Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle.
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OBJECTIVES: Increased afterload and multilevel LV obstruction is constant. We assumed that restoration of normal loading conditions by relief of LV obstructions promotes its growth, provided that part of the cardiac output was preoperatively supported by the LV, whatever the echocardiographic indexes. BACKGROUND: Whether to perform uni- or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal LV (hypoplastic left heart syndrome classes II & III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. METHODS: Twenty ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multilevel LV obstruction. Preoperative echocardiographic assessment showed: mean EDLW of 12.4 +/- 3.03 ml/m2 and mean Rhodes score of -1.73 +/-0.8. Surgery consisted in relief of LV outflow tract obstruction by coarctation repair in all associated to aortic commissurotomy in one and ASD closure in 2. RESULTS: There were 3 early and 2 late deaths. Failure of biventricular repair and LV growth was obvious in patients with severe anatomic mitral stenosis. The other demonstrated growth of the left heart. At hospital discharge the EDLVV was 19.4+/-3.12 ml/m2 (p = 0.0001) and the Rhodes score was -0.38+/-1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were 72.5% and 46%, respectively. CONCLUSIONS: Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal LV provided that all anatomical causes of LV obstruction can be relieved. Secondary growth of the left heart then occurs; however, the reoperation rate is high. (+info)
Cell death in acromegalic cardiomyopathy.
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BACKGROUND: Prolonged untreated acromegaly leads to a nonspecific myopathy characterized by ventricular dysfunction and failure. However, the mechanisms responsible for the alterations of cardiac pump function remain to be defined. Because cell death is implicated in most cardiac disease processes, the possibility has been raised that myocyte apoptosis may occur in the acromegalic heart, contributing to the deterioration of ventricular hemodynamics. METHODS AND RESULTS: Ten acromegalic patients with diastolic dysfunction and 4 also with systolic dysfunction were subjected to electrocardiography, Holter monitoring, 2-dimensional echocardiography, cardiac catheterization, and biventricular and coronary angiography before surgical removal of a growth hormone-secreting pituitary adenoma. Endomyocardial biopsies were obtained and analyzed quantitatively in terms of tissue scarring and myocyte and nonmyocyte apoptosis. Myocardial samples from papillary muscles of patients who underwent valve replacement for mitral stenosis were used for comparison. The presence of apoptosis in myocytes and interstitial cells was determined by confocal microscopy with the use of 2 histochemical methods, consisting of terminal deoxynucleotidyl transferase (TdT) assay and Taq probe in situ ligation. Acromegaly was characterized by a 495-fold and 305-fold increase in apoptosis of myocytes and nonmyocytes, respectively. The magnitude of myocyte apoptosis correlated with the extent of impairment in ejection fraction and the duration of the disease. A similar correlation was found with the magnitude of collagen accumulation, indicative of previous myocyte necrosis. Myocyte death was independent from the hormonal levels of growth hormone and insulin-like growth factor-1. Apoptosis of interstitial cells did not correlate with ejection fraction. CONCLUSIONS: Myocyte cell death, apoptotic and necrotic in nature, may be critical for the development of ventricular dysfunction and its progression to cardiac failure with acromegaly. (+info)
Long-term clinical and echocardiographic follow-up after percutaneous mitral valvuloplasty with the Inoue balloon.
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BACKGROUND: The objective of this study was to assess the long-term clinical outcome and valvular changes (area and regurgitation) after percutaneous mitral valvuloplasty (PMV). METHODS AND RESULTS: After PMV, 561 patients were followed up for 39 (+/-23) months and clinical/echocardiographic data obtained yearly. Kaplan-Meier and Cox regression analyses were performed to estimate event-free survival, its predictors, and the relative risks of several patient subgroups. There were several nonexclusive events: 19 (3.3%) cardiac deaths, 55 (9.8%) mitral replacements, 6 (1%) repeated PMVs, 56 (10%) cases of restenosis, and 108 (19%) cases of clinical impairment. Survival free of major events (cardiac death, mitral surgery, repeat PMV, or functional impairment) was 69% at 7 years, ranging from 88% to 40% in different subgroups of patients. Wilkins score was the best preprocedural predictor of mitral opening, but the procedural result (mitral area and regurgitation) was the only independent predictor of major event-free survival. Mitral area loss, though mild [0.13 (+/-0.21)cm2], increased with time and was >/=0.3 cm2 in 12%, 22%, and 27% of patients at 3, 5, and 7 years, respectively. Regurgitation did not progress in 81% of patients, and when it occurred it was usually by 1 grade. CONCLUSIONS: Seven years after PMV, more than two thirds of patients were in good clinical condition and free of any major event. The procedural result was the main determinant of long-term outcome, although a high score had also negative implications. Mitral area decreased progressively over time, whereas regurgitation did not tend to progress. (+info)
Combined aortic and mitral stenosis in mucopolysaccharidosis type I-S (Ullrich-Scheie syndrome).
