1/14. Critical subaortic stenosis in a newborn caused by accessory mitral valve tissue.A 2-week-old newborn girl underwent successful surgery in our clinic for critical subaortic stenosis caused by accessory mitral valve tissue, which, because of excessive growth, protruded into the left ventricular outflow tract. The preoperative pressure gradient below the aortic valve was 80 mm Hg. The operation consisted of resection of the accessory tissue through a combined aortotomy and atriotomy approach without residual pressure gradient and mitral valve incompetence. This approach is recommended to ensure that accessory tissue is removed without damaging the mitral valve.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/14. Discrete subaortic membranous stenosis--a case report.Isolated subaortic stenosis is a rare type of cardiac anomaly which has been characterized as having two types: the discrete type, including membranous or fibromuscular, and the tunnel type. In the discrete type, a crescent-shaped, fibrous curtain is attached to the ventricular septum or completely encircles the left ventricular outflow tract and can be located anywhere from immediately below the aortic valve to 10 mm or more into the body of ventricle. A 22-year-old female presented at our hospital with a divided PDA, a murmur that was found by incident and progressive exertional dyspnea. echocardiography revealed left ventricular hypertrophy, moderate aortic insufficiency, and severe aortic stenosis characterized by a thickened aortic valve and membranous type subaortic stenosis with a transmembranous high pressure gradient, 121 mmHg. An operation to replace the aortic valve and excise the membranous collar was performed with cardiopulmonary bypass support. The patient did well during the postoperative follow-up period. If the preoperative LVOT pressure gradient had been higher than 45 mmHg, the incidence of recurrent stenosis, progression of aortic regurgitation and the need for reoperation would have been higher. In order to prevent this from happening, we chose to replace the defective valve with a mechanical valve and enucleate the discrete lesion.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/14. The functional defect in amyloid heart disease. The "stiff heart" syndrome.Left ventricular performance was studied in three patients with heart failure due to amyloid deposits. The diagnosis of amyloidosis was proved by cardiac biopsy in two patients and by rectal biopsy in the third. One patient had myelomatosis, but the other two had no other identifiable disease. The investigative technique allowed simultaneous measurements of pressure and volume in the left ventricle. The functional defect with slow cardiac filling at high pressure and greatly reduced left ventricular contraction differed from that of constrictive pericarditis and other heart muscle disease. These features of a "stiff heart" are probably unique to amyloidosis and should make possible positive recognition of the condition on the basis of echocardiographic, angiographic and hemodynamic findings.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/14. chordae tendineae rupture resulting in pulmonary edema in a patient with discrete subvalvular aortic stenosis--a case report and literature review.This report concerns an apparently healthy elderly woman who presented with gradually worsening mitral regurgitation secondary to chordae tendineae rupture leading to pulmonary edema in the presence of discrete subvalvular aortic stenosis with a severe gradient reflecting the left ventricular outflow tract obstruction. The gradual worsening of heart failure took place parallel to the increase in severity of mitral regurgitation in a short period. The patient underwent successful mitral valve replacement with myectomy. Surgical inspection revealed rupture of the chordae tendineae to the posterior leaflets without any significant primary intrinsic disease of the mitral valve. The predominant mechanism of chordae tendineae rupture in this patient with discrete subvalvular aortic stenosis is a severe pressure gradient. It is suggested that increased awareness of chordae tendineae rupture as a cause of mitral regurgitation and the prompt use of appropriate diagnostic tools may facilitate the timely recognition of this potentially fatal, but treatable, cause of mitral regurgitation in patients with left ventricular outflow tract obstruction.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
5/14. Surgical management of tunnel-like subaortic stenosis via ventricular septal defect in a patient with the interrupted aortic arch.A 24-day-old male with interrupted aortic arch (type B), ventricular septal defect, and tunnel-like subaortic stenosis underwent a one-stage surgical treatment. The operative procedure comprised reconstruction of the aortic arch, transatrial excision of the subaortic fibromuscular tissue via the ventricular sepatal defect, and patch closure of the defect. The patient tolerated the procedure well and the postoperative echocardiography demonstrated a residual pressure gradient across the left ventricular outflow tract of 20 mmHg. Our result suggests that the transatrial surgical management of subaortic stenosis via the ventricular sepatal defect produces a safe and promising surgical option.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
