Iatrogenic hydropneumopericardium.

We report a case of iatrogenic hydropneumopericardium in a patient with diffuse scleroderma. The transthoracic echocardiogram revealed classical features of this condition. This case resolved spontaneously after five days using only 60% oxygen, which may have augmented resolution in this instance.

Isolated left ventricular non-compaction with normal ejection fraction.

Isolated left ventricular non-compaction (LVNC) is a genetic disease that is being increasingly recognised in patients presenting with heart failure of unknown origin. In this case report, we describe a patient with classic LVNC without clinical heart failure and with normal left ventricular ejection fraction.

Oral cancer-associated paraneoplastic syndromes.

Paraneoplastic syndromes are cancer-associated endocrinological, haematological, dermatological or neurological disorders, which are directly related neither to the physical effects of the tumour mass, nor to invasion by the primary tumour, nor to metastasis of the tumour; nor are they associated either with the side-effects of anticancer treatment or with any of the complications of cancer. These syndromes are brought about by the ectopic production of biological mediators by the malignant tumour cells, or by immunological responses to the malignancy. Certain cancers are typically associated with specific paraneoplastic disorders. Though uncommonly, oral carcinomata have reportedly been associated with paraneoplastic pemphigus, humoral hypercalcaemia of malignancy, syndrome of inappropriate antidiuretic hormone, and paraneoplastic leukocytosis syndrome.

Chemotherapy- and radiotherapy-induced oral mucositis: pathobiology, epidemiology and management.

Oral mucositis is a debilitating complication of anticancer treatment, characterised by erythematous, atrophic, erosive or ulcerative lesions. Oral mucositis is almost always painful, affects eating, sleeping, and speech and affects the physiological and social well-being of the patient. The pathophysiology of the condition is not well understood. Guidelines to the treatment of oral mucositis are often contradictory so that there is no evidence based standard treatment protocol. Therefore the treatment is empiric. This paper offers a brief review of current knowledge of the pathophysiology and treatment of oral mucositis.

Beta1- and alpha2c-adrenoreceptor variants as predictors of clinical aspects of dilated cardiomyopathy in people of African ancestry.

BACKGROUND: Although the beta1-adrenoreceptor (AR) Gly389Arg and alpha2c-AR Del322-325 gene variants are associated with the response to beta-AR-blocker therapy, whether this effect is associated with the risk for heart failure, or the severity or progression of heart failure is uncertain. AIMS: To assess the relationship between Gly389Arg and Del322-325 variants and the presence, severity and progression of idiopathic dilated cardiomyopathy (IDC) in 403 black South African patients. METHODS: Genotypes were identified using a restriction fragment length polymorphism-based technique and automated sequencing. Left ventricular ejection fraction (LVEF) and dimensions were determined at baseline and in 132 patients after six months of standard medical therapy excluding beta-AR-blockers (not indicated as standard care at the time of completing this study). RESULTS: All patients and controls genotyped for the alpha2c-AR variant were homozygous for the Del322-325 (risk) allele. The Gly389Arg polymorphism was not associated with IDC (control n = 429) (Arg389 allele homozygosity: odds ratio = 1.03, confidence limits = 0.78-1.35), nor did it predict LVEF and cavity dimensions either before or after therapy. CONCLUSION: In patients homozygous for the risk allele of the alpha2c-AR variant, the beta1-AR variant neither increased the risk for IDC nor predicted its severity or progression in patients not receiving beta-AR-blockers.

Quality assurance, benchmarking, assessment and mutual international recognition of qualifications.

