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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.
Left ventricular (LV) outflow obstruction may result from retaining the anterior mitral leaflet when a mitral prosthesis is inserted in the mitral anulus. We retrospectively reviewed the echocardiograms (two-dimensional Doppler and Doppler color flow imaging, or transesophageal with color flow imaging) obtained in seven patients with preoperative mitral regurgitation who had a prosthesis implanted with the native mitral leaflets left intact. Systolic anterior motion of the native anterior mitral leaflet, as seen in dynamic LV outflow tract obstruction, was observed in six of seven patients. LV fractional shortening preoperatively was less than or equal to 0.25 in all (mean 0.20 +/- 0.04) and did not significantly (p = ns) increase postoperatively (mean 0.27 +/- 0.12). Color flow imaging revealed disturbed systolic flow in the LV outflow tract in five patients, and all had systolic anterior motion of the native anterior mitral leaflet. Continuous wave Doppler detected significant systolic LV outflow tract jets in five patients averaging 4.1 +/- 0.9 m/sec. Mitral prosthetic function was normal (pressure half-time of 81 +/- 25 msec and mean gradient of 7 +/- 3 mm Hg +/- SD) in five patients. Clinical follow-up revealed that all had died, six of them within 2 months of their operation. Thus systolic anterior motion of the native anterior mitral leaflet occurs commonly after prosthetic mitral valve insertion with the native leaflets left intact. Continuous wave Doppler often demonstrates increased systolic LV outflow tract velocities consistent with dynamic LV outflow obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)
A case of isolated tamponade of the right side of the heart, seen as an abrupt change in the cardiac silhouette and as right ventricular outflow obstruction following myocardial trauma, is presented, along with a review of the English-language literature on delayed postoperative tamponade and loculated pericardial effusion. The importance of a low, fixed cardiac output and nonspecific physical findings in suggesting the clinical diagnosis is emphasized. The absence of many of the classic signs and symptoms of pericardial tamponade is noted, as is the possibility of unusual changes in the appearance or function of the heart in the presence of a loculated effusion. Finally, the differential diagnosis and the results of a variety of diagnostic techniques are discussed.
We report three-dimensional echocardiographic delineation of a congenital aneurysm of the membranous interventricular septum causing right ventricular outflow tract obstruction in an adult patient. To our knowledge, these findings have not been described before.
Aortic root abscess often complicates the course of aortic valve endocarditis. In severe cases, left ventricular-aortic discontinuity may occur, providing challenging technical problems for the surgeon. Moreover, surgical intervention sometimes takes place in a semi-emergency context, and the patches and prosthesis are sutured into friable tissues and subjected to high systemic pressures. Subsequently, paravalvular leaks and prosthesis dehiscence are not uncommon; postoperative false aneurysm of the aortic root is a much more unusual complication. We report one case of right ventricular outflow obstruction that occurred after surgical treatment of an aortic root abscess. Echocardiographic data were useful, but magnetic resonance imaging provided valuable information about the anatomic extent of the cavity. (ECHOCARDIOGRAPHY, Volume 13, January 1996)
The authors report the medico-surgical experience of Marie Lannelongue hospital of a rare condition: accessory mitral valve tissue. Seven patients aged 2 to 28 years (average: 8.7 years) had left ventricular outflow obstruction due to accessory mitral valve tissue. The diagnostic was not obvious clinically and was based on the association of echocardiographic and angiographic data. This condition was associated with another intra-cardiac malformation in 6 of the 7 patients. Surgical treatment included resection of the accessory mitral valve tissue by an aortic or combined aorto-left atrial approach, together with correction of the associated intracardiac abnormality. The postoperative results were excellent with the regression of the ventriculo-aortic pressure gradient and the physiological integrity of the mitral valve.
To assess left ventricular (LV) diastolic filling in children with pressure overload hypertrophy, 12 patients with LV outflow obstruction (7 with aortic valve stenosis and 5 with aortic coarctation) and 12 healthy, age-matched control subjects were examined. Each child underwent M-mode echocardiography and pulsed Doppler examination of the LV inflow. The patients with LV outflow obstruction had cardiac catheterization and balloon angioplasty. Their echo/Doppler examinations were performed in the catheterization laboratory before and immediately after balloon angioplasty. From the M-mode echocardiogram, the LV cavity dimensions and wall thicknesses, LV mass and shortening fraction were measured. The following measurements were made from the Doppler recording: peak velocities at rapid ventricular filling (peak E) and during atrial contraction (peak A), ratio of peak E to peak A velocities, total area under the Doppler curve, percent of the total Doppler area occurring in the first one-third of diastole (0.33 area fraction), percent of the total area occurring under the E wave (E area fraction), percent of the total area occurring under the A wave (A area fraction) and the ratio of E area to A area.(ABSTRACT TRUNCATED AT 250 WORDS)
The Authors describe a patient with hypertrophic cardiomyopathy (HCM), severe left ventricular outflow tract obstruction and lack of response to beta-blockers or verapamil. Intravenous infusion of disopyramide resulted in a virtual disappearance of the LV pressure gradient, reduction of the systolic anterior motion of the mitral valve and slowing of the LV isovolumetric relaxation. One month after maintenance therapy with oral disopyramide a decrease of the anginal episodes and an improvement of the exercise tolerance were noted. Thus disopyramide, probably because of its negative inotropic action, is useful in the management of patients with HCM when LV outflow obstruction is the main cause of the clinical-hemodynamic findings.
