000; 101:E101.
  13. Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 1998; 339:364.
  14. te Riele AS, James CA, Groeneweg JA, et al. Approach to family screening in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Eur Heart J 2016; 37:755.
  15. Miles C, Finocchiaro G, Papadakis M, et al. Sudden Death and Left Ventricular Involvement in Arrhythmogenic Cardiomyopathy. Circulation 2019; 139:1786.
  16. Pinamonti B, Sinagra G, Salvi A, et al. Left ventricular involvement in right ventricular dysplasia. Am Heart J 1992; 123:711.
  17. Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol 1997; 30:1512.
  18. Tabib A, Loire R, Chalabreysse L, et al. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia. Circulation 2003; 108:3000.
  19. Towbin JA, McKenna WJ, Abrams DJ, et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm 2019; 16:e301.
  20. Cipriani A, Bauce B, De Lazzari M, et al. Arrhythmogenic Right Ventricular Cardiomyopathy: Characterization of Left Ventricular Phenotype and Differential Diagnosis With Dilated Cardiomyopathy. J Am Heart Assoc 2020; 9:e014628.
  21. Bhonsale A, Groeneweg JA, James CA, et al. Impact of genotype on clinical course in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated mutation carriers. Eur Heart J 2015; 36:847.
  22. Mast TP, James CA, Calkins H, et al. Evaluation of Structural Progression in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. JAMA Cardiol 2017; 2:293.
  23. James CA, Bhonsale A, Tichnell C, et al. Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol 2013; 62:1290.
  24. Saberniak J, Hasselberg NE, Borgquist R, et al. Vigorous physical activity impairs myocardial function in patients with arrhythmogenic right ventricular cardiomyopathy and in mutation positive family members. Eur J Heart Fail 2014; 16:1337.
  25. Caforio ALP, Re F, Avella A, et al. Evidence From Family Studies for Autoimmunity in Arrhythmogenic Right Ventricular Cardiomyopathy: Associations of Circulating Anti-Heart and Anti-Intercalated Disk Autoantibodies With Disease Severity and Family History. Circulation 2020; 141:1238.
  26. Chatterjee D, Fatah M, Akdis D, et al. An autoantibody identifies arrhythmogenic right ventricular cardiomyopathy and participates in its pathogenesis. Eur Heart J 2018; 39:3932.
  27. Poller W, Haas J, Klingel K, et al. Familial Recurrent Myocarditis Triggered by Exercise in Patients With a Truncating Variant of the Desmoplakin Gene. J Am Heart Assoc 2020; 9:e015289.
  28. Thiene G, Corrado D, Nava A, et al. Right ventricular cardiomyopathy: is there evidence of an inflammatory aetiology? Eur Heart J 1991; 12 Suppl D:22.
  29. Burke AP, Farb A, Tashko G, Virmani R. Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: are they different diseases? Circulation 1998; 97:1571.
  30. Basso C, Thiene G, Corrado D, et al. Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis? Circulation 1996; 94:983.
  31. Basso C, Thiene G. Adipositas cordis, fatty infiltration of the right ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a matter of fat? Cardiovasc Pathol 2005; 14:37.
  32. Basso C, Czarnowska E, Della Barbera M, et al. Ultrastructural evidence of intercalated disc remodelling in arrhythmogenic right ventricular cardiomyopathy: an electron microscopy investigation on endomyocardial biopsies. Eur Heart J 2006; 27:1847.
  33. Kaplan SR, Gard JJ, Protonotarios N, et al. Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease). Heart Rhythm 2004; 1:3.
  34. Corrado D, Link MS, Calkins H. Arrhythmogenic Right Ventricular Cardiomyopathy. N Engl J Med 2017; 376:61.
  35. Bhonsale A, Te Riele ASJM, Sawant AC, et al. Cardiac phenotype and long-term prognosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia patients with late presentation. Heart Rhythm 2017; 14:883.
