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Provocation of left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy. Comparison of orthostasis testing and nitrate application.

Several provocation maneuvers are described in hypertrophic cardiomyopathy to Doppler echocardiographically distinguish the obstructive from the non obstructive type. No data are available about the value of orthostasis testing in comparison with nitrate application in this disease. In this study, 16 consecutive patients with hypertrophic cardiomyopathy were examined. 11 patients with hypertrophic cardiomyopathy were classified as obstructive, 5 patients with hypertrophic cardiomyopathy as non obstructive. Normal left ventricular outflow tract velocities as detected by the Doppler method were defined as < 2.0 m/s. Doppler echocardiographic measurements were performed after 10 minutes in supine position, within 10 minutes after head-up tilt and again, within 10 minutes in supine position. If systolic blood pressure during this examination exceeded 100 mm Hg 2.5 mg isosorbiddinitrate were sprayed sublingually. Measurements were done after 20 minutes in supine position and within 10 minutes after head-up tilt. Only in 7 of the 11 patients with hypertrophic obstructive cardiomyopathy maximal left ventricular outflow tract velocity in supine position measured > 2.0 m/s (2.2 +/- 0.8). During head-up tilt, all patients showed increased values (3.8 +/- 1.2 m/s). No differences in maximal left ventricular outflow tract velocity between head-up tilt and nitrate application in supine position (3.5 +/- 1.4 m/s) were present. All patients with hypertrophic non obstructive cardiomyopathy showed maximal left ventricular outflow tract velocities < 2.0 m/s in every step of the examination. Consequently, orthostasis testing was able to identify all patients with hypertrophic obstructive cardiomyopathy and demonstrated a diagnostic value similar to nitrate application.

Adult↗

[Ventricular outflow tract obstruction by cardiac tumors during ACTH therapy of an infant with tuberous sclerosis].

In an infant with tuberous sclerosis and West syndrome, ACTH treatment was interrupted because of augmentation of the biventricular outflow tract obstruction. A 5-month-old boy, who had been diagnosed to have multiple cardiac tumors since in utero, manifested West syndrome with typical hypsarhythmia in EEG. Several days after starting low dose ACTH treatment, a systolic murmur due to biventricular outflow tract obstruction intensified with ventricular arrhythmia. A tumor in the left ventricular outflow tract was enlarged. Discontinuation of daily administration of ACTH and administration of a beta-blocking agent improved the cardiac problems. Acceleration of blood velocity in the outflow tracts disappeared two months later. Intracardiac morphology and hemodynamic performance should be monitored to avoid critical complications during ACTH treatment in patients with tuberous sclerosis.

Adrenocorticotropic Hormone↗

Septal myectomy for left ventricular outflow tract obstruction after mitral valve repair.

We describe a patient with a left ventricular outflow tract obstruction after mitral valve repair. Intraoperative transesophageal echocardiography permitted us to recognize the role of a bulging septum in the development of a systolic motion of the mitral valve. A left ventricular septal myectomy and myotomy was able to relieve the left ventricular outflow tract obstruction and the systolic anterior motion of the mitral valve.

Heart Septum↗

Left ventricular outflow tract obstruction with mitral valve replacement in small ventricular cavities.

The inference that mitral valve replacement (MVR) may produce left ventricular outflow tract (LVOT) obstruction has been made, but no comparative hemodynamic studies with various types of prostheses have been done. The purpose of the present study was to compare the gradients created across the LVOT with MVR in young sheep with small left ventricular cavities. Mitral valve replacement was accomplished using cardiopulmonary bypass and hypothermic cardioplegic arrest. Five animals were used for each of the following valves studied: 25-mm Ionescu-Shiley bovine pericardial valve, 25-mm Hancock porcine aortic valve, 2M-6120 28-mm Starr-Edwards ball-valve prosthesis, 25-mm Björk-Shiley 60-degree flat tilting-disc prosthesis, and 25-mm St. Jude Medical hemidisc valve. Gradients across the LVOT were measured after MVR and then during infusion of isoproterenol hydrochloride (0.05 micrograms/kg/min). Following MVR, only the Starr-Edwards valve produced an LVOT gradient (32 +/- 23 mm Hg). Substantial gradients after MVR were seen, however, with isoproterenol administration with the Ionescu-Shiley (47 +/- 4 mm Hg), Hancock (13 +/- 8 mm Hg), and Starr-Edwards (65 +/- 30 mm Hg) valves but not with the low-profile valves (Björk-Shiley and St. Jude Medical). The results of the present study demonstrate that MVR can produce LVOT obstruction. The greatest degree of obstruction was with the high-profile mechanical and bioprosthetic valves.

