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PubMed1997

The left ventricular outflow tract in atrioventricular septal defect revisited: surgical considerations regarding preservation of aortic valve integrity in the perspective of anatomic observations.

OBJECTIVE: The anatomy of the left ventricular outflow tract in hearts with atrioventricular septal defect has been widely investigated, but controversies remain regarding detailed aspects of left ventricular outflow tract anatomy in the perspective of operative techniques to either prevent or relieve outflow tract obstruction. METHODS: We investigated 29 postmortem hearts with an atrioventricular septal defect. Measurements were taken of the circumferences and of the widths of the components that make up the outflow tract, that is, the interventricular septum, the superior bridging leaflet, the left ventricular free wall, and the length of the tendinous cords. RESULTS: The circumference of the left ventricular outflow tract immediately underneath the aortic valve was not different from that at the middle part of the outflow tract. Hearts with the partial type defect, characterized by separate atrioventricular orifices, had a smaller outflow tract than those with the complete variety. Although the anatomic constituents that contribute to left ventricular outflow tract obstruction are complex, this study showed that a reduced width of the interventricular septum was most intimately related to narrowing immediately underneath the aortic valve. Obstruction at the middle part of the left ventricular outflow tract was largely caused by reduced width of the interventricular septum together with short tendinous cords. CONCLUSIONS: On the basis of these observations, we recommend detailed investigation of the anatomy of the left ventricular outflow tract immediately underneath the aortic valve, before surgical attempts to relieve outflow tract obstruction, because in some procedures the integrity of the aortic valve will be at stake.

Aortic Valve↗
PubMed2002

Obstruction of the left ventricular outflow tract after mitral valvuloplasty with implantation of Gregori's ring.

Mitral valvuloplasty is efficient for repairing mitral valve disease with few complications. In some cases, obstruction of the left ventricular outflow tract may occur due to systolic anterior motion of the mitral valve. We report the case of a patient with this complication and a pressure gradient between the left ventricle and the aorta of 130 mm Hg after mitral valvuloplasty with implantation of a Gregori's ring. The management was clinical with suspension of the vasoactive drugs and introduction of a beta-blocker. Two years after the surgery, the patient is asymptomatic and has a normal life.

Heart Valve Prosthesis Implantation↗
PubMed1993

Mechanism of outflow tract obstruction causing failed mitral valve repair. Anterior displacement of leaflet coaptation.

BACKGROUND: Systolic anterior motion of the mitral valve causing left ventricular outflow tract obstruction occurs in 1% to 2% of patients having mitral valve repair, in some cases requiring further surgery to relieve the obstruction, but the mechanism and the geometry involved are not certain. METHODS AND RESULTS: We studied 14 patients who developed systolic anterior motion and left ventricular outflow tract obstruction, all after posterior leaflet resection and annuloplasty, in whom a second repair eliminated systolic anterior motion by complete (n = 6) or partial (n = 8) ring removal. Intraoperative transesophageal echocardiography was recorded before pump, after failed repair during left ventricular outflow tract obstruction, and after a corrective second pump run to relieve the systolic anterior motion. Systolic anterior motion occurred when the mitral valve coaptation to septum distance was reduced (before, 26.5 +/- 4.3; during systolic anterior motion, 17.4 +/- 4.4 versus after second pump, 23.4 +/- 6.9 mm) and the mitral valve coaptation to posterior mitral annulus distance was greater (before, 18.9 +/- 3.4; during systolic anterior motion, 22.2 +/- 4.6 versus after second pump, 17.4 +/- 3.6 mm), both P < .01. Comparing dimensions before pump, during systolic anterior motion, and after the second pump, there were no differences in left ventricular cavity diameter in systole or diastole, the septum to posterior annulus distance, or the angle between the aortic and mitral annular planes. CONCLUSIONS: After mitral repair, left ventricular outflow tract obstruction occurs when the mitral coaptation line is displaced anteriorly. When systolic anterior motion occurs, reduction of the amount of annuloplasty or use of the posterior leaflet sliding procedure may eliminate this problem. Understanding the geometry of this phenomenon may facilitate preoperative echo selection of high-risk patients (those with large redundant posterior leaflets and relatively normal ventricular size) and modification of surgical technique to avoid the problem of outflow tract obstruction after mitral valve repair.

