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[Muscular obstruction of the right ventricular outflow tract. Subvalvular pulmonary stenosis or hypertrophic cardiomyopathy?].

A case is reported of right ventricular outflow obstruction caused by hypertrophic cardiomyopathy. The pressure gradient between the inflow tract and the outflow tract of the right ventricle was 84 mm Hg. Resection of the hypertrophic muscle bundles practically eliminated the obstruction leaving only an insignificant subvalvular gradient of 2 mm Hg. Histologic examination revealed cardiac muscle cell hypertrophy and disorganization.

Adult↗

Anomalous papillary muscle producing dynamic left ventricular outflow tract obstruction.

A patient with a dynamic left ventricular outflow murmur was shown to have an anomalous anterolateral papillary muscle by transthoracic and transesophageal two-dimensional Doppler echocardiography. High late systolic outflow velocity and systolic anterior motion were demonstrated in the lateral outflow tract near the anomalous muscle, suggesting a role for the Venturi effect. This is the first confirmation by Doppler echocardiography of an anomalous papillary muscle contributing to abnormal left ventricular hemodynamics.

Adult↗

Accessory mitral valve tissue causing left ventricular outflow tract obstruction.

Among the various causes of left ventricular outflow tract obstruction accessory mitral valve tissue is the least common. To the best of our knowledge there have been only five such cases reported to date. The aim of presenting two more cases and reviewing the literature is to bring attention to this rare anomaly as it is easy to treat provided it has been diagnosed preoperatively or recognised at surgery.

Child↗

Mechanisms for recurrent left ventricular outflow tract obstruction after septal myectomy for obstructive hypertrophic cardiomyopathy.

BACKGROUND: Left ventricular septal myectomy provides excellent outcomes for symptomatic patients with severe obstructive hypertrophic cardiomyopathy. However, in a few patients, left ventricular outflow tract obstruction may recur and require repeat myectomy. We reviewed this subset of patients to assess the mechanisms of recurrence. METHODS: From 1975 to July 2003, 610 septal myectomies were performed; 13 of these were repeat myectomies after classic myectomies performed at our institution (n = 6) or elsewhere (n = 7). Ages ranged from 4 to 70 years (mean, 32 +/- 22). The interval between initial myectomy and repeat myectomy ranged from 13 months to 11 years (mean, 5.0 +/- 3.7 years). RESULTS: Mechanisms for obstruction included limited myectomy at the initial myectomy (n = 11), septal hypertrophy at the midventricular level (n = 8), and anomalous papillary muscles (n = 3). Mean intraoperative pressure gradients decreased from 82 +/- 24 to 6.2 +/- 4.4 mm Hg. No mitral valve replacement was performed, and there were no early deaths. Mean follow-up was 5.8 +/- 5.8 years. There was one late death. All surviving patients were free from recurrence of outflow tract obstruction and were in the New York Heart Association functional class I or II. CONCLUSIONS: Mechanisms for recurrent obstruction included limited myectomy at the initial operation, midventricular obstruction, anomalies of papillary muscles, and ventricular remodeling, especially in pediatric patients. Repeat myectomy can be performed with excellent outcomes. Need for reoperation may be reduced with current surgical approaches that include a more extended resection of the midventricular septum, relief of papillary muscle anomalies, and routine use of intraoperative transesophageal echocardiography.

Adolescent↗

Accessory mitral valve leaflet causing aortic regurgitation and left ventricular outflow tract obstruction. Case report and review of published reports.

Arrhythmias, aortic regurgitation, and symptoms of severe intermittent ventricular outflow obstruction developed in a 14 year old boy with a heart murmur who had been followed from infancy. These were caused by an accessory mitral leaflet, which was successfully removed at open heart operation. A review of 21 previously reported cases found a high incidence of associated cardiac malformations, appreciable subaortic obstruction in most patients, and a consistent attachment of the accessory tissue to the ventricular aspect of the anterior mitral leaflet. The characteristic echocardiographic appearance of a mobile mass arising from the area of aortic-mitral continuity is sufficient for the diagnosis of accessory mitral leaflet and echocardiographic examination will facilitate the surgical management of this condition.

Adolescent↗

Extending the concept of the autograft for complete repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: a report of ten cases of a modified procedure.

