Inexcitable right ventricle and bilateral bundle branch block in Uhl's disease.

Bharati, Saroja; Ciraulo, Domenic A.; BILITCH, MICHAEL; Rosen, Kenneth M.; Lev, Maurice

doi:10.1161/01.cir.57.3.636

Cited by 54 publications

(12 citation statements)

References 16 publications

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“…ARVD is generally considered to be due to abnormality of devetopment mostly affecting the RV. This interpretation fits quite well with the embryologic development of the myocardium [23]. Thus, involvement of the embryologically adjacent LV seems plausible.…”

Section: Gross Pathology and Histologysupporting

confidence: 85%

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The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy

Fontaine

Fontaliran

et al. 1995

Heart Vessels

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Twenty-four patients presenting with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ventricular tachycardia of right ventricular origin associated with structural abnormalities of the right ventricle) were divided into two groups with left ventricular ejection fraction (LVEF) above or below 45%. The distribution of LVEF in the group with LVEF below 45% was comparable with the distribution in 6 patients with idiopathic dilated cardiomyopathy who had ventricular tachycardia originating in the left ventricle (P = 0.2). They also had the same unfavorable long-term prognosis. Therefore, it is suggested that the term, arrhythmogenic right ventricular cardiomyopathy (ARVC), be restricted to patients with a LVEF below 45%. Histological data obtained in the ARVC group showed signs of acute or chronic myocarditis (in the right and left ventricles). It can be hypothesized that patients with arrhythmogenic right ventricular dysplasia (ARVD) may be prone to develop infectious myocarditis. In patients in whom an abnormal host immune response had been seen, progressive deterioration of right and left ventricular function could be observed. This pattern may be superimposed on the genetically determined background of ARVD. This could explain the wide spectrum of clinical presentation observed in patients with tachycardia originating in an abnormal right ventricle.

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Section: Gross Pathology and Histologysupporting

confidence: 85%

“…usually manifests as varying degrees of infiltration of fat and fibrous tissue that has the same pathological characteristics as in the RV [5,23,[25][26][27][28]. This could be present in a rather limited area of myocardium or could extend to large zones of the LV musculature [29].…”

Section: Fibrofatty Replacement Of Left Ventricular Myocardium Left mentioning

confidence: 99%

The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy

Fontaine

Fontaliran

et al. 1995

Heart Vessels

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“…The one major difference is that in Uhl's case, the patient did not have heart block. However, on careful review of other reported cases, we found that heart block has been seen in Uhl's anomaly in an exceptionally well-studied case in which the conduction system was examined (56) and, furthermore, that heart block is not all that rare in arrhythmogenic right ventricular dysplasia (57, 58), although conduction system studies have been reported in which no anatomical abnormalities were found (59). How all of this may be linked for both Uhl's anomaly and arrhythmogenic right ventricular dysplasia is discussed below.…”

Section: Massive Apoptotic Destruction Of the Right Ventricle And Itsmentioning

confidence: 99%

Normal and Abnormal Consequences of Apoptosis in the Human Heart

James

1998

Annu. Rev. Physiol.

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Knowledge about apoptosis has become essential for understanding many aspects of cardiac structure and function. In the human heart there are major periods of morphogenesis that begin only after birth, and some of these processes recur intermittently for many years. Although the exact mechanisms by which these events are initiated or terminated remain poorly understood, it is clear that their benefits may be mirrored in destructive effects. In this review, selected examples include normal morphogenesis of the cardiac conduction system and the normal postnatal involution of the right ventricle, both of which are mediated by apoptosis. Destructive counterparts include familial heart block ending in fatal arrhythmias, similar results in the long QT syndrome, and the pathogenesis of both Uhl's anomaly and arrhythmogenic right ventricular dysplasia; in each apoptosis is an important factor.

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“…This is in contrast to Uhl's anomaly, where many cases occur in the very young and usually manifest as stillbirth or congestive heart failure in early childhood. 6 The 12-year-old boy described in this report is the youngest patient presenting with this condition in this area. Rheumatic fever is an important and common cause of tricuspid valve regurgitation in our area, and always needs to be ruled out.…”

Section: Discussionmentioning

confidence: 77%

Arrhythmogenic Right Ventricular Dysplasia (ARVD) in Childhood: Case Report with a Review of the Literature

Jarallah

1997

Ann Saudi Med

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AS Al Jarallah, Arrhythmogenic Right Ventricular Dysplasia (Arvd) in Childhood: Case Report with a Review of the Literature. 1997; 17(3): 350-353 Over the last few decades of advances in the field of cardiology, many newly identified diseases have been described. One of these newly recognized conditions was arrhythmogenic right ventricular dysplasia (ARVD), which was first described in 1978 by Frank et al. 1 The clinical spectrum of this condition was described by Marcus et al. in 1982. 2 Estimates of the prevalence of ARVD vary sharply in the literature. Thiene et al. found a startling 20% incidence of ARVD in a study of sudden deaths in young adults originating from northeast Italy. 3 In contrast, in two retrospective studies of sudden deaths in young American adults, the incidence of ARVD was found to be 0.5%-3.0%. 4 In this condition, areas of fatty and fibrous tissues replace the normal myocardium of the right ventricle. The involved myocardium evokes ventricular arrhythmias of right ventricular origin. The diagnosis of ARVD in the past was based on the presence of ventricular arrhythmia with left bundle branch block configuration and morphologic changes or motion abnormalities in the free wall of the right ventricle. 2 Recently, standardized diagnostic criteria have been proposed, based on the presence of major and minor criteria encompassing structural, histological, electrocardiographic, arrhythmia, and genetic factors. 5 Although the long-term prognosis of ARVD is generally good, the prevalence is unknown because of its rarity, especially in the pediatric age group. A male child with ARVD is reported here, with a review of the literature. Case ReportA 12-year-old boy presented with an 18-month history of progressive shortness of breath and recurrent vomiting. Nine months prior to admission, he was seen in a private clinic with severe dyspnea and tachycardia (heart rate of 216 beats/min), where he was diagnosed with heart failure and treated with digoxin, furosemide and spironolactone. There was no history of rheumatic fever or endocarditis and no family history of heart disease.General examination revealed a puffy child, mildly dyspneic, and pink in color. Weight was in the 50th percentile, height in the 10th percentile, temperature was normal, respiratory rate was 22 per minute. Pulse was 110 per minute, regular, good volume, with no radio-femoral delay. Blood pressure was 110/75 mmHg in the right upper arm. Precordial examination revealed no chest deformity, with quiet precordium, no thrill. First and second heart sounds were muffled, third heart sound was audible (gallop rhythm) with a grade 3/6 pansystolic murmur at the left lower sternal border and the lung fields were clear with equal air entry. Abdomen was slightly distended with mild ascites and tender hepatomegaly (6 cm below the right costal margin). There was pitting edema involving both lower limbs.Chest x-ray showed globular enlargement of the heart (cardiothoracic ratio 70%) (Figure 1). The 12-leads electrocardiography showed sinus rh...

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Inexcitable right ventricle and bilateral bundle branch block in Uhl's disease.

Cited by 54 publications

References 16 publications

The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy

The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy

Normal and Abnormal Consequences of Apoptosis in the Human Heart

Arrhythmogenic Right Ventricular Dysplasia (ARVD) in Childhood: Case Report with a Review of the Literature

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