Accessory Mitral Valve Tissue Research Articles

Left ventricular outflow tract obstruction in the presence of asymmetric septal hypertrophy and accessory mitral valve tissue treated with alcohol septal ablation

Redundant or accessory mitral valve tissue (AMVT) is a rare clinical condition. It is an even rarer cause of left ventricular outflow tract obstruction. We report a case of an adult male with medically unresponsive hypertrophic obstructive cardiomyopathy in whom real-time three-dimensional transesophageal echocardiography was used to both diagnose the presence of coexistent asymmetric septal hypertrophy and AMVT as well as confirm the safety and efficacy of treatment with alcohol septal ablation.

A potential source of cardioembolic cerebrovascular event: Accessory mitral valve tissue

Accessory mitral valve is an extremely rare congenital cardiac anomaly which can cause a variety of clinical features. Most of the cases reported in the literature were associated with left ventricular outflow tract obstruction. The clinical signs and symptoms of accessory mitral valve tissue vary according to its location and coexisting cardiac malformations.

Functional systolic anterior motion of the mitral valve due to accessory chordae

Accessory mitral valve tissue is an uncommon finding, usually associated with other congenital cardiac malformations and diagnosed during childhood. We present the case of a 70-year-old patient referred to our hospital for aortic valve and ascending aorta replacement. At preoperative examination, no evidence of mitral valve structural abnormalities was detected. Anaesthesia induction was complicated by sudden haemodynamic impairment. Intraoperative transoesophageal echocardiography showed two anomalous mitral chordae. The presence of abnormal mitral tissue also allowed to assess the mechanism of unexpected haemodynamic worsening: anomalous chordae caused anterior displacement of the anterior mitral leaflet, resulting in left ventricular outflow tract obstruction and mitral regurgitation. Medical therapy was instituted and the planned surgical approach was modified according to the new anatomy observed at echocardiographic examination. A valve-sparing technique was successfully applied. This case provides a good example of the diagnostic usefulness of routine intraoperative transoesophageal echocardiography, especially when facing unexpected findings. It also shows how transoesophageal echocardiography may help guide medical and surgical treatment.

Dynamic mild subaortic left ventricular obstruction caused by an accessory mitral valve attached to the anterior mitral valve in a young pregnant woman

Accessory mitral valve tissue is an extremely rare congenital cardiac anomaly of embryologic development of the endocardial cushion. This anomaly is often associated with left ventricular outflow tract obstruction (LVOTO). A 26-year-old pregnant female was referred to our Department of Cardiology with exertional shortness of breath and tachycardia. Transthoracic and transesophageal echocardiography revealed a flexible circular (1.3 x 1.4 cm), mobile structure attached to the ventricular side of anterior mitral valve leaflet, with chordal attachments structure from anterior papillary muscle. This picture is compatible with a parachute-like accessory mitral valve tissue. We performed an echocardiographic exercise test that shows a systolic flow turbulence starting immediately proximal to this structure, resulting in a small increase in left ventricular outflow tract (LVOT) gradient (30 mmHg). Therefore we started low dose of beta-blocker therapy in order to decrease heart frequency and reduce the future risk of a worsening of an LVOT dynamic obstruction. Transthoracic and transesophageal echocardiography is critical for the differential diagnosis of LVOT and in the management of accessory mitral valve tissue. In patients without rest and only an exertional mild LVOTO and no other cardiac malformations, prophylactic removal of mitral accessory tissue excision is not required; antibiotic prophylaxis for endocarditis can be indicated and a regular follow-up is recommended to identify any progression in LVOTO entity.

The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study☆

To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.

Accessory mitral valve tissue

Accessory mitral valve tissue is a rare congen- ital malformation. It can be found isolated or in combination with other cardiac anomalies and is usually detected in early childhood. We describe the case of a 74-year-old asympto- matic patient who was referred to our clinic for the evaluation of a heart murmur.

Left Ventricular Outflow Obstruction

Left ventricular outflow tract obstructions (LVOTOs) encompass a series of stenotic lesions starting in the anatomic left ventricular outflow tract (LVOT) and stretching to the descending portion of the aortic arch (Figure 1). Obstruction may be subvalvar, valvar, or supravalvar. These obstructions to forward flow may present alone or in concert, as in the frequent association of a bicuspid aortic valve with coarctation of the aorta. All of these lesions impose increased afterload on the left ventricle and, if severe and untreated, result in hypertrophy and eventual dilatation and failure of the left ventricle. LVOTOs are congenital in the vast majority of individuals younger than 50 years in the United States; some variants of subaortic obstruction are the exception. It is imperative to consider all patients with LVOTO at a high risk for developing infective endocarditis, and one should always institute appropriate measures for prophylaxis. The present article is intended as a contemporary review of the causes, manifestations, treatments, and outcomes of LVOTO; it will not address LVOTO in the pediatric population or genetic hypertrophic cardiomyopathy but will focus strictly on congenital malformations in the adult. Figure 1. Artist’s rendering of the LVOTO lesions in sequence as viewed from a superolateral orientation. A, Gradient echo cardiac MR image as viewed from the frontal projection demonstrating flow acceleration at a site of supravalvar aortic stenosis (white arrow) in a patient with Williams syndrome. The black arrow identifies the level of the unrestricted aortic valve. B, Classic radiological signs of coarctation of the aorta: rib notching (white arrows) as seen on a posteroanterior chest x-ray in a patient with coarctation of the aorta. The rib notching is caused by erosion of the inferior rib margins by dilated pulsatile posterior intercostals collateral arteries. The black arrow points to the Figure 3 silhouette that …

