Left ventricular involvement in right ventricular dysplasia (original) (raw)

Right ventricular dysplasia: angiographic study

European Heart Journal, 1989

At the moment, the most reliable method for diagnosing right ventricular dysplasia is considered to be angiography. Morphological alterations such as the presence of akinetic/dyskinetic areas, aneurysmatic dilatations and deep anteroapical fissuring, not necessarily associated with an increase in ventricular volume, are understood to be angiographic criteria indicating dysplasia. To verify their diagnostic value, these abnormalities have been evaluated in: (1) 33 patients suspected of having dysplasia because of PVBs with LBBB morphology and with 'borderline' involvement of the right ventricle or without instrumental evidence of cardiac disease (Group A); (2) 16 subjects with no arrhythmia and normal left ventricular angiography, coronary and bioptic findings (Group B); (3) 36 patients with a clinical, angiographic and bioptic diagnosis of dilated idiopathic cardiomyopathy (Group C). In 48-5% of the patients in Group A, angiography showed localized akinesia/dyskinesia (12 patients), small conical outpouchings persisting during systole (10 patients) and apical deep fissuring (two patients). In 81% of these patients, endomyocardial biopsy showed the presence of fibrous and/or adipose tissue in at least 20% of the examined sample. Angiographic abnormalities suggesting dysplasia were found in none of the normal subjects and only in two of the 36 patients with dilated cardiomyopathy (5-5%).

Right ventricular dysplasia: Right and left ventricular involvement morphometrically evaluated

Cardiovascular Pathology, 1995

Right ventricular dysplasia (RVD) is a cardiac anomaly characterized by replacement of variable amounts of right ventricular myocardium by adipose tissue. This condition is believed to be a selective disorder involving extensively the right ventricle, but there are occasional reports of concomitant "minor" abnormalities of the left ventricle. The object of this report concerns a patient who died after heart transplantation because of an unsuspected RVD of the donor heart. We present a morphometric study of the heart in order to evaluate the distribution of the fat on both ventricles and to understand the structural basis of the heart failure. The results show that a large portion of the right ventricle is replaced by fat with a quite homogeneous distribution; the left ventricle is also largely replaced by adipose tissue that is primarily localized at the apex and decreases from the apex to the basis. The remodeling of the heart is attributable to a conspicuous increase in volume of the right ventricle assocrated with a normal number of myocytes that are longer than normal. For these reasons, according to Starling's law, the heart develops congestive failure.

Right Ventricular Dysplasia: A Report of 24 Adult Cases

Annals of Noninvasive Electrocardiology, 1999

Right ventricular dysplasia is characterized by an abnormality in the development of part of the right ventricular musculature. Patients with right ventricular dysplasia may present with ventricular tachycardia, supraventricular arrhythmias, right-heart failure or asymptomatic cardiomegaly. Twenty-two adult patients with right ventricular dysplasia who had recurrent ventricular tachycardia were seen during a 7-year period. The male/female ratio was 2.7:1. The mean age at the time of hospitalization was 39 years. All but one of the patients had ventricular tachycardia of a left bundle branch block configuration. With few exceptions, the T waves were inverted over the right precordial leads. The heart was usually enlarged and the pulmonary vasculature was usually normal. In six patients who had two-dimensional echocardiograms, all showed increased right ventricular diastolic dimensions. All patients had right ventricular angiography; the diagnosis of right ventricular dysplasia was substantiated during surgery in 12 patients and at autopsy in another. Two other patients who did not have arrhythmias had right ventricular dysplasia diagnosed by right- and left-heart angiography. Our unique experience, when combined with a literature review of 34 adult cases, permits a composite clinical profile of this condition in the adult.

