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Dynamic left ventricular outflow tract obstruction complicating bilateral lung transplantation.

UNLABELLED: Dynamic left ventricular outflow tract obstruction (DLVOTO) has been observed in a variety of clinical circumstances but not previously reported in the setting of orthotopic lung transplantation. Diagnosis and effective management of this adverse event were facilitated by transesophageal echocardiography (TEE). IMPLICATIONS: Dynamic left ventricular outflow tract obstruction is not an uncommon phenomenon, but it is often unrecognized. Its rapid recognition and effective treatment was only possible with transesophageal echocardiography (TEE). This contributes to the support for TEE being routinely available for assessment of hemodynamic instability.

Echocardiography, Transesophageal↗

Echocardiographic predictors of left ventricular outflow tract obstruction after repair of interrupted aortic arch.

OBJECTIVES: This study was designed to identify preoperative echocardiographic predictors of left ventricular outflow tract obstruction after repair of interrupted aortic arch and ventricular septal defect closure. BACKGROUND: Left ventricular outflow tract obstruction becomes apparent in nearly 50% of patients after repair of interrupted aortic arch and ventricular septal defect closure but is seldom recognized preoperatively. METHODS: We analyzed the preoperative echocardiograms of all patients with interrupted aortic arch who had postoperative echocardiographic or catheterization data available. Thirty-seven infants (aged 1 day to 10 months, median 5 days) were included. Off-line measurements were performed on hard copies of selected images. The cross-sectional area (indexed to body surface area) and diameters (indexed to the square root of body surface area) of the left ventricular outflow tract; ascending and descending aorta; ventricular septal defect; and mitral, aortic and pulmonary valves were compared with outcome by using analysis of variance. Outcome was classified according to development of postoperative left ventricular outflow tract Doppler gradient (Group 1 < or = 20 mm Hg, Group 2 > 20 mm Hg). RESULTS: The cross-sectional area of the left ventricular outflow tract was significantly smaller in patients who did than in those who did not develop subaortic obstruction ([mean +/- SD] 0.64 +/- 0.25 vs. 1.7 +/- 1.01 cm2/m2, p < 0.004). Left ventricular outflow tract and aortic valve diameters and aortic valve area were not predictive of postoperative left ventricular outflow tract obstruction. Incidence of postoperative left ventricular outflow tract obstruction was lower (p < 0.03) in interrupted aortic arch type A (0 of 6) than in type B (15 of 31). The incidence of aberrant right subclavian artery was lower (p < 0.02) in Group 1 (6 of 22) than in Group 2 (10 of 15). CONCLUSIONS: The preoperatively measured cross-sectional area of the left ventricular outflow tract is significantly smaller in patients with interrupted aortic arch who develop subaortic obstruction postoperatively, with a left ventricular outflow tract area < or = 0.7 cm2/m2 being a sensitive predictor. Aortic arch anatomy (i.e., type of interrupted aortic arch and presence of aberrant right subclavian artery) is also predictive of postoperative left ventricular outflow tract obstruction, possibly by influencing the volume of blood flow across the left ventricular outflow tract. These data should enable preoperative identification of infants who may require surgical relief of subaortic stenosis.

Aorta, Thoracic↗

Initial results of combined anterior mitral leaflet plication and ventricular septal myotomy-myectomy for relief of left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy.

BACKGROUND: Several operations (most commonly ventricular septal myotomy-myectomy and also mitral valve replacement) have been performed to relieve symptoms and obstruction to left ventricular outflow in patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: In an effort to establish an alternative to mitral valve replacement, combined septal myotomy-myectomy and suture plication of the anterior mitral valve leaflet was performed in a subgroup of 36 severely symptomatic patients with obstructive HCM. These patients were selected primarily because the mitral leaflets were judged morphologically at the time of operation to be of sufficient size relative to that of the outflow tract to represent a risk for residual systolic septal contact and outflow obstruction. The effects of operation were assessed in 34 patients; of the 33 with preoperative symptoms, 28 (85%) reported substantial functional improvement after surgery over an average follow-up of 2.2 years. Of 29 patients with complete hemodynamic data, basal outflow gradient was obliterated or reduced substantially by surgery (to < or = 35 mm Hg) in 26 patients (90%) and for the group from 81 +/- 42 to 16 +/- 24 mm Hg (p < 0.001); provocable gradient with isoproterenol infusion also decreased considerably (from 109 +/- 50 to 47 +/- 25 mm Hg; p < 0.001). Echocardiographic studies showed that plication limited the systolic anterior motion of anterior mitral leaflet. There was no hemodynamic evidence of mitral stenosis, and in only one patient did mitral regurgitation increase after surgery. CONCLUSIONS: Mitral valve plication combined with myotomy-myectomy in obstructive HCM 1) can be performed safely and does not adversely alter mitral valve function, 2) relieves symptoms and outflow obstruction under basal and provocable conditions, and 3) may represent an alternative to mitral valve replacement in selected patients with elongated and enlarged mitral leaflets.