Echocardiography, Transesophageal↗
PubMed2000

Repeated procedure after radical surgery for tetralogy of Fallot.

BACKGROUND: Although the immediate results of radical operation for tetralogy of Fallot are excellent, long-term follow-up has shown that the number of repeated procedures has increased in many institutions. We describe patients who underwent a second or third procedure after radical operation for tetralogy of Fallot. METHODS: Between April 1981 and August 1996, we operated on 44 patients for the second time and on 4 for the third time after radical operation for tetralogy of Fallot. Indications for a second and third procedure included right ventricular outflow tract obstruction in 38 patients, infective endocarditis in 4, and isolated residual ventricular septal defect in 3. RESULTS: One patient died after concomitant replacement of the pulmonary and tricuspid valves. Three patients who underwent sternotomy more than twice (before the second or third operation) underwent accidental cardiovascular trauma during this procedure (30%). Moreover, when patients underwent more than two sternotomy procedures before the repeated operation for tetralogy of Fallot, the total bypass time, interval between cessation of the cardiopulmonary pump to completion of the operation, amount of blood transfusion, and length of intensive care unit stay were significantly higher compared with those who underwent less than two sternotomy procedures (p < 0.05). Right ventricular outflow tract obstruction was the main indication for a second operation. After the second operation for right ventricular outflow tract obstruction in 35 patients, the preoperative right ventricle to left ventricle pressure ratio decreased significantly (from 0.75 +/- 0.13 to 0.51 +/- 0.12; p < 0.0001). However, the right ventricle to left ventricle pressure ratio did not significantly decrease in patients who underwent a third procedure to treat right ventricular outflow tract obstruction. CONCLUSIONS: The surgical results of a second procedure after radical operation for tetralogy of Fallot were acceptable. However, the risk of accidental cardiovascular trauma during dissection was high among patients who underwent sternotomy more than twice before repeat operation.

Adolescent↗
PubMed1983

Anatomic obstruction of the right ventricular outflow tract in transposition of the great arteries.

In 126 hearts with transposition of the great arteries (TGA), morphologic features of the right ventricular (RV) outflow tract were analyzed. Distinct anatomic outflow tract obstruction was found in 15 of the 55 hearts with a ventricular septal defect (VSD) (27%): 12 had a perimembranous defect (predominantly extending into the infundibulum), 2 had an inlet, and 1 had an infundibular defect. In 12 hearts, outflow tract obstruction was caused by anterior displacement of infundibular septum and ventriculoinfundibular fold; in 3 hearts, it was caused by bulging of the trabecula septomarginalis or ventriculoinfundibular fold, or both. Of 71 hearts with an intact ventricular septum, only 2 (3%) had RV outflow tract obstruction: 1 had a trabecula in front of the aortic ostium and in the other an aberrant hypertrophic muscle bundle crossed the RV cavity, obstructing the entrance to the infundibulum. Associated malformations included aortic arch malformations (6 cases) and tricuspid valve anomalies (6 cases). Thus, anatomic RV outflow tract obstruction may constitute a complicating factor in TGA, notably in cases with VSD. A systemic RV blood pressure may trigger hypertrophy, resulting in progression of the obstruction; establishing normal RV pressure by a "switch" procedure might prevent this progression.

