Clinical sustained uniform ventricular tachycardia in hypertrophic cardiomyopathy: association with left ventricular apical aneurysm

Of 51 patients with hypertrophic cardiomyopathy who had episodes of ventricular tachycardia detected during ambulatory electrocardiographic monitoring only two had clinical sustained uniform ventricular tachycardia that required medical treatment because of worsening symptoms. In both patients the arrhythmia was associated with the uncommon finding of an apical aneurysm with angiographically normal coronary arteries.     

Clinical sustained uniform ventricular tachycardia in hypertrophic cardiomyopathy: association with left ventricular apical aneurysm.

Of 51 patients with hypertrophic cardiomyopathy who had episodes of ventricular tachycardia detected during ambulatory electrocardiographic monitoring only two had clinical sustained uniform ventricular tachycardia that required medical treatment because of worsening symptoms. In both patients the arrhythmia was associated with the uncommon finding of an apical aneurysm with angiographically normal coronary arteries.     

Myocardial infarction with normal coronary angiogram. Possible mechanism of smoking risk in coronary artery disease.

The coronary angiograms of 120 consecutive patients under 40 years of age were examined. Ten new cases of myocardial infarction with normal coronary arteriogram were identified (group 1) and compared with 30 cases of myocardial infarction and obstructive coronary disease (group 2). Heavy cigarette smoking was the sole major risk factor in group 1. Patients in group 2 smoked as well but most also had hypercholesterolaemia or hypertension. Pre- and postinfarction angina was rare among the patients with myocardial infarction and normal coronary arteriogram, and recanalisation after smoking-induced thrombotic occlusion is thought to be the most likely mechanism. Smoking-induced thrombosis is only likely to be recognised in special circumstances, when it develops in apparently normal coronary arteries, is followed by recanalisation, and is complicated by infarction as a permanent marker of previous obstruction to regional myocardial blood flow. Thrombotic occlusion of a "normal" coronary artery without recanalisation will only be recognised when infarction is fatal. If smoking can predispose to thrombosis in "normal" coronary arteries, it may be even more likely to accelerate thrombosis in atheromatous coronary arteries. The importance of recognising group 1 may well be in relation to the much commoner group 2.     

A radioreceptor assay for quantitating plasma factor VIII/von Willebrand's protein

A sensitive and precise radioreceptor assay for determining plasma levels of human factor VIII/von Willebrand's factor (FVIII/vWF) has been developed by taking advantage of the FVIII/vWF receptor sites on human platelets. Paraformaldehyde-fixed platelets, which were processed and then stored, retained FVIII/vWF binding activity and therefore could be used as a convenient source of receptors. The human plasma samples to be tested were initially filtered on 4% agarose columns to concentrate the FVIII/vWF protein in the void volume and to remove the factor(s) that interferes with the assay. The percent recovery of FVIII/vWF in the pooled eluent was measured by the recovery of added trace 125I-FVIII/vWF. The coefficients of intra- and interassay variation were 6% and 10%, respectively. The plasma FVIII/vWF concentrations determined by the assay for pooled normal plasma, hemophilia A plasma, and plasmas from two patients with von Willebrand's disease were 16.3 +/- 0.5, 52.6 +/- 1.5, 6.8 +/- 0.8, and 3.2 +/- 0.2 microgram/ml, respectively. The range of plasma FVIII/vWF concentrations varied between 8.3 microgram/ml and 24.9 microgram/ml for 10 normal adults. The plasma FVIII/vWF concentrations determined by the radioreceptor assay correlated well with levels measured by the ristocetin-induced platelet aggregation method, thus demonstrating the functional relevancy of the radioreceptor assay for plasma FVIII/vWF.     

Myocardial disarray in Noonan syndrome

Objective—To characterise the histopathology of the left ventricular hypertrophy commonly associated with Noonan syndrome by assessing the extent of myocyte disarray and therefore to define one aspect of the relation between this disease and idiopathic hypertrophic cardiomyopathy.

Design—Blinded histological analysis.

Setting—Hospital medical school.

Patients—Six hearts of children with the Noonan phenotype and isolated ventricular hypertrophy were compared with age and sex matched controls.

Methods—Histological analysis was performed with an image analyser under light microscopy. Representative sections from the entire left ventricular free wall were examined. Results were expressed as the percentage of fields showing disarray related to the number of fields evaluated: 100 fields were examined for each patient.

Results—In the patients with Noonan syndrome myocardial disarray was present in the ventricular septum in 24 (5·7)% (mean (SD)) of fields and in the free wall in 22·2 (6·8)%. In the controls disarray was present in the septum in 3·8 (2·3)% of fields and in the free wall in 2·4 (2·8)%. In both regions the extent of disarray was significantly greater in patients with Noonan syndrome (p < 0·0005; 95% confidence interval 14 to 26·3 for the septum: p < 0·005, 95% confidence interval 11·4 to 28·2 for the free wall).

Conclusions—The ventricular hypertrophy associated with Noonan syndrome is histologically similar to hypertrophic cardiomyopathy but whether the two diseases are the expression of the same genetic defect remains to be determined.

     

Sclerosing Mesenteritis With Occult Ileal Perforation: Report of a Case Simulating Extensive Intra-Abdominal Malignancy

Sclerosing mesenteritis is a rare condition that is characterized by fibrosis affecting mainly small-bowel mesentery, which in extensive cases may mimic advanced intra-abdominal malignancy. Establishing the diagnosis in such cases is a clinical and histopathologic challenge. We report the successful management of a case of extensive sclerosing mesenteritis with occult ileal perforation, which was possibly the triggering cause. Severe complications occurred as a result of both the disease itself and its surgical treatment. Despite the complex course and life-threatening complications, a good prognosis can be expected. Although occasional recovery has been attributed to spontaneous regression and response to immunosuppressive therapy, a search for, and full eradication of, possible triggering focus is of paramount importance.Reprints are not available.