Nonobstructing accessory mitral valve tissue and ventricular septal defect

A 4-month-old boy with ventricular septal defect was found to have accessory mitral valve tissue attached to the anterior leaflet of the mitral valve. Operation was successfully performed to excise the accessory mitral tissue in the left ventricular outflow tract and close the ventricular septal defect. Most previously reported cases with accessory mitral valve tissue were associated with left ventricular outflow tract obstruction. This boy had no pressure gradient across the left ventricular outflow tract. The indications for prophylactic excision of nonobstructing accessory mitral valve tissue in a patient with other forms of congenital cardiac disease are discussed.     

Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction due to anomalous papillary muscle

BACKGROUND: Ventricular septal myotomy/myectomy (Morrow procedure) is the standard surgical option for severely symptomatic patients with hypertrophic cardiomyopathy (HCM) and marked basal obstruction to left ventricular outflow due to mitral valve systolic anterior motion. In some patients, however, congenital malformations of the mitral apparatus may be responsible for outflow obstruction; the failure to recognize this morphology before operation could have adverse consequences. METHODS AND RESULTS: We recently evaluated 2 patients with obstructive HCM operated on at Mayo Medical Center in 1997 who demonstrated direct anomalous papillary muscle insertion into the anterior mitral leaflet, producing muscular midcavity obstruction. This anomaly is potentially identifiable with echocardiography by exaggerated anterior displacement of hypertrophied papillary muscles within the left ventricular cavity and the direct continuity between papillary muscle and anterior leaflet associated with a rigid motion pattern of the mitral apparatus. Echocardiographic diagnosis, however, was confused in both patients by the association of systolic anterior motion of the mitral valve, probably produced by freely mobile margins of the mitral leaflet unencumbered by papillary muscle insertion, and in 1 patient probably representing a second and more basal level of obstruction. Because outflow tract morphology was judged unsuitable for conventional myotomy/myectomy, a novel surgical strategy was designed to remove the outflow gradient in which an extensive myectomy trough (wider at its apical than basal extent) was created within the ventricular septum to papillary muscle level; also, in 1 patient, attachment of anterolateral papillary muscle with the lateral free wall was partially severed to increase mobility of the mitral apparatus. After surgery, both patients reported substantial relief of symptoms and improved exercise tolerance and also showed reduced or abolished basal outflow obstruction. CONCLUSIONS: In HCM, outflow obstruction due to anomalous papillary muscle insertion directly into anterior mitral leaflet is challenging to identify but should always be contemplated before operative intervention. This important (but often unsuspected) congenital malformation may require alternative surgical strategies to standard myotomy/myectomy, similar to those described here.     

The cumulative risks of prolapsing mitral valve. 40 years of follow-up

Prolapsing mitral valve is a common cardiac condition, occurring in approximately 16 million people in the United States alone. Primary prolapsing mitral valve may be familial or nonfamilial and may be associated with myxomatous degeneration of the mitral valve leaflets, such as occurs in Marfan syndrome and other connective tissue disorders. Secondary forms may be associated with such entities as rheumatic fever (especially after commissurotomy) and coronary artery disease (in the presence of ruptured chordae tendineae), and with such congenital conditions as interatrial defect and primary cardiomyopathy with outflow tract obstruction. Prolapsing mitral valve is characterized by late systolic murmur, mid-systolic click, or both. Arrhythmias occur in the form of benign premature atrial contraction, premature nodal contraction, and paroxysmal atrial tachycardia. As the patient ages, atrial flutter and atrial fibrillation tend to develop. In some chronic cases, especially those involving atrial fibrillation, systemic emboli may occur. Rare premature ventricular contractions may be largely benign, whereas more frequent premature ventricular contractions may lead to severe arrhythmic complexes such as ventricular tachycardia or ventricular fibrillation. With advancing age, atrioventricular conduction defects of varying degrees or sick sinus syndrome may necessitate a pacemaker installation. About one quarter of prolapsing mitral valve cases progress, with increasing mitral insufficiency and increasing enlargement of the left atrium and left ventricle, which at times leads to congestive heart failure. Coronary artery disease may occur with the severity commensurate with the patient's age group. About three quarters of patients with prolapsing mitral valve syndrome lead normal lives.     