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The genetic mucopolysaccharidosis syndromes (MPS) are autosomal recessive inborn errors of metabolism. Heart valve involvement in MPS is not uncommon but only a few case reports of successful cardiac surgery are available. In particular, reports of combined aortic and mitral stenosis associated with MPS type I-S are very rare. Both type I and type VI MPS are associated with significant left sided valvar heart disease that requires surgical valve replacement because of irregular valve thickening, fibrosis, and calcification. A 35 year old man had severe mitral valve stenosis after successful surgical replacement of a stenotic aortic valve. Valvar heart disease was investigated by cardiac ultrasound and left heart catheterisation. Histomorphological characterisation of the affected mitral valve was performed. The case illustrates typically associated clinical features of cardiac and extracardiac abnormalities found in MPS type I-S. (+info)
Predictors of clinical events or restenosis during follow-up after percutaneous mitral balloon valvotomy.
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AIMS: The purpose of this study is to define predictors of events or restenosis during follow-up after percutaneous mitral balloon valvotomy. METHODS AND RESULTS: Percutaneous mitral balloon valvotomy was attempted in 137 patients with severe mitral valve stenosis. In 127 patients follow-up was complete with a mean of 4.2 +/- 2.6 years. Events during follow-up were defined as death, mitral valve surgery or repeat percutaneous mitral balloon valvotomy. Restenosis was defined as a decrease in mitral valve area from > or = 1.5 cm2 following percutaneous mitral balloon valvotomy to < 1.5 cm2. There was 80 +/- 4% event-free survival 4 years after percutaneous mitral balloon valvotomy. Multivariate analysis showed chronic atrial fibrillation at baseline (P = 0.039, relative risk (RR) = 2.5) and a high residual maximal gradient after percutaneous mitral balloon valvotomy (P = 0.004, RR = 2.0 per 5 mmHg) to be independent predictors of an event during follow-up. The restenosis rate was 28.3% after 4 years. Chronic atrial fibrillation at baseline (P = 0.0338, RR = 2.2), a small mitral valve area after percutaneous mitral balloon valvotomy (P = 0.0003, RR = 0.8/0.1 cm2) and a high residual maximal transmitral gradient (P = 0.0252, RR = 1.6/5 mmHg) were all independent predictors of restenosis. CONCLUSION: Patients with chronic atrial fibrillation and a high maximal transmitral gradient after percutaneous mitral balloon valvotomy have a higher risk for events during follow-up. Restenosis is related to the presence of chronic atrial fibrillation at baseline and a suboptimal percutaneous mitral balloon valvotomy result. (+info)
Late results of percutaneous mitral commissurotomy in a series of 1024 patients. Analysis of late clinical deterioration: frequency, anatomic findings, and predictive factors.
