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(Circulation. 2000;101:e101.)
© 2000 American Heart Association, Inc.

Circulation Electronic Pages

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Need for an International Registry

Domenico Corrado, MD; Guy Fontaine, MD; Frank I. Marcus, MD; William J. McKenna, MD; Andrea Nava, MD; Gaetano Thiene, MD; Thomas Wichter, MD; for the 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

From the Departments of Cardiology (D.C., A.N.) and Pathology (G.T.), University of Padova, Italy; Department of Cardiology (G.F.), Hôpital Jean Rostand, Ivry-sur-Seine, Paris, France; Section of Cardiology (F.I.M.), University of Arizona, Tucson, Az; Department of Cardiological Sciences (W.J.M.), St George’s Hospital Medical School, London, UK; and Department of Cardiology and Angiology (T.W), T. Medizinische Klinik und Poliklinik, Innere Medizin, Westfälische Wilhelms-Universität, Münster, Germany.

Correspondence to Domenico Corrado, MD, Department of Cardiology, University of Padua Medical School, Via N. Giustiniani 2—35121 Padova, Italy. E-mail cardpath{at}ux.1.unipd.it

	Abstract

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
Abstract—Arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar RV involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group on ARVD/C 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 is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since it first was described in 1977. The present article focuses on important but still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed and thus answer pending questions. The registry also will facilitate pathological, molecular, and genetics research on the causes and pathogenesis of the ARVD/C. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C.

Key Words: cardiomyopathy • electrophysiology • genetics • death, sudden • tachyarrhythmias

	Introduction

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
Arrhythmogenic right ventricular (RV) dysplasia or cardiomyopathy (ARVD/C) is a heart muscle disease, often familial, characterized by structural and functional abnormalities of the RV due to replacement of the myocardium by fatty and fibrous tissue.^{1 2 3 4 5 6 7} Clinical presentation of ARVD/C usually consists of arrhythmias of RV origin that range from isolated premature ventricular beats to sustained ventricular tachycardia or ventricular fibrillation that leads to sudden death.^{1 3 4 5 6 7 8 9 10 11} Other clinical manifestations include global or regional dysfunction and structural alterations of the RV; ECG depolarization/repolarization changes, characteristically in right precordial leads; and evolution to right or biventricular heart failure that mimics dilated cardiomyopathy.^{1 3 4 5 6 7 9 12}

In the 22 years since ARVD/C was first described,¹³ previously reported as auricularization of the RV,¹⁴ considerable progress has been made in our understanding of the pathogenesis, morbid anatomy, and clinical presentation of this condition.^{6 7 15 16} However, a great lack of information still exists with regard to genetics, clinical diagnosis, natural history, risk stratification, outcome, and comparative efficacy of antiarrhythmic treatment and prophylaxis of life-threatening ventricular arrhythmias and prevention of death in patients with ARVD/C.

The present report focuses on important but still unanswered clinical problems, mostly regarding outcome and management of ARVD/C patients. The need to accumulate information in a centralized international registry to answer pending questions is stressed.

	Morbid Anatomy and Histology

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
The most striking morphological feature of ARVD/C is diffuse or segmental lack of myocardium in the RV free wall, which is replaced by fatty or fibrofatty tissue.^{1 2 3 4 5} Only subendocardial layers are preserved, in which some myocardium appears to be interspersed with fibrosis. Persistent strands of cardiomyocytes bordered by or embedded in a variable area of fibrosis are observed also in the epicardial and mediomural layers inside the fat. Patchy acute myocarditis with myocyte death and focal round cell inflammatory infiltrates (mostly lymphocytes)¹⁷ are present in two thirds of cases.⁴ This disease is largely overlooked by routine autopsy, because the limit between normal and pathological fatty infiltration is unclear.^{4 18 19} Certain studies now recognize that presence of fat interspersed with cardiomyocytes is observed in a sizable numbers of normal adults.^{18 19}

The purely adipose form of ARVD/C is characterized by partial or almost total replacement of RV wall by fatty tissue, with predominant involvement of the apex and infundibulum, in absence of fibrosis and inflammatory infiltrates.^{3 4 5 6 7 19} In this variant, risk of sudden death in the absence of other concomitant heart disease is controversial.¹⁹

The fibrofatty form was the original type of description, characterized by fibrosis that borders or is embedded with cardiomyocytes, RV wall thinning with aneurysmal dilatation, and inflammatory infiltrates.^{1 2 3 4 5 6 7} Aneurysms that typically affect inflow, apical, and outflow portions of the RV ("triangle of dysplasia")¹ have been reported in 50% of the cases in the autopsy series.⁴ In this variant, the LV and, more rarely, the ventricular septum may be involved to a lesser extent.^{3 4 5 6 7 17}