The aim of this report is to provide guidance to assist in the international convergence of quality assurance, benchmarking and assessment systems to improve dental education. Proposals are developed for mutual recognition of qualifications, to aid international movement and exchange of staff and students including and supporting developing countries. Quality assurance is the responsibility of all staff involved in dental education and involves three levels: internal, institutional and external. Benchmarking information provides a subject framework. Benchmarks are useful for a variety of purposes including design and validation of programmes, examination and review; they can also strengthen the accreditation process undertaken by professional and statutory bodies. Benchmark information can be used by institutions as part of their programme approval process, to set degree standards. The standards should be developed by the dental academic community through formal groups of experts. Assessment outcomes of student learning are a measure of the quality of the learning programme. The goal of an effective assessment strategy should be that it provides the starting point for students to adopt a positive approach to effective and competent practice, reflective and lifelong learning. All assessment methods should be evidence based or based upon research. Mutual recognition of professional qualifications means that qualifications gained in one country (the home country) are recognized in another country (the host country). It empowers movement of skilled workers, which can help resolve skills shortages within participating countries. These proposals are not intended to be either exhaustive or prescriptive; they are purely for guidance and derived from the identification of what is perceived to be 'best practice'.

Impact of beta2-adrenoreceptor gene variants on cardiac cavity size and systolic function in idiopathic dilated cardiomyopathy.

In heart failure, the Arg16Gly and Gln27Glu polymorphisms of the beta2-adrenoreceptor (beta2-AR) gene are associated with exercise-capacity, clinical outcomes and response to beta-AR blocker therapy. Whether beta2-AR gene variants mediate these effects in-part through an impact on cardiac structural remodeling and pump function independent of the effects of beta-blockers is uncertain. We evaluated whether the Arg16Gly and Gln27Glu variants of the beta2-AR gene predict left ventricular ejection fraction (LVEF) and LV end diastolic diameter (LVEDD) in patients with idiopathic dilated cardiomyopathy (IDC) before and 6 months after receiving standard medical therapy other than beta-AR blockers. In all, 394 patients with IDC and 393 age and gender-matched controls were genotyped for the beta2-AR gene variants using restriction-fragment length polymorphism-based techniques. LVEF and dimensions were determined in 132 patients (of whom 71 were newly diagnosed) both at baseline and after 6 months. Genotype of neither variant was associated with the presence of IDC. Moreover, beta2-AR genotype did not determine LVEF or LV dimensions prior to initiating therapy. After 6 months of therapy, LVEF increased by 7.1+/-1.0 absolute units (P<0.0001) and LVEDD decreased by 0.27+/-0.06 cm (P<0.02). Adjusting for baseline values as well as gender, age, and type of angiotensin-converting enzyme inhibitor therapy received, genotype was associated with neither final LVEF and LVEDD, nor change in LVEF and LVEDD. In conclusion, these data suggest that in heart failure, the functional Arg16Gly and Gln27Glu variants of the beta2-AR gene have no independent effect on adverse structural remodeling and pump function.

A feeding adaptation by an infant with a cleft palate.

One of the primary problems in the management and care of infants born with cleft palates is that of achieving an adequate seal of the cleft to allow proper swallowing to take place. This article describes an interesting case in which a 14-month-old baby, who had received no surgical or prosthodontic treatment for her cleft palate, had developed her own 'obturation' mechanism to enable her to swallow efficiently.

Long-term follow-up of rheumatic patients undergoing left-sided valve replacement with tricuspid annuloplasty--validity of preoperative echocardiographic criteria in the decision to perform tricuspid annuloplasty.

Between September 1989 and December 1991, modified De Vega tricuspid annuloplasty was performed in 43 patients who survived surgery for mitral or mitral plus aortic valve replacement. The preoperative indications for tricuspid annuloplasty were moderate to severe tricuspid regurgitation (TR) in 33 patients and mild or no TR but with a dilated tricuspid annulus (> or =30 mm) as measured by 2-dimensional echocardiography at end-diastole in 10 patients. The mean age was 31 +/- 13 years. The mean duration of follow-up was 57 +/- 18 months. Overall long-term mortality was 12%. On Doppler color flow mapping, postoperative severe TR was present in 1 patient and moderate TR in 4 patients at latest follow-up. The tricuspid annulus diameter decreased from 37 +/- 5 mm preoperatively to 24 +/- 6 mm at latest follow-up. During the study period, an additional 77 patients underwent mitral valve replacement or double valve replacement, but without tricuspid annuloplasty. Within this group, 38 patients had a preoperative tricuspid annulus diameter of > or =30 mm, and 5 of these patients (13%) developed moderate or severe TR in the postoperative period, which may have been prevented had clinicians adhered to the preoperative indications for tricuspid annuloplasty. Thus, preoperative echocardiographically documented moderate or severe TR or a tricuspid annulus diameter of > or =30 mm are valid indications for performing tricuspid annuloplasty; modified De Vega tricuspid annuloplasty is a durable procedure in rheumatic patients; it appears that reducing the diastolic tricuspid annulus diameter to 24 mm is adequate to prevent residual TR in the long term.