Animals↗

[Sigmoid septum causing left ventricular outflow tract obstruction: a case report].

A 67-year-old man with a sigmoid septum causing the left ventricular outflow obstruction by inotropic stimulation was reported. This patient was admitted to the Hospital of the University of Tsukuba because of chest pain. Phonocardiography revealed a systolic ejection murmur which was intensified by amyl nitrite inhalation. A carotid pulse tracing showed a mid-systolic dip and a secondary slow wave during amyl nitrite inhalation. M-mode echocardiography demonstrated neither systolic anterior motion of the mitral valve (SAM) nor mid-systolic closure of the aortic valve at rest. Two-dimensional echocardiography revealed a basal interventricular septum markedly protruding into the left ventricle (sigmoid septum). The remainder of the septum and the left ventricular free wall were not hypertrophied, and no enlargement of the left ventricular cavity was observed. During exercise tests, blood pressure dropped significantly. Cardiac catheterization showed a pressure gradient within the left ventricle with isoproterenol infusion and post-extrasystolic potentiation. These findings suggest that left ventricular outflow tract obstruction could occur in a patient with sigmoid septum by inotropic stimulation, producing a fall of blood pressure during exercise.

Aged↗

Left ventricular outflow tract obstruction due to valve myxoma.

A life-threatening left ventricular outflow tract obstruction developed in a 26-year-old man because of a very uncommon myxoma emerging from the ventricular side of the mitral valve. Immediate surgical treatment was indicated because of high-risk factors reported herein. After transaortic resection of a single myxoma, the progress of this patient has been excellent, especially as a familial myxoma could be excluded.

Adult↗

Thrombus causing fatal left ventricular outflow tract obstruction in a heart transplant patient.

A 60 year male, orthotopic heart transplant recipient developed a fatal left ventricular outflow obstruction secondary to thrombus at 38 months post transplant. Although he had episodes of mild to moderate rejection at 2 and 16 months post transplant, subsequent biopsies were negative and annual cardiac catheterizations showed mild left ventricular hypokinesis and normal coronary arteries. This case represents a catastrophic complication of transplant rejection and illustrates the problems with identifying rejection using current diagnostic methods.

Coronary Angiography↗

One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction.

BACKGROUND: Surgical repair of obstructive lesions of the right ventricular outflow tract (RVOT) in children commonly creates pulmonary valve incompetence that may eventually require pulmonary valve replacement (PVR). We reviewed our experience with PVR late after RVOT reconstruction. METHODS: We performed 100 PVRs in 93 children 1.1 months to 22.4 years (median 8) after RVOT reconstruction. Children with right ventricular to pulmonary artery conduits and primary PVRs were excluded. Age at PVR was 4.5 months to 27.9 years (median 9.5 years). Initial diagnosis was tetralogy of Fallot and variants, 62; critical pulmonary stenosis, 15; pulmonary atresia with intact ventricular septum, 7; and others, 9. Eleven patients had a redo PVR. A total of 62 PVRs were homografts; 38 were porcine valves. RESULTS: There was one early death. On follow-up of 5 months to 12.4 years (mean 4.9 years) there were no late deaths although 1 child underwent cardiac transplantation. Actuarial freedom from redo PVR at 8 years was 100% for porcine valves but 70% for homograft valves (p = 0.17). For children younger than 3 years at PVR, freedom from reoperation was 76% at 1 year and 39% at 8 years compared with freedom from redo PVR at 8 years of 100% for children older than 3 years. On latest echocardiogram 97% of porcine valves had mild or no pulmonary regurgitation compared with 72% of homograft valves. CONCLUSIONS: PVR after RVOT reconstruction can be performed with low risk. Porcine valves may be superior to homograft valves although this advantage may be due to older age at time of PVR.

Adolescent↗

Echocardiographic diagnosis of left ventricular outflow tract obstruction after mitral valve replacement with subvalvular preservation.

We describe two cases of left ventricular outflow tract obstruction after mitral valve replacement with complete retention of the subvalvular apparatus. The first patient deteriorated immediately after insertion of a high-profile bioprosthesis. In the second patient, chronic left ventricular outflow tract obstruction developed after the insertion of a low-profile mechanical prosthesis. The clinical course of left ventricular outflow tract obstruction after mitral valve replacement with complete retention of the subvalvular apparatus may differ greatly. Evaluation of the left ventricular outflow tract by perioperative transesophageal echocardiography or epicardial echocardiography is essential in the prevention and treatment of this complication.