	Genetics and Epidemiology

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
A familial history of ARVD/C is present in 30% to 50% of cases.^{20 21 22 23 24 25 26} The most common pattern of inheritance is autosomal-dominant, although an autosomal-recessive pattern has also been reported. Linkage analysis has located the genetic abnormality on chromosomes 1, 2, 3, and 14 for the dominant form^{21 22 23 24 25} and on chromosome 17 for the recessive variant of the disease.²⁶ This latter form is characteristically associated with epidermal abnormalities such as palmoplantar keratosis and woolly hair ("Naxos disease"). In this condition, signs of the disease are more severe and penetrance in family members is 90%.²⁶ Some families are not linked to these loci, which suggests further genetic heterogeneity. A genetic test is not currently available.

Incidence and prevalence of ARVD/C are unknown. Patients with a clinical diagnosis of ARVD/C based on symptoms, right precordial ECG changes, RV arrhythmias, and structural and functional RV abnormalities represent only one extreme of the disease spectrum. Several cases have not been recognized because patients were asymptomatic until first presentation with sudden death or were difficult to diagnose by conventional noninvasive methods.^{1 5 27} In this regard, a prospective investigation of sudden death in the young in the Veneto region of Italy showed that 20% of fatal events in young people and athletes were due to previously undiagnosed ARVD/C.^{28 29} At the other extreme of the spectrum are patients in whom the diagnosis of ARVD/C was not recognized at onset of symptoms but who present years later with congestive heart failure with or without ventricular arrhythmias and are often wrongly diagnosed as having dilated cardiomyopathy.^{1 4 5 9 12 30}

The high incidence of ARVD/C in Northern Italy has not been confirmed in studies from North America.¹⁹ Prevalence of the disease observed in different parts of the world could be due either to clustering of the disease in some geographic areas or, more likely, to the fact that this entity is being underdiagnosed both pathologically and clinically.¹⁶

	Etiopathogenesis

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
ARVD/C has been added to the cardiomyopathies group in a recent revision of classifications of the Task Force of the World Health Organization/International Society and Federation of Cardiology.³¹ Replacement of the RV myocardium by fibrofatty tissue has been related to 3 basic mechanisms:

1. Apoptosis or programmed cell death^{32 33} ;
   
2. Inflammatory heart disease with a spectrum of clinical presentations that range from acute myocarditis⁴ to fibrous healing, which in severe forms may involve both RV and LV and may lead to congestive heart failure that mimics dilated cardiomyopathy^{3 4 5 6 7 12 30} ; and
   
3. Myocardial dystrophy that seems independent of myocarditis and might reflect genetically determined atrophy.⁴
   

	Clinical Diagnosis

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
Standardized diagnostic criteria have been proposed by the Study Group on ARVD/C of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.³⁴ Diagnosis of ARVD/C is based on presence of major and minor criteria that encompass structural, histologic, electrocardiographic, arrhythmic, and genetic factors (Table). On the basis of this classification, diagnosis of ARVD/C is fulfilled in the presence of 2 major criteria, or 1 major plus 2 minor criteria, or 4 minor criteria from different groups. Although these guidelines represent a useful clinical approach to ARVD/C diagnosis, optimal assessment of diagnostic criteria requires prospective evaluation obtained by international registry from a large population.³⁵ The phenotype of ARVD/C is probably broader than has been recognized to date, and minor electrocardiographic or RV structural abnormalities that overlap with normality may represent disease expression, particularly in context of proven disease within families. Therefore, current guidelines on diagnosis present criteria that are specific but may lack sensitivity. Ultimately, a molecular genetic diagnosis should be the gold standard against which clinical diagnostic criteria can be tested.

View this table: [in this window] [in a new window]   Table 1. Criteria for Diagnosis of ARVD/C

Diagnosis of ARVD/C at its early stages remains a clinical challenge.^{1 4 5 6 7 27} Diagnosis of ARVD/C is difficult in patients with minimal RV abnormalities at echocardiographic or angiographic examination.^{36 37} Endomyocardial biopsy has the potential for in vivo demonstration of typical fibrofatty replacement of the RV myocardium However, sensitivity of this test is low because, for reasons of safety, samples are usually taken from septum, a region uncommonly involved by the disease.³⁸ MRI is a promising technique for delineation of RV anatomy and function as well as for characterizing the composition of the RV wall, especially with regard to the presence of fatty tissue.^{39 40 41 42} However, diagnostic sensitivity and specificity of MRI still need to be defined, because the quality of images detected is at present operator dependent and results largely subject to individual interpretation.