Hypertrophic obstructive cardiomyopathy in pediatric patients: results of surgical treatment

Between April 1975 and May 1995, 25 pediatric patients on one hospital service underwent extended left ventricular septal myectomy because of hypertrophic obstructive cardiomyopathy. Ages ranged from 2 months to 20 years (mean, 11.2 years). Seventeen patients had moderate to severe mitral valve insufficiency. Medical therapy had failed in all patients and one patient had undergone dual-chamber pacemaker implantation without improvement. Left ventricular outflow tract gradients ranged from 50 to 154 mm Hg (mean, 99.9 +/- 25.2). Concomitant cardiac procedures included mitral valve repair (n = 2), automatic implantable cardioverter defibrillator implantation (n = 1), and closure of atrial septal defect (n = 1). Intraoperative premyectomy left ventricular outflow tract gradients ranged from 20 to 117 mm Hg (mean, 60.4 + 26.2) and postmyectomy gradients ranged from 0 to 20 mm Hg (mean, 6.6 +/- 5.9). Postmyectomy mitral insufficiency was reduced to a regurgitant fraction of 0% to 12%, and no patient required mitral valve replacement. One patient required a pacemaker because of complete heart block; on subsequent follow-up, normal sinus rhythm had returned. There was no early mortality and no instance of aortic or mitral valve injury or ventricular septal defect. Follow-up ranged from 10 months to 20 years (mean, 6.4 years). There were no late deaths. Left ventricular outflow tract gradients by echocardiography were a mean of 14.2 mm Hg with a median of 5.0 mm Hg. All patients had normal sinus rhythm. Reoperation because of recurrent left ventricular outflow tract obstruction was necessary in two patients at 3.2 years and 12.4 years after initial myectomy, respectively. All patients but one have New York Heart Association class I or II function. We conclude that extended septal myectomy is a safe and effective means of relieving cardiac symptoms and left ventricular outflow tract obstruction in pediatric patients with severe hypertrophic obstructive cardiomyopathy unresponsive to medical management, and late survivorship compares favorably with the natural history of the disease.     

Aortic outflow obstruction in visceral heterotaxy: a study based on twenty postmortem cases

Aortic outflow tract obstruction can complicate the clinical course and surgical management of patients with heterotaxy syndromes, but its anatomic basis has not been described in detail. In 20 postmortem cases with asplenia (n = 4) or polysplenia (n = 16), the anatomic causes of aortic outflow tract obstruction were absence of the subaortic conus in association with (1) narrowing of the subaortic outflow tract between the conal septum anteriorly and the common atrioventricular (AV) valve posteriorly in six (30%) patients; (2) aortic valvar atresia in four (25%), three with asplenia and one with polysplenia; (3) redundant AV valve leaflets in four (20%); (4) excessive AV valve fibrous tissue in four (20%); (5) marked hypoplasia of the mitral valve and left ventricle in two (10%); and (6) aneurysm of membranous septum in one (5%). One patient belonged to group (1) and (4). Aortic outflow tract obstruction was much more common with polysplenia (28%) than with asplenia (4%) (p < 0.001).     

Pulmonary arterial compression syndrome caused by false aneurysm of left ventricular outflow tract

A 29 year old man experienced exertional dyspnea and coughing 3 1/2 years after insertion of a Brauwald-Cutter aortic valve prosthesis. Clinical examination suggested pulmonary arterial hypertension, and cardiac catheterization revealed a saccular lesion apparently arising from the left ventricular outflow tract and producing compression of the right pulmonary artery. Origin from the left ventricular outflow tract just under the aortic ring was confirmed at operation. The lesion apparently arose from an anular excavation related to previous endocarditis with abscess formation. Reported cases of similar aneurysmal lesions are briefly reviewed, and other known causes of the pulmonary arterial compression syndrome are discussed.     

