Discrete subvalvular aortic stenosis is a relatively uncommon cause of the left ventricular outflow obstruction, requiring early intervention. Conventional transthoracic echocardiography may fail in some patients due to insufficient imaging quality. In particular, in patients with a discrete fibrous membrane close to the aortic valve without narrowing of the left ventricular outflow tract, the echocardiographic detection of the membrane may be difficult. Transesophageal echocardiography allows a clear visualization of the aortic valve and the left ventricular outflow tract in virtually all patients, it can be performed rapidly with almost no risk, and it may therfore be helpful in establishing the diagnosis of discrete subaortic stenosis, in particular in patients where the conventional transthoracic approach fails. We have experienced two cases of discrete subaortic stenosis. One case of them was combined with hypertrophic obstructive cardiomyopathy in this report we discussed the utility of multiplane transesophageal echocardiography in patients with discrete subvalvular aortic stenosis.
Aortic Stenosis, Subvalvular* ; Aortic Valve ; Cardiomyopathy, Hypertrophic ; Diagnosis ; Discrete Subaortic Stenosis ; Early Intervention (Education) ; Echocardiography* ; Echocardiography, Transesophageal ; Humans ; Membranes ; Ventricular Outflow Obstruction

During the period of 4 years from May, 1981 to April, 1985, 71 cases of obstructing subinfundibular muscular bundles of the right ventricle in congential cardiovascular anomalies were diagnosed by cariac catheterization and angiography at Hanyang University. The finding of right ventricular angiogram and degree of the right ventricular outflow obstruction, clinical data, electrocardiographic data and hemodynamic data were correlated irrespectively. The summary of this article is as follows. 1) The age of patients was ranged from 1 to 22 years old with a mean age of 8 years old. There were 39 males and 32 females with M:F ratio of 1.2:1. The incidence of obstructing subinfundibular muscular bundles of the right ventricle in congenital cardiovascular anomalies was higher with increasing age. 2) The associated cardic anomalies were as follows: 40 cases (56%) of isolated ventricular septal defect(VSD), 13(18%) of tetralogy of Fallot physiology, 7(10%) of patent ductus arteriosus(PDA), 3(4%) of pulmonary stenosis, 1 aortic stenosis, 1 double outlet of right ventricle(DORV), 1 trilogy, 1 ostium secundum defect, etc. The incidence of VSD with or without other associated cardiovascular anomalies was 56 cases(79%) out of 71 cases. 3) Maximum systolic pressure gradient between proximal and distal chamber of the right ventricle were under 25 mmHg in 32 cases, between 25 and 50 mmHg in 13 and above 50 mmHg in 26. Pressure gradients of all 7 cases with PDA were under 25 mmHg. 4) Correlative assesment of angiographic manifestation(2 indicies:Diameter of right ventricular outflow tract(systolic phase)/diameter of tricuspid valvular annulus(diastolic phase)=OT/TV, Diameter of right ventricular outflow tract(systolic phase)/Length of right ventricular diaphragmatic surface(systolic phase)=OT/RV) according to pressure gradient, OT/Tv and OT/RV values were lower the increasing pressure gradient, between proximal and distal chamber of the right ventricle. These were reverse correlations but coefficients of correlation(r) were-0.49and -0.48. Therefore, the degree of right ventricular outflow obstruction could be predicted, using 2 indices of right ventricular angiogram in individual cases, but could not be calculated accurately. 5) This indicated that pressuer gradient was also affected by technical errors, variable cardic anomalies, development of sinusoid, age and the other factors. 6) We assumed that non-invasive Doppler echocardiography could be useful in making the diagnosis and follow up of the patient with obstructing subinfundibular muscle bundles in right ventricle.
Angiography ; Aortic Valve Stenosis ; Blood Pressure ; Catheterization ; Catheters ; Child ; Diagnosis ; Echocardiography, Doppler ; Electrocardiography ; Equidae ; Female ; Heart Ventricles* ; Hemodynamics ; Humans ; Incidence ; Male ; Physiology ; Pulmonary Valve Stenosis ; Tetralogy of Fallot ; Ventricular Outflow Obstruction ; Young Adult

Discrete subaortic stenos is one of the subtype of congenital left ventricular outflow obstruction and frequently associated with other defect such as ventricular septal defect, coarctation of the aorta, inturrupted aortic arch, double-chambered right ventricle and atrioventricular canal. It is progressive disease from the neonatal period and can be a cause of secondary endocarditis or aortic regurgitation. The authors have experienced a case of discrete subaortic stenosis without other associated lesions in 8 years old male patient who has complained chest discomfort. The diagnosis was established by the findings of echocardiography and cardiac catheterization. We report this case with a brief review of related literatures.
Aorta, Thoracic ; Aortic Coarctation ; Aortic Valve Insufficiency ; Cardiac Catheterization ; Cardiac Catheters ; Child ; Diagnosis ; Discrete Subaortic Stenosis* ; Echocardiography ; Endocarditis ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Humans ; Male ; Thorax ; Ventricular Outflow Obstruction

Supravalvular aortic stenosis is a rare cause of left ventricular outflow obstruction in adults. It occurs as an isolated defect sporadically or as a hereditary basis with an autosomal dominant trait without further phenotypical anomalies, or as a part of the Williams syndrome with mental retardation and multiple anomalies. In this report, we present a case of williams syndome associated with megacoronary artery.
Adult ; Aortic Stenosis, Supravalvular ; Arteries* ; Humans ; Intellectual Disability ; Ventricular Outflow Obstruction ; Williams Syndrome*

