TTE with Valsalva manoeuvre is as good as TEE in diagnosing shunts. Valsalva manoeuvre increases the size of shunt. Both techniques produce false negative results.

Objective-To assess whether the size of the cytosine-thymine-guanine (CTG) expansion mutation in myotonic dystrophy predicts progression of conduction system disease and cardiac events. Design-Longitudinal study involving ECG and clinical follow up over (mean (SD)) 4.8 (1.8) and 6.2 (1.9) years, respectively, of patients stratified by CTG expansion size (E0 to E4). Patients-73 adult patients under annual review in a regional myotonic dystrophy clinic. Patients were grouped into E0/E1 (n = 25), E2 (n = 34), and E3/E4 (n = 14). Results-The proportion of patients with a QRS complex > 100 ms at baseline increased with the size of the CTG expansion (EO/E1, 4%; E2, 12%; E3/E4, 36%; p = 0.02). This trend was more pronounced at follow up (E0/E1, 4%; E2, 21%; E3/E4, 57%; p = 0.0004). The rate of widening of the QRS complex (ms/year) was similarly related to the size of the mutation (EO/E1, 0.4 (1.3); E2, 1.4 (2.5); E3/E4, 1.5 (1.6); p = 0.04). First degree atrioventricular block was present in 23% of patients at baseline and 34% at follow up, with no significant relation to expansion size. Seven patients suVered a cardiac event during follow up (sudden death in two, permanent pacemaker insertion in three, chronic atrial arrhythmia in two), of whom six were in CTG expansion group E2 or greater. Patients who experienced a cardiac event during follow up had more rapid rates of PR interval increase (9.9 (11.1) v 1.6 (2.9) ms/year; p = 0.008) and a trend to greater QRS complex widening (3.6 (4.5) v 0.9 (1.5) ms/year; p = 0.06) than those who did not. Conclusions-Larger CTG expansions are associated with a higher rate of conduction disease progression and a trend to increased risk of cardiac events in myotonic dystrophy. (Heart 2001;86:411-416)

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Primary cardiac sarcomas are rare and typically undergo aggressive local spread. There is no reliable definitive treatment, although radical surgical resection can provide palliation in the medium term. A case of a pleomorphic leiomyosarcoma with dramatic images is presented. The relative usefulness of transoesophageal echocardiography and cardiovascular magnetic resonance imaging to define the extent of tumour involvement, allowing planning of treatment, is demonstrated.

Coronary ischaemia in acute endocarditis is usually due to pre-existing coronary disease or occasionally as a result of embolism from vegetations. A 68 year old man with known mixed aortic valve disease presented with a four week history of progressive exertional angina, which became unstable. He was apyrexial with no peripheral signs of endocarditis. Three sets of blood cultures were negative. Transthoracic echocardiography with suboptimal windows confirmed moderate mixed aortic valve disease. Marked reversible ST segment depression with angina recurred at rest. Aortography showed severe aortic regurgitation with a distorted aortic root. Coronary angiography showed severe proximal narrowing of the left anterior descending and circumflex arteries with an unusual long and tapering contour. Emergency surgery revealed a large anterior aortic root abscess which had destroyed the left and right coronary cusps. Aortic root abscess and other rare causes of extrinsic coronary compression are discussed. CASE REPORTA 68 year old man with known mixed aortic valve disease presented with a four week history of progressive exertional angina, which became unstable. A month previously he experienced rigors but spontaneously improved without treatment. He had no risk factors for coronary disease. Examination revealed moderate mixed aortic valve disease and blood pressure 140/70 mm Hg.He was apyrexial with no peripheral signs of endocarditis. The resting electrocardiogram showed left ventricular hypertrophy, left axis deviation, and 1 mm of ST depression in V6. The chest radiograph showed mild cardiomegaly and upper lobe venous diversion. Haemoglobin concentration was 93 g/l, mean cell volume 70 fl, serum iron 0.9 µmol/l, total iron binding capacity 55 µmol/l (45-75), white cell count 10.8 × 10 9 /l, platelets 316 × 10 9 /l, erythrocyte sedimentation rate (ESR) 100 mm/hour, and C-reactive protein 60 mg/dl. Three sets of blood cultures and serial cardiac enzymes were negative. Transthoracic echocardiography with suboptimal windows revealed overall moderate left ventricular systolic function, posterioapical hypokinesis and a calcified, immobile aortic valve. Doppler predicted a peak aortic valve gradient of 60 mm Hg with moderate aortic regurgitation. Despite oral metoprolol, nitrates, intravenous unfractionated heparin and a three unit blood transfusion, marked reversible ST segment depression with angina recurred at rest accompanied by hypotension. Emergency coronary angiography showed severe proximal narrowing of the left anterior descending (LAD) and circumflex arteries with an unusual long and tapering contour (fig 1). Aortography showed severe aortic regurgitation with a distorted aortic root and a peak to peak withdrawal gradient of 45 mm Hg across the aortic valve.Emergency surgery revealed a large anterior aortic root abscess which had destroyed the left and right coronary cusps with penetration into but not through the interventricular septum. The aortic root was replaced with a 25 mm Carbomedics valved conduit with reimp...