  36. Basso C, Ronco F, Marcus F, et al. Quantitative assessment of endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy/dysplasia: an in vitro validation of diagnostic criteria. Eur Heart J 2008; 29:2760.
  37. Link MS, Laidlaw D, Polonsky B, et al. Ventricular arrhythmias in the North American multidisciplinary study of ARVC: predictors, characteristics, and treatment. J Am Coll Cardiol 2014; 64:119.
  38. Protonotarios N, Tsatsopoulou A, Anastasakis A, et al. Genotype-phenotype assessment in autosomal recessive arrhythmogenic right ventricular cardiomyopathy (Naxos disease) caused by a deletion in plakoglobin. J Am Coll Cardiol 2001; 38:1477.
  39. Antoniades L, Tsatsopoulou A, Anastasakis A, et al. Arrhythmogenic right ventricular cardiomyopathy caused by deletions in plakophilin-2 and plakoglobin (Naxos disease) in families from Greece and Cyprus: genotype-phenotype relations, diagnostic features and prognosis. Eur Heart J 2006; 27:2208.
  40. Maron BJ, Carney KP, Lever HM, et al. Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003; 41:974.
  41. Thiene G, Nava A, Corrado D, et al. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988; 318:129.
  42. Quarta G, Muir A, Pantazis A, et al. Familial evaluation in arrhythmogenic right ventricular cardiomyopathy: impact of genetics and revised task force criteria. Circulation 2011; 123:2701.
  43. Ector J, Ganame J, van der Merwe N, et al. Reduced right ventricular ejection fraction in endurance athletes presenting with ventricular arrhythmias: a quantitative angiographic assessment. Eur Heart J 2007; 28:345.
  44. Kirchhof P, Fabritz L, Zwiener M, et al. Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice. Circulation 2006; 114:1799.
  45. Haissaguerre M, Le Métayer P, D'Ivernois C, et al. Distinctive response of arrhythmogenic right ventricular disease to high dose isoproterenol. Pacing Clin Electrophysiol 1990; 13:2119.
  46. Leclercq JF, Potenza S, Maison-Blanche P, et al. Determinants of spontaneous occurrence of sustained monomorphic ventricular tachycardia in right ventricular dysplasia. J Am Coll Cardiol 1996; 28:720.
  47. Wichter T, Hindricks G, Lerch H, et al. Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy. An analysis using 123I-meta-iodobenzylguanidine scintigraphy. Circulation 1994; 89:667.
  48. Wichter T, Schäfers M, Rhodes CG, et al. Abnormalities of cardiac sympathetic innervation in arrhythmogenic right ventricular cardiomyopathy : quantitative assessment of presynaptic norepinephrine reuptake and postsynaptic beta-adrenergic receptor density with positron emission tomography. Circulation 2000; 101:1552.
  49. Paul M, Wichter T, Kies P, et al. Cardiac sympathetic dysfunction in genotyped patients with arrhythmogenic right ventricular cardiomyopathy and risk of recurrent ventricular tachyarrhythmias. J Nucl Med 2011; 52:1559.
  50. Maron BJ, Ackerman MJ, Nishimura RA, et al. Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol 2005; 45:1340.
  51. Sawant AC, Bhonsale A, te Riele AS, et al. Exercise has a disproportionate role in the pathogenesis of arrhythmogenic right ventricular dysplasia/cardiomyopathy in patients without desmosomal mutations. J Am Heart Assoc 2014; 3:e001471.
  52. Sawant AC, Te Riele AS, Tichnell C, et al. Safety of American Heart Association-recommended minimum exercise for desmosomal mutation carriers. Heart Rhythm 2016; 13:199.
  53. Camm CF, James CA, Tichnell C, et al. Prevalence of atrial arrhythmias in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Heart Rhythm 2013; 10:1661.
  54. Chu AF, Zado E, Marchlinski FE. Atrial arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and ventricular tachycardia. Am J Cardiol 2010; 106:720.
