Detection of soluble suppression of tumorigenicity 2 and N-terminal B-type natriuretic peptide in a rat model of aortic regurgitation: differential responses to omecamtiv mecarbil.

OBJECTIVES: Both N-terminal fragment of B-type natriuretic peptide (NT-proBNP) and soluble isoform of ST2 (sST2) have been identified as biomarkers of heart failure. We evaluated the plasma levels of NT-proBNP and sST2 in a rat model of severe aortic valve regurgitation (AR) and correlated these findings with echocardiographic measurements. We also examined the impact of omecamtiv mecarbil (OM) on these parameters. METHODS: The plasma levels of NT-proBNP and sST2 were measured in 18 rats both before and 2 months after surgical induction of AR, and at these same time points, in six rats assigned to a sham-procedure control group. Plasma biomarkers were then measured again after infusion of OM or placebo in rats with AR (n=8 and 10, respectively) and OM alone in the sham control rats (n=6). Echocardiographic measurements were collected before and 2 months after induction of AR. RESULTS: Our results revealed increased levels of plasma NT-proBNP (219 ± 34 pg/mL vs. 429 ± 374 pg/mL; p<0.001) in rats with AR at day 7 after infusion of placebo, whereas plasma levels of sST2 were higher in this cohort after infusion of either OM or placebo. We identified a significant positive correlation between plasma sST2 with posterior wall thickness in diastole (r=0.34, p<0.05) and total body weight (r=0.45, p<0.01) in rats with surgically induced AR. CONCLUSIONS: Because sST2 increased markedly, whereas NT-proBNP remained unchanged, when OM was administered, we hypothesize that sST2 has a distinct capability to detect deleterious effects of passive muscle tension, not reliably assessed by NT-proBNP, in the setting of AR.

Effects of the cardiac myosin activator Omecamtiv-mecarbil on severe chronic aortic regurgitation in Wistar rats.

BACKGROUND: Aortic regurgitation (AR) is a valvular disease that can lead to systolic heart failure. Treatment options besides cardiac surgery are limited and consequently severe AR is associated with higher mortality and morbidity when not operated. In this investigation, we examined the effects of a novel cardiac myosin activator, Omecamtiv-mecarbil (OM), in rats with chronic severe AR. METHODS: AR was created by retrograde puncture of the aortic valve leaflets in 20 adults Wistar rats. 12 animals survived the acute AR phase and were randomized 2 months thereafter into OM (n = 7) or placebo groups (n = 5). Two rats underwent a sham operation and served as controls. Equal volumes of OM or placebo (NaCl 0.9%) were perfused in the femoral vein by continuous infusion (1.2 mg/kg/hour) during 30 min. Doppler-echocardiography was performed before and at the end of the infusion periods. RESULTS: OM increased indices of global cardiac function (cardiac output, stroke volume), and increased systolic performance (fractional shortening, ejection fraction, left ventricular end systolic diameter) (all p < 0.05). These effects concurred with decreases in indices of LV preload (left atrial size, left ventricular end diastolic diameter) as well in the aortic pre-ejection period / left ventricular ejection time ratio (all p < 0.05). The severity score of the regurgitant AR jet did not change. Placebo infusion did not affect these parameters. CONCLUSION: The cardiac myosin activator OM exerts favorable hemodynamic effects in rats with experimental chronic AR.

Warfarin-related nephropathy with acute kidney injury in a patient with immunoglobulin A nephropathy.

A 55-year-old man with Marfan syndrome taking warfarin for anticoagulant therapy after aortic valve replacement developed acute kidney injury (serum creatinine level of 9.01 mg/dL) and gross macrohematuria. Renal biopsy showed red cell casts in the renal tubules, glomerular crescent formation in the glomeruli with immunoglobulin A deposition, and global sclerosis. Based on these findings, the patient was diagnosed with warfarin-related nephropathy with acute kidney injury characterized by immunoglobulin A nephropathy with crescents. The warfarin was withdrawn, and his hematuria and renal function improved without immunosuppressive agents.

Aortic Regurgitation Presenting with Recurrent Detachment of a Prosthetic Valve, as the First Presenting Symptom of Cardiovascular Behçet's Disease.