Heart Septum↗
PubMed1988

Hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy is a diverse clinical and pathophysiologic disorder of unknown cause that principally involves the left ventricle and is manifested as asymmetric or concentric hypertrophy. If asymmetric, the hypertrophy is usually greatest in the ventricular septum, but variations occur in which the hypertrophy may be maximal at the mid-ventricular level, at the apex, or rarely, in the free wall of the left ventricle. Right ventricular involvement is usually less evident. The principal abnormality in systole is the obstruction to left ventricular outflow caused by systolic anterior motion (SAM) of the anterior or posterior mitral leaflet(s) with mitral leaflet-septal contact. SAM occurs as the result of the Venturi forces created by the rapid ejection of blood through an outflow tract that is narrowed by upper septal hypertrophy, drawing the mitral leaflet(s) anteriorly. The time of onset and duration of mitral leaflet-septal contact determine the magnitude of the pressure gradient. Mitral regurgitation invariably accompanies the obstruction to outflow. Ventriculomyectomy surgery, by thinning the septum and widening the outflow tract, abolishes the abnormal mitral leaflet motion and, consequently, the obstruction to outflow and the mitral regurgitation. In symptomatic patients with resting obstruction this form of surgery more dramatically relieves the systolic abnormalities and the accompanying symptoms than any form of medical therapy currently available. The extent of hypertrophy is believed to be the principal determinant of impaired left ventricular relaxation and increased chamber stiffness that characterize diastole in hypertrophic cardiomyopathy. Diastolic dysfunction is common to most such patients irrespective of the presence or absence of outflow obstruction. Calcium entry blockers may improve the left ventricular relaxation process and relieve symptoms in patients with hypertrophic cardiomyopathy, particularly the subgroup with no obstruction to outflow. Atrial and ventricular arrhythmias are responsible for a significant proportion of the morbidity and mortality, and their prevalence appears to depend on the presence of obstruction and the extent of hypertrophy. Thus, the major manifestations of hypertrophic cardiomyopathy in systole and diastole, as well as the disturbances in rhythm, appear to be related to the site and/or extent of the hypertrophic process. We have learned much about hypertrophic cardiomyopathy in the 30 years since its modern description. The vast majority of symptomatic patients can now be improved with specific medical or surgical therapy.(ABSTRACT TRUNCATED AT 400 WORDS)

Arrhythmias, Cardiac↗
PubMed1998

Altered autonomic cardiac control in hypertrophic cardiomyopathy. Role of outflow tract obstruction and myocardial hypertrophy.

AIM: The goal of this study was to investigate the role of left ventricular outflow tract obstruction and myocardial hypertrophy on autonomic cardiac function in patients with hypertrophic cardiomyopathy. METHODS AND RESULTS: The sympatho-vagal function was evaluated by spectral analysis of heart rate variability in 28 patients with hypertrophic obstructive cardiomyopathy, 22 patients with hypertrophic non-obstructive cardiomyopathy, 12 with systemic hypertension and left ventricular hypertrophy and 28 healthy subjects. Left ventricular out-flow tract pressure gradient in patients with hypertrophic cardiomyopathy was evaluated by echo-Doppler methods and the quantitative assessment of left ventricular hypertrophy was based on an echocardiographic index. At rest, patients with hypertrophic non-obstructive cardiomyopathy showed normal spectral patterns, while in patients with hypertrophic obstructive cardiomyopathy and in patients with systemic hypertension we observed, respectively, a significant reduction and increase in the low frequency component relative to the control (P < 0.05). During tilt, the physiological increases in the low frequency component and in the low to high frequency ratio were markedly blunted, or even reverted, only in patients with hypertrophic obstructive cardiomyopathy. In these patients, the heart rate increase during tilt was delayed in comparison to the other groups. Finally, in the hypertrophic obstructive cardiomyopathy group, the impairment of sympathetic activation (lack of increase in the low frequency component during tilt) was significantly correlated to the echocardiographic index of left ventricular hypertrophy (r = -0.800, P < 0.001) rather than to the left ventricular outflow tract pressure gradient (r = 0.295, P: ns). CONCLUSION: Among patients with hypertrophic cardiomyopathy, only those with outflow tract obstruction show spectral signs of altered autonomic cardiac control. Within this group, the autonomic dysfunction appears to be correlated to myocardial hypertrophy rather than to left ventricular outflow tract obstruction.

Adult↗
PubMed2003

Plasma levels of brain natriuretic peptide in various forms of obstruction to the left ventricular outflow tract.