  55. Zghaib T, Bourfiss M, van der Heijden JF, et al. Atrial Dysfunction in Arrhythmogenic Right Ventricular Cardiomyopathy. Circ Cardiovasc Imaging 2018; 11:e007344.
  56. Wu L, Yao Y, Chen G, et al. Intracardiac thrombosis in patients with arrhythmogenic right ventricular cardiomyopathy. J Cardiovasc Electrophysiol 2014; 25:1359.
  57. Perrin MJ, Angaran P, Laksman Z, et al. Exercise testing in asymptomatic gene carriers exposes a latent electrical substrate of arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol 2013; 62:1772.
  58. Sen-Chowdhry S, Syrris P, Ward D, et al. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression. Circulation 2007; 115:1710.
  59. Tandri H, Saranathan M, Rodriguez ER, et al. Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging. J Am Coll Cardiol 2005; 45:98.
  60. Sen-Chowdhry S, Prasad SK, Syrris P, et al. Cardiovascular magnetic resonance in arrhythmogenic right ventricular cardiomyopathy revisited: comparison with task force criteria and genotype. J Am Coll Cardiol 2006; 48:2132.
  61. Sen-Chowdhry S, Syrris P, Prasad SK, et al. Left-dominant arrhythmogenic cardiomyopathy: an under-recognized clinical entity. J Am Coll Cardiol 2008; 52:2175.
  62. Dechering DG, Kochhäuser S, Wasmer K, et al. Electrophysiological characteristics of ventricular tachyarrhythmias in cardiac sarcoidosis versus arrhythmogenic right ventricular cardiomyopathy. Heart Rhythm 2013; 10:158.
  63. Quarta G, Husain SI, Flett AS, et al. Arrhythmogenic right ventricular cardiomyopathy mimics: role of cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2013; 15:16.
  64. UHL HS. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 1952; 91:197.
  65. Gerlis LM, Schmidt-Ott SC, Ho SY, Anderson RH. Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia. Br Heart J 1993; 69:142.
  66. Chimenti C, Pieroni M, Maseri A, Frustaci A. Histologic findings in patients with clinical and instrumental diagnosis of sporadic arrhythmogenic right ventricular dysplasia. J Am Coll Cardiol 2004; 43:2305.
  67. Vasaiwala SC, Finn C, Delpriore J, et al. Prospective study of cardiac sarcoid mimicking arrhythmogenic right ventricular dysplasia. J Cardiovasc Electrophysiol 2009; 20:473.
  68. Philips B, Madhavan S, James CA, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. Circ Arrhythm Electrophysiol 2014; 7:230.
  69. Corrado D, Basso C, Leoni L, et al. Three-dimensional electroanatomic voltage mapping increases accuracy of diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 2005; 111:3042.
  70. Corrado D, Basso C, Buja G, et al. Right bundle branch block, right precordial st-segment elevation, and sudden death in young people. Circulation 2001; 103:710.
  71. Le Guludec D, Gauthier H, Porcher R, et al. Prognostic value of radionuclide angiography in patients with right ventricular arrhythmias. Circulation 2001; 103:1972.
  72. Jaoude SA, Leclercq JF, Coumel P. Progressive ECG changes in arrhythmogenic right ventricular disease. Evidence for an evolving disease. Eur Heart J 1996; 17:1717.
  73. Hoffmayer KS, Machado ON, Marcus GM, et al. Electrocardiographic comparison of ventricular arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy and right ventricular outflow tract tachycardia. J Am Coll Cardiol 2011; 58:831.
  74. Niroomand F, Carbucicchio C, Tondo C, et al. Electrophysiological characteristics and outcome in patients with idiopathic right ventricular arrhythmia compared with arrhythmogenic right ventricular dysplasia. Heart 2002; 87:41.
  75. Hoffmayer KS, Bhave PD, Marcus GM, et al. An electrocardiographic scoring system for distinguishing right ventricular outflow tract arrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy from idiopathic ventricular tachycardia. Heart Rhythm 2013; 10:477.
Topic 4931 Version 33.0

References

آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