Clinical and hemodynamic follow-up of left ventricular to aortic conduits in patients with aortic stenosis

To assess the long-term results of left ventricular outflow tract reconstruction utilizing an apical left ventricular to aortic valved (porcine) conduit the clinical and hemodynamic data were reviewed from 24 patients who had placement of an apico-aortic conduit. Eighteen of the patients are asymptomatic and taking no cardiac medications. Three patients were reoperated on, one patient 1.5 years after his original operation for subacute bacterial endocarditis and two patients 3 to 4 years after their original operation for severe conduit valve insufficiency. None of the patients is taking anticoagulants and no thromboembolic events have occurred. Postoperative catheterization has been performed 1 to 1.5 years (mean 1.2) after repair in 15 of 21 patients. The rest left ventricular outflow tract gradient has decreased from 102.5 +/- 20 mm Hg preoperatively to 14.8 +/- 9.9 mm Hg postoperatively (probability [p] less than 0.001). Some degree of conduit obstruction was demonstrated by catheter passage in 11 of the 15 patients. In these 11 patients, the obstruction occurred at three distant sites: at the egress of the left ventricle in 9, at the porcine valve in 5 and at the aortic to conduit junction in 1. Isometric exercise in five and supine bicycle exercise in six patients increased the left ventricular outflow tract gradient by 2.5 +/- 1.1 and 20.8 +/- 11.8 mm Hg, respectively, despite an increase in cardiac index of 1 +/- 0.3 and 3.7 +/- 0.4 liters/min per m2, respectively. The data suggest that a left ventricular to aortic conduit is an effective form of therapy for severe left ventricular outflow tract obstruction.     

Vitamin D receptor stable transfection restores the susceptibility to 1,25-dihydroxyvitamin D3 cytotoxicity in a rat glioma resistant clone

BACKGROUND: Visceroatrial heterotaxy syndrome is characterized by abnormality of visceral laterality and complex cardiovascular anomalies usually involving both the outflow and inflow tract. Morishima et al. (1995) showed that mouse embryos treated with all-trans retinoic acid at embryonic day 6.5 (primitive streak stage) induces this syndrome. METHODS: To investigate the morphogenetic process of visceroatrial heterotaxy syndrome, we examined retinoic acid-treated mouse embryos at embryonic days 9-15 using scanning electron microscopy. RESULTS: The sinoatrial connection was first distinguished for the determination of situs as early as at embryonic day 10.5. Normal visceroatrial situs was found in 57% of all treated embryos, and the rest had abnormal situs, in which right isomerism was found in 81%. In the right-isomeric mouse, the cardiac morphology was characterized by abnormal looping together with dysplasia of the inflow and outflow tract cushion; that is, the primitive right ventricle was usually deviated cranially to various degrees, the atrioventricular cushion appeared trilobed in a half of them, and unilateral ventricular hypoplasia was noted in about one-third of them after embryonic day 14.5. CONCLUSIONS: An anomalous relation between the atrioventricular cushions and the interventricular septum appeared to have caused a restrictive inflow to the unilateral ventricle, leading to ventricular chamber hypoplasia on the ipsilateral side. Thus, we clarified that retinoic-acid treatment at the primitive streak stage disturbed cardiac looping and formation of atrioventricular cushion development, which secondarily influenced ventricular chamber development.     

Hypercalcemic cardiomyopathy associated with primary hyperparathyroidism mimicking primary obstructive hypertrophic cardiomyopathy

A 72-year-old woman presented to hospital with rapidly progressive dyspnea and chest pain on exertion. Physical findings included a grade 3/6 systolic murmur increased by the Valsalva manoeuvre. Transthoracic echocardiography revealed concentric left ventricular hypertrophy, systolic anterior motion of the mitral valve and critical dynamic outflow tract obstruction. The myocardium was strikingly heterogeneous with hyperdynamic left ventricular systolic function. Laboratory findings included severe hypercalcemia secondary to primary hyperparathyroidism. The patient's outcome was unfavourable with nephrogenic diabetes insipidus, pancreatitis, shock, severe acidosis and death. Postmortem examination confirmed the presence of severe concentric left ventricular hypertrophy, a narrowed left ventricular outflow tract and localized endocardial fibrosis of the left interventricular septum. Microscopic findings showed diffuse calcium deposits of the myocardium, coronary arteries, kidneys and lungs. This appears to be the first report of two-dimensional and Doppler echocardiographic findings in hypercalcemic cardiomyopathy mimicking obstructive hypertrophic cardiomyopathy.     