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BACKGROUND: The optimal use of percutaneous mitral commissurotomy (PMC) in a wide range of patients requires accurate evaluation of late results and identification of their predictors. METHODS AND RESULTS: Late results of PMC were assessed in 1024 patients whose mean age was 49+/-14 years. Echocardiography showed that 141 patients (14%) had pliable valves and mild subvalvular disease, 569 (55%) had extensive subvalvular disease, and 314 (31%) had calcified valves. A single balloon was used in 26 patients, a double balloon in 390, and the Inoue Balloon in 608. Good immediate results were defined as valve area >/=1.5 cm2 without regurgitation >2/4 (Sellers' grade) and were obtained in 912 patients. Median duration of follow-up was 49 months. The 10-year actuarial rate of good functional results (survival with no cardiovascular death and no need for surgery or repeat dilatation and in New York Heart Association [NYHA] class I or II) was 56+/-4% in the entire population. Follow-up echocardiography was available in 90% of the patients who experienced poor functional results after good immediate results and showed restenosis in 97% of these. In multivariate analysis, the predictors of poor functional results were old age (P=0.0008), unfavorable valve anatomy (P=0.003), high NYHA class (P<0.0001), atrial fibrillation (P<0.0001), low valve area after PMC (P=0.001), high gradient after PMC (P<0.0001), and grade 2 mitral regurgitation after PMC (P=0.04). CONCLUSIONS: PMC can be performed with good late results in a variety of patient subsets. Prediction of late events is multifactorial. Knowledge of these predictors can improve patient selection and follow-up. (+info)
Is redo percutaneous mitral balloon valvuloplasty (PMV) indicated in patients with post-PMV mitral restenosis?
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OBJECTIVES: The purpose of this study was to assess the immediate and long-term outcome of repeat percutaneous mitral balloon valvuloplasty (PMV) for post-PMV mitral restenosis. BACKGROUND: Symptomatic mitral restenosis develop in 7% to 21% of patients after PMV. Currently, most of these patients are referred for mitral valve replacement. However, it is unknown if these patients may benefit from repeat PMV. METHODS: We report the immediate outcome and long-term clinical follow-up results of 36 patients (mean age 58+/-13 years, 75% women) with symptomatic mitral restenosis after prior PMV, who were treated with a repeat PMV at 34.6+/-28 months after the initial PMV. The mean follow-up period was 30+/-33 months with a maximal follow-up of 10 years. RESULTS: An immediate procedural success was obtained in 75% patients. The overall survival rate was 74%, 72% and 71% at one, two, and three years respectively. The event-free survival rate was 61%, 54% and 47% at one, two, and three years respectively. In the presence of comorbid diseases (cardiac and noncardiac) the two-year event-free survival was reduced to 29% as compared with 86% in patients without comorbid diseases. Cox regression analysis identified the echocardiographic score (p = 0.03), post-PMV mitral valve area (p = 0.003), post-PMV mitral regurgitation grade (p = 0.02) and post-PMV pulmonary artery pressure (p = 0.0001) as independent predictors of event-free survival after repeat PMV. CONCLUSIONS: Repeat PMV for post-PMV mitral restenosis results in good immediate and long-term outcome in patients with low echocardiographic scores and absence of comorbid diseases. Although the results are less favorable in patients with suboptimal characteristics, repeat PMV has a palliative role if the patients are not surgical candidates. (+info)
Long-term clinical and echocardiographic outcome in patients with mitral stenosis treated with percutaneous transvenous mitral commissurotomy.
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Long-term follow-up after percutaneous transvenous mitral commissurotomy (PTMC) is limited. Ninety-four middle-aged (51+/-9 years) mitral stenosis patients who underwent successful PTMC were followed up with annual echocardiography for 6.1+/-1.4 years. PTMC success was defined as either mitral valve area (MVA) >1.5 cm2 or a MVA of more than twice the pre-procedural value, together with no worsening of mitral regurgitation >grade 2+. Mitral valve replacement (MVR), worsening of congestive heart failure (CHF), and thromboembolism were sought for survival analysis. Restenosis was defined as loss of more than 50% of the initial procedural MVA gain. Functional limit of daily activities was assessed through a questionnaire. The study population was divided into group 1 (post-procedural MVA >2.0 cm2), group 2 (MVA > 1.5 cm2 and < or = 2.0 cm2) and group 3 (MVA < or = 1.5 cm2). The 6-year survival with freedom from MVR, CHF, thromboembolism, and combined events (MVR+CHF) was 92%, 95%, 91%, and 88%, respectively. No group 1 patient experienced MVR or CHF. Restenosis was predominant in group 3. Deterioration of daily activities during follow-up was not observed in group 1; however, it was significant in group 2 (p<0.05) and group 3 (p<0.001). These results demonstrated that patients who attained a large MVA (>2.0cm2) immediately after PTMC maintained their procedural benefit with less clinical complication and with less limitation of daily activity. (+info)