Cardiomyopathy, Hypertrophic↗

Long-term follow-up of patients with left ventricular outflow tract obstruction after Carpentier ring mitral valvuloplasty.

Left ventricular outflow tract (LVOT) obstruction is a complication of Carpentier ring mitral valvuloplasty that may occur only when this procedure is used to correct mitral regurgitation attributable to myxomatous degeneration of the mitral valve. LVOT obstruction has not been observed among approximately 300 patients undergoing this procedure to correct mitral regurgitation attributable to other causes. Among 200 patients with degenerative mitral regurgitation who underwent Carpentier valvuloplasty, LVOT obstruction was found in 12 patients (6%). Five of these patients demonstrated severe LVOT obstruction during intraoperative echocardiography immediately after repair, which was corrected by mitral valve replacement in four and removal of the Carpentier ring in one. The remaining seven patients were followed-up for a mean period of 27 months with history, physical examination, and Doppler echocardiography. Systolic anterior motion of the mitral valve was the echocardiographic hallmark of LVOT obstruction. Doppler echocardiographic and catheter-measured LVOT gradient paralleled the severity of the systolic anterior motion of the mitral valve. The severity of the motion decreased, but still could be provoked, with amyl nitrite at late follow-up. Mitral regurgitation tended to recur at late follow-up. Despite the presence of LVOT obstruction and hemodynamic features resembling hypertrophic cardiomyopathy at late follow-up, none of the patients had left ventricular hypertrophy or asymmetric septal hypertrophy, and early postoperative functional class improvement was sustained.

Echocardiography↗

Role of echocardiography in the diagnosis and surgical management of accessory mitral valve tissue causing left ventricular outflow tract obstruction.

We report an unusual case of left ventricular outflow tract obstruction caused by accessory mitral valve tissue. This case illustrates the importance of transthoracic echocardiography in preoperative evaluation and the usefulness of transesophageal echocardiography for intraoperative assessment. If the accessory mitral valve has no functional significance it may be surgically excised, thereby relieving the outflow tract obstruction.

Child↗

Dynamic left ventricular outflow tract obstruction in senile cardiac amyloidosis.

Dynamic left ventricular outflow tract (LVOT) obstruction is classically seen in hypertrophic obstructive cardiomyopathy (HOCM). This can also be seen in cardiac amyloidosis. We describe a rare case of senile systemic amyloidosis with dynamic LVOT obstruction and concomitant three vessel coronary artery disease presenting with clinical and echocardiographic findings similar to those seen in HOCM. We also highlight the importance of distinguishing the sub-types of amyloidosis so that the appropriate therapy can be offered to patients with cardiac involvement, including coronary artery bypass grafting and septal myotomy/myectomy to relieve LVOT obstruction in the more benign forms of cardiac amyloidosis.

Aged↗

Apico-aortic conduits in children with severe left ventricular outflow tract obstruction.

BACKGROUND: Infrequently, congenital heart defects are complicated by left ventricular outflow tract obstruction (LVOTO) not amenable to conventional reconstruction. Apico-aortic conduits provide a means of palliating such patients until definitive repair is possible. The purpose of this study was to review a single institution's current experience with apico-aortic conduits. METHODS: The medical records of pediatric patients receiving apico-aortic conduits were reviewed. Demographics, operative techniques, preoperative and postoperative physiologic variables, morbidity, mortality, and functional class were recorded. Off-pump and on-pump procedures were categorized for comparison. RESULTS: Ten cases of apico-aortic conduits for left ventricular outflow tract obstruction were identified. Indications included congenital aortic stenosis, aortic atresia, and subaortic stenosis. Six procedures were performed off-pump and four required median sternotomy with cardiopulmonary bypass as necessitated by concomitant procedures. There was one operative death. The remaining patients demonstrated hemodynamic improvements and are all alive to date. One patient required conduit valve replacement. All patients are in New York Heart Association classification I or II at the time of last follow-up. CONCLUSIONS: Apico-aortic conduits provide a safe and effective treatment alternative for select cases of left ventricular outflow tract obstruction. Off-pump techniques are feasible in the majority of cases. This valuable adjunct should be considered whenever conventional repair of left ventricular outflow tract obstruction is considered prohibitive.