Gonococcal endocarditis in a patient with systemic lupus erythematosus.

There is mounting evidence that patients with systemic lupus erythematosus (SLE) are prone to disseminated neisserial infections. We describe the first proven case of gonococcal endocarditis affecting the pulmonary valve in a patient known to have SLE. The clinical clues and pitfalls in diagnosis are discussed, and the role of echocardiography is highlighted. Possible reasons for the association of gonococcal endocarditis with SLE include pre-existing Libman. Sacks endocarditis, complement deficiency and abnormalities of the reticuloendothelial system.

Long-term changes in left ventricular performance following mitral valve replacement for pure rheumatic mitral regurgitation.

Our data indicate that MVR, with or without chordal preservation, for pure severe MR in symptomatic younger rheumatic patients with a good preoperative ejection fraction results in normalization of LV size and performance by 1 year. Normalization of LV performance was only achieved at 1 year after surgery, and it is therefore essential to extend the assessment of LV function to at least 1 year postoperatively.

Can the long-term outcomes of percutaneous balloon mitral valvotomy and surgical commissurotomy be expected to be similar?

The ultimate role of percutaneous balloon mitral valvotomy will depend on its potential for sustained improvement. Long-term outcome data including survival, reoperation and thromboembolism are available for surgical commissurotomy. However, length of follow up for percutaneous balloon mitral valvotomy is inadequate to acquire similar end-point data. We therefore hypothesized that comparison of changes in mitral valve area following balloon or surgical commissurotomy would serve as a useful surrogate end-point by which the long-term benefit of percutaneous balloon mitral valvotomy could be determined. Mitral valve area was determined by Doppler echocardiography following percutaneous balloon mitral valvotomy (N = 230) and surgical commissurotomy (N = 241, 130 closed and 111 open mitral commissurotomy). Regression lines of mitral valve area versus interval from intervention were constructed for each of the two groups. Nine clinical and echocardiographic variables were also analyzed to determine their predictive value for low mitral valve areas. Both groups showed similar and significant negative correlations for mitral valve area versus time (r = -0.48, r = -0.6, balloon vs. surgical commissurotomy respectively, p = 0.001 for both groups). The slopes of the regression lines for both groups were also similar (y = -0.007 x +1.9, y = -0.005 x +1.8, y = -0.006 x +1.8, p = NS). There were no differences in the prevalence of mitral regurgitation. Independent predictors of mitral restenosis according to multivariate analysis were time interval from surgery (p < 0.03), composite mitral valve morphology score (p < 0.04) and subvalvular disease (p < 0.04). Thus, there is a progressive decrease in mitral valve area following percutaneous mitral balloon valvotomy that, at least for the available duration of follow up, appears to parallel changes in valve area following closed or open mitral commissurotomy. A less pliable valve and more subvalvular disease are independent predictors of smaller valve areas. These data suggest that the long term clinical outcome following percutaneous balloon mitral valvotomy may be expected to be similar to the available data for surgical commissurotomy.

Long-term (3-month) effects of a new beta-blocker (nebivolol) on cardiac performance in dilated cardiomyopathy.

