Double outlet right ventricle with left ventricular outflow tract obstruction due to small ventricular septal defect

This is a report of the seventh and eighth known cases of double outlet right ventricle with left ventricular outflow tract stenosis due to a small ventricular septal defect. Case 1 is the second patient with this uncommon anomaly who has undergone successful surgical correction by enlargement of the ventricular septal defect and reconstruction of the outflow tract from the left ventricle to the aorta by means of a teflon tunnel. Case 2 was studied at autopsy and illustrates well the anatomic features of this malformation. It is suggested that this type with left ventricular outflow tract stenosis be incorporated into current classifications of double outlet right ventricle.     

Critical left ventricular outflow tract obstruction due to accessory mitral valve tissue

Left ventricular outflow tract (LVOT) obstruction due to anomalous tissue tag arising from the mitral valve is a rare congenital cardiac anomaly. It generally becomes symptomatic during the first decade of life as exercise intolerance, chest pain, or syncope at effort. To date, only a few cases of critical systemic obstruction due to isolated mitral valve anomaly in neonates have been reported. We report the case of a neonate who was a few hours old and was referred in severe clinical condition due to critical left ventricular outflow obstruction resulting from an anomalous tissue tag of mitral valve origin.     

Hypovolemia-induced reversible severe mitral regurgitation due to left ventricular outflow tract obstruction

We describe a hypertensive patient who developed profound cardiogenic shock after treatment with nitrates and diuretics. Echocardiography revealed a marked left ventricular outflow tract obstruction with severe mitral regurgitation that were reversible after aggressive fluid therapy.     

Double outlet right ventricle with l-malposition and uncommitted ventricular septal defect.

A case is described which exhibited Double Outlet Right Ventricle (DORV) with l-malposition of the aorta in association with a ventricular septal defect of the noncommitted, atrioventricular canal variety. When the ventriculoarterial relationship of DORV is encountered, it is more usual to find the aorta to the right of the pulmonary artery. Review of the literature shows that examples of DORV and l-malposition have been described with subaortic, subpulmonary and doubly committed defects, but we are unaware of any previous example reported with a noncommitted defect. These findings indicate that DORV with l-malposition should be anticipated in association with the same accompanying defects as those described in DORV with d-malposition, and emphasize the necessity for adopting a segmental approach to nomenclature. They also have morphogenetic significance, suggesting that DORV with l-malposition is closely linked with the anomalies for anatomically corrected malposition and concordant (complete) l-transposition. The present case exhibited an electrocardiographic superior axis, which is probably related to the presence of the atrioventricular canal deformity. The coexistence of hypoplasia of the aortic isthmus is speculatively related to the presence of conal and valvar factors, which can be considered to have reduced aortic blood flow.     

Double outlet left ventricle with intact ventricular septum

Two cases of double outlet left ventricle with intact ventricular septum are reported. Both patients presented with neonatal cyanosis and signs of right ventricular outflow tract obstruction. Both have hypoplastic right ventricles with suprasystemic pressures, pulmonary to mitral valve continuity and the aorta in left-sided position. These seem to be the second and third cases of the malformation to be described thus far.     

Double outlet left ventricle with subpulmonary ventricular septal defect and pulmonary hypertension

A two-month old male infant with the rare occurrence of double outlet left ventricle, subpulmonary ventricular septal defect and pulmonary hypertension is presented. The infant was managed temporarily with banding of the pulmonary trunk, with a favorable result, and is scheduled for definitive intraventricular repair.     

Accessory mitral valve tissue causing left ventricular outflow tract obstruction

Although left ventricular outflow tract obstruction is commonly associated with congenitally corrected transposition of the great vessels, this obstruction is seldom caused by accessory mitral valve tissue. Three cases in which accessory mitral valve tissue caused left ventricular outflow tract obstruction in children are described. Two had congenitally corrected transposition and one had normally connected great vessels. The accessory leaflet tissue, which was identified by echocardiography and angiography, was attached by chordae tendineae to normally sited papillary muscles and herniated into the left ventricular outflow tract during systole. Operation was successful in these patients. The accessory valve tissue was excised via an arteriotomy in the great vessel that arose from the left ventricle. The obstructive tissue was excised close to its peripheral attachments in the outflow tract and its chordae tendineae were divided. Resection was performed without injury to the abnormally placed conduction system or to the normal valve structures.     

Double outlet right ventricle with anomalous left pulmonary artery

We describe a rare case of anomalous origin of the left pulmonary artery from the ascending aorta with concomitant double-outlet right ventricle in a 2-year-old boy. He underwent successful 2-stage surgical treatment with transluminal balloon pulmonary valvuloplasty, followed by complete repair. A follow-up examination at 4 years after the operation showed good results.     