One practical problem is differentiation between ARVD/C and RV outflow-tract tachycardia, a usually benign and nonfamilial arrhythmic condition. Whether RV outflow-tract tachycardia represents a forme fruste of ARVD/C, as suggested by RV structural abnormalities often detected by MRI, is still a matter of debate.⁴³

	Disease Progression and LV Involvement

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
Clinical and pathological evidence suggests that ARVD/C is a progressive heart muscle disease. Long-term follow-up data from clinical studies indicate that the RV may become more diffusely involved with time.^{44 45 46 47} As the disease progresses, the LV may be progressively affected with subsequent biventricular failure. Recently, a multicenter clinicopathological investigation was performed to further define the anatomoclinical profile of ARVD/C, with special reference to disease progression and LV involvement.⁵ Through examination of 42 affected, whole hearts, which included those removed at transplant, and by correlating pathological findings with patient clinical history, the study demonstrated that, at least in this subgroup that represents an extreme of the disease spectrum, ARVD/C can no longer be regarded as an isolated disease of the RV. Macroscopic or histologic involvement of the LV was found in 76% of hearts with ARVD/C. This involvement was age dependent, more common in patients with long-standing clinical history, and progressive, as evaluated by serial echocardiographic examinations. Moreover, LV lesions were associated with clinical arrhythmic events, more severe cardiomegaly, inflammatory infiltrates, and heart failure.

	Natural History

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
Natural history of ARVD/C is a function of both cardiac electrical instability and progressive ventricular dysfunction.^{1 3 4 5 6 7 45 46 47 48} Ventricular arrhythmias range from isolated premature ventricular beats to nonsustained or sustained ventricular tachycardia, which may lead to ventricular fibrillation and sudden death at any time during the disease.^{1 3 4 5 10} An imbalance of adrenergic innervation has been documented and may contribute to arrhythmogenesis in ARVD/C because it increases propensity for ventricular tachyarrhythmias by dispersion of refractoriness and generation of delayed afterdepolarizations, particularly during exercise and catecholamine exposure.⁴⁹ Later in the disease evolution, progressive impairment of ventricular contractility may result in right or biventricular heart failure.^{3 4 5 6 7 12 45 46 47 48}

At present, information is limited regarding the clinical course of ARVD/C even in patients with overt disease and significant ventricular arrhythmias, and even less is known about asymptomatic, affected family members. However, the following clinicopathological phases should be considered⁴⁸ : (1) the concealed phase, characterized by subtle RV structural changes, with or without minor ventricular arrhythmias, during which sudden death may occasionally be the first manifestation of the disease (mostly in young people during competitive sports or intense physical exercise)^{3 4 5 20 28 29} ; (2) an overt electrical disorder, in which symptomatic RV arrhythmias that may cause cardiac arrest are associated with overt RV functional and structural abnormalities^{1 3 4 5 6 7 8} ; (3) RV failure due to progression and extension of RV muscle disease that provokes global RV dysfunction with relatively preserved LV function^{1 4 5 47} ; (4) the final stage of biventricular pump failure due to significant LV involvement.^{4 5 44 45 46 47 48} At this stage, ARVD/C mimics biventricular dilated cardiomyopathy of other causes that leads to congestive heart failure and such related complications as atrial fibrillation and thromboembolic events.

	Therapy

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
Because clinical findings that predict long-term outcome of patients with ARVD/C are incompletely known, no precise guidelines exist to select patients who should be treated with ß-blockers, antiarrhythmic drugs, or an implantable cardioverter-defibrillator.⁵⁰ Moreover, how to best predict the efficacy of both pharmacological and nonpharmacological therapy in patients with ARVD/C is unclear. Management of patients with ARVD/C is individualized, and strategies are based on local experience of the different centers. Patients with well-tolerated and non-life-threatening ventricular arrhythmias are usually treated empirically with antiarrhythmic drugs, including amiodarone, sotalol, ß-blockers, flecainide, and propafenone, alone or in combination.⁵¹ In patients with sustained ventricular tachycardia or ventricular fibrillation, antiarrhythmic drug therapy guided by programmed ventricular stimulation with serial drug testing may be more reliable than the empirical approach for the suppression of ventricular arrhythmias,⁵¹ even if efficacy on prevention of sudden death remains unproved. Nonpharmacological therapy, including catheter ablation and implantable cardioverter–defibrillator use, is reserved for patients with life-threatening ventricular arrhythmias in whom drug therapy is ineffective, is associated with serious side effects, or is not applicable because clinical ventricular arrhythmias cannot be reproducibly induced during electrophysiological study.^{52 53 54} Although the implantable cardioverter-defibrillator is the most effective safeguard against sudden arrhythmic death,^{53 54} its precise role in ARVD/C needs to be evaluated in a large series of patients.

