Independent Clinical and Echocardiographic Predictors of Restenosis After Percutaneous Mitral Balloon Commissurotomy in a Large, Consecutive Cohort Followed for 24 Years.

OBJECTIVE: To enlighten preprocedural risk factors of mitral valve restenosis in a large, single-center cohort of patients submitted to percutaneous mitral balloon commissurotomy (PMBC) for the treatment of mitral stenosis (MS) secondary to rheumatic heart disease. METHODS: This is a database analysis of a single-center, high-volume tertiary institution involving all consecutive PMBC procedures performed in the mitral valve (MV). Restenosis was diagnosed when MV area was <1.5 cm² and/or loss of 50% or more of the immediate procedural result aligned with the return/worsened symptoms of heart failure. The primary endpoint was to determine the preprocedural independent predictors of restenosis after PMBC. RESULTS: Among a total of 1921 PMBC procedures, 1794 consecutive patients without previous intervention were treated between 1987 and 2010. Throughout 24 years of follow-up, MV restenosis was observed in 483 cases (26%). Mean age was 36 years and most (87%) were female. Median follow-up duration was 9.03 years (interquartile range, 0.33-23.38). Restenosis population, however, presented a significantly lower age at the procedure time as well as a higher Wilkins-Block score. At multivariate analysis, independent preprocedure predictors of restenosis were left atrium diameter (hazard risk [HR], 1.03; 95% confidence interval [CI], 1.02-1.05; P<.04), preprocedure maximum gradient (HR, 1.02; 95% CI, 1.00-1.03; P=.04), and higher Wilkins-Block score (>8) (HR, 1.38; 95% CI, 1.14-1.67; P<.01). CONCLUSIONS: At long-term follow-up, MV restenosis was observed in a quarter of the population undergoing PMBC. Preprocedure echocardiographic findings, including left atrial diameter, maximum MV gradient, and Wilkins-Block score were found to be the only independent predictors.

Independent clinical and echocardiographic predictors of restenosis after percutaneous mitral balloon commissurotomy in a large, consecutive cohort followed for 24 years

OBJECTIVES: to enlighten preprocedural risk factors of mitral valve restenosis in a large, single-center cohort of patients submitted to percutaneous mitral balloon commissurotomy (PMBC) for the treatment of mitral stenosis (MS) secondary to rheumatic heart disease. METHODS: this is a database analysis of a single-center, high-volume tertiary institution involving all consecutive PMBC procedures performed in the mitral valve (MV). Restenosis was diagnosed when MV area was <1.5 cm2 and/or loss of 50% or more of the immediate procedural result aligned with the return/worsened symptoms of heart failure. The primary endpoint was to determine the preprocedural independent predictors of restenosis after PMBC. Results: among a total of 1921 PMBC procedures, 1794 consecutive patients without previous intervention were treated between 1987 and 2010. Throughout 24 years of follow-up, MV restenosis was observed in 483 cases (26%). Mean age was 36 years and most (87%) were female. Median follow-up duration was 9.03 years (interquartile range, 0.33-23.38). Restenosis population, however, presented a significantly lower age at the procedure time as well as a higher Wilkins-Block score. At multivariate analysis, independent preprocedure predictors of restenosis were left atrium diameter (hazard risk [HR], 1.03; 95% confidence interval [CI], 1.02-1.05; P<.04), preprocedure maximum gradient (HR, 1.02; 95% CI, 1.00-1.03; P=.04), and higher Wilkins-Block score (>8) (HR, 1.38; 95% CI, 1.14-1.67; P<.01). CONCLUSIONS: at long-term follow-up, MV restenosis was observed in a quarter of the population undergoing PMBC. Preprocedure echocardiographic findings, including left atrial diameter, maximum MV gradient, and Wilkins-Block score were found to be the only independent predictors.

Long-term follow-up in patients with rheumatic mitral stenosis and severe pulmonary artery hypertension submitted to percutaneous mitral balloon commissurotomy

BACKGROUND: Percutaneous balloon mitral commissurotomy (PMBC) is an attractive therapeutic approach in patients with mitral stenosis. The aim of this study was to assess the immediate and long-term clinical, echocardiographic and haemodynamic outcomes of PMBC in patients with severe pulmonary hypertension (PH). METHODS: Among all procedures (in more than two decades of experience), PMBC was performed from 1987 until 2011 at a single-center in 147 patients who had significant PH defined as baseline pulmonary artery mean pressure (PAMP) (systolic pulmonary pressure > 75 mmHg). All-cause mortality, need for mitral valve replacement (MVR) or new PMBC, and valve restenosis were evaluated during follow-up yearly. RESULTS: Mean age was 33.8 ± 12.8 years and 83.6% (123 patients) were women. Primary success was achieved in 89.8% of the patients (132 patients). Mitral valve area (MVA) increased from 0.83 ± 0.17 cm2 to 2.03± 0.35 cm2 (p<0.001), and at 20-years, mitral valve area was 1.46± 0.34 cm2 (p=0.235). Systolic pulmonary artery pressure decreased from 87.0 ± 6.0 mmHg to 60.0 ± 0.9 mmHg (p<0.001) The rates of all-cause mortality, need for MVR, new PMV, and valve restenosis were 0.67%, 20.0%, 8.78% and 30.4%, respectively, in long-term follow- up (mean 15.6 ± 4.9 years). CONCLUSIONS: PMBC is a safe and effective technique for the treatment of patients with mitral stenosis and PH. A significant decrease in pulmonary pressure was observed after commissurotomy. Although there was a gradual decrease of MVA at long-term follow-up, most patients remained asymptomatic and without major adverse events.