Real-time three-dimensional transthoracic echocardiographic visualisation of accessory mitral valve tissue in a 22-year-old man with multiple sclerosis

Ieva, Riccardo; Correale, Michele; Guaricci, Andrea Igoren; Di Biase, Matteo Author Information

Understanding Left Ventricular Outflow Obstruction: Anatomoechocardiographic Correlation

Echocardiography is a valuable noninvasive technique for identifying the site and type of obstruction in the left ventricular outflow tract. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. This study was undertaken to provide improved understanding of the anatomy of left ventricular outflow obstruction with corresponding side-by-side echocardiographic images. Specimens were part of the collection of the Department of Embryology of the Instituto Nacional de Cardiología "Ignacio Chávez," Mexico City, Mexico. Twenty-eight patients were studied by echocardiography, and 22 specimens with different types of left ventricular outflow tract obstruction were analyzed. Echocardiographic characteristics: Eighteen (64.3%) patients were women and 10 (35.7%) men. Three had fibrous rings, 19 with obstructive asymmetric septal hypertrophic cardiomyopathy, 1 with posterior displacement of the infundibular septum, 2 with accessory mitral valve tissue, 2 with anomalous insertion of mitral valve chordae tendineae, and 1 with malposition of the mitral valve and papillary muscles. Anatomic characteristics: Twelve had fibrous ring, 2 asymmetric septal hypertrophic cardiomyopathy, 2 posterior displacement of the infundibular septum, 1 ectopic mitral tissue, 1 anomalous insertion of mitral valve chordae tendineae in the ventricular septum, 3 malposition of the mitral valve and papillary muscles, and 1 subaortic infundibulum. The anatomoechocardiographic correlation clearly demonstrates the mechanism of subaortic obstruction. The comparison with corresponding echocardiographic images of different subaortic obstruction types is potentially quite valuable in enhancing the echocardiographer's understanding especially of complex subaortic obstruction.

Left Ventricular Outflow Tract Obstruction Caused by Accessory Mitral Valve Tissue

This report describes the echocardiographic diagnosis of accessory mitral valve tissue causing left ventricular outflow tract obstruction in a symptomatic man. The accessory tissue was surgically removed and the mitral valve replaced with complete resolution of left ventricular outflow tract gradient and symptoms. The case illustrates the importance of transthoracic and transesophageal echocardiography in the diagnosis and preoperative evaluation of this rare but treatable cause of subaortic valve obstruction.

Left Ventricular Outflow Obstruction Caused by Accessory Mitral Valve Tissue with Associated History of Coarctation of the Aorta.

Left Ventricular Outflow Obstruction Caused by Accessory Mitral Valve Tissue with Associated History of Coarctation of the Aorta.

Recurrence of cerebrovascular thromboembolic event in a woman with accessory mitral valve during puerperium period

Accessory mitral valve tissue is an extremely rare congenital cardiac anomaly. Most of the cases reported in the literature were associated with left ventricular outflow tract obstruction. In this report, a second cerebrovascular event is presented in the same patient two years after an operation in which septal myectomy was performed but accessory mitral valve tissue was not excised.

Accessory Mitral Valve Tissue Causing Mitral Valve Obstruction and Left Ventricular Outflow Tract Obstruction in a Young Adult

Accessory mitral valve (AMV) is a rare cause of left ventricular outflow tract (LVOT) obstruction. This case report demonstrates the progression from a hypertrophic cardiomyopathy to a mid-cavity obstruction; abnormal mitral valve architecture, including AMV with increasing obstruction; progressive symptoms; and full documentation of details by transesophageal echocardiogram. The patient underwent a successful operation for removal of the AMV at age 21 years.

Accessory mitral valve tissue causing severe left ventricular outflow tract obstruction in an adult

Accessory mitral valve (AMV) is a rare cause of left ventricular outflow tract (LVOT) obstruction and is extremely rare in adults. We report a case of an older adult with an AMV that caused severe LVOT obstruction. A parachute-like piece of tissue (the AMV) protruding into the LVOT during systole was first detected in a 45-year-old woman by echocardiography. Because the pressure gradient and dyspnea gradually progressed, she finally underwent a successful operation for removal when she was 48 years old.