The growth of secondary infections of Hymenolepis microstoma in mice: the effect of various primary infection regimes

Two patients, ages 7 and 17, with unresectable obstructions within the left ventricular cavity, have been managed by interposing a conduit bearing a porcine aortic valve between the apex of the left ventricle and the infra-renal abdominal aorta. The younger child had idiopathic hypertrophic subaortic stenosis (IHSS) recognized in infancy. At the age of three, a right ventricular myomectomy and a trans-aortic left ventricular myotomy were performed. Symptoms were progressive with congestive failure, diaphoresis, syncope , and angina pectoris. Following construction of a second left ventricular outflow tract with relief of intraventricular obstruction, the patient has become asymptomatic. The second patient has fibrous tunnel obstruction of the left ventricular outflow tracting providing a 100 mm Hg gradient. Fibrous tissue was resected in part through the transaortic route, and a second outflow tract was constructed. A postoperative cardiac catheterization revealed an obliteration of the previous intraventricular gradients and an equal distribution of left ventricular output through the two available outflow tracts. She remains asymptomatic.     

The effect of the ingestion of ethanol on obstruction of the left ventricular outflow tract in hypertrophic cardiomyopathy

BACKGROUND: Ethanol causes vasodilation, which might have an adverse effect, due to increased obstruction of the left ventricular outflow tract, in patients with hypertrophic obstructive cardiomyopathy. We assessed the hemodynamic effects of the ingestion of ethanol, in an amount commonly consumed socially, in patients with hypertrophic cardiomyopathy. METHODS: We performed echocardiography in 36 patients before and several times after the ingestion of either 50 ml of 40 percent ethanol or an isocaloric placebo with the aroma of rum. Each patient received both ethanol and placebo, on different days. The patients, but not the physicians, were blinded to the content of the drink. We measured the sizes of the left atrium and left ventricle, the left-ventricular-wall thickness, blood pressure, heart rate, the degree of systolic anterior motion of the mitral valve, and the pressure gradient across the left ventricular outflow tract. RESULTS: The ingestion of ethanol regulated in a significant drop in the mean (+/- SD) systolic blood pressure (from 130.5 +/- 18.6 to 122.5 +/- 20.3 mm Hg, P<0.001), a significant increase in systolic anterior motion of the mitral valve (from a grade of 2.1 to a grade of 2.5, P<0.001), and a 63 percent increase in the mean gradient across the left ventricular outflow tract (from 38.1 +/- 26.5 to 62.2 +/- 42.4 mm Hg, P<0.001). These changes, which were not associated with symptoms, did not occur after the ingestion of placebo. CONCLUSION: The ingestion of a small amount of ethanol caused an increase in the gradient across the left ventricular outflow tract in patients with hypertrophic obstructive cardiomyopathy, which could have and adverse clinical effect.     

Flow reversal in the descending aorta: a guide to intraoperative assessment of aortic regurgitation with transesophageal echocardiography

This study assessed the value of biplane transesophageal echocardiographic assessment of diastolic flow reversal in the descending aorta as an alternative to Doppler color flow imaging in determining severity of aortic regurgitation. In 45 patients undergoing cardiac operations, the severity of aortic regurgitation was assessed by semiquantitative grading of the width of the Doppler color flow regurgitant jet relative to the left ventricular outflow tract, and the presence of diastolic flow reversal was assessed with pulsed-wave Doppler measurements at three sites in the descending aorta. In four patients, the diastolic flow reversal method was the only available form of assessment because of inadequate visualization of the left ventricular outflow tract beneath a mitral valve prosthesis. Diastolic flow reversal in the descending aorta was not observed in patients without aortic regurgitation and was always present in patients with severe aortic regurgitation. Aortic valve replacement successfully eliminated descending aortic flow reversal in all 19 patients in whom it was present before valve replacement. Identification of diastolic flow reversal at multiple sites in the descending aorta with biplane transesophageal echocardiography helps to confirm the presence of severe aortic regurgitation and can serve as an alternative method of assessment when visualization of the left ventricular outflow tract is impaired.     

Echocardiographic features of congenital left ventricular inflow obstruction

The echocardiographic features of congenital left ventricular inflow obstruction are described in six patients. The echocardiograms in two patients with cor triatriatum were distinguished by normal mitral valve motion and an abnormal echo within the left atrium. In two patients with supravalvar mitral ring, in addition to abnormal mitral valve motion, an abnormal echo, presumably originating from the obstructive membrane, was located between the anterior and posterior mitral leaflets. In two cases of parachute mitral valve, mitral valve motion was abnormal. In one of these cases there were multiple mitral valve echoes similar to those found in supravalvar mitral ring. The echocardiographic identification of an obstructive membrane within the left atrium is difficult because of the occurrence of artifacts. However, membranes may be identified if careful scanning techniques are employed in patients in whom left ventricular inflow obstruction is suspected. The echocardiogram is useful in detecting mitral valve abnormalities in these patients and is valuable in cases where mitral valve replacement is contemplated.     