Preditores independentes de reestenose da valva mitral após valvoplastia mitral percutânea por balão em uma grande coorte consecutiva com seguimento clínico por mais de duas décadas / Independent predictors of mitral valve restenosis after percutaneous balloon mitral valvuloplasty in a large consecutive cohort with clinical follow-up for more than two decades

INTRODUÇÃO: A valvoplastia mitral percutânea com balão (VMPB), sempre que tecnicamente viável, é a opção de tratamento preferencial para a estenose mitral, particularmente aquelas secundárias à doença cardíaca reumática. No entanto, a reestenose valvar mitral pode se desenvolver em um número significativo de pacientes submetidos a esse procedimento, com fatores de risco ainda pouco claros para tal ocorrência. OBJETIVOS: O objetivo deste estudo foi elucidar os fatores de risco da reestenose valvar mitral em um número significativo de pacientes submetidos à comissurotomia mitral percutânea por balão para tratamento da estenose mitral (EM), principalmente quando secundária à cardiopatia reumática. MÉTODOS: Trata-se de uma análise de centro único de uma coorte grande e consecutiva de pacientes tratados com VMP entre 1987 e 2010, que desenvolveram reestenose. O desfecho primário foi determinar os preditores independentes desse evento, definido como perda de mais de 50% do aumento original na área valvar mitral máxima (AVM) ou AVM menor que 1,5cm2. RESULTADOS: Um total de 1.794 pacientes consecutivos submetidos a VMP em um único centro, instituição terciária de alto volume, foram incluídos neste registro. Reestenose da valva mitral foi observada em 26% dos casos (n=483). A média de idade da população foi de 36 anos, com a maioria dos pacientes sendo do sexo feminino (87%). A duração média do acompanhamento foi de 4,8 anos. Na análise multivariada, os preditores independentes de reestenose foram: diâmetro atrial esquerdo [RR (risco relativo): 1,03; IC (intervalo de confiança) 95%: 1,01-1,04; p <0,01]; gradiente máximo pré-procedimento (RR: 1,01; IC 95%: 1,00-1,03; p=0,02) e Wilkinsscore > 8 (RR: 1,37; IC 95%: 1,13-1,66; p<0,01). CONCLUSÕES: No seguimento em longo prazo, a reestenose da valva mitral foi observada em até 25% da população submetida à VMP. Os achados ecocardiográficos pré-procedimento, incluindo o diâmetro do átrio esquerdo, o gradiente valvar máximo e o escore de Wilkins, foram os únicos preditores independentes desse desfecho desfavorável.

Seguimento a longo prazo de valvuloplastia mitral percutânea em pacientes com estenose reumática mitral e hipertensão arterial pulmonar grave / Long-term follow-up of percutaneous mitral valvuloplasty in patients with rheumatic mitral stenosis and severe pulmonary arterial hypertension

INTRODUÇÃO: A hipertensão arterial pulmonar pode ser um fator de complicação em pacientes com estenose valvar reumática e que serão submetidos a valvuloplastia mitral percutânea por balão (VMPB), sendo esta uma abordagem terapêutica atraente em pacientes com estenose mitral de origem reumática. OBJETIVOS: O objetivo deste estudo foi avaliar os resultados imediatos e de longo prazo em pacientes com hipertensão pulmonar (HAP) submetidos à VMPB e estenose mitral (EM) reumática. MÉTODOS: Entre os 1.794 pacientes consecutivos, de 1987 a 2010, a VMP foi realizada em um único centro em 147 pacientes que tinham HAP significativa definida como pressão arterial média basal (pressão pulmonar sistólica > 75mmhg). Mortalidade por todas as causas, necessidade de substituição valvar mitral ou nova VMP e reestenose valvar foram avaliados durante o acompanhamento anual. RESULTADOS: A média de idade foi de 33,8±12,8 anos e 83,6% (123 pacientes) eram mulheres. O sucesso foi alcançado em 89,8% dos pacientes (132 pacientes). A área valvar mitral (AVM) aumentou de 0,83±0,17cm2 para 2,03±0,35cm2 (p <0,001) e, aos 20 anos, a área valvar mitral foi de 1,46±0,34cm2 (p=0,235). A pressão sistólica da artéria pulmonar diminuiu de 87,0±6,0mmHg para 60,0±0,9mmHg (p <0,001) As taxas de mortalidade por todas as causas, necessidade de substituição da valva mitral, nova VMP e reestenose valvar foram de 0,67%, 20,0%, 8,78% e 30,4%, respectivamente, em seguimento a longo prazo (média de 15,6±4,9 anos). CONCLUSÕES: Observou-se que houve diminuição significativa da pressão arterial pulmonar após o procedimento e a VMPB é considerada segura e eficaz em pacientes com EM reumática. Embora tenha havido uma diminuição gradual da AVM a longo prazo, a maioria dos pacientes permaneceu assintomática e sem grandes eventos adversos.