BACKGROUND: In most cases of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, a Lecompte procedure (réparation à l'étage ventriculaire) is possible without interposition of a conduit between the right ventricle and pulmonary artery. However, the anterior location of the pulmonary arteries after the Lecompte maneuver may be a potential cause for right ventricular outflow obstruction, which continues to be reported in 5% to 25% of cases. We have used a tubular segment of aortic autograft to connect the pulmonary artery, left in the orthotopic posterior position (without the Lecompte maneuver), to the right ventricle in 10 consecutive patients with transposition, ventricular septal defect, and left ventricular outflow tract obstruction. METHODS: Ten consecutive patients aged 2 months to 11 years (mean 32 months) have undergone a modified Lecompte operation. Eight had severe pulmonary stenosis, two had pulmonary atresia, and four had a restrictive ventricular septal defect at the time of the operation. Two had multiple ventricular septal defects. Seven had undergone one (n = 5) or two (n = 2) previous modified Blalock-Taussig shunts. All patients underwent a total correction with left ventricular-aortic intraventricular connection (four needed a ventricular septal defect enlargement), connection between the right ventricle and pulmonary arteries with a tubular segment of autograft aorta, without the Lecompte maneuver (anterior location of the bifurcation of the pulmonary arteries) on the right (n = 6) or the left (n = 4) of the aorta. No valvular device was used for the right ventricular outflow repair. RESULTS: No early or late deaths occurred. One patient with multiple ventricular septal defects needed an early (2 weeks) reoperation for a residual muscular ventricular septal defect. All patients are currently in New York Heart. Association class I, without medications, in sinus rhythm, at a mean follow-up of 30 months. Late results up to 3.6 years show no calcification on the chest roentgenogram, and at the most recent echocardiogram, right ventricular pressures were low (25 to 40 mm Hg, mean 33 mm Hg) and no significant gradient (over 10 mm Hg) was found between the right ventricle and pulmonary arteries. Left and right ventricular function was satisfactory. CONCLUSION: This modification of the Lecompte operation using a segment of autograft allows an excellent early and late result, with no danger of compression of anteriorly placed pulmonary arteries, no significant right ventricular outflow obstruction, and normal appearance of the tubular autograft. In view of laboratory and clinical evidence, normal growth of the autograft can be anticipated. It allows an elective correction of transposition, ventricular septal defect, and left ventricular outflow tract obstruction without a previous Blalock-Taussig shunt (three patients) and correction at a young age (three patients younger than 1 year).

Aorta↗

Tunnel subaortic stenosis: left ventricular outflow tract obstruction produced by fibromuscular tubular narrowing.

The clinical and morphologic features of tunnel subaortic stenosis, an unusual form of obstruction to left ventricular outflow, are described in 11 patients. Although patients with tunnel subaortic stenosis demonstrate a variety of cardiovascular malformations, the most characteristic anatomic feature is fibromuscular tubular narrowing of the outflow tract that remains relatively unchanged during the cardiac cycle. The aortic anulus was abnormally small in six of the 11 patients, including one who also had a hypoplastic ascending aorta. Evidence of a small mitral orifice was present in two patients, and two other patients had asymmetric septal hypertrophy. Although operation was successful in significantly reducing the outflow gradient in two of the seven operated patients, all seven patients had gradients of 50 mm Hg or more at the most recent postoperative evaluation. Three patients (two with previous operation) died suddenly; each of these patients had mild or no symptoms. Because of the apparent ineffectiveness of current operative methods in patients with tunnel subaortic stenosis, it is important to differentiate this condition from obstructions to left ventricular outflow.

Adolescent↗

Modified Damus-Kaye-Stansel procedure using aortic flap technique for systemic ventricular outflow tract obstruction in functionally univentricular heart.

Damus-Kaye-Stansel procedure is a useful method to relieve the systemic ventricular outflow tract obstruction in functionally univentricular heart. Regurgitation of pulmonary valve and recurrence of systemic ventricular outflow obstruction are the major concerns at the late phase of this procedure. Modification of original Damus-Kaye-Stansel procedure that can prevent the use of prosthetic materials is evaluated. The modified Damus-Kaye-Stansel procedure using aortic flap technique was performed in eight patients with functionally univentricular heart. Patients' ages ranged from 3 to 28 months (mean 14 months). Follow-up period was 37 months as a mean (9-71 months), and the follow-up was complete. There was no operative mortality and no late death. In addition, there was no recurrence of systemic ventricular outflow tract obstruction throughout the follow-up period. Regurgitation of the pulmonary valve estimated by echocardiography at the latest follow-up was none to trivial in seven patients and mild in one. The modified Damus-Kaye-Stansel procedure using aortic flap technique is a safe, useful and reproducible technique to solve systemic ventricular outflow tract obstruction in functionally univentricular heart, and it can be an alternative for original technique or the so-called double-barrel modification.