A 33-year-old man with severe aortic regurgitation underwent initial aortic valve replacement (AVR). During the 2 years after AVR, 3 reoperations for prosthetic valve detachment were required. During hospitalization, he had no typical clinical findings, with the exception of a persistent inflammatory reaction; a pseudo-aneurysm around the Bentall graft developed 27 days after the 4th operation. This unique clinical course suggested the possibility of Behçet's disease. In the 8 years of follow-up after the administration of prednisolone, the pseudo-aneurysm did not become enlarged and the detachment of the prosthetic valve was not observed. We herein present a case of cardiovascular Behçet's disease, with a review of the literature.

Comparison of effects of losartan and metoprolol on left ventricular and aortic function at rest and during exercise in chronic aortic regurgitation.

Aortic regurgitation (AR) increases the hemodynamic load on both the left ventricle (LV) and the aorta. Vasodilators and beta-blockers both reduce systemic blood pressure, but their relative effects on the LV and aortic function and aortic regurgitant fraction in chronic AR are uncertain. We aimed to compare short-term effects of losartan and metoprolol on LV and aortic function in asymptomatic patients with chronic moderate to severe AR, both at rest and during exercise, using cardiac magnetic resonance (CMR) imaging. 17 chronic AR patients were randomized to 4-6 weeks losartan followed by metoprolol, or vice versa, in a cross-over design. Aortic regurgitant fraction, aortic distensibility, pulse wave velocity and LV function were assessed at rest and after moderate exercise stress (29 ± 7 W, heart rate increase 25 ± 6 bpm) using CMR. Chronic AR patients on metoprolol had a significantly lower mean heart rate, cardiac power index and rate-pressure product, than on losartan (all p < 0.01). However, aortic regurgitant fraction was greater on metoprolol compared to losartan (by 7 ± 11%, p = 0.02). Metoprolol was also associated with a greater reduction in aortic distensibility during exercise than losartan (- 2.4 ± 1.5 × 10-3 vs - 1.7 ± 2.1 × 10-3 mmHg-1 respectively, p = 0.04). End-diastolic volume index was higher on metoprolol than losartan at exercise (difference 6.6 ± 7.8 ml/m2, p < 0.01), as was end-systolic volume index (difference 4.0 ± 5.2 ml/m2, p < 0.01). Losartan and metoprolol have significantly different short-term effects on aortic regurgitation and LV and aortic function in chronic AR. Further research is required to determine the long-term clinical significance of these changes.

Ascending aortic aneurysm caused by Mycobacterium tuberculosis.

BACKGROUND: Tuberculous aortitis is an unusual presentation of a common disease in Sri Lanka. There were no reported cases of tuberculous aortitis from Sri Lanka. Here we report a case of a 40-year-old woman who developed an ascending aortic aneurysm with severe aortic regurgitation caused by Mycobacterium tuberculosis. CASE PRESENTATION: A 40-year-old Sri Lankan female who presented with exertional breathlessness (NYHA II) and weight loss for 4 weeks duration was found to have collapsing pulse and early diastolic murmur at left sternal edge. Transthoracic and transesophageal echocardiogram showed ascending aortic aneurysm with severe aortic regurgitation. Computed tomographic aortography confirmed the diagnosis of aneurysmal dilatation of the ascending aorta. She underwent successful aortic valve replacement and aortic root replacement. The final diagnosis of tuberculous aortitis was made on the basis of macroscopic appearance of inflammation and microscopic confirmation of caseating granuloma. She made a good clinical recovery with category 1 antituberculous chemotherapy. CONCLUSIONS: Although most cases of aortitis are non-infectious in Sri Lanka, an infectious etiology must be considered in the differential diagnosis because therapeutic approaches differ widely. Tuberculous aortitis may be under diagnosed in Sri Lanka, a country with intermediate tuberculosis burden, as the histological or microbiological diagnosis is not possible in most cases. The clinical and radiological diagnostic criteria for tuberculous aortitis need to be set out in case of aneurysmal aortic disease in the absence of apparent etiology.

Anemia in a middle aged female with aortitis: a case report.

