Cardiac assessment of patients for haematopoietic stem cell transplantation.

The expanding role of haematopoietic stem-cell transplantation (HSCT) renders the previous policy of avoiding transplantation in high-risk cardiac patients obsolete. Patients with amyloid, autoimmune conditions, sickle-cell disease, or thalassaemia, and patients over the age of 60 years are increasingly being offered HSCT. It is evident that the policy of avoiding transplantation in patients with impaired systolic function fails to identify all high-risk patients in such groups, and will deprive some patients of the benefits of HSCT unnecessarily. The development of an appropriate algorithm for cardiac pre-assessment and peri-transplant management is hampered by an inadequate understanding of the predictive value of various tests of cardiovascular function, the rapid evolution of advanced management strategies for cardiac dysfunction, and the development of non-cardiotoxic conditioning regimens. To meet this need we propose that an algorithm based on evidence from other clinical situations - already been found to be successful in the management of HSCT in patients with systemic sclerosis - should be used uniformly, and registry studies should be undertaken to distinguish those aspects of the algorithm that positively help to expand the remit of HSCT from those that add little of value.

Angiographically proven coronary artery disease in scleroderma.

OBJECTIVE: It has been suggested that macrovascular disease is more common in patients with scleroderma (SSc). We investigated the prevalence of coronary artery disease (CAD) in SSc using coronary angiography. METHODS: Coronary angiography was performed in 172 patients with SSc and suspected CAD to examine the prevalence of significant CAD. The prevalence of CAD was estimated in the whole group and also according to age, gender and type of symptoms (typical angina, atypical angina and non-anginal pain or breathlessness). Standardized prevalence ratios (SPRs) were calculated in each symptomatic group in order to compare CAD rates amongst our observed population with those predicted using the Diamond and Forrester (D & F) probability analysis. This analysis provides an estimate of the probability of CAD based on gender, age and symptoms in subjects aged between 30-69 yrs. RESULTS: The observed prevalence of CAD in the whole population was 22% (38/172); 17% (6/36) in males and 23% (32/136) in females. A total of 41 patients were excluded because they were outside the age range for D & F analysis. Compared with the reference population, the SPRs for CAD in the three SSc groups were: 47% (95% CI 21.7-89.9) in the typical angina group (22 patients), 50% (95% CI 13.6-128) in the atypical angina group (22 patients) and 93% (95% CI 49.4-158.8) in the non-anginal pain or breathlessness group (87 patients). CONCLUSION: The prevalence of CAD in patients with SSc is similar and not greater to that expected in individuals without SSc.

Systemic sclerosis associated pulmonary hypertension: improved survival in the current era.

OBJECTIVES: To measure survival, haemodynamic function and functional class in patients with systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) in two treatment eras. METHODS: Six year longitudinal study of 92 consecutive patients with SSc-PAH diagnosed by cardiac catheterisation. Data were collected both prospectively and retrospectively. Patients were given basic treatment (diuretics, digoxin, oxygen and warfarin). Where clinically indicated, a prostanoid was used as advanced treatment (historical control group). From 2002, the range of treatments available expanded to include bosentan, which was generally the preferred treatment (current treatment era group). Survival was measured from the date of diagnosis of pulmonary hypertension by cardiac catheterisation. Six minute walking distance and haemodynamic function were measured at the time of diagnosis and at least one month after treatment was started. RESULTS: The historical control group comprised 47 patients, all of whom received basic treatment; 27 of these were also treated with prostanoids. The current treatment era group comprised 45 patients, all of whom received bosentan as preferred treatment. Kaplan-Meier survival in the historical control group was 68% at one year and 47% at two years. Survival in the current treatment era group was 81% and 71% (p = 0.016) at one and two years, respectively. Pulmonary vascular resistance increased in the historical control group (by 147 dyn.s.cm(-5)), whereas in the current treatment era group, it remained stable over an average of nine months (decrease of 16 dyn x s x cm(-5), p < 0.006). CONCLUSION: Survival of selected patients with SSc-PAH has improved in the current treatment era. In contrast to patients treated historically with basic drugs and prostanoids, patients treated in the current treatment era had improved survival associated with a lack of deterioration in cardiac haemodynamic function.

QT dispersion in athletic left ventricular hypertrophy.

OBJECTIVE: To assess whether physiologic left ventricular hypertrophy as a result of physical training is associated with an increased QT length or dispersion. METHODS: Thirty-three subjects were assessed. These consisted of a group of international endurance athletes (including 8 rowers, 2 cyclists, and 1 triathlete), a group of 12 professional soccer players, and a further group of 10 control subjects. Each underwent 2-dimensional echocardiography and 12-lead electrocardiographic examination. RESULTS: Left ventricular mass index was considerably greater in both the endurance athlete (163.3 +/- 14.4 g/m2; P <.01) and soccer player groups (144.2 +/- 5.5 g/m 2; P <.05) compared with the controls (109.2 +/- 6.3 g/m2). In spite of these large differences in cardiac structure there were no significant differences in QT parameters between the groups (QT dispersion 56.9 +/- 5.5, 68.5 +/- 9.5, and 67.2 +/- 12.6 ms; QTc dispersion 61.4 +/- 9.2, 69.4 +/- 13.3, and 54.2 +/- 6.5 ms; maximum QT 402 +/- 10.3, 404 +/- 9.6, and 392 +/- 14.0 ms; and maximum QTc 404 +/- 7.0, 413 +/- 9.3, and 399 +/- 9.9 ms among endurance athletes, soccer players, and controls, respectively). CONCLUSION: Left ventricular hypertrophy occurring as a consequence of athletic training does not appear to be associated with a major increase in QT length or QT dispersion.