Reduction of left ventricular outflow tract obstruction with transcatheter mitral valve repair

Many patients with severe mitral regurgitation cannot undergo conventional mitral valve surgery due to prohibitive surgical risk and are candidates for transcatheter repair with an edge‐to‐edge technique. Prior reports suggest efficacy with this approach for mitral regurgitation due to hypertrophic cardiomyopathy with left ventricular outflow obstruction. We present a case report of transcatheter mitral valve repair for posterior leaflet prolapse with concomitant left ventricular outflow tract obstruction due to systolic anterior motion of the mitral valve in the absence of hypertrophic cardiomyopathy.     

Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We report the case of an 18-day-old infant with DORV and extremely rare anatomical features, such as anterior and left-sided aorta and subpulmonary ventricular septal defect (VSD). In addition to the anatomic features, the role of the echocardiogram for guiding the diagnosis and the surgical therapy of this congenital heart disease are discussed.     

Left ventricular outflow tract obstruction due to anomalous mitral valve: successful mitral valve replacement in a four month old infant

A four month old infant was investigated for heart failure was found to have mitral incompetence and severe subvalvar aortic stenosis. The left ventricular outflow tract obstruction was found to be due to an anatomically anomalous mitral valve. The obstruction could only be relieved by removal of the mitral valve and its replacement with a St Jude's prosthesis. Two years after operation the child is fit and active. There have been no difficulties with anticoagulant treatment.     

Accessory mitral valve tissue causing left ventricular outflow tract obstruction.

Although left ventricular outflow tract obstruction is commonly associated with congenitally corrected transposition of the great vessels, this obstruction is seldom caused by accessory mitral valve tissue. Three cases in which accessory mitral valve tissue caused left ventricular outflow tract obstruction in children are described. Two had congenitally corrected transposition and one had normally connected great vessels. The accessory leaflet tissue, which was identified by echocardiography and angiography, was attached by chordae tendineae to normally sited papillary muscles and herniated into the left ventricular outflow tract during systole. Operation was successful in these patients. The accessory valve tissue was excised via an arteriotomy in the great vessel that arose from the left ventricle. The obstructive tissue was excised close to its peripheral attachments in the outflow tract and its chordae tendineae were divided. Resection was performed without injury to the abnormally placed conduction system or to the normal valve structures.     

Left ventricular outflow tract obstruction due to anomalous mitral valve: successful mitral valve replacement in a four month old infant.

A four month old infant was investigated for heart failure was found to have mitral incompetence and severe subvalvar aortic stenosis. The left ventricular outflow tract obstruction was found to be due to an anatomically anomalous mitral valve. The obstruction could only be relieved by removal of the mitral valve and its replacement with a St Jude's prosthesis. Two years after operation the child is fit and active. There have been no difficulties with anticoagulant treatment.     

An accessory mitral valve leaflet causing left ventricular outflow tract obstruction and associated with severe aortic incompetence

This case report describes a 20-year-old woman with Turner's syndrome who presented with reduced effort tolerance limited by dyspnea. She had previously been on pediatric cardiology follow-up for congenital subvalvular aortic stenosis first diagnosed at age 7. Unfortunately she defaulted after two visits before any intervention could be done. Transthoracic echocardiography demonstrated severe aortic incompetence (AI) with a membrane-like structure in the left ventricular outflow tract (LVOT). The mean pressure gradient across the LVOT on continuous wave Doppler was 41 mmHg. The membranous interventricular septum appeared aneurysmal and it was observed that the "subaortic membrane" had a connection to the anterolateral papillary muscle via a strand of chordal tissue. Further images were captured using two-dimensional and three-dimensional transthoracic and transesophageal echocardiography (iE33, Philips Medical Systems, Andover, MA, USA). After a review of the literature it was concluded that this appeared to be an accessory mitral valve (AMV) leaflet causing LVOT obstruction associated with AI. AMV tissue is a rare congenital malformation causing LVOT obstruction. Because it is so unusual, it may not be immediately recognizable even in a high volume echocardiography laboratory. The clue which helped with the diagnosis was the strand of chordal tissue which connected the mass to the papillary muscle. This anomaly is often associated with LVOT obstruction.     

Unruptured sinus of Valsalva aneurysm with right ventricular outflow tract obstruction associated with ventricular septal defect

We describe an aneurysm originating from the right sinus of Valsalva, with right ventricular outflow tract obstruction associated with ventricular septal defect, in a 9-year-old boy. Diagnosis was made by echocardiography and cardiac catheterization. Successful surgical repair of ventricular septal defect and aortic valvuloplasty resulted in symptomatic improvement. © 1996 Wiley-Liss, Inc.     

Percutaneous alcohol septal ablation to acutely reduce left ventricular outflow tract obstruction induced by transcatheter mitral valve replacement

Transcatheter implantation of balloon expandable valves in native mitral valves (MV) has been performed in patients with severe MV annular calcification who are not good candidates for standard surgical MV replacement. Significant left ventricular outflow tract (LVOT) obstruction with hemodynamic compromise has been described as one of the potential complications of transcatheter MV replacement. Surgical rescue carries significant risk in this high‐risk patient population. We describe a percutaneous technique to acutely decrease transcatheter MV replacement‐induced LVOT obstruction. © 2016 Wiley Periodicals, Inc.