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[Arrhythmogenic dysplasia of the right ventricle].

Arrhythmogenic right ventricular dysplasia is a disease of the cardiac muscle of unknown etiology. Landmarks of this disease are the presence of muscular atrophy and replacement of ventricular myocardium by adipous and fibroadipous tissue. This disease was originally described by Fontaine et al in 1977 during surgical ablation of drug refractory ventricular tachycardias in patients without evident structural heart disease. During surgery anomalies in contractility of the right ventricle and the presence of adipous tissue were documented. Some years later, Markus et al reported the first clinical series of patients with arrhythmogenic right ventricular dysplasia. Since then, this disease has been widely recognized and must be considered in the differential diagnosis of all patients with ventricular arrhythmias originating in the right ventricle.

Arrhythmias, Cardiac↗

The physiologic basis for the management of ventricular assist devices.

Mechanical ventricular assist devices are now approved as destination therapy for terminal heart failure. It is the purpose of this review to discuss the physiology of this technology that is considered in outpatient care. The currently available pulsatile devices are solely dependent of preload volume and, when placed in the automatic mode, can maintain physiologic cardiac outputs with exercise. However, because of their dependence on preload volume, there are unique physiologic consequences; device bradycardia represents volume depletion, device tachycardia reflects volume overload. The differential diagnosis of left ventricular assist device dysfunction includes native right ventricular failure, native left ventricular recovery, or other technical considerations. The management of biventricular mechanical support as well as arrhythmia management and the role of echocardiographic assessment in this unique patient population will be discussed. Expertise in outpatient management of such devices is now a requisite for subspecialists in heart failure, In the future, technical innovations may simplify management for professionals, patients, and their families.

Blood Pressure↗

Arrhythmogenic right ventricular cardiomyopathy: clinicopathologic correlation based on a revised definition of pathologic patterns.

Different morphologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) have been described. However, it is still unclear whether they correspond to distinct forms of the same disease. A pathologic study was performed on a series of ARVC (15 from heart transplant and 12 from autopsy) from 2 Italian referral university hospitals. Based on both myocellular features and the nature of myocardial replacement, hearts were divided into 2 groups: infiltrative, with a lacelike pattern of transmural fatty infiltration and strands of normal residual cardiomyocytes (n = 11); and cardiomyopathic, with massive myocardial replacement by fibro fatty tissue and cardiomyopathic changes (such as hypertrophy and myofibril loss) of residual cardiomyocytes (n = 16). Hearts from the infiltrative group were mostly obtained at autopsy of patients who died suddenly. Fatty substitution was limited almost exclusively to the right ventricle. Mitral valve dysplasia (prolapse or cleft) was frequently present. Hearts from the cardiomyopathic group came mainly from heart transplants for congestive heart failure. Fibro fatty replacement was more extensive, usually biventricular. Active myocarditis and features suggestive of myocardial transdifferentiation were also observed. Despite these differences in clinical outcome and morphologic features, patients from the 2 groups showed similar mean age, sex distribution, occurrence of threatening ventricular arrhythmias, and prevalence of family history of sudden death, arrhythmias, or cardiomyopathy. Infiltrative and cardiomyopathic patterns represent different clinical and pathologic subsets of ARVC. Myocellular features are an important clue in the distinction between the two entities. The differentiation between the 2 patterns is feasible on endomyocardial biopsy and could give important prognostic information.

Adipose Tissue↗

Reduction of QRS duration after pulmonary valve replacement in adult Fallot patients is related to reduction of right ventricular volume.

AIMS: Late after total correction, Fallot patients with a long QRS duration are prone to serious arrhythmias and sudden cardiac death. Pulmonary regurgitation is a common cause of right ventricular (RV) failure and QRS lengthening. We studied the effects of pulmonary valve replacement (PVR) on QRS duration and RV volume. METHODS AND RESULTS: Twenty-six consecutive Fallot patients were evaluated both pre-operatively and 6-12 months post-operatively by cardiac magnetic resonance (CMR). In this study, we present the computer-assisted analysis of the standard 12-lead electrocardiograms closest in time to the CMR studies. For the whole group, QRS duration shortened by 6+/-8 ms, from 151+/-30 to 144+/-29 ms (P=0.002). QRS duration decreased in 18 of 26 patients by 10+/-6 ms, from 152+/-32 to 142+/-31 ms. QRS duration remained constant or increased slightly in eight of 26 patients by 3+/-3 ms, from 148+/-27 to 151+/-25 ms. CMR showed a decrease in RV end-diastolic volume from 305+/-87 to 210+/-62 mL (P=0.000004). QRS duration changes correlated with RV end-diastolic volume changes (r=0.54, P=0.01). CONCLUSION: Our study shows that PVR reduces QRS duration. The amount of QRS reduction is related to the success of the operation, as expressed by the reduction in RV end-diastolic volume.