Treatment of Isolated Idiopathic Growth Hormone Deficiency in Children and Thyroid Function: Is the Need for LT4 Supplementation a Concern in Long-Term Therapy?

Introduction Recombinant human growth hormone (rhGH) replacement therapy might be able to induce hypothyroidism, but this is a controversial issue. Previous studies evaluated the effects of rhGH replacement therapy on thyroid function, but little information is available in the subset of children with isolated idiopathic growth hormone deficiency (GHD). Our aim was to assess the effects of rhGH replacement therapy on thyroid function in children with isolated idiopathic GHD. Methods Retrospective analysis of the medical files of 64 children with confirmed GHD treated with rhGH. After review, 56 children with isolated idiopathic GHD and treated with rhGH for at least one year were included. Auxological (weight standard deviation score [SDS], height SDS, growth velocity [GV] SDS) and biochemical (free thyroxine [FT4], thyroid-stimulating hormone [TSH], and insulin-like growth factor 1 [IGF-1]) parameters were recorded before, during, and after treatment with rhGH. Results FT4 and TSH levels decreased significantly during rhGH therapy in children with isolated idiopathic GHD. Twenty-one percent (n=12) of the children developed hypothyroidism, on average 47 months after initiation of rhGH. Higher baseline FT4 levels were protective against the need for levothyroxine (LT4) (OR=0.8, CI 0.592-0.983; p=0.036). Hypothyroidism was reversed after interruption of rhGH, except in one patient; FT4 levels returned to baseline in the first year after completing the treatment. Final height SDS of the children who developed hypothyroidism was not different from their counterparts without hypothyroidism (-1.24 [-1.52 to -1.10] vs -1.13 [-1.78 to -0.74], p=1.000). Predicted adult height (PAH) SDS in patients who completed rhGH treatment was similar in both LT4 supplemented (n=7; final Ht SDS -1.16 [-1.31 to -1.10] vs PAH -1.00 [-1.42 to -0.48]; p=0.398) and not supplemented patients (n=25; final Ht SDS -1.46 [-1.83 to -0.78] vs PAH SDS -0.88 [-1.35 to -0.56]; p=0.074). Conclusions Our results show that patients with isolated idiopathic GHD may transiently need LT4 during GH treatment. Properly supplemented patients achieved PAH.

Independent clinical and echocardiographic predictors of restenosis after percutaneous mitral balloon commissurotomy followed for 24 years

INTRODUCTION: Mitral valve stenosis (MVS) is one of the most common structural heart diseases in developing countries, primarily due to rheumatic disease. Percutaneous mitral balloon valvuloplasty (PMBV) has been, since its introduction in 1984, the preferred option of treatment for such disease. However, restenosis is presented with an approximate incidence of 20%. Echocardiographic scoring of the mitral apparatus has been the main tool used to indicate and foresee the possible result of the procedure. OBJECTIVE: The objective of this study was to enlight risk factors of mitral valvular restenosis in a significant number of patients submitted to percutaneous mitral balloon commissurotomy for the treatment of mitral stenosis (MS), particularly when secondary to rheumatic heart disease. METHODS: This study reports the vast experience of a single center high volume tertiary institution where 1.794 consecutive patients were treated with PMBC between 1987 and 2011. The primary endpoint was to determine the independent predictors of this untoward event, defined as loss of over 50% of the original increase in maximum valve area (MVA) or MVA < 1.5 cm2. RESULTS: Mitral valve restenosis was observed in 26% of the cases (n = 483). Mean population age was 36 years old, with most patients being female (87%). Mean follow up duration was 4.8 years. At multivariate analysis independent pre-procedural predictors of restenosis were: left atrial diameter (HR: 1.03, 95% ci: 1.01-1.04, p < 0.01), pre procedure maximum gradient (HR: 1.01, 95% ci: 1.00-1.03, p = 0.02) and higher wilkins scores (HR: 1.37, 95% ci: 1.13-1.66, p < 0.01). CONCLUSION: In the very long term follow-up, mitral valve restenosis was observed in a quarter of the population undergoing PMBC. Preprocedure echocardiographic findings, including left atrial diameter, maximum valve gradient and high Wilkins scores were found to be the only independent predictors of this deleterious event.

