Surgery for ventricular tachycardia associated with right ventricular dysplasia: disarticulation of right ventricle in 9 of 10 cases.

Ten patients (nine men, one woman; mean age 39 years) with arrhythmogenic right ventricular dysplasia underwent surgery to control life-threatening drug refractory ventricular arrhythmias. All had ventricular tachycardia causing syncope and six had a history of cardiac arrest. In all a minimum of three antiarrhythmic drugs (mean five) had been ineffective. At operation, the right ventricle was grossly diseased in all patients. Ventricular tachycardias were induced and mapped intraoperatively in all patients. The surgical plan was to ablate the arrhythmogenic focus if it was less than 4 cm2; one patient was so managed. Of the remaining nine, four underwent partial (approximately 40% of the right ventricular free wall) and five underwent total right ventricular disarticulation. All survived the operation and are alive at a mean follow-up interval of 24 months (range 5 to 67). Two patients developed new sustained ventricular tachycardias. These were well tolerated and, unlike the original arrhythmias, were easily controlled by drug treatment. All patients who underwent right ventricular disarticulation manifested signs of right heart failure in the early postoperative period, but these lessened progressively with the development of systolic septal movement into the right ventricular cavity. All 10 patients are in New York Heart Association class I or II at last review. In selected patients with arrhythmogenic right ventricular dysplasia, surgery offers a curative treatment for ventricular tachycardia and should be considered for patients whose arrhythmias are life-threatening and refractory to drug treatment.

Risk-benefit profiles of antiarrhythmic therapy.

Whereas much has been learned of the mechanisms and pathophysiology of cardiac arrhythmias, treatment remains controversial and is largely empiric. Cardiac arrhythmias range in severity from innocuous to fatal; antiarrhythmic agents offer differing degrees of efficacy, inefficacy or toxicity. For an arrhythmia that needs treatment, there is no reliable or easy method for selecting the most appropriate antiarrhythmic drug. Antiarrhythmic treatment is justified for arrhythmias that are symptomatic or hemodynamically significant, the risk of lethality dictating the degree of drug toxicity that might be tolerated. The treatment of prognostically important arrhythmias is indicated if their suppression is rewarded by an improved prognosis. However, these arrhythmias are often merely indicators of a bad prognosis rather than the cause of death. Assessment of the risk-benefit ratio of antiarrhythmic treatment principally depends on the perceived risk and symptomatology of the arrhythmia and the efficacy and toxicity of the antiarrhythmic drug. Beta-adrenoceptor blocking drugs offer optimal risk-benefits for the prevention of ventricular fibrillation (infarct survivors and long QT syndrome); disopyramide and the class IC agents offer acceptable risk-benefit ratios for the treatment of ventricular tachycardia, whereas no antiarrhythmic drug offers an acceptable risk-benefit for the suppression of asymptomatic "cosmetic" atrial or ventricular ectopic beats.

Echocardiographic features of mitral valve prolapse in Libyan patients.

Echocardiographic observations in 200 subjects with mitral valve prolapse (MVP) are presented. The diagnostic criteria used were: (1) abrupt late systolic posterior motion of one or both leaflets of the mitral valve, and (2) holoor pansystolic posterior motion of 3 mm of one or both leaflets of the mitral valve. Most of the subjects were young--72% were aged less than 30 years. Prolapse of posterior leaflets was noted in 98% of subjects--69.5% late systolic, 28.5% pansystolic, and 2% had prolapse of the anterior mitral leaflet only. Mitral valve prolapse was considered to be primary--being the only abnormality in 78.5% of the subjects. In the remaining 21.5% MVP was associated with other cardiac lesions, the commonest being, atrial septal defect (2.5%), dilated aortic root (2%), bicuspid aortic valve (2%), cardiomyopathy (5%), rheumatic heart disease (4%) and ischaemic heart disease (1.5%). Mitral valve prolapse was considered to be important enough to result in haemodynamically significant mitral regurgitation in only 8% of subjects. Mitral valve prolapse was the commonest single echocardiographic abnormality (16%) observed in patients referred to this university hospital, which is the referral centre for approximately half of Libya. Although this does not indicate the prevalence of MVP in the general population, this study indicates MVP to be the commonest valvular abnormality seen in hospital practice in Libya.

Coexistence of red cell aplasia and autoimmune haemolytic anaemia in a family.

Red cell aplasia is thought to have an immunological basis and has been reported to exist in association with various immunological abnormalities including autoimmune haemolytic anaemia in the same patient. A case is reported of a patient with pure red cell aplasia probably of acquired primary variety; but laboratory tests revealed only slight immunological abnormalities. On the other hand, the mother of the patient had autoimmune haemolytic anaemia. It is believed that such a familial coexistence of the diseases may further support the immunological basis of red cell aplasia.

Acute and chronic hemodynamic impact of total right ventricular disarticulation.

Right ventricular disarticulation is a radical operation to control ventricular arrhythmias in patients with arrhythmogenic right ventricular dysplasia. This report describes the acute and chronic hemodynamic impact of the procedure based on our experience of five patients with life-threatening arrhythmias unresponsive to medical therapy who have undergone total disarticulation of the right ventricle. Although all patients suffered acute postoperative hemodynamic problems, all survived and returned to an excellent functional class. Right ventricular disarticulation should be considered in patients with drug refractory ventricular tachycardias due to arrhythmogenic right ventricular dysplasia when the arrhythmia either poses a life threat or results in chronic morbidity.