BACKGROUND: Idiopathic aortitis is among the most common causes of non-infectious aortitis, which rarely presents with anemia. CASE PRESENTATION: Here we report a case of a 49-year-old muhajir female who presented with shortness of breath and easy fatigability for the past 6 months. Physical examination revealed pallor and a diastolic murmur in the aortic region. Echocardiography showed thickened and calcified aortic and mitral valves, severe aortic regurgitation and dilatation of ascending aorta. She was advised aortic valve replacement and was referred to a haematologist due to concomitant anemia. Complete blood counts revealed haemoglobin: 7.7 gm/dl, mean corpuscular volume (MCV): 78 fl, mean corpuscular haemoglobin (MCH):23 pg, total white cell count: 9.0 × 10(9)/L and platelet count: 227 × 10(9)/L. Erythrocyte sedimentation rate (ESR) was 100 mm/hr. There was suspicion of myelodysplastic syndrome, but could not be confirmed as the patient refused bone marrow and cytogenetic studies. She was given erythropoietin, folic acid and ferrous sulphate. Following relatively prolonged therapy, her haemoglobin level increased to approximately 9.0 gm/dL. She was transfused with packed red cells and underwent aortic valve and ascending aorta replacement. The ascending aorta was dilated and aortic wall markedly thick and irregular. Histopathology of the resected aorta revealed granulomatous aortitis. She was prescribed prednisolone, which resulted in further incremental rise of haemoglobin to 13.1 gm/dL. One month later, she developed complaints of blurred vision in the right eye and was diagnosed with central retinal vein occlusion. She was treated with antiplatelet agents and her vision improved. After 3 months, she was asymptomatic and her haemoglobin level rose to 11.2 gm/dL without hematinic therapy or blood transfusion. She was begun on anticoagulant therapy and remains clinically stable. CONCLUSION: We report a case of idiopathic aortitis with presumed diagnosis of anemia of chronic disease exhibiting a transient response towards steroid therapy post-valvuloplasty.

Transesophageal Echocardiography Guidance of Antegrade Cardioplegia Delivery for Cardiac Surgery.

OBJECTIVES: The initial volume of antegrade cardioplegia used to induce asystole during aortic cross-clamp still is based on empiric methods and may be excessive, potentially leading to hyperkalemia, myocardial edema, and acute left ventricular distention from aortic regurgitation. The objectives were to determine whether the volume of cardioplegia required to induce asystole is proportional to left ventricular mass, and whether the degree of left ventricular distention is proportional to the severity of aortic regurgitation. DESIGN: Prospective observational study. SETTING: Two tertiary university hospitals. INTERVENTIONS: Transesophageal echocardiography was used to estimate left ventricular mass (prolate ellipse revolution formula), quantify aortic regurgitation, and monitor for distention during initial antegrade cardioplegia delivery. The volume of cardioplegia required for asystole was recorded. PARTICIPANTS: Fifty-eight patients aged over 18 years scheduled for cardiac surgery requiring aortic cross-clamping. MEASUREMENTS AND MAIN RESULTS: There was a weak correlation of left ventricular mass and antegrade cardioplegia volume required for asystole (r = 0.35, p = 0.047). The degree of left ventricular distention correlated moderately with the severity of aortic regurgitation (r = 0.55, p = 0.007) and was excessive and stopped early (aborted) in 24% of all patients, including 18% of 39 patients without aortic regurgitation. An aortic regurgitation vena contracta of 0.3 cm predicted aborted cardioplegia with modest accuracy (AUC 0.81, 0.66-0.99, p = 0.02, sensitivity 71%, specifity 81%). CONCLUSIONS: Estimated left ventricular mass is not a useful predictor of the initial volume of antegrade cardioplegia required to induce asystole. However transesophageal echocardiography can predict and monitor for left ventricular distention, which is common.

Advantages of early replacement therapy for mucopolysaccharidosis type VI: echocardiographic follow-up of siblings.

Mucopolysaccharidosis type VI (Marateaux-Lamy syndrome) is an autosomal recessive disorder caused by deficient activity of the enzyme N-acetylgalactosamine-4-sulphatase (arylsulphatase B). Cytoplasmic vacuoles full of dermatan sulphate are observed in endothelial cells, myocyte, and fibroblasts, compromising the function of cardiovascular structures and contributing significantly towards morbidity and mortality. The primary objective of this study was to assess the advantages of early replacement therapy with recombinant human arylsulphatase B through the echocardiographic follow-up of sisters who started treatment at quite different ages: one at 9 years and the other at 1 year and 7 months. The older sibling showed striking mitral and aortic valve compromise when she was only 2 years old and finally needed cardiac surgery at the age of 8, even before starting enzyme replacement. Differently, the younger one has developed only mild mitral and aortic lesions throughout the follow-up period of 3 years. The two siblings had left ventricle cardiomyopathy, but partial reverse remodelling was induced by enzyme replacement therapy in both cases. The younger sibling has never received any cardiovascular drugs, whereas the older one has been using ß-blockers and diuretics in addition to enzyme therapy to cope with heart failure. Comparing the outcomes of these two sisters with a very aggressive phenotype of mucopolysaccharidosis type VI, the conclusion was that early onset of therapy may slow down the disease progression and prevent severe cardiac lesions to be established. Moreover, patients' compliance is essential for the success of treatment, as sequential echocardiographic evaluation demonstrated worsening of some cardiac lesions whenever infusions were missed.

