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OBJETIVO: Identificar dados clínicos e laboratoriais que diferenciam os casos com síndrome de Klinefelter de acordo com a faixa etária. CASUÍSTICA E MÉTODOS: Foram incluídos todos os casos de hipogonadismo, ginecomastia e/ou infertilidade avaliados em hospital universitário cujo cariótipo foi realizado entre janeiro de 1989 e dezembro de 2011, totalizando 105 pacientes. Foram avaliados: idade na primeira consulta, relação entre envergadura e altura, pilificação pubiana, ginecomastia, tamanho testicular, hormônio luteinizante (LH), hormônio folículo-estimulante (FSH), testosterona e espermograma. RESULTADOS: Foram diagnosticados três casos com síndrome de Klinefelter (SK+) e 72 sem a síndrome (SK-). Dos casos com síndrome de Klinefelter, apenas sete (21,2%) foram diagnosticados antes dos 20 anos e dois (6,1%) antes dos 10 anos de idade. A idade na primeira consulta (em anos) foi semelhante nos dois grupos (SK+ = 31,3±12,9 e SK- = 27,6±12,1), o mesmo ocorrendo com a relação entre envergadura e altura e a presença de ginecomastia. No entanto, a pilificação pubiana foi menor no grupo SK+, o mesmo ocorrendo com a média do volume bitesticular e a testosterona, enquanto que o LH e o FSH foram mais elevados neste grupo, o mesmo ocorrendo com a frequência de azoospermia. CONCLUSÕES: A síndrome de Klinefelter ainda é pouco e tardiamente diagnosticada em nosso meio, sendo os dados de tamanho testicular, LH, FSH, testosterona e presença de azoospermia no espermograma os mais importantes para o seu diagnóstico, principalmente na puberdade e na vida adulta.

Background: Systolic anterior motion of the mitral valve causing left ventricular outflow tract obstruction is an uncommon complication of mitral valve repair that may necessitate immediate additional surgical action. We prospectively evaluated the technique of the edge-to-edge suture on post-mitral repair systolic anterior motion, which persisted despite conservative treatment., Methods: From March 2002 to March 2004, 4 of 112 patients requiring mitral valve repair surgery for chronic degenerative mitral regurgitation had systolic anterior motion with severe left ventricular outflow tract obstruction and mitral regurgitation. All 4 patients (mean age, 50 years) had posterior leaflet prolapse with chordal rupture with a billowing anterior leaflet, but without chordal rupture. Repair was achieved through a quadrangular resection of the posterior leaflet, completed by plication of the annulus in 2 patients and leaflet sliding in the other 2. All patients had mitral annuloplasty; two patients had a complete CE Physio ring (Edwards Lifesciences, Irvine, CA) inserted, whereas the other 2 patients had an open CG Future band (Medtronic, Minneapolis, MN). Routine perioperative transesophageal echocardiography showed systolic anterior motion, severe left ventricular outflow tract obstruction (> 50 mm Hg), and mitral regurgitation. After resuming cardiopulmonary bypass, all patients had an edge-to-edge suture at the middle part of the free edge of the anterior and posterior leaflets., Results: The control transesophageal echocardiography showed in all cases disappearance of the systolic anterior motion, of the left ventricular outflow tract obstruction and of mitral regurgitation. Mean follow-up was 14 months (range, 6 to 28 months). All patients were in New York Heart Association's functional class I., Conclusions: With the edge-to-edge repair, the early and 2-year results were satisfactory with total disappearance of the systolic anterior motion, of the left ventricular outflow tract obstruction and of the recurrent mitral regurgitation.