Blood Vessel Prosthesis Implantation↗

Patients with mild hypertensive heart disease and left ventricular outflow tract obstruction: treatment with angiotensin II antagonists.

Systolic anterior motion of the mitral leaflet (SAM) combined with obstruction at the left ventricular (LV) outflow tract is often observed on echocardiography in elderly hypertensive patients with severe concentric LV hypertrophy. We experienced, however, two patients with mild hypertension who had an ejection systolic murmur, SAM, and LV outflow tract obstruction with a pressure gradient of 46 and 45 mmHg, respectively, despite very mild symmetric hypertrophy of LV wall (12 mm) by echocardiography. Treatment with angiotensin II type 1 receptor blocker improved intraventricular obstruction and LV hypertrophy in both patients. Left ventricular outflow tract obstruction should be suspected in hypertensive patients with mild LV hypertrophy, particularly in those with an ejection systolic murmur. Angiotensin II antagonists could be considered as the treatment of choice for such patients.

Aged↗

Left ventricular outflow tract obstruction due to tricuspid valve prolapse through a high ventricular septal defect.

A rare case is described of fixed subaortic obstruction due to a prolapsed redundant fibrous septal leaflet of the tricuspid valve, herniated through a membranous ventricular septal defect in a boy aged 11 years. The type of obstruction was not suspected before surgery and there was no shunt through the septal defect. Left ventriculography, was attempted and abandoned due to dangerous arrhythmia. Echocardiogram showed definite fixed subvalvular left ventricular outflow obstruction. Corrective surgery was carried out through aortotomy and right ventriculotomy. Ruptured chordae were resected and the tricuspid valve was replaced into the right ventricle. Redundant fibrous tissue was used to close the septal defect. The postoperative recovery was uneventful after complete AV block in the first 5 days.

Aorta↗

Mitral valve anomalies obstructing left ventricular outflow.

This paper reports on two cases of more uncommon types of subaortic stenosis. A 2-year-old boy was found with accessory mitral valve leaflet (AMVL) attaching to the anterior leaflet, ballooning into the subaortic ventricular septum associated with a discrete subaortic membrane. The obstruction was successfully relieved by removal of the AMVL and resection of the membrane. A 19-day-old newborn with accessory tissue on the mitral valve (AMVT) causing subaortic stenosis, subaortic ventricular septal defect (VSD) and patent ductus arteriosus was operated on successfully. Accessory tissue excision through the VSD, VSD patch closure and ductus ligation were performed.

Abnormalities, Multiple↗

Repair of complex left ventricular outflow tract obstruction in children.

BACKGROUND: Complex obstruction of the left ventricular outflow tract (LVOTO) in children may be treated with surgical procedures like the Ross-Konno or Koncz-Konno procedures, or modifications thereof. We present our results from the last 10 years. MATERIAL AND METHOD: During the period from December 1991 to April 2002, 17 patients/children with complex LVOTO were operated on. Patients treated with balloon valvuloplasty, open commisurotomy or simple valve replacement are excluded. The procedures performed were: Koncz-Konno (KK) or modified KK (n = 10), Ross-Konno (RK) (n = 2) and Ross (RO) (n = 5). Concomitant procedures included mitral valve replacement (n = 1), aortic arc plasty (n = 1), subvalvular myotomi (n = 1), resection of subvalvular membrane (n = 1) and aorto-coronary bypass (n = 1). Patients' ages ranged from 1 to 178 months, median 71 months. Body weights ranged from 3.8 to 50.0 kg, median 19.4 kg. Eight cases were redo procedures. RESULTS: Three early deaths occurred. One patient in the KK group, one in the modified KK group and one in the RK group. One patient died 8 years postoperatively of unknown reasons; no autopsy was performed. The rest of our patients who survived the initial major surgical treatment show normal physical performance and their quality of life seems to be good. CONCLUSION: Patients with complex LVOTO can be operated with biventricular repair in selected cases. Early and late mortality are acceptable.

Adolescent↗

Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction.