A new technique is described for relief of diffuse obstruction in the left ventricular outflow tract without aortic valve replacement. Left ventricular septoplasty was performed, preserving the aortic valve. The supra-valve stenosis was repaired using a Y-shaped extension of the aortotomy proximally and a pantaloon-shaped patch of woven Dacron fabric. A 10-year-old girl with "tunnel" or diffuse stenosis obtained striking relief of left ventricular hypertension by this technique.
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A case of left ventricular outflow tract stenosis resulted from compression of the heart is presented. This symptomatic cardiovascular lesion was produced by extensive growth of mediastinal tumour. Numerous noninvasive methods were necessary to establish this difficult diagnosis. The patient underwent surgery that disclosed a benign process (of neurofibroma). After tumour's resection marked hemodynamic signs subsided.
Two neonates had unusually rapid development of necrotizing enterocolitis within 24 hours of birth. Both patients had decreased systemic perfusion secondary to aortic atresia. Onset of either clinical or radiographic manifestations of necrotizing enterocolitis in the first day of life should alert one to the possible presence of severe left ventricular outflow obstruction.
OBJECTIVES: The aim of this study was to investigate whether the prognostic importance of left ventricular (LV) outflow obstruction in hypertrophic cardiomyopathy (HCM) is influenced by other predictors of morbidity and mortality. BACKGROUND: It remains unknown whether the effect of outflow obstruction on clinical outcome in HCM is influenced by other determinants of survival. METHODS: We assessed the impact of severity of symptoms, LV wall thickness, atrial fibrillation, and age on the prognostic importance of outflow obstruction in a large HCM population. RESULTS: Of 526 consecutive HCM patients, 141 (27%) had outflow obstruction. During a follow-up of 4.5 +/- 4.1 years, 34 patients died of HCM. The incidence of cardiovascular death was significantly higher among patients with LV obstruction than among those without obstruction (relative risk [RR] = 2.14; p = 0.02). The prognostic power of the outflow gradient changed in relation to severity of symptoms (p = 0.024). At initial evaluation, LV obstruction was a significant predictor of cardiovascular mortality only in New York Heart Association (NYHA) functional class I to II patients (hazard ratio [HR] = 2.38; p = 0.025). During follow-up, at time of development of severe symptoms, the outflow gradient lost its prognostic significance (HR = 1.18; p = 0.66), whereas NYHA functional class III to IV was associated with an eight-fold increase in risk of cardiovascular mortality (HR = 7.90; p < 0.001). CONCLUSIONS: In patients with HCM, the prognostic importance of LV outflow obstruction varies in relation to the severity of symptoms. In patients with mild or no symptoms, obstruction is an important predictor of cardiovascular death. After development of severe symptoms, NYHA functional class becomes the dominant marker of prognosis independently of the presence of an outflow gradient.
We report a 1-year-old girl who developed ventricular outflow tract obstruction early after a Fontan operation, necessitating surgical relief using the Damus-Kaye-Stansel procedure. The patient had a complete atrioventricular septal defect complicated by a muscular ventricular septal defect (VSD) and a small left ventricle, a morphology that has not previously been reported in cases of systemic outflow tract obstruction after the Fontan procedure. Postoperative systemic outflow obstruction must be considered as a possible sequela following Fontan surgery in patients with an atrioventricular septal defect and a small left ventricle.
Accessory mitral valve tissue, a rare cause of left ventricular outflow tract obstruction, can be difficult to diagnose preoperatively and confusing even at surgery. The reported case illustrates how preoperative evaluation can be made using two-dimensional echocardiography combined with Doppler flow analysis. The intraoperative technique for removing the accessory tissue without causing damage to the native mitral valve is discussed.
We interposed valved conduits between the left ventricular apex and aorta in 38 patients over a 10-year period. Indications included tunnel subaortic stenosis, aortic annular hypoplasia, tubular supravalvular aortic hypoplasia, and severe calcification of the ascending aorta. Operative mortality was 11%, but 78% of the survivors were alive at 5 years, and 70% had had no major complication. The results were better in adolescents and adults than in young children. Although complications included calcific degeneration of the valve and disruption of the conduit at the site of insertion into the ventricular apex, we continue to believe in the utility of this procedure in a few patients with complex left ventricular outflow tract obstruction.
This report described 56-year-old male with cardiac amyloidosis, whose echocardiographic and hemodynamic findings suggested hypertrophic obstructive cardiomyopathy. M-mode echocardiography demonstrated asymmetric septal hypertrophy (2.1 cm/1.6 cm), systolic anterior motion of the mitral valve, and pericardial effusion. Two-dimensional echocardiography revealed marked hypertrophy of the septum and papillary muscles with characteristic fine grannular sparkling appearance of the myocardium. Bilateral heart catheterization showed normal hemodynamics except a pressure gradient of 35 mmHg within the left ventricular outflow tract. Moreover, no hemodynamic evidence of restrictive cardiomyopathy was observed. Myocardial biopsy was performed at the time of pericardiectomy, and diffuse amyloid deposits were identified between myocardial fibers. It is of particular interest that cardiac amyloidosis presented the form of ventricular hypertrophy associated with left ventricular outflow obstruction, probably due to uneven deposition of amyloid.
The recommended operative management of unruptured sinus of Valsalva aneurysm consists of closure of the mouth of the aneurysm with or without aortic valve surgery. We report a case of unruptured aneurysm producing right ventricular outflow tract obstruction. Closure of the mouth of the aneurysm failed to relieve the obstruction, which was subsequently achieved by excising the aneurysmal wall overlying the outflow tract.