Adult↗

NT-proBNP reflects right ventricular structure and function in pulmonary hypertension.

The aim of the current study was to investigate whether alterations in N-terminal pro brain natriuretic peptide (NT-proBNP) reflect changes in right ventricular structure and function in pulmonary hypertension patients during treatment. The study consisted of 30 pulmonary hypertension patients; 15 newly diagnosed and 15 on long-term treatment. NT-proBNP, right heart catheterisation and cardiac magnetic resonance imaging measurements were performed, at baseline and follow-up. There were no significant differences between newly diagnosed patients and those on treatment at baseline or follow-up with respect to NT-proBNP, haemodynamics and right ventricular parameters. Relative changes in NT-proBNP during treatment were correlated to the relative changes in right ventricular end-diastolic volume index (r = 0.59), right ventricular mass index (r = 0.62) and right ventricular ejection fraction (r = -0.81). N-terminal pro brain natriuretic peptide measurements reflect changes in magnetic resonance imaging-measured right ventricular structure and function in pulmonary hypertension patients. An increase in N-terminal pro brain natriuretic peptide over time reflects right ventricular dilatation concomitant to hypertrophy and deterioration of systolic function.

Adult↗

The management of mechanical hearts.

Mechanical ventricular assist devices are now approved as destination therapy for terminal heart failure. It is the purpose of this review to discuss the physiology of this technology that is considered in outpatient care. The currently available pulsatile devices are solely dependent of preload volume, and when placed in the automatic mode, can maintain physiologic cardiac outputs with exercise. However, because of their dependence on preload volume, there are unique physiologic consequences; device bradycardia represents volume depletion, device tachycardia reflects volume overload. The differential diagnosis of left ventricular assist device dysfunction includes native right ventricular failure, native left ventricular recovery, or other technical considerations. The management of biventricular mechanical support will be discussed, as well as arrhythmia management and the role of echocardiographic assessment in this unique patient population. Expertise in outpatient management of such devices is now a requisite for subspecialists in heart failure. In the future, technical innovations may simplify management for professionals, patients and their families.

Arrhythmias, Cardiac↗

[Infarction in the inferior myocardium with and without damage to the right ventricle. Treatment and prognosis].

Acute inferior infarction often involves the right ventricle as well. For these patients, the prognosis is not as good as for patients with inferior infarction alone. Right ventricular infarction can be diagnosed with a high level of sensitivity and specificity by recording right precordial leads. We used V4R. 100 patients admitted to the Coronary Care Unit with an electrocardiographic diagnosis of inferior infarction were registrated consecutively. V4R was recorded in 95 patients; the right ventricle was affected in 35 of them. The mortality during the stay in hospital was 3% in the group with inferior infarction and 11% when the right ventricle was involved (p > 0.05). An unusually large proportion of the patients in both groups received thrombolytic therapy (more than 70%). The result may indicate that thrombolytic therapy is of particular benefit to patients with inferior infarction and involvement of the right ventricle.

Adult↗

Hyperuricaemia in patients with right or left heart failure.

Based on the clinical observation that patients with right or left heart failure often present with hyperuricaemia, the relation between serum urate values and haemodynamic variables was studied in patients with primary pulmonary hypertension (PPH) as well as in patients with advanced ischaemic heart disease or dilated cardiomyopathy. The study was a retrospective analysis of 39 patients with PPH and 36 patients with left heart disease, examining serum urate levels in association with haemodynamic variables. Elevated urate concentrations were found in 79% of the PPH patients. There was no association between serum urate levels and mean pulmonary artery pressures, but a significant correlation was found between urate levels and the cardiac index (r=0.48; p=0.0021) and an even stronger correlation between serum urate levels and mean right atrial pressures (r=0.83; p<0.0001). A similar association was found in a subgroup of 21 PPH patients not receiving diuretics. In 36 patients with ischaemic heart disease or dilated cardiomyopathy, hyperuricaemia was present in 78% and was significantly associated with elevated right atrial pressures (r=0.40; p=0.031) and even more so with elevated left atrial pressures (r=0.55; p=0.0005) but not with the cardiac index (r=0.034; p=0.86). The data show that hyperuricaemia in patients with cardiac dysfunction is closely related to elevated right or left atrial filling pressures.

Adult↗