Angiotensin II-converting enzyme inhibition improves survival, ventricular remodeling, and myocardial energetics in experimental aortic regurgitation.

BACKGROUND: Aortic valve regurgitation (AR) is a volume-overload disease causing severe eccentric left ventricular (LV) hypertrophy and eventually heart failure. There is currently no approved drug to treat patients with AR. Many vasodilators including angiotensin-converting enzyme inhibitors have been evaluated in clinical trials, but although some results were promising, others were inconclusive. Overall, no drug has yet been able to improve clinical outcome in AR and the controversy remains. We have previously shown in an animal model that captopril (Cpt) reduced LV hypertrophy and protected LV systolic function, but we had not evaluated the clinical outcome. This protocol was designed to evaluate the effects of a long-term Cpt treatment on survival in the same animal model of severe aortic valve regurgitation. METHODS AND RESULTS: Forty Wistar rats with AR were treated or untreated with Cpt (1 g/L in drinking water) for a period of 7 months to evaluate survival, myocardial remodeling, and function by echocardiography as well as myocardial metabolism by µ positron emission tomography scan. Survival was significantly improved in Cpt-treated animals with a survival benefit visible as soon as after 4 months of treatment. Cpt reduced LV dilatation and LV hypertrophy. It also significantly improved the myocardial metabolic profile by restoring the level of fatty acids metabolic enzymes and use. CONCLUSIONS: In a controlled animal model of pure severe aortic valve regurgitation, Cpt treatment reduced LV remodeling and LV hypertrophy and improved myocardial metabolic profile and survival. These results support the need to reevaluate the role of angiotensin-converting enzyme inhibitors in humans with AR in a large, carefully designed prospective clinical trial.

Fenofibrate reduces cardiac remodeling and improves cardiac function in a rat model of severe left ventricle volume overload.

AIMS: Fenofibrate is a peroxisome proliferator-associated receptor alpha agonist (PPARα) used clinically for the management of dyslipidemia and is a myocardial fatty acid oxidation stimulator. It has also been shown to have cardiac anti-hypertrophic properties but the effects of fenofibrate on the development of eccentric LVH and ventricular function in chronic left ventricular (LV) volume overload (VO) are unknown. This study was therefore designed to explore the effects of fenofibrate treatment in a VO rat model caused by severe aortic valve regurgitation (AR) with a focus on cardiac remodeling and myocardial metabolism. MAIN METHODS: Male Wistar rats were divided in four groups (13-15 animals/group): Shams (S) treated with fenofibrate (F; 100 mg/kg/d PO) or not (C) and severe AR receiving or not fenofibrate. Treatment was started one week before surgery and the animals were sacrificed 9 weeks later. KEY FINDINGS: AR rats developed severe LVH (increased LV weight) during the course of the protocol. Fenofibrate did not reduce LV weight. However, eccentric LV remodeling was strongly reduced by fenofibrate in AR animals. Fractional shortening was significantly less affected in ARF compared to ARC group. Fenofibrate also increased the myocardial enzymatic activity of enzymes associated with fatty acid oxidation while inhibiting glycolytic enzyme phosphofructokinase. SIGNIFICANCE: Fenofibrate decreased LV eccentric remodeling associated with severe VO and helped maintain systolic function. Studies with a longer follow-up will be needed to assess the long-term effects of fenofibrate in chronic volume overload caused by aortic regurgitation.

Beta-blocker therapy for valvular disorders.