Discrete subaortic stenosis: incidence, morphology and surgical impact of associated subaortic anomalies

Background The association between discrete subaortic stenosis and other subaortic anomalies is a well known but rarely reported occurrence. The aim of this study is to define the incidence, morphology, and surgical impact of associated anomalies of the left ventricular outflow tract in children operated on for discrete subaortic stenosis. Methods Between 1994 and 2000, 45 consecutive children were operated on for discrete subaortic stenosis. Patients were divided in two groups according to the obstructive lesion detected by echocardiography. Results A localized shelf was found as an isolated lesion in 31 patients (group A), whereas additional subaortic anomalies were found in 14 cases (31%) and were multiple in 5 cases (group B). The anomalies included anomalous septal insertion of mitral valve (7 cases); accessory mitral valve tissue (2 cases); anomalous papillary muscle (2 cases); anomalous muscular band (8 cases); and muscularization of the anterior mitral valve leaflet (1 case). Cardiopulmonary bypass and aortic cross-clamping times were significantly shorter in group A. There were no operative deaths nor major complications or deaths during follow-up. A gradient of 15 mm Hg or more was found at follow-up in 5 cases whereas aortic regurgitation was estimated to be not clinically significant in all but 1 patient. Six cases of recurrent subaortic stenosis were found in our series, 3 of them with other subaortic anomalies. Conclusions This study shows that discrete subaortic stenosis can often be associated with other subaortic abnormalities. Surgical treatment of these anomalies produces excellent early and mid-term relief of obstruction without any increase in mortality and morbidity.

Accessory Mitral Valve Tissue Obstructing Left Ventricular Outflow Tract

A 6-year-old girl underwent surgical excision of accessory mitral valve tissue causing significant subaortic stenosis. Preoperative 2-dimensional echocardio-graphy gave the necessary information on this isolated anomaly. The approach through an aortotomy provided adequate exposure. Postoperatively, there was no residual gradient across the left ventricular outflow tract, or mitral regurgitation.

Critical subaortic stenosis in a newborn caused by accessory mitral valve tissue

A 2-week-old newborn girl underwent successful surgery in our clinic for critical subaortic stenosis caused by accessory mitral valve tissue, which, because of excessive growth, protruded into the left ventricular outflow tract. The preoperative pressure gradient below the aortic valve was 80 mm Hg. The operation consisted of resection of the accessory tissue through a combined aortotomy and atriotomy approach without residual pressure gradient and mitral valve incompetence. This approach is recommended to ensure that accessory tissue is removed without damaging the mitral valve.

Clinical and pathological features of accessory valve tissue

Background. Accessory valve tissue is a rare congenital cardiac malformation causing a variety of clinical features. We describe our experience with the surgical treatment of accessory valve tissue to clarify clinical and pathological features of this anomaly.Methods. Between December 1990 and February 1999, 11 children underwent resection of accessory valve tissue together with repair of coexisting intracardiac malformations. Eight patients had accessory tricuspid valve, while 3 had accessory mitral valve tissue. Associated intracardiac malformations included tetralogy of Fallot in 6 patients, transposition of the great arteries in 3, coarctation complex in 1, and ventricular septal defect in 1.Results. Accessory valve tissue was completely resected in all but 1 patient with accessory mitral valve and transposition of the great arteries. As total resection of the accessory valve tissue through the pulmonary artery was unsuccessful, partial resection and bidirectional Glenn shunt operation was performed instead of arterial switch operation in this patient.Conclusions. The clinical signs and symptoms of accessory valve tissue vary according to its location and coexisting cardiac malformations. Congenital heart surgeons should be familiar with the clinical and pathological features of accessory valve tissue, so as not to overlook it during operation.

Congenital subaortic stenosis by accessory mitral valve tissue, recognition and management

Accessory mitral valve tissue as the single cause for left ventricular outflow tract obstruction is a very rare cardiac malformation in normally connected hearts. We report a case in which this condition was present as single cause for left ventricular outflow tract obstruction. The surgical technique is described and a review of the literature presented.

Estenosis subaórtica progresiva causada por tejido mitral accesorio

Accessory mitral valve tissue is a rare cause of subaortic obstruction. The reported case correspond to a two days old patient diagnosed by 2D-Echocardiography. Serial doppler flow analysis showed progressive left ventricular outflow obstruction. A cardiac catheterization at the age of 9 months confirmed the obstruction without defining the cause. Five months later, transesophageal echocardiography clearly defined the intraventricular connections of the accessory mitral tissue. At the age of 18 months the patient suffered from cardiac failure and underwent surgery for removing the accessory tissue, assisted by intraoperative transesophageal echocardiography pre and post by-pass. Follow-up echocardiography at 25 months showed no left ventricle outflow obstruction and the patient is now asymptomatic.