Aged↗

Physiological and clinical consequences of relief of right ventricular outflow tract obstruction late after repair of congenital heart defects.

BACKGROUND: Right ventricular outflow tract obstruction (RVOTO) is a common problem after repair of congenital heart disease. Percutaneous pulmonary valve implantation (PPVI) can treat this condition without consequent pulmonary regurgitation or cardiopulmonary bypass. Our aim was to investigate the clinical and physiological response to relieving RVOTO. METHODS AND RESULTS: We studied 18 patients who underwent PPVI for RVOTO (72% male, median age 20 years) from a total of 93 who had this procedure for various indications. All had a right ventricular outflow tract (RVOT) gradient >50 mm Hg on echocardiography without important pulmonary regurgitation (less than mild or regurgitant fraction <10% on magnetic resonance imaging [MRI]). Cardiopulmonary exercise testing, tissue Doppler echocardiography, and MRI were performed before and within 50 days of PPVI. PPVI reduced RVOT gradient (51.4 to 21.7 mm Hg, P<0.001) and right ventricular systolic pressure (72.8 to 47.3 mm Hg, P<0.001) at catheterization. Symptoms and aerobic (25.7 to 28.9 mL.kg(-1).min(-1), P=0.002) and anaerobic (14.4 to 16.2 mL.kg(-1).min(-1), P=0.002) exercise capacity improved. Myocardial systolic velocity improved acutely (tricuspid 4.8 to 5.3 cm/s, P=0.05; mitral 4.7 to 5.5 cm/s, P=0.01), whereas isovolumic acceleration was unchanged. The tricuspid annular velocity was not maintained on intermediate follow-up. Right ventricular end-diastolic volume (99.9 to 89.7 mL/m2, P<0.001) fell, whereas effective stroke volume (43.7 to 48.3 mL/m2, P=0.06) and ejection fraction (48.0% to 56.8%, P=0.01) increased. Left ventricular end-diastolic volume (72.5 to 77.4 mL/m2, P=0.145), stroke volume (45.3 to 50.6 mL/m2, P=0.02), and ejection fraction (62.6% to 65.8%, P=0.03) increased. CONCLUSIONS: PPVI relieves RVOTO, which leads to an early improvement in biventricular performance. Furthermore, it reduces symptoms and improves exercise tolerance. These findings have important implications for the management of this increasingly common condition.

Adolescent↗

Isolated thrombus-producing right ventricular outflow tract obstruction: an unusual presentation of primary antiphospholipid antibody syndrome.

Right ventricular outflow tract obstruction secondary to an isolated organized thrombus is a rare entity. Proper diagnosis of this condition is essential for the survival of such patients. We describe the case of a middle aged woman who presented with dyspnea and decreased effort tolerance, and was managed with surgical excision of a firm mass which was identified as organized thrombus.

Adult↗

Left ventricular outflow tract obstruction due to mitral annulus calcification in patient with mild concentric left ventricular hypertrophy.

The combination of the systolic anterior motion of the anterior leaflet of the mitral valve and/or the mitral annular calcification in the case of asymmetrical septal hypertrophy has been fully recognized. However, in concentric left ventricular hypertrophy the systolic anterior motion of the anterior mitral valve and the massive posterior submitral calcification have not been commonly reported. We present a case with mild concentric left ventricular hypertrophy and massive posterior submitral calcification which displace the entire mitral ring anteriorly, namely, toward the left ventricular outflow tract. In this case, typical left ventricular outflow obstruction with systolic anterior motion of the anterior mitral valve was seen. Thus, we considered that this rare condition may have contributed to the formation of the systolic anterior motion of the anterior mitral leaflet in this case. We have provided additional information regarding the possible causes of systolic outflow obstruction in hypertrophic obstructive cardiomyopathy.

Aged↗

Left ventricular outflow tract obstruction in TGA: treatment with LV-to-PA valved conduit.