Unusual Clinical Presentation of a Patient With an Extreme Form of Right Ventricular Dysplasia

Circulation, 2001

A previously healthy 42-year-old woman presented with recurrent reversible neurological deficits. On admission, the surface ECG showed a very low voltage QRS pattern along with prominent P-waves ( ). Computerized tomography of the brain was normal. Transthoracic echocardiography showed massive dilatation and extensive dyskinesia of the right ventricle and evidence of fatty deposits in the myocardial structure, suggesting a diagnosis of right ventricular dysplasia. The patient had a patent foramen ovale, which could have caused paradoxical emboli to the brain. A coronary angiogram was normal. Right ventriculography showed sacculations that are typically observed in patients with right ventricular dysplasia. Left ventriculography showed normal myocardial function. In electrophysiological testing, no arrhythmias could be induced by programmed ventricular stimulation. MRI showed fatty replacement of right ventricular myocardium ). In addition, there was massive dilatation of the right heart chambers, which essentially encircled the left heart chambers . Because the left ventricle makes the most significant contribution to QRS forces, these findings would explain the discrepancy between the markedly attenuated QRS complexes and the prominent P-waves, a rarely observed ECG pattern in clinical practice. Figure 1. A 12-lead ECG showing low-voltage QRS complexes and prominent P-waves.

ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA

Annual Review of Medicine, 1999

Arrhythmogenic right ventricular dysplasia (ARVD) is a new form of cardiomyopathy probably more frequent than commonly reported. It is a rare but important cause of sudden arrhythmic death in young, otherwise healthy persons, as well as a subtle cause of congestive heart failure. It may lead to temporary incapacitation with catastrophic consequences. Proper electrocardiographic criteria, echocardiography, nuclear medicine, or magnetic resonance imaging could identify most of these individuals. With the exception of full-thickness histological examination of the right ventricular free wall, contrast ventriculography remains the most definitive standard for a positive diagnosis. The wide clinical spectrum of arrhythmogenic right ventricular cardiomyopathies/dysplasia appears to be the result of one or possibly two factors: (a) replacement of most of the right ventricular myocardium by fat and (b) genetic susceptibility to environmental agents (myocarditis). Current treatment modalities include drug therapy, catheter or surgical ablative techniques, and modern treatments of congestive heart failure. Heart transplant is exceptional. Implantable defibrillators, used alone or in combination with drug therapy, will probably play an increasing role in ARVD and related cardiomyopathies.

Right Ventricular Dysplasia With Biventricular Involvement

Circulation, 1998

A 23-year-old man was admitted to the hospital with severe heart failure and cachexia. Ventricular arrhythmias and progressive heart failure (predominantly right heart) had been observed in the previous 3 years. Physical examination was unremarkable except for a widely split second heart sound, a systolic left precordial lift, third and fourth heart sounds, and signs of increased venous pressure. Chest radiography showed significant cardiomegaly. The ECG was characterized by right atrial enlargement, low QRS voltages, wide complexes in the right precordial leads (epsilon From the

Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Screening, diagnosis, and treatment

Heart Rhythm, 2006

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disorder characterized pathologically by fatty or fibrofatty replacement and electrical instability of the right ventricular myocardium. Clinical manifestations include structural and functional malformations (fatty infiltration, dilatation, aneurysms) of the right ventricle, ECG abnormalities, and presentation with ventricular tachycardias with left bundle branch block pattern or sudden death. The disease often is familial with an autosomal inheritance. The typical hallmarks of ARVD/C are distributed in the so-called "triangle of dysplasia." The functional and morphologic characteristics are relevant to clinical imaging approaches such as contrast angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and cardiovascular magnetic resonance imaging. Evident forms of the disease are straightforward to diagnose based on a series of diagnostic criteria proposed by the International Task Force for Cardiomyopathy. However, the diagnosis of early and mild forms of the disease often is difficult. Treatment is directed toward preventing life-threatening ventricular arrhythmias in which radiofrequency ablation and implantable defibrillators play an increasing role. Despite new diagnostic and therapeutic approaches in ARVD/C, uncertainties about the etiology of the disease, the genetic basis, the appropriate diagnosis and therapy, and the clinical course of patients with ARVD/C have resulted in several registries to increase our knowledge of this intriguing disease.