Long-term follow-up in patients with rheumatic mitral stenosis and severe pulmonary artery hypertension submitted to percutaneous mitral balloon commissurotomy

INTRODUCTION: Percutaneous balloon mitral commissurotomy (PMBC) is an attractive therapeutic approach in patients with mitral stenosis. OBJECTIVE: The aim of this study was to assess the immediate and long-term clinical, echocardiographic and haemodynamic outcomes of PMBC in patients with severe pulmonary hypertension (PH). METHODS: Among all procedures (in more than two decades of experience), PMBC was performed from 1987 until 2011 at a single-center in 147 patients who had significant PH defined as baseline pulmonary artery mean pressure (PAMP) (systolic pulmonary pressure > 75 mmHg). All-cause mortality, need for mitral valve replacement (MVR) or new PMBC, and valve restenosis were evaluated during follow-up yearly. RESULTS: Mean age was 33.8 ± 12.8 years and 83.6% (123 patients) were women. Primary success was achieved in 89.8% of the patients (132 patients). Mitral valve area (MVA) increased from 0.83 ± 0.17 cm2 to 2.03 ± 0.35 cm2 (p < 0.001), and at 20-years, mitral valve area was 1.46 ± 0.34 cm2 (p = 0.235). Systolic pulmonary artery pressure decreased from 87.0 ± 6.0 mmHg to 60.0 ± 0.9 mmHg (p < 0.0001). The rates of all-cause mortality, need for MVR, new PMV, and valve restenosis were 0.67%, 20.0%, 8.78% and 30.4%, respectively, in long-term follow- up (mean 15.6 ± 4.9 years). CONCLUSIONS: PMBC is a safe and effective technique for the treatment of patients with mitral stenosis and PH. A significant decrease in pulmonary pressure was observed after commissurotomy. Although there was a gradual decrease of MVA at long-term follow-up, most patients remained asymptomatic and without major adverse events.

Seguimento de longo prazo em pacientes com estenose mitral reumática e hipertensão arterial pulmonar grave submetidos a valvuloplastia mitral percutânea por balão / Long-term follow-up in patients with rheumatic mitral stenosis and severe pulmonary arterial hypertension undergoing mitral valvuloplasty percutaneous balloon

INTRODUÇÃO: A valvuloplastia mitral percutânea por balão (VMPB) é uma abordagem terapêutica de escolha em pacientes com estenose mitral. OBJETIVO(s): O objetivo deste estudo foi avaliar os resultados clínicos, ecocardiográficos e hemodinâmicos imediatos e de longo prazo pacientes com hipertenso pulmonar (HP) grave submetidos a VMPB. MÉTODOS: Entre todos os procedimentos (em mais de duas décadas de experiência), a VMPB foi realizada de 1987 a 2011 em um único centro em 147 pacientes que tinham HP significativa definida como pressão média da artéria pulmonar basal (PAMP) (pressão pulmonar sistólica >75mmHg). Mortalidade por todas as causas, necessidade de substituição da válvula mitral (TVM) ou nova VMPB e reestenose da válvula foram avaliadas durante o acompanhamento anual. RESULTADOS: A média de idade foi 33,8±12,8 anos e 83,6% (123 pacientes) eram mulheres. O sucesso primário foi alcançado em 89,8% dos pacientes (132 pacientes). A área valvar mitral (AVM) aumentou de 0,83±0,17cm2 para 2,03±0,35cm2 (p<0,001) e, aos 20 anos, a AVM era de 1,46±0,34cm2 (p=0,235). A pressão sistólica da artéria pulmonar diminuiu de 87,0± 6,0mmHg para 60,0±0,9mmHg (p<0,0001). As taxas de mortalidade por todas as causas, necessidade de trova valvar mitral, nova VMPB e reestenose valvar foram de 0,67%, 20,0%, 8,78% e 30,4%, respectivamente, no seguimento de longo prazo (média 15,6±4,9 anos). CONCLUSÃO: VMPB é uma técnica segura e eficaz para o tratamento de pacientes com estenose mitral e HP. Uma diminuição significativa da pressão pulmonar foi observada após o procedimento. Embora tenha havido uma diminui. O gradual de AVM no acompanhamento de longo prazo, a maioria dos pacientes permaneceu assintomática e sem eventos adversos maiores.