Progressive or recurrent left ventricular outflow tract obstruction after a previous Mustard or Senning operation represents a rare but challenging problem. The obstruction can be resected in some patients, but abnormal attachment of the mitral valve or a long fibromuscular tunnel represents a difficult surgical problem. Between 1979 and 1993, we encountered this type of left ventricular outflow tract obstruction in 10 patients, 4 to 13 years after the atrial repair. They ranged in age from 5 to 15 years (mean, 8.8 years) and weighed between 11.5 and 47 kg (mean, 25.3 kg). Operations were performed through a left thoracotomy with the patient on hypothermic cardiopulmonary bypass but without aortic cross-clamping. The left atrial appendage and descending aorta were cannulated. Good relief of the gradient was obtained in all patients (mean residual gradient, 14.8 mm Hg). All patients survived the operation. One patient died suddenly at home 6 months later; 2 patients required conduit replacement. All 9 long-term survivors are asymptomatic as of 6 months to 8 years after their conduit placement or replacement. We recommend the placement of a left ventricle-to-pulmonary artery valved conduit for the relief of severe left ventricular outflow tract obstruction arising after a Senning or Mustard operation that cannot be managed by other means.

Adolescent↗

Use of the Alfieri edge-to-edge technique to eliminate left ventricular outflow tract obstruction caused by mitral systolic anterior motion.

A 68-year-old woman with concentric left ventricular hypertrophy, prosthetic valve endocarditis with aortic root abscess, and sepsis had aortic root replacement with an aortic allograft. On weaning from cardiopulmonary bypass, she had hemodynamic instability caused by systolic anterior motion of the mitral valve, which resulted in a left ventricular outflow tract obstruction; the peak pressure gradient across the left ventricular outflow tract was 130 mm Hg, and there was moderately severe (3+) mitral regurgitation. After reinstitution of cardiopulmonary bypass, a central Alfieri edge-to-edge stitch was placed between the anterior and posterior leaflets of the mitral valve. This reduced the gradient across the left ventricular outflow tract to 10 mm Hg and eliminated the mitral regurgitation, which enabled successful separation from cardiopulmonary bypass.

Aged↗

Doppler echocardiographic effects of medetomidine on dynamic left ventricular outflow tract obstruction in cats.

OBJECTIVE: To evaluate the effects of medetomidine on dynamic left ventricular outflow tract (LVOT) obstruction in cats with left ventricular hypertrophy. DESIGN: Clinical trial. ANIMALS: 6 domestic shorthair cats with echocardiographic evidence of dynamic LVOT obstruction. PROCEDURE: Cats were restrained in lateral recumbency, and baseline M-mode and Doppler echocardiographic examinations were performed. An ECG was recorded continuously, and blood pressure was measured indirectly with Doppler instrumentation. Medetomidine (20 microg/kg 19.1 microg/lb]) was then administered i.m., and examinations were repeated 15 minutes later. RESULTS: Significant decreases in heart rate, LVOT velocity, and the LVOT pressure gradient were documented following medetomidine administration. After adjusting for the effects of heart rate by ANCOVA, there were no significant differences in any other systolic or diastolic indices of left ventricular function. CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that administration of medetomidine to cats with dynamic LVOT obstruction may result in elimination of outflow tract obstruction; medetomidine may be a suitable sedative and analgesic agent in this subpopulation of cats.

Animals↗

Management of complex left ventricular outflow tract obstruction with pulmonary autografts.

The surgical relief of complex multilevel left ventricular outflow tract (LVOT) obstruction remains a challenging surgical problem. We present a new operation which combines the concepts of aortoventriculoplasty, extended aortic root replacement, and the use of a pulmonary autograft. Sixteen patients underwent this operation: 11 patients after previous attempts to relieve diffuse subvavalvular stenosis and 5 patients who presented excessive gradients over a previous aortic valve prosthesis. All patients except one survived the operation. One patient developed massive right ventricular infarction and underwent transplantation after 5 days. Another patient developed complete heart block and transient tricuspid regurgiation after a septal infarction. One patient remained in congestive heart failure and died suddenly after 17 months. All other patients are in NYHA class I after a mean follow-up of 23 +/- 12 months. One patient developed progressive dilatation of the neo-aortic root and underwent reoperation after 4 years. All other patients showed laminar flow in the LVOT and excellent function of the autograft and homograft valve at follow-up. This operation offers a more durable or even a definitive solution in the management of these complex left ventricular outflow tract obstructions.

Adolescent↗

Right ventricular outflow tract obstruction secondary to small intestinal lymphoma.

A 71-year-old man developed symptoms and signs of right ventricular outflow obstruction. After angiography, which showed multiple lobulated and well-defined filling defects in the right ventricular outflow tract, endomyocardial biopsy disclosed infiltration of the myocardium by a poorly differentiated malignant neoplasm. At necropsy he was found to have a small intestinal lymphoma.

Aged↗