OBJECTIVES: This study examined the long-term (3-month) effects of nebivolol, a new beta-adrenergic blocking agent, on cardiac performance in patients with dilated cardiomyopathy. BACKGROUND: Several beta-blocking drugs have been reported to have a beneficial hemodynamic effect in patients with dilated cardiomyopathy, but few data obtained in a placebo-controlled randomized study have addressed the mechanisms of improvement. METHODS: Twenty-four patients with dilated idiopathic (n = 22) or ischemic (n = 2) cardiomyopathy (ejection fraction 0.15 to 0.40) in stable New York Heart Association functional class II or III were entered into a double-blind randomized trial of nebivolol, a new, potent, selective beta 1-antagonist. Exercise time, invasive hemodynamic data (12- and 24-h monitoring) and variables of left ventricular function were examined at baseline and after 3 months of orally administered nebivolol (1 to 5 mg/day, n = 11) or placebo (n = 13). RESULTS: Heart rate decreased (group mean 85 to 71 beats/min vs. 87 to 87 beats/min with placebo) and stroke volume increased significantly (group mean 43 to 55 ml vs. 42 to 43 ml) with nebivolol; decreases in systemic resistance, systemic arterial pressure, wedge pressure and pulmonary artery pressure were not significantly different from those with placebo. Similar hemodynamic results were obtained in the catheterization laboratory. Analysis of high fidelity measurements of left ventricular pressure showed a decrease in left ventricular end-diastolic pressure in the nebivolol group (group mean 21 to 15 vs. 24 to 20 mm Hg with placebo) but no change in the maximal rate of pressure development or in two variables of left ventricular relaxation (maximal negative rate of change of left ventricular pressure [dP/dtmax] and the time constant tau). Left ventricular mass decreased (p = 0.04). Despite a decrease in heart rate with nebivolol, there was a slight decrease in left ventricular end-diastolic volume (p = NS). End-systolic volume tended to decrease (p = 0.07) despite no reduction in end-systolic stress. The net result was a significant increase in ejection fraction (group mean 0.23 to 0.33 vs. 0.21 to 0.23 with placebo), presumably as a result of an increase in contractile performance. This effect was corroborated by an increase in a relatively load-independent variable of myocardial performance. CONCLUSIONS: Nebivolol improved stroke volume, ejection fraction and left ventricular end-diastolic pressure, not through a measurable reduction in afterload or a lusitropic effect, but by improving systolic contractile performance.

Pulmonary hypertension is a contraindication to beta-blockade in patients with severe mitral stenosis.

Intravenous atenolol was given to 31 patients just before balloon mitral valvotomy to assess the hemodynamic efficacy and safety of beta-blockade in mitral stenosis complicated by pulmonary hypertension. Hemodynamic response in patients with pulmonary resistance > 600 dynes.sec.cm-5 (group 2, n = 17) was compared with those (group 1, n = 14) with a resistance below this value. In addition to a higher pulmonary arterial resistance (by design), patients in group 2 had a higher systemic resistance, lower cardiac index, and smaller mitral valve area compared with those in group 1. After atenolol infusion, transmitral gradient and left atrial pressure improved similarly. In spite of the decline in left atrial pressure, pulmonary vascular resistance increased in both groups, more in group 2 (847 +/- 398 dynes.sec.cm-5 to 135 +/- 648 dynes.sec.cm-5) than in group 1 (291 +/- 149 dynes.sec.cm-5 to 363 +/- 200; p < 0.001 for drug effect and p = 0.027 for group effect by two-way analysis of variance). Cardiac index declined similarly from 2.77 +/- 0.51 L/min/m2 to 2.37 +/- 0.37 L/min/m2 in group 1 and from 2.33 +/- 0.58 L/min/m2 to 1.92 +/- 0.54 L/min/m2 in group 2. Systemic pressure tended to decline only in group 2 (mean aortic pressure, 89 +/- 12 mm Hg to 89 +/- 12 mm Hg in group 1 and 90 +/- 9 mm Hg to 83 +/- 12 mm Hg in group 2; p = 0.06 for group effect).(ABSTRACT TRUNCATED AT 250 WORDS)

Excessive vasoconstriction in rheumatic mitral stenosis with modestly reduced ejection fraction.