Anastomosis, Surgical↗

Long-term results of apical aortic conduits in children with complex left ventricular outflow tract obstruction.

BACKGROUND: Apical aortic conduits were used in children with complex left ventricular outflow tract obstruction at our institution before we adopted the Ross and Ross-Konno procedures. The early results with apical aortic conduits were quite good when we used a porcine xenograft conduit and were less satisfactory when we converted to an aortic homograft valve as the conduit valve. This report summarizes our clinical experience with apical aortic conduits in children with an emphasis on hemodynamic results, reoperation, and long-term follow-up. METHODS: Records of 28 patients (age range, 2 weeks to 19 years) who underwent insertion of apical aortic conduit between September 1979 and June 1993 were reviewed. All patients had complex multilevel left ventricular outflow tract obstruction. All were symptomatic, and 22 (79%) had had one or two previous aortic valvotomies or left ventricular outflow tract operations, or both. RESULTS: Hospital mortality was 11% (3 of 28). Twenty-five children survived the perioperative period and improved, and 21 have had one or more cardiac catheterization from 6 to 18 months (mean, 1.2 years) after the initial operation. Reduction or resolution of resting mean left ventricular-to-aortic peak gradient in the early postoperative period from 81.8 +/- 24.0 to 15.4 +/- 8.9 mm Hg was demonstrated (p < 0.001). Overall 25-year survival was 57%. Fourteen surviving patients (56%) have undergone subsequent procedures (n = 18) from 5 months to 16 years postoperatively (mean, 6.9 years) because they developed a recurrent left ventricular-to-aortic gradient of 58 +/- 28 mm Hg (p < 0.002). One patient underwent heart transplantation. All other late survivors have normal left ventricular function as determined by serial echocardiography. CONCLUSIONS: Apical aortic conduit is effective in relieving complex left ventricular outflow tract obstruction and improving left ventricular performance with acceptable short-term and midterm results, but late complications caused primarily by conduit tissue valve dysfunction are frequent in children. Since the early 1990s, the apical aortic conduit procedure has been largely replaced with the Ross or Ross-Konno procedure in our pediatric practice.

Adolescent↗

Left ventricular outflow tract obstruction after mitral valve replacement.

We describe a patient with left ventricular outflow tract obstruction after mitral valve replacement preserving the anterior subvalvular apparatus. Postoperative transesophageal echocardiography demonstrated systolic narrowing of the left ventricular outflow tract by a bulging septum and systolic anterior motion of the preserved anterior mitral leaflet. Septal myectomy and transaortic mitral apparatus resection enabled us to relieve the left ventricular outflow tract obstruction. This suggests that septal hypertrophy might be a relative contraindication to the preservation of the anterior mitral subvalvular apparatus in mitral replacement.

Aged↗

Liposarcoma causing left ventricular outflow tract obstruction and syncope: a case report and review of the literature.

Metastatic cardiac myxoid liposarcoma is rare. We present the case of a 56-year-old woman who was admitted for angina and syncope, and in whom a mobile intracardiac mass obstructing the left ventricular outflow tract and protruding into the aortic root during systole was detected by transesophageal echocardiography (TEE). She reported an antecedent of the complete excision of a myxoid liposarcoma of the thigh 12 years before. A surgical resection procedure was performed through an excision of the anterior wall of the left ventricle. The histopathological analysis of the specimens obtained during surgery revealed a low-grade malignancy myxoid liposarcoma, similar to the one resected in the thigh 12 years before. The present case is the first one to report syncope due to left ventricular outflow tract as a cause for admission. TEE was able to visualize the metastatic tumor to the heart, accurately localize its position and allow for prompt surgical treatment that produced relief of the symptoms.

Angina Pectoris↗

Transesophageal echocardiographic monitoring of critical left ventricular outflow tract obstruction following mitral xenograft replacement--a case report.

Left ventricular outflow tract obstruction (LVOTO) is a critical complication after mitral bioprosthesis. Intraoperative transesophageal echocardiographic (TEE) monitoring enables early diagnosis and guides prompt subsequent treatment. Here we report a case of mitral heart disease who developed critical LVOTO following mitral xenograft replacement. The values of intraoperative TEE in the disclosure of the LVOTO and its facilitation of successful management are discussed.

Animals↗