BACKGROUND AND AIM OF THE STUDY: The study aim was to evaluate the relationship between plasma concentrations of brain natriuretic peptide (BNP) and the type or degree of stenosis in the left ventricular outflow tract (LVOT). METHODS: The relationship between BNP plasma level and pressure gradient (PG) in the LVOT and LV wall thickness (LVWth) was analyzed in 25 patients with a PG > or = 30 mmHg in the LVOT from the mid-left ventricle to the aortic valve. Among patients, 14 had aortic valve stenosis (AS), five had subaortic type hypertrophic obstructive cardiomyopathy (HOCM), three had mid-ventricular type HOCM, and three had angled ventricular septum. Three patients with AS showed LV systolic dysfunction (ejection fraction (EF) < 50%). All patients were in sinus rhythm. LV peak-systolic pressure (LVPSP) was derived by adding maximum PG to cuff systolic arterial pressure. RESULTS: In AS patients without LV systolic dysfunction and HOCM patients, there was a significant positive correlation between BNP and LVPSP (r = 0.78, p = 0.001; r = 0.76, p = 0.007, respectively). In AS patients without LV systolic dysfunction, BNP was positively correlated with LVWth (r = 0.79, p = 0.001), but no correlation was found between BNP and LVWth in patients with HOCM. In AS patients including systolic LV dysfunction, BNP was negatively correlated with LVEF (r = -0.87, p < 0.0001), but no correlation was found between BNP and LVEF in patients with HOCM. CONCLUSION: These results suggest that BNP level is closely associated with severity of stenosis in patients with HOCM, but mainly with severity of stenosis and also degree of LV systolic dysfunction in patients with AS. The BNP-LVWth relationship appeared to differ between AS (a fixed stenosis with uniform myocardial hypertrophy) and HOCM (a dynamic stenosis with uneven myocardial hypertrophy).

Aged↗
PubMed1990

Obstruction of the left ventricular outflow tract in childhood--improved diagnosis by transoesophageal echocardiography.

We used transoesophageal echocardiography in 4 children with either documented or suspected obstruction of the left ventricular outflow tract to determine whether this technique may provide additional morphologic information when compared to praecordial ultrasound. Transoesophageal studies defined unsuspected mitral valvar abnormalities in two patients and involvement of the subvalvar mitral apparatus in one. In one patient, the study documented a suspected fibrous ridge associated with malalignment of the outlet septum. Transoesophageal echocardiography would appear to be a new technique with definite advantages in defining the spectrum of lesions which cause obstruction to the left ventricular outflow tract in children.

Child↗
PubMed1994

Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960-91): a study of 313 patients.

OBJECTIVE: To determine the incidence of the various types of obstruction of the left ventricular outflow tract in patients born in the five health districts of Liverpool and to compare their prognosis into early adult life. DESIGN: Notes of all patients with obstruction of the left ventricular outflow tract born in the study area between 1960 and 1991 were reviewed. Patients with hypoplastic left ventricle, mitral valve atresia, and those with discordant atrioventricular or ventriculoarterial connections were excluded. Survivors were traced and assessed clinically; eight were lost to follow up. RESULTS: Obstruction of the left ventricular outflow tract occurred in 313 patients (67% male), giving an incidence of 6.1/10,000 live births. The median (range) age at presentation was 13.9 months (0-20 yr). Aortic valve stenosis occurred in 71.2%: subvalve in 13.7%, supravalve in 7.7%, and multilevel in 7.4%. The median (range) duration of follow up was 10.0 (1-29) yr. Aortic regurgitation at presentation occurred more often (p < 0.001) in patients with subvalve stenosis than in those with other types of obstruction, but there was an increased incidence (p < 0.001) at follow up in patients with valve stenosis. Ninety eight patients (31.3%) underwent operation. The reoperation rate was 27% for valve stenosis and 9% for subvalve obstruction. No patients with supravalve stenosis underwent reoperation. The median duration from first operation to aortic valve replacement (17 patients) was 12.3 years. Hazard analysis confirmed that the risk of death was higher in patients presenting at a younger age, with more severe stenosis, and those with subaortic, multilevel obstruction or a syndrome. Hazard analysis also showed that the risk of a clinical event (surgery, balloon dilatation, or endocarditis) was greater in patients who presented at a younger age, with more severe stenosis or aortic regurgitation, and in those with subvalve or multilevel obstruction. CONCLUSIONS: Aortic valve stenosis was the most common type of obstruction. Hazard analysis indicates that the age and severity of obstruction at presentation have a significant effect on survival and freedom from a clinical event. The risk of premature death in patients presenting with moderately severe valve stenosis is reasonably small, but increases considerably in those with subvalve, supravalve, and multilevel obstruction. Patients who present with mild valve stenosis have a good prognosis. The risk of sudden death is less than previous predictions. Patients with subvalve and multilevel obstruction, even when mild at presentation, are more likely to undergo intervention or develop endocarditis than those with valve or supravalve stenosis. Follow up into adult life is essential.