The complex between retinol and retinol-binding protein is formed in the rough microsomes of liver following repletion of vitamin A-depleted rats.

Retinol-binding protein (RBP), the plasma transport protein for vitamin A, is primarily synthesized in the rough endoplasmic reticulum of the liver. RBP then passes through the smooth endoplasmic reticulum and into the Golgi apparatus where vesicles form and transport the protein to the cell membrane. When rats were depleted of their vitamin A stores, RBP accumulated in the liver microsomes, particularly in the rough microsomes. To identify the organelle(s) where retinol initially binds to RBP, vitamin A-depleted rats were given an i.v. injection of [3H]retinol suspended in Tween 40. After intervals of 2, 3, 4, 5, 6, 8, 10, 15 and 20 min, liver fractions enriched in rough and smooth microsomes and Golgi apparatus were prepared. The retinol/RBP complex (holoRBP) was detected in the rough microsomes within 3 min post injection. HoloRBP later appeared in the smooth microsomes and Golgi fraction, and then the serum at time intervals consistent with the known secretion rate for RBP. HoloRBP was detected in the rough microsomes at all times after 3 min, whether or not the complex was present in the other subcellular fractions. Thus, the holoRBP complex can form in the rough endoplasmic reticulum of the liver.

Transbrachial coil occlusion of the first intercostal branch of an internal mammary artery bypass graft for angina.

Percutaneous transbrachial insertion of a metallic coil into the first intercostal branch of the left internal mammary artery resulted in resolution of severe angina in a 60-year-old man who had coronary artery bypass surgery 14 years previously. The diagnosis of coronary artery steal was made clinically. This case illustrates the importance of recognising coronary steal in patients who redevelop angina after coronary artery surgery with the use of an incompletely prepared left internal mammary artery as a conduit. The diagnosis can be made clinically and the condition treated without the need for further surgery.

Pulmonary artery reconstruction for tuberculosis.

A 35-year-old woman underwent reconstruction of her right pulmonary artery for treatment of acquired right pulmonary artery stenosis. The stenosis was secondary to tuberculosis causing both an extrinsic and an intrinsic obstructive component. After her reconstruction, the patient made an uneventful recovery, and perfusion to the right lung was subsequently restored.

Dobutamine echocardiography and thallium-201 imaging predict functional improvement after revascularisation in severe ischaemic left ventricular dysfunction.

OBJECTIVES: To evaluate the concordance between thallium-201 uptake and echocardiographic wall thickening, which are both indicators of potentially reversible myocardial dysfunction, in patients with chronic ischaemic left ventricular failure and to assess their relative contribution to predicting improvement in regional function after revascularisation in a subgroup. PATIENTS AND METHODS: 45 patients with chronic ischaemic left ventricular dysfunction (mean (SD) ejection fraction 25 (8)%) underwent echocardiography before and after dobutamine infusion (10 micrograms/kg/min). Of these, 22 patients underwent rest echocardiography at a mean (SD) of 9 (1) weeks after revascularisation. 201Tl imaging was performed during dobutamine echocardiography and at rest, 1, and 4 h after treatment with sublingual glyceryl trinitrate on two separate days. Potentially reversible dysfunction was thought to be present when a myocardial segment contained a Tl score of > or = 3 (ascending score 1-4), or showed improved wall thickening of a dysynergic segment during dobutamine stimulation. RESULTS: Of the 201Tl protocols, the redistribution scan 1 h after treatment with glyceryl trinitrate best demonstrated myocardial viability. Concordance between 201Tl and dobutamine induced wall thickening was 82% (kappa = 0.59) for detecting potentially reversible myocardial dysfunction before revascularisation (n = 45). Regional function improved in 18 of 22 patients after revascularisation. There were 168 dysynergic segments before intervention. The sensitivity of echocardiography and 201Tl imaging for detecting "recoverable" or viable segments after revascularisation was 87% and 92% respectively and specificity was 82% and 78% respectively (P = NS). CONCLUSIONS: Dobutamine echocardiography and 201Tl imaging may be used to predict mechanical improvement in dysynergic segments after revascularisation in patients with chronic ischaemic left ventricular dysfunction.

Disappearing left atrial vegetation in an intravenous drug abuser.