Mobilization of the left and right fibrous trigones for relief of severe left ventricular outflow obstruction

BACKGROUND: There is still no agreement about the optimal method of surgical relief of fixed subaortic stenosis, particularly the severe forms. OBJECTIVES: The purpose of this study was to describe a new technique for the relief of subaortic stenosis based on analysis of the functional anatomy of the left ventricular outflow tract and pathophysiologic features of subaortic stenosis. Methods and patients: We propose that one of the basic abnormalities in subaortic stenosis is interference with the hinge mechanism provided by the 2 fibrous trigones with progressive deposition of fibrous tissue in these angles. The technique described in this paper consists of excision of all components of the fibrous "ring," with mobilization of the left and right fibrous trigones. This results in the restoration of the normal dynamic behavior of the left ventricular outflow tract with maximal widening of the outflow tract as the result of backward displacement of the subaortic curtain and anterior leaflet of the mitral valve. This technique has been used in 57 consecutive patients who ranged in age between 5 months and 56 years (mean, 15.5 +/- 10.6 years). Gradients across the left ventricular outflow tract were between 45 and 200 mm Hg (mean, 86.7 mm Hg). Additional lesions were present in 10 patients, and 7 patients had had 8 previous operations on the left ventricular outflow tract. At operation, in addition to resection of subaortic stenosis, 3 patients had aortic valvotomy, 2 patients had homograft replacement of the aortic valve, 7 patients had patch closure of a ventricular septal defect, and 1 patient had open mitral valvotomy. RESULTS: There were 2 early deaths and 1 late sudden death during the follow-up period that ranged from 1 month to 25 years (mean, 15. 2 years). One patient experienced the development of endocarditis on the aortic valve 7 years after operation, which was successfully treated by homograft replacement. Postoperative gradients across the left ventricular outflow tract varied from no gradient to 30 mm Hg (mean, 8 mm Hg). There were no instances of recurrence of a gradient across the left ventricular outflow tract. CONCLUSION: It is concluded that mobilization of the left and right fibrous trigones results in durable relief of subaortic stenosis.     

Role of transesophageal echocardiography in the diagnosis of obstructive hypertrophic myocardiopathy. Report of 2 cases and review of the literature

Two cases of obstructive hypertrophic cardiomyopathy, in which the transthoracic echocardiography was not enough to reach the right diagnosis, are reported. The transesophageal echocardiography revealed to be the most useful technique to reach the exact diagnosis and more information about the mechanism of the left ventricular outflow tract obstruction. Moreover, this technique could be useful to direct to a surgical treatment and to monitorize the mitral valve function and the possible complications during operation.     

End-on aortogram: improved identification of important coronary artery anomalies in tetralogy of Fallot

OBJECTIVE: To identify coronary artery anomalies in patients with tetralogy of Fallot with an aortogram taken with steep caudal and left oblique angulation ("end-on" aortogram). DESIGN: Prospective evaluation of end-on aortogram in the preoperative angiographic assessment of consecutive patients with tetralogy of Fallot. SETTING: Regional paediatric cardiology centre. PATIENTS: 34 patients, aged 3 months to 12 years (median age 9 months). METHODS: An aortogram was performed with steep caudal (38 degrees-45 degrees) and left oblique (0 degrees-30 degrees) angulation under general anaesthetic as part of routine preoperative angiographic assessment. RESULTS: The origins and courses of the coronary arteries were visualised in all patients and important coronary artery anomalies were identified in four patients: single left coronary artery; single right coronary artery (two patients); separate high origin of left anterior descending. These anomalous coronary vessels crossed the right ventricular outflow tract. CONCLUSIONS: It is important to identify preoperatively coronary arteries that may interfere with right ventricular outflow tract reconstruction. An aortogram with steep caudal and left oblique angulation is useful in identifying anomalous coronary arteries and more importantly it defines the relation of these vessels to the right ventricular outflow tract.     