Adolescent↗
PubMed2005

Fabry disease: percutaneous transluminal septal myocardial ablation markedly improved symptomatic left ventricular hypertrophy and outflow tract obstruction in a classically affected male.

Fabry disease (alpha-galactosidase A deficiency) is an X-linked recessive lysosomal storage disease in which left ventricular hypertrophy (LVH) is common, and if severe, may mimic hypertrophic obstructive cardiomyopathy. Alcohol-induced percutaneous transluminal septal myocardial ablation (PTSMA) has been used as a safe and effective method to alleviate LVH obstruction in patients with hypertrophic obstructive cardiomyopathy (HCM). We describe a case of a classically affected Fabry 53-year-old male with symptomatic HCM (NYHA class III with exertional angina) who was treated with PTSMA. The procedure safely and effectively alleviated symptomatic left ventricular outflow tract obstruction at long-term follow-up, and the patient's NYHA classification was reduced to NYHA class I to II.

Cardiac Catheterization↗
PubMed1998

Spontaneous variability of left ventricular outflow tract gradient in hypertrophic obstructive cardiomyopathy.

BACKGROUND: Improvement in the left ventricular outflow tract (LVOT) gradient has been used as a means of assessing response to therapy in patients with hypertrophic obstructive cardiomyopathy (HOCM). To our knowledge, no data exist regarding the spontaneous day-to-day variability of the LVOT gradient in patients with HOCM. Defining the magnitude of such variability is critical to properly understand how much improvement in LVOT gradient must be present to invoke a therapeutic response. METHODS AND RESULTS: We studied the spontaneous variation in the continuous-wave, Doppler-derived pressure gradient on 5 consecutive days in 12 HOCM patients and 5 aortic stenosis control subjects. While in some patients the day-to-day variability in resting gradient was small, in others it varied markedly. The 95% confidence interval for attributing a change in LVOT gradient to factors other than random variation is +/-32 mm Hg for resting gradient and +/-50 mm Hg for provoked gradient. The mean coefficient of variation for gradient across 5 days for the group was 0.52+/-0.33 for resting gradient and 0.46+/-0.16 for provoked gradient. The day-to-day variability in pressure gradient could not be explained by changes in heart rate, blood pressure, or left ventricular end-diastolic dimension, each of which had a coefficient of variation <.11. Moreover, technical factors related to the performance or interpretation of the studies did not account for it because the coefficient of variation for gradient in aortic stenosis was <10% and interobserver and intraobserver agreement was excellent (r=.96 and .98, respectively). CONCLUSIONS: The LVOT pressure gradient varies considerably from day to day in stable patients with HOCM. A single measurement of pressure gradient is not adequate to define the severity of dynamic LVOT obstruction in HOCM.

Adult↗
PubMedDate not supplied

Right ventricular outflow tract and pulmonary artery obstruction by postoperative mediastinal hematoma: delineation by multiplane transesophageal echocardiography.

A 53-year-old man with osteogenesis imperfecta underwent valve replacement and coronary artery bypass surgery. Unexplained symptoms of dyspnea and fatigue in the late postoperative period prompted further evaluation. Transthoracic echocardiography demonstrated obstruction of the right ventricular outflow tract and pulmonary artery by a mass, with a maximum gradient of 50 mm Hg. Multiplane transesophageal echocardiography revealed that the mediastinal hematoma was more extensive than was suggested by the transthoracic echocardiogram. This finding prompted the decision to resort to a more extensive surgical procedure. Surgical drainage continued until no residual hematoma could be visualized by multiplane transesophageal echocardiography. This case report demonstrates the value of multiplane transesophageal echocardiography in the assessment of mediastinal masses.

Aortic Valve↗
PubMed1991

Disopyramide as a negative inotrope in obstructive cardiomyopathy in children.

Two children with left ventricular outflow tract obstruction due to myocardial hypertrophy received oral disopyramide as a negative inotrope. Both showed rapid improvement in clinical signs and by echo Doppler examination. Nearly complete abolition of severe left ventricular outflow tract obstruction was documented in each case. This experience may prompt further application of disopyramide as a therapeutic agent to relieve dynamic muscular subaortic obstruction in children.