OBJECTIVE: Pulmonary regurgitation after valveless repair of right ventricular outflow tract obstruction (RVOTO) results in progressive right ventricular (RV) dilatation and dysfunction in an increasing number of patients. Since 1989, we have exclusively used cryopreserved homografts to restore pulmonary valve competence in these patients. Our 9-year-experience with pulmonary valve insertion (PVI) in such cases has been reviewed to evaluate the indications for this procedure and its benefits. METHODS: From 1989 to 1998, 49 patients (original diagnosis: tetralogy of Fallot in 42 patients and pulmonary stenosis in seven) aged from 3 to 42 years (mean 18 +/- 9 years) underwent PVI with homografts late (mean 13 +/- 7 years) after valveless repair of RVOTO (transannular patch, n = 38; pulmonary valvulotomy therefore tau chi infundibular patch, n = 11). Preoperatively, all patients had severe pulmonary regurgitation, cardiomegaly, significant to severe RV dilatation and dysfunction, fatigue, reduced exercise tolerance, and were in NYHA class II (n = 43) or III (n = 6). Ten patients had ventricular arrhythmia. RESULTS: There was one early death, due to air embolism, and one late death, due to ventricular arrhythmia. All survivors but one, who subsequently underwent heart transplant, had symptomatic improvement after homograft insertion. The mean RV end-diastolic diameter decreased from 38 +/- 9 to 26 +/- 8 mm (P < 0.01), and cardiothoracic ratio decreased from 0.62 +/- 0.07 to 0.54 +/- 0.04 (P < 0.01). Good late homograft function was the rule, with all the survivors being free of reoperation for valve failure. At a mean follow-up of 42 +/- 28 months, 41 patients (87% of the survivors) were in New York Heart Association (NYHA) class I and six in class II. Within this group three patients are still in treatment for RV failure and five for ventricular arrhythmias. In these patients, the average interval between RVOTO repair and PVI was significantly longer than in the others (18 +/- 7 vs. 12 +/- 6 years, P < 0.01). CONCLUSION: Homograft PVI is safe and provides clinical improvement with a significant reduction in RV volume overload and excellent mid-term results in most patients with severe PR late after RVOTO repair. This procedure should be undertaken early in symptomatic patients, before severe RV failure and ventricular arrhythmias ensue.

Adolescent↗

Beta-blocker therapy for dynamic left ventricular outflow tract obstruction induced by exercise.

BACKGROUND: In patients who present with angina, dynamic left ventricular outflow tract obstruction may be responsible of symptoms. The aim of our study was to evaluate the effect of negative inotropic therapy on ventricular physiology and symptoms of patients with dynamic left ventricular outflow tract obstruction induced by exercise in the absence of hypertrophic cardiomyopathy. METHODS: Seventy eight patients with symptoms of angina, normal exercise SPECT test and normal resting left ventricular systolic function were prospectively analysed with exercise echocardiography. Fifteen of them developed high outflow tract velocities (greater than 2.57 m/s) on effort. After treatment with bisoprolol (mean dose 6.9+/-3.5 mg) in these patients, symptoms and dynamic left ventricular outflow tract obstruction during exercise were re-evaluated. RESULTS: After negative inotropic therapy, there was a significant reduction of 86.6% in the development of intracavitary gradient (15 to 2 patients). In all patients the left ventricular outflow tract velocities decreased (2.91+/-0.4 m/s to 1.92+/-0.46 m/s, p=0.001). However, the oxygen consumption during exercise was higher (7.3+/-1.7 METs to 8.5+/-1.8 METs, p=0.005), and symptoms improved significantly following therapy, with a reduction in mean NYHA class from 1.9+/-0.5 to 1.0+/-0.0 (p=0.001). CONCLUSIONS: Negative inotropic therapy may represent a beneficial therapeutic approach in selected patients with dynamic left ventricular outflow tract obstruction induced by exercise and identified during exercise echocardiography.

Adrenergic beta-Antagonists↗

Edge-to-edge technique to treat post-mitral valve repair systolic anterior motion and left ventricular outflow tract obstruction.