In patients in whom ARVD/C has progressed to severe RV or biventricular systolic dysfunction, treatment consists of current therapy for heart failure, including diuretics, angiotensin-converting enzyme inhibitors, and digitalis, as well as anticoagulant therapy. These patients may become candidates for heart transplantation.

	International Registry

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
An international registry has been established by the Study Group on ARVD/C 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 and it will officially start on January 1, 2001. The ARVD/C International Registry is necessary to collect a large group of patients and prospectively evaluate the accuracy of clinical diagnosis, long-term outcome, and efficacy of therapy. Availability of this population could facilitate evaluation of new diagnostic and therapeutic approaches. The registry will also facilitate pathological, molecular, and genetic research on the causes and pathogenesis of ARVD/C. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among cardiologists.

The primary objective of the ARVD/C International Registry will be follow-up of a large patient population for 10 years, with the following aims:

Clinical Diagnosis—To prospectively validate criteria for clinical diagnosis of ARVD/C proposed by the Study Group of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.³⁴

Natural History—To assess the natural course of the disease, particularly occurrence of sudden death and heart failure, in different clinical ARVD/C subgroups of patients and in asymptomatic family members.

Risk Stratification—To identify clinical markers of sudden death and poor prognosis.

Antiarrhythmic Therapy—To improve clinical management by evaluating long-term efficacy of empiric and electrophysiologically guided antiarrhythmic drug therapy and nonpharmacological treatment, including catheter ablation and use of the implantable cardioverter defibrillator.

The ARVD/C International Registry clearly will enhance diagnostic accuracy, help in understanding of the natural history of the disease, allow risk stratification, and improve clinical management of patients with ARVD/C as well as enhance scientific collaboration on behalf of patients with this disease and their families. Cardiologists are urged to cooperate by entering their patients into the ARVD/C International Registry. Enrollment forms with detailed entry criteria and semiannual follow-up forms will be provided for each patient. Of note, patients will be treated according to the best judgment of their personal physicians without interference by the registry. However, participating physicians will be able to query the data bank regarding clinicotherapeutic questions. Contact the ARVD/C International Registry at the addresses listed in the Appendix.

	Appendix 1

Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
ARVD/C International Registry Contact Information
Domenico Corrado, Department of Cardiology, University of Padua Medical School, via Giustiniani 2, 35121, Padova, Italy. Telephone 39 049 827 2286; Fax 39 049 827 2284.

Guy Fontaine, Hopital Jean Rostand, 39-41 rue jean Le Galleu, 94200, Ivry-sur-Seine Paris, France. Telephone 33-1-495-97062; Fax 33-1-495-97073.

Frank I. Marcus, Section of Cardiology, University of Arizona. 1501 N Campbell Ave, PO Box 24-5037, Tucson, AZ 85724-5037. Telephone 520-626-6358; Fax 520-626-4333.

William J. McKenna, Department of Cardiological Sciences, St George’s Hospital Medical School, Cranmer Terrace, London SW 17 0RE, United Kingdom. Telephone 44-181-725-5913; Fax 44-181-682-0944.

Andrea Nava, Department of Cardiology, University of Padua Medical School, via Giustiniani 2, 35121, Padova, Italy. Telephone 39-049-821-2426; Fax 39-049-875-4179.

Gaetano Thiene, Department of Pathology, University of Padua Medical School, via Gabelli 61, 35121 Padova, Italy. Telephone 39-049-827-2283; Fax 39-049-827-2284.

Thomas Wichter, Department of Cardiology and Angiology, T. Medizinische Klinik und Poliklinik, Innere Medizin, Westfälische Wilhelms-Universität, Albert-Schweitzer-Strasse 33, D-48129 Münster, Germany. Telephone 49-251-834-7617; Fax 49-251- 834-7864.

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Top Abstract Introduction Morbid Anatomy and Histology Genetics and Epidemiology Etiopathogenesis Clinical Diagnosis Disease Progression and LV... Natural History Therapy International Registry Appendix 1 References

 
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