Preditores clínicos e ecocardiográficos independentes de reestenose após valvuloplastia mitral percutânea por balão após seguimento de até 24 anos / Clinical and echocardiographic predictors independent of restenosis after valvuloplasty percutaneous mitral valve balloon after follow-up up to 24 years old

INTRODUÇÃO: A estenose da válvula mitral (EM) é uma das doenças cardíacas estruturais mais comuns nos países em desenvolvimento, principalmente devido à doença reumática. A valvoplastia mitral percutânea por balão (VMPB) tem sido, desde sua introdução em 1984, a opção preferencial de tratamento para essa doença. Porém, a reestenose apresenta-se com incidência aproximada de 20%. A pontuação ecocardiográfica do aparelho mitral tem sido a principal ferramenta utilizada para indicar e prever o possível resultado do procedimento. OBJETIVO(s): O objetivo deste estudo foi elucidar os fatores de risco da reestenose valvar mitral em um número significativo de pacientes submetidos à comissurotomia mitral percutânea por balão para tratamento da estenose mitral (EM), principalmente quando secundária à cardiopatia reumática. MÉTODOS: Este estudo relata a vasta experiência de uma instituição terciária de alto volume de centro único, onde 1.794 pacientes consecutivos foram tratados com PMBC entre 1987 e 2011. O desfecho primário foi determinar os preditores independentes deste evento adverso, definido como perda de mais de 50% do aumento original na área máxima da válvula (AVM) ou AVM <1,5cm2. RESULTADOS: Reestenose valvar mitral foi observada em 26% dos casos (n=483). A média de idade da população foi de 36 anos, sendo a maioria dos pacientes do sexo feminino (87%). A duração média do acompanhamento foi de 4,8 anos. Na análise multivariada, os preditores pré-procedimento independentes de reestenose foram: diâmetro do átrio esquerdo (HR: 1,03, IC 95%: 1,01-1,04, p<0,01), gradiente máximo pré-procedimento (HR: 1,01, IC 95%: 1,00-1,03, p=0,02) e pontuações de Wilkins mais altas (HR: 1,37, IC 95%: 1,13-1,66, p<0,01). CONCLUSÃO: No seguimento de muito longo prazo, a reestenose da válvula mitral foi observada em um quarto da população submetida a PMBC. Os achados ecocardiográficos pré-procedimento, incluindo diâmetro do átrio esquerdo, gradiente valvar máximo e altos escores de Wilkins, foram considerados os únicos preditores independentes desse evento deletério.

Independent mitral valve predictors of restenosis after percutaneous mitral balloon commissurotomy in a large, consecutive cohort followed for more than two decades

AIMS: the objective of this study was to enlight risk factors of mitral valvular restenosis in a significant number of patients submitted to percutaneous mitral balloon commissurotomy for the treatment of mitral stenosis (ms), particularly when secondary to rheumatic heart disease. METHODS AND RESULTS: this study reports the vast experience of a single center high volume tertiary institution where 1,794 consecutive patients were treated with PMBC between 1987 and 2011. the primary endpoint was to determine the independent predictors of this untoward event, defined as loss of over 50% of the original increase in maximum valve area (mva) or mva < 1.5 cm2. mitral valve restenosis was observed in 26% of the cases (n=483). average population age was 36 years old, with most patients being female (87%). mean follow up duration was 4.8 years. at multivariate analysis independent pre-procedural predictors of restenosis were: left atrial diameter (hr: 1.03, 95% ci: 1.01-1.04, p<0.01). pre procedure maximum gradient (hr: 1.01, 95% ci: 1.00-1.03, p=0.02) and higher wilkins scores (hr: 1.37, 95% ci: 1.13-1.66, p<0.01). CONCLUSIONS: In the very long term follow-up, mitral valve restenosis was observed in a quarter of the population undergoing PMBC. Preprocedure echocardiographic findings, including left atrial diameter, maximum valve gradient and high wilkins scores were found to be the only indepen dent predictors of this deleterious event.

Long-term follow-up in patients with rheumatic mitral stenosis and severe pulmonary artery hypertension submitted to percutaneous mitral balloon commissurotomy

AIMS: Percutaneous balloon mitral commissurotomy (PMBC) is an attractive therapeutic approach in patients with mitral stenosis. The aim of this study was to assess the immediate and long-term clinical, echocardiographic and haemodynamic outcomes of PMBC in patients with severe pulmonary hypertension (PH). METHODS AND RESULTS: Among all procedures (in more than two decades of experience), PMBC was performed from 1987 until 2011 at a single-center in 147 patients who had significant PH defined as baseline pulmonary artery mean pressure (PAMP) (systolic pulmonary pressure > 75 mmHg). All-cause mortality, need for mitral valve replacement (MVR) or new PMBC, and valve restenosis were evaluated during follow-up yearly. Mean age was 33.8 ± 12.8 years and 83.6% (123 patients) were women. Primary success was achieved in 89.8% of the patients (132 patients). Mitral valve area (MVA) increased from 0.83 ± 0.17 cm2 to 2.03± 0.35 cm2 (p<0.001), and at 20-years, mitral valve area was 1.46 ± 0.34 cm2 (p=0.235). Systolic pulmonary artery pressure decreased from 87.0 ± 6.0 mmHg to 60.0 ± 0.9 mmHg (p<0.0001). The rates of all-cause mortality, need for MVR, new PMV, and valve restenosis were 0.67%, 20.0%, 8.78% and 30.4%, respectively, in long-term follow- up (mean 15.6 ± 4.9 years). CONCLUSIONS: PMBC is a safe and effective technique for the treatment of patients with mitral stenosis and PH. A significant decrease in pulmonary pressure was observed after commissurotomy. Although there was a gradual decrease of MVA at long-term follow-up, most patients remained asymptomatic and without major adverse events.