6/14. Aortic valve reconstruction in Rubinstein-Taybi-syndrome: the valuable aid of transesophageal echocardiography.A fourteen year old boy with Rubinstein-Taybi-syndrome presented with a severe congenital subvalvular fibrous aortic stenosis with associated aortic regurgitation. Transthoracic echocardiographic imaging and left heart angiography showed a transvalvular peak systolic pressure gradient of 90 mmHg and a regurgitant fraction of 30%. The surgical treatment consisted of resection of the subvalvular fibrous tissue and subsequent aortic valvuloplasty. Intraoperative transesophageal echocardiography revealed a wide systolic opening of the aortic valve and good coaptation of the aortic valve leaflets in diastole. Two-dimensional color-coded and contrast echocardiography were successfully used to confirm a satisfactory reconstruction. Transesophageal echocardiography therefore represents a valuable tool in the assessment of the morphologic and haemodynamic status, especially in rare cases of congenital heart disease in older children.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
7/14. Uncommon type of mitral insufficiency caused by perivalvular communication between left ventricle and left atrium.This case report describes a patient with an uncommon type of mitral incompetence caused by a perivalvular communication between the left ventricle (LV) and the left atrium (LA) masked by a considerable fibrotic subvalvular aortic stenosis, endocarditis and congestive heart failure (CHF). A 64 year old farmer with a history of a systolic murmur since childhood complaining of increasing fatigue and dyspnoea, temperature over 39 degrees C, and signs of CHF was admitted and transferred to a cardiological unit. Invasive examination and continuing clinical deterioration caused urgent transfer for surgery under suspicion of a decompensated hypertrophic obstructive cardiomyopathy. Clinical investigation revealed a decompensated subvalvular aortic stenosis and a mild mitral insufficiency. At surgery the advanced fibrotic subvalvular stenosis was resected. After coming off bypass severe mitral insufficiency was detected by intraoperative analysis of the simultaneous intracavitary-pressure tracings. A midsystolic maximum of a high V-wave of the LA-pressure tracing was suggestive of an unusual reason of the mitral insufficiency. Reexploration indicated a perivalvular broad communication from the LA groove to the LV with an otherwise normal mitral valve. The communication was closed using buttressed mattress-sutures. This uncommon type of mitral incompetence via a perivalvular LA-LV communication was probably caused by endocarditis and an intramyocardial abscess in the LA-wall which subendocardially led to LV-LA communication.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/14. The Damus-Stansel-Kaye procedure. Should the aortic valve or subaortic valve region be closed?Two patients (one with transposition of the great arteries and another with Taussig-Bing anomaly) underwent the Damus-Stansel-Kaye procedure (Group I). Significant aortic valve insufficiency developed postoperatively in both patients. In contrast, seven patients with a univentricular heart and subaortic stenosis from a variety of reasons underwent creation of an aortopulmonary window (Group II), a procedure very similar to the proximal main pulmonary artery-aortic root anastomosis of the Damus-Stansel-Kaye procedure. aortic valve insufficiency had not developed after up to 7 years of follow-up in this group (average 43 months). Postoperative angiograms suggest that aortic valve incompetence in Group I may have been caused by prolapse of the aortic valve. The valvular structures are subjected to high systolic pressures and face a dilated, low-pressure right ventricle. Aortic root distortion may have contributed, as well. In Group II patients, the aortic valve structures face a small, thick-walled chamber. The orientation of the aortic valve vis-a-vis the right ventricle changed postoperatively in Group I but not in Group II patients. Our experience suggests that the aortic valve or subaortic valve region should be closed at the initial repair in patients with low pulmonary vascular resistance who are undergoing the Damus-Stansel-Kaye procedure, to minimize the need for reoperation for aortic valve insufficiency.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
9/14. Fragmentation hemolysis in idiopathic hypertrophic subaortic stenosis.A 69-year-old woman with previously asymptomatic IHSS developed fragmentation hemolytic anemia in association with bacterial endocarditis when the calculated pressure gradient across the left ventricular outflow tract was 82 mm Hg. Partial correction of the anemia by transfusion resulted in a prompt reduction in fragmentation. The interplay of anemia and increased pressure gradient in the production of red cell fragmentation is discussed.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/14. Pseudo subaortic stenosis--a catheter-induced artifact.Three cases are reported of valvular aortic stenosis in which artifactual subvalvular chambers were recorded on pressure tracings. The pressure tracings were obtained during withdrawal of multiholed catheters from the left ventricle to the aortic root. Several recent reports have described the coexistence of valvular and subvalvular aortic stenosis. The cases reported here re-emphasize that a multiholed catheter, which is the type commonly used for retrograde left ventricular entry in a patient with valvular aortic stenosis, is unsatisfactory for the detection of coexisting subvalvular obstruction.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
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