BACKGROUND: Systolic anterior motion of the mitral valve causing left ventricular outflow tract obstruction is an uncommon complication of mitral valve repair that may necessitate immediate additional surgical action. We prospectively evaluated the technique of the edge-to-edge suture on post-mitral repair systolic anterior motion, which persisted despite conservative treatment. METHODS: From March 2002 to March 2004, 4 of 112 patients requiring mitral valve repair surgery for chronic degenerative mitral regurgitation had systolic anterior motion with severe left ventricular outflow tract obstruction and mitral regurgitation. All 4 patients (mean age, 50 years) had posterior leaflet prolapse with chordal rupture with a billowing anterior leaflet, but without chordal rupture. Repair was achieved through a quadrangular resection of the posterior leaflet, completed by plication of the annulus in 2 patients and leaflet sliding in the other 2. All patients had mitral annuloplasty; two patients had a complete CE Physio ring (Edwards Lifesciences, Irvine, CA) inserted, whereas the other 2 patients had an open CG Future band (Medtronic, Minneapolis, MN). Routine perioperative transesophageal echocardiography showed systolic anterior motion, severe left ventricular outflow tract obstruction (> 50 mm Hg), and mitral regurgitation. After resuming cardiopulmonary bypass, all patients had an edge-to-edge suture at the middle part of the free edge of the anterior and posterior leaflets. RESULTS: The control transesophageal echocardiography showed in all cases disappearance of the systolic anterior motion, of the left ventricular outflow tract obstruction and of mitral regurgitation. Mean follow-up was 14 months (range, 6 to 28 months). All patients were in New York Heart Association's functional class I. CONCLUSIONS: With the edge-to-edge repair, the early and 2-year results were satisfactory with total disappearance of the systolic anterior motion, of the left ventricular outflow tract obstruction and of the recurrent mitral regurgitation.

Cardiopulmonary Bypass↗

Left ventricular outflow tract obstruction in complete transposition of the great arteries with intact ventricular septum. A cross sectional echocardiography study.

The roles of posterior bulging of the interventricular septum (septal bulge) and of systolic septal mitral apposition in patients with simple transposition of the great arteries are not known. Cross sectional echocardiograms of 40 such patients were reviewed (after exclusion of those with fixed left ventricular outflow tract obstruction) and haemodynamic findings were compared with long and short axis measurements within the left ventricle. There was no significant correlation between the degree of septal bulge and systolic gradient across the left ventricular outflow tract, but septal bulge correlated weakly with systolic right ventricular pressure and inversely with pulmonary arteriolar resistance index. Systolic left ventricular outflow gradient was inversely related to the minimum systolic distance between the anterior mitral leaflet and interventricular septum. No patients without complete systolic apposition of the anterior mitral leaflet and interventricular septum had a left ventricular outflow gradient greater than 20 mm Hg. Conversely, even when cross sectional echocardiography showed apparently total obstruction of the left ventricular outflow tract at some time in systole there was often no significant gradient detected during haemodynamic study. In the short axis cuts closeness of the papillary muscles to the interventricular septum or to each other was unrelated to systolic gradient. This study shows that (a) cross sectional echocardiography can identify fixed obstruction of the left ventricular outflow tract in simple transposition of the great arteries; (b) the degree of septal bulge, unless complicated by fibrous thickening of the anterior mitral leaflet and interventricular septum, is unrelated to the gradient across the left ventricular outflow tract; (c) the absence of systolic septal/mitral apposition excludes a significant gradient at that site across the left ventricular outflow tract; and (d) papillary muscle geometry is unrelated to dynamic gradients across the left ventricular outflow in this condition.

Coronary Circulation↗

Coarctation of the aorta in early infancy. Left ventricular outflow tract obstruction and its clinical implications.

In order to clarify the clinical significance of left ventricular outflow tract obstruction, 11 infants with coarctation of the aorta and ventricular septal defect were subjected to cineangiography (or autopsy) analysis. Three infants without left ventricular outflow tract obstruction were salvaged by aortoplasty using a subclavian flap or a Dacron patch with concomitant pulmonary artery banding. The remaining 8 infants died surgically or nonsurgically, and were confirmed retrospectively to have had left ventricular outflow tract obstruction. It seems worthwhile to stress that the presence of an abnormal muscle bundle in the left ventricle can be demonstrated angiographically, especially by the axial technique. From our present study, though the number of cases examined is limited, we have an impression that left ventricular outflow tract obstruction may be a determining factor with regard to the appropriateness of pulmonary artery banding in the two-stage operation for infantile coarctation associated with a large ventricular septal defect.

Aortic Coarctation↗

Dynamic left ventricular outflow tract obstruction in a patient with pheochromocytoma.

Symmetric left ventricular hypertrophy or asymmetric septal hypertrophy associated with pheochromocytoma simulating hypertrophic obstructive cardiomyopathy have been rarely reported. In this report, we present a case with pheochromocytoma that had dynamic left ventricular outflow tract obstruction without asymmetric septal hypertrophy. A surface echo revealed resolution of the systolic anterior motion of the mitral valve and all Doppler evidence of left ventricular outflow tract obstruction following removal of the tumor. Dynamic left ventricular outflow tract obstruction seen in this patient was probably due to excessive secretion of cathecolamines by the tumor.

Adrenal Gland Neoplasms↗