Avascular femoral necrosis as part of Cushing syndrome presentation: a case report.

BACKGROUND: The clinical characteristics and complications of Cushing syndrome (CS) are well known and described in the literature. Nevertheless, rare, atypical presentations may go unnoticed. Osteonecrosis is a well-documented complication of glucocorticoid therapy. However, endogenous hypercortisolism is a rare, but relevant, cause of bone avascular necrosis. We describe the case of a woman with CS undiagnosed for 2 years after presenting with femoral avascular necrosis. CASE PRESENTATION: A 38-year-old Caucasian woman was referred for evaluation of secondary amenorrhea, associated with oral contraception withdrawal in the context of deep venous thrombosis (DVT). She had a previous right hip arthroplasty for treatment of avascular necrosis of the femoral head, diagnosed after 3 years of progressive right hip pain and limited mobility. She also had high blood pressure (HBP) of 5 years' duration, and reported weight gain (4 kg in 2 years). There was no history of infertility (gravida 2, para 2). Physical examination revealed buffalo hump, truncal obesity, facial plethora, muscular atrophy and proximal myopathy, and easy bruising (under anticoagulant treatment for DVT). Workup showed abnormal overnight dexamethasone suppression test (DST) (serum cortisol 21.5 µg/dL; normal < 1.8 µg/dL), elevated 24-hour urinary free cortisol (UFC) (728.9 µg/day; reference range 36.0-137.0 µg/day), and suppressed plasma adrenocorticotropic hormone (ACTH) (< 1.0 pg/mL), findings consistent with ACTH-independent CS. Urinary metanephrines and catecholamines were normal, and the remaining analytical study showed no major changes, apart from glycated hemoglobin (HbA1c) of 6.8%. Adrenal computed tomography (CT) scan showed a 25 mm lesion in the left adrenal gland, with density non-suggestive of adenoma. The patient underwent unilateral adrenalectomy and started steroid replacement. Histology revealed an adrenal cortex adenoma. Three months after surgery the patient presented with resolution of HBP and hypercortisolism (UFC 37.4 µg/day; reference range 36.0-137.0 µg/day). CONCLUSION: In some cases, CS signs may go unnoticed and the diagnosis postponed. Avascular necrosis is a rare presenting feature of endogenous hypercortisolism, and, if left untreated, complete collapse of the femoral head may ensue, rendering the need for hip replacement in up to 70% of patients. Suspicion and recognition of atypical features is therefore important in avoiding complications and delay in treatment of CS.

Novel prognostic score for immediate and late success after percutaneous mitral balloon commissurotomy in patients with mitral stenosis

Abstract: Objectives. Percutaneous mitral balloon commissurotomy (PMBC) remains the preferred treatment for patients with severe symptomatic rheumatic mitral stenosis (MS) and suitable anatomy. The objective of this study was to propose a new score for the prediction of immediate and late success. Methods. This is a single-center, retrospective analysis of all 1582 patients with severe mitral stenosis who underwent PMBC from August 1987 to July 2010. The composite outcome was cardiovascular death, new PMBC, or mitral valve repair surgery up to 24 years of follow-up. Results. Mean patient age was 36.8 ± 12.9 years, most (86.4%) were female, and Wilkins score was between 9-11 in 49.1% of patients. In the multivariate analysis, the predictors of immediate success were age (odds ratio [OR], 0.98; 95% confidence interval [CI], 0.96-0.99; P=.01), left atrium size (OR, 0.96; 95% CI, 0.93-0.99; P=.01), mean preprocedure mitral gradient (OR, 0.93; 95% CI, 0.89-0.96; P<.001), intermediate Wilkins score 9-11 (OR, 0.62; 95% CI, 0.40-0.94; P=.02), and high Wilkins score ≥12 (OR, 0.35; 95% CI, 0.16-0.76; P<.01). For prediction of late events, age (hazard ratio [HR], 0.98; 95% CI, 0.97-0.98; P<.001), New York Heart Association class III-IV (HR, 1.50; 95% CI, 1.18-1.92; P<.001), left atrium size (HR, 1.02; 95% CI, 1.02-0.04; P<.01), and high Wilkins score ≥12 (HR, 2.02; 95% CI, 1.30-3.15; P<.01) were significant. Two nomograms were developed using significant predictors from the model. Conclusions. In this large population, not only the Wilkins score, but also clinical and hemodynamic features, seem to be relevant in predicting immediate and late success for patients with rheumatic MS who underwent PMBC.