Valvular disorders are common, and result in a neurohormonal milieu similar to the heart failure state. Although valve surgery is the therapy of choice in symptomatic severe lesions, many patients do not receive surgery for a variety of reasons. Beta-blockers have a role in the management of many patients with valvular disorders, especially in the case of patients with mitral stenosis, where they reduce the transmitral gradient. They may also serve as life-saving therapy in pregnant women with pulmonary edema. Other uses of beta-blockers include the reduction of valve-related hemolysis, the prevention of atrial fibrillation, and the relief of dynamic left and right ventricular outflow tract obstruction. The prevention of aortic root dilation, potentially with beta-receptor blockade, may reduce the risk of aortic insufficiency in Marfan syndrome, and also in those with bicuspid aortic valves or following the Ross procedure. In this review, the potential role of beta-blockers is explored for the treatment of severe mitral and aortic regurgitation and asymptomatic severe aortic stenosis.

Congestive heart failure complicating aortic regurgitation with medical and surgical management: a prospective study of traditional and quantitative echocardiographic markers.

OBJECTIVE: Congestive heart failure complicating aortic regurgitation is poorly described, and predictive roles of quantitative versus traditional (symptoms or low ejection fraction) surgical markers are unclear. METHODS: We prospectively enrolled 287 patients with aortic regurgitation (age, 61 +/- 17 years; 68% male) in whom we performed quantitative Doppler echocardiographic analysis and personal physicians conducted management. RESULTS: After diagnosis, 40 congestive heart failure episodes occurred under medical management (10-year, 23% +/- 4%) causing high subsequent mortality (hazard ratio [HR], 2.8; 95% confidence interval [CI], 1.2-6.8; P = .02). Patients with traditional surgical markers (symptoms or ejection fraction <50%) were surprisingly followed 1.4 +/- 3.3 years under medical management with frequent congestive heart failure (adjusted risk, 4.9; 95% CI, 2.1-11.0; P < .001) and excess postoperative mortality (HR, 3.0; 95% CI, 1.3-7.1; P = .01). Quantitative American Society of Echocardiography aortic regurgitation grading and left ventricular end-systolic volume index independently predicted congestive heart failure (quantitative American Society of Echocardiography severe aortic regurgitation: HR, 3.6; 95% CI, 1.3-13.0; P = .015; end-systolic volume index >or=45 mL/m(2): HR, 2.1; 95% CI, 1.03-4.4; P = .04) or death-congestive heart failure with incremental predictive value (P < .001). Higher congestive heart failure rates occurred with quantitative American Society of Echocardiography severe aortic regurgitation (regurgitant volume of >or=60 mL/beat or orifice of >or=30 mm(2)) versus quantitative American Society of Echocardiography mild aortic regurgitation (10-year: 44% +/- 10% vs 15% +/- 7%, P < .001) and end-systolic volume index of 45 mL/m(2) or greater versus less than 45 mL/m(2) (33% +/- 7% vs 9% +/- 2%, P < .001). Traditional markers (symptoms and ejection fraction <50%) had lower sensitivity for congestive heart failure than quantitative echocardiography (all P < .001). Cardiac surgery for aortic regurgitation markedly reduced congestive heart failure in quantitative American Society of Echocardiography severe aortic regurgitation (HR, 0.23; 95% CI, 0.08-0.68; P = .008) without excess mortality (P = .10). CONCLUSION: This prospective study of aortic regurgitation shows frequent congestive heart failure under conservative management. Traditional surgical markers (symptoms and ejection fraction <50%) predict subsequent congestive heart failure but are insensitive, and rescue operations are often delayed and associated with excess mortality. Quantitative echocardiography provides congestive heart failure predictors that are independent, incremental, and more sensitive than traditional markers. Cardiac surgery for aortic regurgitation markedly reduces congestive heart failure rates in high-risk patients with aortic regurgitation.

[Aortic insufficiency under weak doses of cabergoline for non-tumoral hyperprolactinemia]. / Insuffisance aortique sous faibles doses de cabergoline pour hyperprolactinémie non tumorale.

Used in its neurological indication, cabergoline is known to induce cardiac valve regurgitations, essentially mitral and aortic valvular diseases, by its action on the 5HT2b receptors. Until now, it was assumed that the dose and the duration of exposure were the major factors of appearance. We describe a case of aortic insufficiency which developed in a patient given low doses of cabergoline (0.5 mg weekly) for non-tumoral hyperprolactinemia. Because of previous use of appetite suppressants and of bromocriptine, the exclusive responsibility of cabergoline remained uncertain. The potential gravity of these valvular heart diseases emphasizes the importance of careful cardiologic examination before and during treatment.

Vasodilator therapy in patients with aortic insufficiency: a systematic review.