Mitral valve replacement with total preservation of native valve and subvalvular apparatus

BACKGROUND AND AIMS OF THE STUDY: Preservation of the mitral valve and subvalvular apparatus was introduced clinically in the early 1960s, but for two decades the technique for mitral valve replacement included excision of both leaflets and their attached chordae tendineae. Lately, emphasis has been replaced on retaining the mitral subvalvular apparatus during valve replacement because of its role in left ventricular function. Hence, during the past six years, when performing mitral valve replacement we have, when possible, preserved the valvular and sub-valvular mitral apparatus. METHODS: Between January 1990 and November 1996, complete retention of all mitral tissue in connection with mitral valve replacement was performed in 58 patients (23 women and 35 men). Mean age was 63 years (range: 23 years to 77 years). Coronary bypass was a concomitant procedure in 19 patients; both the mitral and aortic valve was replaced in four cases. Calcified and/or stenotic valves were not a contraindication for the procedure; calcified plaques were removed. Adhesion between anterior and posterior leaflets was treated with sharp dissection. Valve and subvalvular tissue were preserved. The leaflets were reefed within the valve-sutures and compressed between the sewing ring and the native annulus when implanting the valve prosthesis. Chordal tension on the ventricle is thus maintained and the chordae pulled away from the valve effluent. RESULTS: Six patients died in the postoperative period and three had transient neurological symptoms. In no patient was death or transient neurological symptoms a consequence of the retention of mitral leaflets with subvalvular apparatus. CONCLUSIONS: We find the described technique to be useful not only in valve insufficiency but also in valve stenosis when preserving the mitral leaflets with sub-valvular apparatus during valve replacement. The technique is without procedure-related complications and prevents obstruction of left ventricular outflow tract.     

Early results of AV sequential pacing on left ventricular outflow obstruction after Senning procedure

Dual chamber pacing was shown to decrease left ventricular outflow tract (LVOT) obstruction in patients with hypertrophic cardiomyopathy 30 years ago. We report early results of AV sequential pacing from the LV apex in a patient with transposition of the great arteries who is post-Senning procedure. LVOT obstruction resulted from septal deviation and systolic anterior motion of the mitral valve. Pacing was indicated for sinus node dysfunction. AV sequential pacing with a short optimal AV interval of 60 ms demonstrated a 45% reduction in the degree of LVOT obstruction. This article suggests that LVOT obstruction after the Senning procedure can be palliated by asynchronous septal contraction induced by AV sequential pacing, even if the activation is from LV apex, and avoid or postpone surgery in selected situations.     

Teams and seams: skill mix in primary care

OBJECTIVES: We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality. BACKGROUND: Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality. METHOD: All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed. RESULTS: A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), pulmonary outflow tract obstruction (84%), anomalous pulmonary venous drainage (87%) and pulmonary vein obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p < 0.05). The surgical mortality rate for patients undergoing pulmonary vein repair was 95%. Overall survival estimates were 71% at 1 month, 49% at 1 year and 35% at 5 years. Independent risk factors for decreased time to death included the absence of pulmonary outflow obstruction (relative risk [RR] 2.23, p < 0.03), presence of major AV valve anomaly (RR 5.23, p < 0.03) and obstructed pulmonary veins (RR 5.43, p < 0.0001). CONCLUSIONS: Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of pulmonary vein obstruction remains a serious problem and is associated with high mortality.     

Cross-sectional echocardiography in evaluating patients with discrete subaortic stenosis

Ten patients with discrete subvalvular aortic stenosis were examined using a real time, high resolution cross-sectional echocardiographic scanner. There were two patients (Group I) with a thin discrete subvalvular membrane, five (Group II) with a more extensive area of subvalvular narrowing and three with a residual area of outflow tract obstruction after surgical revision (Group III). In patients with a thin obstructing membrane (Group I), two distinct linear echoes were observed in the outflow tract. These echoes were not continuous with the walls of the outflow tract and showed some dynamic motion during the cardiac cycle. In four of the five patients with diffuse outflow tract narrowing (Group II), a relatively extensive area of inward bowing of both the anterior and posterior margins of the outflow tract was noted. In the fifth case, there was a prominent localized shelf-like increase in thickness of the basal portion of the muscular septum with a corresponding echo projecting anteriorly from the mid-portion of the anterior or mitral leaflet. The three cases examined after surgical revision of the outflow tract (Group III), had different patterns of outflow tract narrowing, but narrowing was clearly demonstrated. This study suggests that cross-sectional echocardiography offers an alternative and probably improved method for the noninvasive visualization of the left ventricular outflow tract.