An unruptured sinus of Valsalva aneurysm is rare and is usually asymptomatic until a symptom associated with its complication develops. Hence, an unruptured sinus of Valsalva aneurysm is not infrequently missed unless echocardiogram is performed with other indications. An unruptured sinus of Valsalva aneurysm rarely protrudes into the right ventricular outflow tract, causing the right ventricular outflow tract obstruction. In this report, I describe a rare case of unruptured sinus of Valsalva aneurysm producing the right ventricular outflow tract obstruction, which was incidentally detected by echocardiography.
Aneurysm ; Echocardiography ; Sinus of Valsalva ; Ventricular Outflow Obstruction

Many cases have been reported hemolytic anemia and left ventricular outflow obstruction with systolic anterior motion developing after bioprosthetic valve replacement. We report a case of hemolytic anemia and left ventricular outflow tract obstruction occured after mitral valve repair using Duran ring and were resolved by preservative therapy.
Anemia, Hemolytic* ; Mitral Valve* ; Ventricular Outflow Obstruction

BACKGROUND: Supravalvular aortic stenosis is a rare form of congenital cardiac anomaly involving ascending aorta distal to coronary orifice. MATERIALS AND METHODS: We operated 12 cases of supravalvular aortic stenosis between July 1986 and March 1997. Age ranged from 4 to 17 (mean 10.2) years and 11 of them were male. Nine patients had clinical features of Williams syndrome. We experienced two types of supravalvular aortic stenosis, including 10 hour glass type and 2 diffuse type. RESULTS: Preoperative transaortic pressure gradient ranged from 40 to 180 (mean 92) mmHg by cardiac catheterization. Pulmonary stenosis was associated in 5 and 2 of them required angioplasty. Operative techniques included 6 standard aortoplasty with elliptical patch, 4 extended aortoplasty with inverted Y shaped patch, and 2 modified Brom's repair. There were no operative deaths. Postoperative echocardiographic evaluation was done at a mean interval of 12 months. Grade I or II aortic regurgitation was found in 3 cases. Postoperative cardiac catheterization revealed a mean transaortic pressure gradient of 26 (range 0 to 75) mmHg. A mean pressure drop was 78 (range 30 to 114) mmHg. All patients were followed up for a mean of 40 (range 1 to 67) months with uneventful clinical course. CONCLUSIONS: Our data proved the low mortality and excellent hemodynamic improvement after surgical relief of supravalvular aortic stenosis in children.
Angioplasty ; Aorta ; Aortic Stenosis, Supravalvular* ; Aortic Valve Insufficiency ; Aortic Valve Stenosis ; Cardiac Catheterization ; Cardiac Catheters ; Child ; Echocardiography ; Glass ; Hemodynamics ; Humans ; Male ; Mortality ; Pulmonary Valve Stenosis ; Williams Syndrome

In small children with left ventricular outflow tract obstruction, a few methods of surgical treatment could be considrred. The pulmonary autogrart provides a promising options for aortic valve replacement as part of the aortoventriculoplasty procedure in children. We report a successfully treated congenital aortic stcnoinsufricicncy with severe left ventricular dysfunction in an early infant with the aortoventriculoplasty using thc pulmonary autograft (the Ross-Konno procedure).
Aortic Valve ; Autografts* ; Child ; Dronabinol ; Humans ; Infant ; Transplantation, Autologous ; Ventricular Dysfunction, Left ; Ventricular Outflow Obstruction

OBJECTIVETo introduce the experience of diagnosis and surgical treatment of Williams syndrome combined with cardiovascular disease.

METHODSBetween October 1996 and June 2003, 8 patients of Williams syndrome with cardiovascular disease were admitted in Fuwai hospital. Seven patients underwent surgical correction. One didn't undergo surgical procedure. There were 6 male and 2 female ranging from 1.5 to 12.0 years old (medium age 6.4). Three had localized type supravalvular aortic stenosis and 5 diffused type supravalvular aortic stenosis. In them, 2 patients were combined with peripheral pulmonary stenosis. Single patch aortoplasty were performed in 6 cases, and inverted bifurcated patch aortoplasty in one patient.

RESULTSOne patient died and one patient suffered renal insufficiency. In the early postoperative period, the mean speed of flow was reduced to 1.7 m/s from 4.6 m/s, and the mean systolic pressure gradient was reduced from 91 mm Hg to 18 mm Hg. Six patients were followed up 16 to 91 months. There were 5 cases in NYHA function class I, and one in class II.

CONCLUSIONSatisfied result can be achieved in surgical treatment of Williams syndrome with supravalvular aortic stenosis, but it is not in combined with peripheral pulmonary stenosis.

Aortic Stenosis, Supravalvular ; complications ; congenital ; surgery ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Pulmonary Valve Stenosis ; complications ; congenital ; surgery ; Treatment Outcome ; Williams Syndrome ; complications ; surgery

Sinus of Valsalva aneurysm (SVA) is an uncommon anomaly of the aorta. Rupture of SVA often precipitates dramatic clinical complications, including heart failures. Right SVAs are the most common type, and when they rupture, they usually rupture into the right ventricle or right atrium. Rupture into left ventricle or interventricular septum is rare. Herein, we report a case of right SVA rupture with dissection into interventricular septum, which produced significant left ventricular outflow tract obstruction and aortic regurgitation. The case was successfully treated by surgical operation.
Aneurysm ; Aorta ; Aortic Valve Insufficiency ; Heart ; Heart Atria ; Heart Ventricles ; Rupture* ; Sinus of Valsalva* ; Ventricular Outflow Obstruction