Isolated left atrial mural endocarditis is rare. We report a case where the diagnosis was made clinically and supported by blood cultures and transoesophageal echocardiography. Appropriate intravenous antibiotics were administered and serial transoesophageal echocardiograms helped in monitoring the decrease in size and final disappearance of the vegetation, thus avoiding the need for surgical intervention.

General practitioners' requirements from a hospital cardiology department.

The requirements of 500 general practitioners from a hospital department of cardiology were sought and assessed from a questionnaire. One hundred and twenty two general practitioners caring for 766,384 patients responded. The most important inpatient service requirement was that cardiologists should be responsible for the management of patients with acute myocardial infarction and other acute cardiac conditions. The expertise and reputation of the consultant cardiologist and the waiting time for an appointment were the two most important outpatient service requirements. The least important aspect was the time patients waited in clinic to see a doctor. Fundholders considered price of outpatient consultations to be unimportant. Waiting times for cardiac catheterisation and non-invasive cardiac investigations together with the quality of the investigation equipment and the technical staff were all considered to be important requirements. Most general practitioners wanted protocols for managing common cardiac conditions. The majority wanted an open access investigation service to reduce outpatient waiting times. Those who did not want an open access service explained that they would not be confident in interpreting the results or deciding subsequent management. An understanding of the requirements of general practitioners is helpful in planning and developing hospital services.

Comparison of radionuclide ventriculography and 2D echocardiography for the measurement of left ventricular ejection fraction following acute myocardial infarction.

To compare the measurement of left ventricular ejection fraction obtained by two-dimensional echocardiography and by radionuclide ventriculography in patients following acute myocardial infarction, 49 consecutive patients with acute myocardial infarction underwent echocardiography and radionuclide ventriculography on the same day, pre-discharge. Left ventricular ejection fraction was assessed by two blinded observers for each method and reproducibility was also assessed for each technique. The limits of agreement for the differences in ejection fraction (%) between the two methods was--11.4, 12.2; the mean difference 0.4 was not significantly different from zero. The limits of agreement for the intra- and inter-observer differences in ejection fraction by radionuclide ventriculography were--9.4, 7.6 and -8.6, 11.0, respectively; the mean differences--0.9 and 1.2 were not significantly different from zero. The limits of agreement for the intra- and inter-observer differences by echocardiography were--5.8, 6.6 and--8.9, 9.5 respectively; the mean differences 0.4 and 0.3 were not significantly different from zero. Thus, two-dimensional echocardiography compares well with radionuclide ventriculography for the assessment of ejection fraction without the disadvantage of radiation.

Coxiella burneti endocarditis in a patient with positive chlamydial serology.

A 41-year-old man who habitually slept in a car park presented with a culture-negative endocarditis. Serological tests indicated infection with both Coxiella burneti and Chlamydia psittaci. He was treated with doxycycline and clindamycin and required aortic valve replacement. Culture of the excised value for both agents was negative but electron microscopy was suggestive of coxiella endocarditis.

Sinus arrest and asystole due to severe lithium intoxication.

We report the occurrence of sinus arrest and asystole due to severe lithium intoxication in a patient with preexisting conduction tissue disease. Lithium should be used with extreme caution and frequent monitoring in patients with conduction tissue disease.

Permanent transvenous pacing after a Mustard procedure.

We report the case of a 20-year-old man born with transposition of the great arteries who underwent emergency balloon septostomy and subsequently a Mustard procedure. When aged 20 years, he had several syncopal attacks due to sinoatrial disease for which he was simply and successfully paced transvenously in VVI mode.

Atrial natriuretic peptide release responds to atrial stretch and not to atrial pressure: observations during pericardiocentesis in a young woman.

The effect of pericardiocentesis on the plasma concentration of atrial natriuretic peptide was studied in a patient with cardiac tamponade. Plasma atrial natriuretic peptide concentrations rose sharply from 42 pmol l-1 to a maximum of 150 pmol l-1 with a corresponding fall in mean right atrial pressure from 20 mmHg to 6 mmHg. Our results suggest that atrial 'stretch' (or transmural pressure) rather than atrial pressure per se, is the primary stimulus for atrial natriuretic peptide release in man.

Permanent transvenous pacing after a Mustard procedure.

We report the case of a 20-year-old man born with transposition of the great vessels who underwent emergency balloon septostomy and subsequently a Mustard procedure. When aged 20 years, he had several syncopal attacks due to sinoatrial disease for which he was simply and successfully paced transvenously in VVI mode.

Coronary ostial stenosis after radiotherapy: angioplasty or coronary artery surgery?

A thirty year old man underwent coronary angioplasty for an isolated ostial stenosis of the ostium of the right coronary artery after mediastinal radiotherapy given ten years previously. Despite an angiographically acceptable angioplasty result, he had a myocardial infarction two months later and coronary artery surgery was performed. The most effective form of myocardial revascularisation for radiotherapy related coronary artery lesions remains to be established.

Congenital diverticulum of the left ventricle presenting as heart failure and diagnosed by magnetic resonance imaging.

We report a 25-year-old man with a probable congenital diverticulum of the heart. The diverticulum was clearly shown using magnetic resonance imaging.