Novel Prognostic Score for Immediate and Late Success After Percutaneous Mitral Balloon Commissurotomy in Patients With Mitral Stenosis.

OBJECTIVES: Percutaneous mitral balloon commissurotomy (PMBC) remains the preferred treatment for patients with severe symptomatic rheumatic mitral stenosis (MS) and suitable anatomy. The objective of this study was to propose a new score for the prediction of immediate and late success. METHODS: This is a single-center, retrospective analysis of all 1582 patients with severe mitral stenosis who underwent PMBC from August 1987 to July 2010. The composite outcome was cardiovascular death, new PMBC, or mitral valve repair surgery up to 24 years of follow-up. RESULTS: Mean patient age was 36.8 ± 12.9 years, most (86.4%) were female, and Wilkins score was between 9-11 in 49.1% of patients. In the multivariate analysis, the predictors of immediate success were age (odds ratio [OR], 0.98; 95% confidence interval [CI], 0.96-0.99; P=.01), left atrium size (OR, 0.96; 95% CI, 0.93-0.99; P=.01), mean preprocedure mitral gradient (OR, 0.93; 95% CI, 0.89-0.96; P<.001), intermediate Wilkins score 9-11 (OR, 0.62; 95% CI, 0.40-0.94; P=.02), and high Wilkins score ≥12 (OR, 0.35; 95% CI, 0.16-0.76; P<.01). For prediction of late events, age (hazard ratio [HR], 0.98; 95% CI, 0.97-0.98; P<.001), New York Heart Association class III-IV (HR, 1.50; 95% CI, 1.18-1.92; P<.001), left atrium size (HR, 1.02; 95% CI, 1.02-0.04; P<.01), and high Wilkins score ≥12 (HR, 2.02; 95% CI, 1.30-3.15; P<.01) were significant. Two nomograms were developed using significant predictors from the model. CONCLUSIONS: In this large population, not only the Wilkins score, but also clinical and hemodynamic features, seem to be relevant in predicting immediate and late success for patients with rheumatic MS who underwent PMBC.

Cushing's syndrome: Consequences of late diagnosis after bariatric surgery.

Prior to bariatric surgery, endocrine causes of obesity must be excluded. The diagnosis of osteoporosis in a male requires the study of secondary causes of this condition. The diagnostic delay of Cushing's syndrome may have irreversible consequences.

Interaction of Genetic Variations in NFE2L2 and SELENOS Modulates the Risk of Hashimoto's Thyroiditis.

Background: Several single-nucleotide polymorphisms (SNPs) are known to increase the risk of Hashimoto's thyroiditis (HT); such SNPs reside in thyroid-specific genes or in genes related to autoimmunity, inflammation, and/or cellular defense to stress. The transcription factor Nrf2, encoded by NFE2L2, is a master regulator of the cellular antioxidant response. This study aimed to evaluate the impact of genetic variation in NFE2L2 on the risk of developing HT. Methods: In a case-control candidate gene association study, functional SNPs in the NFE2L2 promoter (rs35652124, rs6706649, and rs6721961) were examined either as independent risk factors or in combination with a previously characterized HT risk allele (rs28665122) in the gene SELENOS, encoding selenoprotein S (SelS). A total of 997 individuals from the north of Portugal (Porto) were enrolled, comprising 481 HT patients and 516 unrelated healthy controls. SELENOS and NFE2L2 SNPs were genotyped using TaqMan® assays and Sanger sequencing, respectively. Odds ratios (ORs) were calculated using logistic regression, with adjustment for sex and age. Expression of SelS was analyzed by immunohistochemistry in thyroid tissue from HT patients and control subjects. Molecular interactions between the Nrf2 and SelS pathways were investigated in thyroid tissues from mice and in rat PCCL3 thyroid follicular cells. Results: When all three NFE2L2 SNPs were considered together, the presence of one or more minor alleles was associated with a near-significant increased risk (OR = 1.43, p = 0.072). Among subjects harboring only major NFE2L2 alleles, there was no increased HT risk associated with heterozygosity or homozygosity for the SELENOS minor allele. Conversely, in subjects heterozygous or homozygous for the SELENOS risk allele, the presence of an NFE2L2 minor allele significantly increased HT risk by 2.8-fold (p = 0.003). Immunohistochemistry showed reduced expression of SelS in thyroid follicular cells of HT patients. In Nrf2 knockout mice, there was reduced expression of SelS in thyroid follicular cells; conversely, in PCCL3 cells, reducing SelS expression caused reduced activity of Nrf2 signaling. Conclusions: The NFE2L2 promoter genotype interacts with the SELENOS promoter genotype to modulate the risk of HT in a Portuguese population. This interaction may be due to a bidirectional positive feedback between the Nrf2 and SelS pathways.