The aim of this study was to assess the results of mitral valvuloplasty for chronic asymptomatic or paucisymptomatic mitral regurgitation. Of 584 patients operated for chronic mitral regurgitation between January 1989 and December 1994, 175 were in NYHA Classes I and II and made up the study population. All had chronic grade 3 or 4/4 mitral regurgitation suitable for mitral valvuloplasty. The average follow-up was 34.3 months. Mitral valvuloplasty was performed in 174 patients, the other patient requiring mitral valve replacement. Three patients died (1.7%) and the actuarial 5 year survival was 98.2 +/- 1.0%. The probability of absence of reoperation and absence of thrombo-embolic complications at 5 years were 97 +/- 0.8% and 96.3 +/- 1.7% respectively. The residual regurgitation at Doppler echocardiography was minimal or absent in 94% of patients at the last follow-up control. The mean end-systolic and end-diastolic left ventricular dimensions decreased from 40.0 +/- 6.8 mm and 64.8 +/- 7.0 mm before surgery to 34.6 +/- 6.7 mm (p < 0.001) and 52.7 +/- 7.4 mm (p < 0.001) at the last control. The authors conclude that conservative mitral valve surgery for NYHA Classes I and II patients with chronic mitral regurgitation is feasible with a low risk and is associated with a significant reduction in ventricular volumes and stability of valvular continence at medium-term. When performed by teams trained in techniques of mitral valvuloplasty, these results suggest that surgery should be performed early.

Unlabelled: A Doppler echocardiographic study was performed to evaluate the hemodynamic performance of three 19 mm size currently used bileaflet valve prosthesis (St Jude Medical Hydrodinamic Plus, Sorin-Bicarbon and Carbomedic Reduced) implanted in aortic position., Methods: Patients, 30, with the same profile receiving 19 mm size valve (ten for each valve type) were selected when body surface area (BSA) was > 1.7 m2. Doppler echocardiography was carried out at rest and after exercise, 60 days after surgery. Peak (Pg) and mean (Mg) gradients across the valve were recorded: the effective orifice area (EOA), and performance index (PI) were calculated., Results: No significant differences were observed between St Jude Medical and Sorin Bicarbon as far as peak and mean gradient, effective orifice area and performance index at rest and after exercise. A significant difference (P < 0.05) was demonstrated in the above mentioned parameters when Carbomedics-R valve were tested. This type of valve showed a lower EOA and PI with higher Pg and Mg gradient both at rest and after exercise., Conclusion: The St Jude Hydrodymanic plus (Hp) and Sorin Bicarbon valves had similar performance and a better hemodynamic trend when compared to the Carbomedics-R valve in patients with large body surface areas. The Carbomedics-R valve shows a ineffective use of the total area of the prosthesis both at rest and after exercise.

Objective: The purpose of this study was to review the risk-benefit ratio of mitral valve repair in patients with severe mitral regurgitation and no or mild symptoms., Methods: From January 1989 to December 1994, 584 patients were operated on for mitral regurgitation. Of these, 175 patients were in New York Heart Association class I or II with grade 3 to 4 isolated chronic mitral regurgitation. They comprise our study population. Mean age was 51.3 +/- 14.3 years. Principal causes of mitral regurgitation were degenerative in 128 (73%) and rheumatic in 26 patients (15%). Leaflet prolapse was the mechanism responsible for regurgitation in 152 patients (86%). Mitral valve repair was performed in 174 patients, and one patient required initial valve replacement. Mean follow-up was 34.3 +/- 18.8 months., Results: Three patients died, for an overall mortality of 1.7%. Five patients were reoperated on, for an actuarial freedom from reoperation of 97.0% +/- 0.8% at 5 years. Actuarial freedom from thromboembolism and endocarditis was 96.3% +/- 1.7% and 99.4% +/- 0.6%, respectively, for an event-free survival of 91.0% +/- 2.0% at 5 years. Left atrial diameter decreased from 54.3 +/- 11.6 mm to 43.6 +/- 10.5 mm (p < 0.001). Left ventricular end-systolic and end-diastolic diameters decreased from 40.0 +/- 6.8 mm and 64.8 +/- 7.0 mm to 34.6 +/- 6.7 mm (p < 0.001) and 52.7 +/- 7.4 mm (p < 0.001), respectively. Mean residual mitral regurgitation was 0.44 +/- 0.6., Conclusion: Mitral valve repair for chronic mitral regurgitation in patients having mild or no symptoms was performed with low mortality and morbidity, good valve function, and preserved late left ventricular performance. Early repair may be advocated on the basis of severity of regurgitation and valve repairability, regardless of symptoms.