BACKGROUND: The use of vasodilators to improve long-term outcomes in asymptomatic patients with chronic aortic insufficiency (AI) is controversial. METHODS: We reviewed MEDLINE, PREMEDLINE, Current Contents, and Cochrane databases to identify relevant clinical trials on asymptomatic patients with chronic AI of at least moderate severity. We included those studies that involved long-term vasodilator therapy (including hydralazine, calcium-channel blockers, and angiotensin-converting enzyme inhibitors) and assessed either hemodynamic and structural parameters or clinical outcomes. Data on patient demographics, study protocols, and outcomes were abstracted. RESULTS: Ten studies with 544 asymptomatic patients with chronic AI were identified. Treatment duration with vasodilators ranged from 12 weeks to 7 years. Of these, 8 studies compared vasodilators with placebo or no therapy, with 5 demonstrating improvements in at least 1 hemodynamic or structural parameter with vasodilators and 3 showing little or no apparent benefit. The remaining 2 studies directly compared outcomes between 2 different vasodilators. Both of these studies demonstrated greater improvements in hemodynamic and structural parameters with angiotensin-converting enzyme inhibitors compared with hydralazine and nifedipine. Clinical outcomes were primarily reported in only 2 of the 10 studies. Although one study suggested that the use of vasodilators slowed the rate of progression to surgery for aortic valve replacement, another showed no difference. CONCLUSIONS: Vasodilators inconsistently improve hemodynamic and structural parameters in asymptomatic patients with chronic AI. In addition, the impact of vasodilators on clinical outcomes is largely uncertain and requires further study.

[Brucella endocardititis: clinical particularities and therapeutic modalities]. / Endocardite brucellienne: particularités cliniques et modalités thérapeutiques.

Brucella infective endocarditis is an uncommon, but serious complication of brucellosis. The aortic valve is the most commonly affected cardiac valve. Due to characteristics of the infection, medical therapy alone is not sufficient in treating the disease and best results are obtained with surgery combination. We describe a case of Brucella endocarditis involving the aortic valve suspected in front of the clinical data and the results of serology, confirmed by the culture of the native valves. In association with the medical treatment, management valve replacement lead to a favorable medium-term evolution.

Avaliacão clínico-cardiológica e ecocardiográfica, seqüencial, em criancas portadoras da síndrome de Marfan / Serial clinical and echocardiographic evaluation in children with Marfan syndrome

OBJETIVO: Descrever a apresentacão clínica cardiológica e a evolucão temporal, estimar a incidência de ectasia ânulo-aórtica e de prolapso da valva mitral, e avaliar a tolerância e a efetividade dos betabloqueadores em criancas com síndrome de Marfan. MÉTODOS: Foram submetidas a exame clínico e ecocardiográfico seriado, durante um ano, 21 criancas com síndrome de Marfan. No ecocardiograma foram analisados: presenca de prolapso mitral, diâmetro da raiz aórtica, refluxos das valvas mitral e aórtica, e o crescimento dos diâmetros aórticos na vigência de betabloqueadores. Em 11 pacientes foi possível obter duas medidas da raiz aórtica no intervalo de um ano. RESULTADOS: Durante o estudo as criancas não apresentaram sintomas. Prolapso mitral foi encontrado em 11 (52 por cento) criancas. Ectasia ânulo-aórtica ocorreu em 16 (76 por cento) pacientes, sendo de grau discreto em 42,8 por cento, moderado em 9,5 por cento, e importante em 23,8 por cento. Um desses pacientes foi submetido com sucesso à cirurgia de Bentall DeBono. Com o uso de betabloqueador a freqüência cardíaca diminuiu 13,6 por cento (de 85 para 73 bpm; p < 0,009), mas houve um crescimento da raiz aórtica de 1,4 mm/ano (p < 0,02). Uma crianca não pôde receber betabloqueador em razão de asma brônquica, e não foram observados efeitos colaterais significativos nas outras criancas, incluindo uma com asma brônquica. CONCLUSAO: Os resultados obtidos sugerem que, no período observado, as criancas permaneceram assintomáticas, o uso de betabloqueadores diminuiu significativamente a freqüência cardíaca e não se acompanhou de efeitos adversos significativos. Ao contrário da literatura, a incidência de ectasia ânulo-aórtica foi elevada e maior do que a de prolapso valvar mitral, tendo crescimento mesmo na vigência de uso eficaz de betabloqueador.