Preditores independentes de reestenose da valva mitral após valvoplastia mitral percutânea por balão em uma grande coorte consecutiva com seguimento clínico por mais de duas décadas / Independent predictors of mitral valve restenosis after percutaneous balloon mitral valvuloplasty in a large consecutive cohort with clinical follow-up for more than two decades

INTRODUÇÃO: A valvoplastia mitral percutânea com balão (VMP), sempre que tecnicamente viável, é a opção de tratamento preferencial para a estenose mitral, particularmente aquelas secundárias à doença cardíaca reumática. No entanto, a reestenose valvar mitral pode se desenvolver em um número significativo de pacientes submetidos a esse procedimento, com fatores de risco ainda pouco claros para tal ocorrência. MÉTODOS: Trata-se de uma análise de centro único de uma coorte grande e consecutiva de pacientes tratados com VMP entre 1987 e 2010, que desenvolveram reestenose. O desfecho primário foi determinar os preditores independentes desse evento, definido como perda de mais de 50% do aumento original na área valvar mitral máxima (AVM) ou AVM menor que 1,5 cm2. RESULTADOS: Um total de 1.794 pacientes consecutivos submetidos a VMP em um único centro, instituição terciária de alto volume, foram incluídos neste registro. Reestenose da valva mitral foi observada em 26% dos casos (n = 483). A média de idade da população foi de 36 anos, com a maioria dos pacientes sendo do sexo feminino (87%). A duração média do acompanhamento foi de 4,8 anos. Na análise multivariada, os preditores independentes de reestenose foram: diâmetro atrial esquerdo [RR (risco relativo): 1,03; IC (intervalo de confiança) 95%: 1,01-1,04; p <0,01]; gradiente máximo pré-procedimento (RR: 1,01; IC 95%: 1,00-1,03; p = 0,02 ) e Wilkins score maior que 8 (RR: 1,37; IC 95%: 1,13-1,66; p <0,01). CONCLUSÕES: No seguimento em longo prazo, a reestenose da valva mitral foi observada em até 25% da população submetida à VMP. Os achados ecocardiográficos pré-procedimento, incluindo o diâmetro do átrio esquerdo, o gradiente valvar máximo e o escore de Wilkins, foram os únicos preditores independentes desse desfecho desfavorável. (AU)

Seguimento a longo prazo de valvuloplastia mitral percutânea em pacientes com estenose reumática mitral e hipertensão arterial pulmonar grave / Long-term follow-up of percutaneous mitral valvuloplasty in patients with mitral rheumatic stenosis and severe pulmonary arterial hypertension

INTRODUÇÃO: O objetivo deste estudo foi avaliar os resultados imediatos e de longo prazo em pacientes com hipertensão pulmonar (HAP) submetidos à valvuloplastia mitral percutânea por balão (VMP) e estenose mitral (EM) reumática. MÉTODOS: Entre os 1.794 pacientes consecutivos, de 1987 a 2010, a VMP foi realizada em um único centro em 147 pacientes que tinham HAP significativa definida como pressão arterial média basal (pressão pulmonar sistólica > 75 mmhg). Mortalidade por todas as causas, necessidade de substituição valvar mitral ou nova VMP e reestenose valvar foram avaliados durante o acompanhamento anual. RESULTADOS: A média de idade foi de 33,8 ± 12,8 anos e 83,6% (123 pacientes) eram mulheres. O sucesso foi alcançado em 89,8% dos pacientes (132 pacientes). A área valvar mitral (AVM) aumentou de 0,83 ± 0,17 cm2 para 2,03 ± 0,35 cm2 (p <0,001) e, aos 20 anos, a área valvar mitral foi de 1,46 ± 0,34 cm2 (p = 0,235). A pressão sistólica da artéria pulmonar diminuiu de 87,0 ± 6,0 mmHg para 60,0 ± 0,9 mmHg (p <0,0001). As taxas de mortalidade por todas as causas, necessidade de substituição da valva mitral, nova VMP e reestenose valvar foram de 0,67%, 20,0%, 8,78% e 30,4%, respectivamente, em seguimento a longo prazo (média de 15,6 ± 4,9 anos). CONCLUSÕES: Observou-se que houve diminuição significativa da pressão arterial pulmonar após o procedimento e a VMP é considerada segura e eficaz em pacientes com EM reumática. Embora tenha havido uma diminuição gradual da AVM a longo prazo, a maioria dos pacientes permaneceu assintomática e sem grandes eventos adversos. (AU)