Rapid progression of primary cardiac leiomyosarcoma with obstruction of the left ventricular outflow tract and mitral stenosis.
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We report a 73-year-old woman with primary cardiac leiomyosarcoma in the left atrium and ventricle. The tumor progressed very rapidly for 2 months after initial clinical evaluation. Obstruction of the left ventricular outflow tract and mitral stenosis were induced by the tumor. Urgent surgical resection was performed because she had cardiogenic shock due to paroxysmal atrial fibrillation. We could not resect the tumor completely because of severe invasion. She refused postoperative chemotherapy and radiotherapy, and died suddenly at home 89 days after surgery. To our knowledge, this is the first observation of mitral stenosis concomitant with obstruction of the left ventricular outflow tract in a patient with primary cardiac leiomyosarcoma. (+info)
Echocardiographic assessment of right ventricular obstruction in hypertrophic cardiomyopathy.
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Echocardiography was used to evaluate the incidence, flow dynamics and morphological characteristics of right ventricular obstruction in 91 patients with hypertrophic cardiomyopathy. Color flow mapping was used to define the sites of obstruction in the left and right ventricles. Ventricular obstruction was considered to be present if the flow velocity was less than 2.0 m/s as measured by continuous wave Doppler. The thickness of both the right ventricular free wall and anterior ventricular septum was measured to assess the magnitude and extent of hypertrophy. Right ventricular obstruction was present in 14 patients of whom 6 (43%) had left ventricular obstruction also. The right ventricular obstructions were found in the outflow tract (9 patients), mid-base septal bulge (2 patients) and apical trabecular region (3 patients). Doppler waveform was confined to systole in all patients with obstruction in the outflow tract and in one of the patients with mid-base septal bulge. Moreover, the flow wave persisted into early diastole in 4 patients, including 2 with apical trabecular obstruction. The thickness of both the right ventricular free wall and anterior ventricular septum suggested that these hypertrophied regions were the sites of right ventricular obstruction. Thus, echocardiography was useful in evaluating right ventricular obstruction in hypertrophic cardiomyopathy. (+info)
Discrete subaortic stenosis: surgical outcomes and follow-up results.
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Discrete subaortic stenosis, which is an obstructing lesion of the left ventricular outflow tract, remains a surgical challenge. The recurrence rate is high despite sufficient conventional resection. We retrospectively reviewed the results of surgery for discrete subaortic stenosis at our institution from September 1995 through March 2001. Twenty-one patients with this lesion underwent surgical treatment during this period. Excision of the fibromuscular membrane with myectomy was performed in all of the patients. Follow-up in all patients ranged from 7 to 67 months (mean follow-up period, 39.57 +/- 15.46 months). The mean systolic gradient between the left ventricle and the aorta decreased from 59.23 +/- 35.38 mmHg preoperatively to 9.47 +/- 9.91 mmHg postoperatively. There was no instance of heart block that required a permanent pacemaker, nor of bacterial endocarditis. There was no early or late postoperative death. A 22nd patient, who had 3+ aortic regurgitation, required aortic valve replacement and was excluded from the study. Two of the patients (9.5%) underwent reoperation because of recurrent gradient and residual ventricular septal defect. Our results suggest that fibromuscular membrane excision combined with myectomy in patients with discrete subaortic stenosis produces sufficient relief of obstruction with low morbidity. (+info)
Predictors of outcome after alcohol septal ablation therapy in patients with hypertrophic obstructive cardiomyopathy.
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BACKGROUND: Alcohol septal ablation (ASA) therapy results in clinical and hemodynamic improvement in patients with hypertrophic obstructive cardiomyopathy. However, a subset remains symptomatic afterward, requiring additional procedures. We sought to examine the determinants of an unsatisfactory outcome, defined as unchanged symptoms with <50% reduction of baseline left ventricular outflow tract (LVOT) gradient. METHODS AND RESULTS: Of 173 consecutive hypertrophic obstructive cardiomyopathy patients who underwent ASA, 39 had an unsatisfactory outcome after the first procedure. Patients with an unsatisfactory outcome had a higher baseline LVOT gradient, fewer septal arteries injected with ethanol, lower peak creatine kinase (CK), smaller septal area opacified by contrast echocardiography, and higher residual gradient in the catheterization laboratory after ASA (all P<0.05). Symptoms, septal thickness, mitral regurgitation severity, and ventricular function were not determinants of outcome. On multiple logistic regression, LVOT gradient reduction after ASA in the catheterization laboratory to > or =25 mm Hg (OR, 5.5; P=0.01) and peak CK <1300 U/L (OR, 2.5; P=0.04) were the independent predictors of an unsatisfactory outcome. CONCLUSIONS: The residual LVOT gradient in the catheterization laboratory and peak CK leak after ASA are the independent predictors of ASA outcome. (+info)
Aortic regurgitation secondary to back-and-forth intimal flap movement of acute type A dissection.