OBJECTIVES: The primary hypothesis examined was that underfilling due to inflow obstruction accounts for modestly depressed ejection performance in mitral stenosis. Having found little evidence to support this hypothesis, we sought to determine other factors that might differentiate patients with different levels of ejection performance. METHODS: Ventricular load and performance were compared in two groups of patients before and immediately after successful balloon valvuloplasty that was not complicated by mitral regurgitation: those in whom prevalvuloplasty ejection fraction was > or = 0.55 (group I, n = 10) and those in whom it was < 0.55 (group II, n = 11). RESULTS: Before valvuloplasty, mitral valve area was less in group II (0.65 cm2) than in group I (0.84 cm2, p = 0.02), but end-diastolic pressure (12 vs. 12 mm Hg in group I), end-diastolic wall stress (46 vs. 44 kdynes/cm2 in group I) and end-diastolic volume (152 vs. 150 ml in group I) were not less in group II, nor were these variables significantly reduced compared with those of a normal control group. In group II, end-systolic volume was larger (77 vs. 55 ml in group I, p = 0.001) and cardiac output was less (3.1 vs. 3.6 liters/min in group I, p = 0.03), possibly owing to higher systemic vascular resistance (2,438 vs. 1,921 dynes.s.cm-5 in group I, p = 0.05) and end-systolic wall stress (273 vs. 226 kdynes/cm2 in group I, p = 0.06), although mean arterial pressure in the two groups was similar (91 vs. 84 mm Hg in group I, p = 0.22). Group II patients also had higher values for pulmonary vascular resistance (712 vs. 269 dynes.s.cm-5 in group I, p = 0.03) and mean pulmonary artery pressure (47 vs. 29 mm Hg in group I, p = 0.02) despite similar values for mean left atrial pressure (20 vs. 18 mm Hg in group I, p = 0.35). After valvuloplasty, mitral valve area increased by 2.5- and 3-fold, respectively, in group I (to 2.1 cm2) and group II (to 2.0 cm2). Modest increases in left ventricular end-diastolic pressure, end-diastolic stress and end-diastolic volume (+9%) after valvuloplasty were statistically significant only for group II. End-systolic wall stress did not decline in either group II (281 kdynes/cm2) or group I (230 kdynes/cm2), and ejection fraction failed to increase significantly (0.49 to 0.51 for group II and 0.62 to 0.61 for group I) after valvuloplasty. Contractile performance estimated with a preload-corrected ejection fraction-afterload relation was within or near normal limits in all 19 patients in whom it was assessed. CONCLUSIONS: Excessive vasoconstriction may account for the higher afterload, lower ejection performance and lower cardiac output observed in a subset of patients with mitral stenosis because contractile dysfunction could not be detected and left ventricular filling--which was not subnormal despite severe inflow obstruction--improved only modestly after valvuloplasty.

The use of transoesophageal echocardiography for detecting early recurrence of atrial myxoma.

A comparison between praecordial and transoesophageal cross-sectional echocardiography was undertaken in the follow-up of 14 patients who had previously undergone surgical excision of atrial myxoma. The mean interval between surgery and follow-up was 39 months. Evidence of recurrent tumour was seen in two patients by transoesophageal echocardiography but went undetected in one of these using the praecordial approach. Clear visualisation of the atria and interatrial septum was possible in all remaining cases using transoesophageal echocardiography and this allowed confident exclusion of tumour recurrence. Using praecordial echocardiography, technically inadequate studies meant that this was not possible in 4 patients. The significant late recurrence rate of excised atrial myxomas, emphasises the need for serial, postoperative echocardiographic studies. Praecordial echocardiography may be unreliable in the detection of recurrent atrial myxoma in its early stages and for this reason transoesophageal echocardiographic follow-up is justified in high risk patients.