Cardiomyopathy, Hypertrophic↗
PubMed2004

Severe volume depletion leading to mitral insufficiency.

We report a patient with profound hypovolemia who developed dynamic left ventricular outflow tract obstruction and severe mitral regurgitation. Both the outflow tract obstruction and mitral regurgitation resolved with volume replacement. Unlike previous reports of dynamic left ventricular outflow obstruction and mitral regurgitation, the degree of mitral regurgitation was severe. Possible mechanisms are discussed.

Acute Disease↗
PubMed2004

Chronic renal failure in an English bull terrier with polycystic kidney disease.

An entire female English bull terrier, aged five years and one month, was diagnosed with polycystic kidney disease by renal ultrasonography. It had thickening and abnormal motion of the mitral valve on 2D and M mode echocardiography, and left ventricular outflow tract obstruction, characterised by turbulence in the left ventricular outflow tract and elevated aortic blood flow velocity, detected by colour flow and spectral Doppler echocardiography, respectively. Two years later, haematology, serum biochemistry and urinalysis data suggested the presence of compensated renal failure. The dog was euthanased at 10 years and eight months of age, with haematology, serum biochemistry and urinalysis data Indicating decompensated chronic renal failure. Postmortem examination confirmed polycystic kidney disease, chronic renal disease, mitral and aortic valvular myxomatous degeneration, and mixed mammary neoplasia. This case demonstrates that bull terriers with polycystic kidney disease may develop associated chronic renal failure.

Animals↗
PubMed2002

Surgical substrates of postoperative junctional ectopic tachycardia in congenital heart defects.

BACKGROUND: Junctional ectopic tachycardia is a major cause of postoperative morbidity after surgery for congenital cardiac disease. To elucidate the mechanism of junctional ectopic tachycardia, surgical correlations were studied in four types of congenital heart defects involving closure of a ventricular septal defect, relief of right ventricular outflow tract obstruction, or both. METHODS: Between 1997 and 1999, a total of 343 consecutive patients underwent repair of tetralogy of Fallot (n = 114), common truncus arteriosus (n = 10), ventricular septal defect (n = 161), and atrioventricular septal defect (n = 58). Variables studied included demographic and bypass data, surgical approaches toward ventricular septal defect closure and relief of right ventricular outflow tract obstruction, and resection as opposed to division of muscle bundles. RESULTS: Junctional ectopic tachycardia occurred most frequently after repair of tetralogy of Fallot (n = 25; 21.9%), with no cases occurring after repair of common trunk, 6 occurring after repair of ventricular septal defect (3.7%), and 6 occurring after repair of atrioventricular septal defect (10.3%). Stepwise logistic regression revealed that resection of muscle bundles (P <.0001), higher bypass temperatures (P <.03), and relief of right ventricular outflow tract obstruction through the right atrium (P <.05) significantly and independently predicted postoperative junctional ectopic tachycardia. CONCLUSIONS: Relief of right ventricular outflow tract obstruction appears to be more important in the causation of junctional ectopic tachycardia than does ventricular septal defect closure, which may explain the higher incidence of this complication after tetralogy of Fallot repair. Muscular resection seems to be more arrhythmogenic than is simple division. Increased traction through the right atrium for relief of right ventricular outflow tract obstruction would fit the hypothesis that enhanced automaticity of the His bundle, the morphologic substrate for junctional ectopic tachycardia, may result from direct trauma or infiltrative hemorrhage of the conduction system. When feasible, techniques avoiding both extensive muscle resection and excessive traction should be applied during resection of right ventricular outflow tract obstruction.

Adolescent↗
PubMed2004

Cardiac angiosarcoma arising from pulmonary artery: endovascular treatment.

We report a case of unusual origin of cardiac angiosarcoma rising from the pulmonary trunk. The tumor caused severe obstruction of the right ventricular outflow tract with serious symptoms of right ventricular failure and tricuspid insufficiency. The angiosarcoma was surgically unresectable because of infiltration of the pulmonary artery and cardiac structures. To relieve the patient's symptoms we chose an endovascular stent treatment to dilate the right ventricular outflow tract obstruction. Consequently this palliative treatment changed the short-term prognosis of the patient, improving his quality of life, and at 6 months follow-up he remains asymptomatic.

Aged↗