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We present an unusual case of acute type A dissection complicated with severe aortic valve insufficiency caused by prolapse of the tubular intimal flap into the left ventricular outflow tract, which was shown legibly by transesophageal echocardiography in the diastolic phase and by intraoperative macroscopic findings. The dissected ascending aorta was excised completely and replaced without any repairing of the aortic valve, resulting in a favorable outcome for the patient. Prolapse of an intimal flap from the aorta into the left ventricle represented a rare pathophysiology of aortic regurgitation in patients with aortic dissection. (+info)
Characterization of left ventricular diastolic function by tissue Doppler imaging and clinical status in children with hypertrophic cardiomyopathy.
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BACKGROUND: Conventional transmitral Doppler indices are unreliable in assessing clinical status in patients with hypertrophic cardiomyopathy (HCM) because they are affected by loading conditions. This study sought to determine whether tissue Doppler velocities are predictive of adverse clinical outcomes including death, cardiac arrest, ventricular tachycardia (VT), significant cardiac symptoms, and exercise capacity in children with HCM. METHODS AND RESULTS: We studied 80 consecutive children (median age 12 years, median follow-up 26 months) evaluated at 1 hospital from January 1999 to August 2003 compared with 80 age- and gender-matched controls. Patients underwent echocardiography, ambulatory Holter monitoring, and upright exercise testing. Children with HCM had significantly decreased early diastolic tissue Doppler velocities at the lateral mitral (13.2 versus 19.3 cm/s), tricuspid (13.3 versus 16.3 cm/s), and septal (9.4 versus 13.5 cm/s) annuli compared with controls (P<0.001 for each comparison). By forward stepwise regression analysis, early transmitral left ventricular filling velocity (E)/septal Ea ratio predicted death, cardiac arrest, or VT (r=0.610, R2=0.37, P<0.001). Peak oxygen consumption (VO2) was most predictive of children who developed symptoms (r=0.427, R2=0.182, P<0.001). Peak VO2 correlated inversely with E/Ea septal ratio (r=-0.740, P<0.01). CONCLUSIONS: Transmitral E/septal Ea ratio predicts children with HCM who are at risk of adverse clinical outcomes including death, cardiac arrest, VT, and significant cardiac symptoms. Peak VO2 correlated with peak exercise capacity in HCM patients. (+info)
Percutaneous pulmonary valve replacement in a large right ventricular outflow tract: an experimental study.
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OBJECTIVES: We report our initial experience with percutaneous pulmonary valve replacement in animals with large pulmonary trunks, using a modified percutaneous approach. BACKGROUND: Percutaneous pulmonary valve replacement has recently been introduced, and early clinical experience has been reported. This technique is presently limited to patients with a right ventricular outflow tract no bigger than 22 mm in diameter. METHODS: In seven animals (groups 1 and 3), we implanted a newly designed nitinol stent in the shape of a conduit with a central restriction of its diameter, containing an 18-mm bovine valve, as a one-step procedure. The animals in groups 1 and 3 were sacrificed after valve implantation and after two-month follow-up, respectively. In the second group (n = 3), we expected to percutaneously reduce the diameter of the pulmonary artery. Eight weeks later, we implanted an 18-mm valve mounted in a balloon-expandable stent. These animals were sacrificed after valve implantation. RESULTS: Eight of 10 devices were successfully delivered and were functioning perfectly at the initial evaluation and after two months. We failed to cross the tricuspid valve in two cases. The downsize mechanism allowed the pulmonary diameter to be reduced from 30 to 18 mm, without an impact on right ventricular function in any of the animals. CONCLUSIONS: Non-surgical implantation of a pulmonary valve is possible in ewes with all types of pulmonary trunk, regardless of its size. A "downsize" stent is needed to allow valve implantation in a large trunk. Further refinements will make this technique feasible in humans. (+info)
A rare case of left ventricular outflow obstruction.
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We report a patient with a mass originating from the anterior mitral valve leaflet causing severe left ventricular outflow tract obstruction. Noninvasive imaging provided the best diagnostic tools for diagnosis. Histological findings showed a very rare giant blood cyst of the mitral valve. (+info)