Combination of an extremely rare and a common congenital valvular anomalies: Accessory mitral valve chordae from left atrium and bicuspid aortic valve.

Accessory chordae tendineae is an extremely rare anomaly. In this case report, we described a 61-year-old female patient newly diagnosed with the combination of an accessory mitral valve chordae extending from left atrium which is an extremely rare congenital anomaly and a bicuspid aortic valve. In our patient, three-dimensional echocardiography showed incremental value over two-dimensional echocardiography in the assessment of the exact localization and the extend of accessory chordea.

A rare congenital anomaly: Anomalous fibromuscular cord of the left atrium.

BACKGROUND: Left atrial anomalous fibromuscular cord is a rare congenital anomaly, which exists in a small proportion of the general population. Although its clinical significance remains largely unknown, it is generally considered a benign entity. We report a case of incidental finding of left atrial fibromuscular cord without structural cardiac abnormalities or hemodynamic obstruction. METHODS AND RESULTS: A-39-year-old female presented with palpitations for more than 10 years. Electrocardiogram and laboratory tests showed no unremarkable. Transthoracic echocardiography revealed an abnormal linear structure connecting the interatrial septum and the left atrial free wall, color Doppler flow imaging did not show hemodynamic obstruction. Cardiac contrast-enhanced computed tomography images showed the string-like structure associated with calcification, connecting the interatrial septum and the ridge around the orifice of the left inferior pulmonary vein. Sagittal multiplanar reconstructed image showed a dot-like structure located in the left atrial cavity. DISCUSSION: Left atrial anomalous fibromuscular cord is a rare congenital anomaly, which is also known as left atrial anomalous fibromuscular cord, left atrial false tendon, accessory chordae tendineae, or left atrial aberrant band. The clinical significance is unclear. Some cases have been reported that the fibromuscular cord, which do not have pathological significance. It has also been reported that it may be associated with supraventricular arrhythmias, patent foramen ovale, and Chiai's network. In some patients, attachment to the mitral chord can lead to mitral valve insufficiency and murmur. Nevertheless, a detailed understanding the anomalous anatomical characteristics of the anomalous cord may help us to better predict an unexpected difficulty in catheter manipulation, and potential arrhythmogenicity. CONCLUSION: Transthoracic echocardiography and cardiac computed tomography angiography have an important imaging value for the diagnosis of the left atrial anomalous fibromuscular cord, including its origin, course, or whether associated with other cardiovascular malformations.

Isolated anterior mitral cleft.

Anterior mitral valve clefts are unusual anomalies particularly associated with endocardial cushion defects. A dysplastic subvalvular apparatus causing left ventricular outflow tract obstruction may result in diagnostic confusion. We present an isolated anterior mitral cleft associated with subvalvular dysplasia.

A late presentation of congenital cardiac anomaly: Accessory chordae from the left atrium causing severe mitral regurgitation.

Mitral regurgitation secondary to accessory mitral valve (MV) chordae of the left atrium is an extremely rare congenital disease. A 85-year-old female (NYHA I-II) was hospitalized for investigations. An echocardiogram showed calcification of the MV with mild stenosis and moderate regurgitation. Transesophageal three-dimensional echocardiogram revealed a band-like structure extending from the distal third of the anterior wall of the left atrium to the MV. This accessory chordae determined severe systolic regurgitation and mild mitral stenosis. The patient was referred for consideration of cardiac surgery but was refused for comorbidities and anatomy. Usually aberrant chordae determinant valvulopathies are detected and treated at a much younger age. The delay of the symptoms could be explained in our case with the progressive growth and dilatation of the left atrium causing traction of the aberrant chord resulting in an increase in the leaflet prolapse and regurgitation.

Mitral Valve Repair for Mitral Regurgitation with Congenital Anomalies of the Papillary Muscles.

BACKGROUND: True parachute mitral valve and parachute-like asymmetric mitral valve are associated with congenital anomalies of the papillary muscles, which involves an abnormal anatomy of the papillary muscles and chordae. METHODS: Two patients are described with mitral valve regurgitation and papillary muscle anomalies. Mitral valve repair using artificial chordae reconstruction and ring annuloplasty was attempted in both cases. RESULTS: The first patient had a true parachute mitral valve, while the second patient had a parachute-like asymmetric mitral valve. In both patients, widespread leaflet prolapse induced mitral valve regurgitation, and the anterior mitral leaflet was thickened because of long-term regurgitation. Mitral valve repair using artificial chordae reconstruction and ring annuloplasty were successfully performed. At midterm follow up the patients did not experience any progression of significant regurgitation or stenosis. CONCLUSIONS: Mitral valve repair using artificial chordae reconstruction combined with ring annuloplasty is effective for treating regurgitant parachute mitral valve in adults.

Incidental finding of aberrant mitral valve chorda during a transesophageal echocardiography evaluation for suspected endocarditis.

Congenital defects involving anomalous chordae of the mitral valve with attachment in the left atrium are an extremely rare finding and may result in valvular insufficiency. Few cases have been reported in the literature with multiple variations in anatomical location and insertion of aberrant chordae within the left ventricle and left atrium. Reported cases have presented with cardiovascular symptoms leading to diagnosis of anomalous mitral valve chordae. We present a case of a young female in which an aberrant mitral valve chorda was an incidental finding on transesophageal echocardiography.

STRUCTURAL HEART ANOMALIES (REVIEW).

Structural heart anomalies (SHA) are congenital abnormalities of cardiovascular system, characterized by various anatomical departures of heart and great vessels from normal conditions. SHA are a part of connective tissue dysplasia syndrome (CTDS), one of the most common congenital autosomal-dominant diseases in people of young and middle age. The most common SHA are a mitral valve prolapse, abnormal chords of left ventricle and their combinations. The clinical significance of these anomalies depends on a degree of severity and impact on intracardial hemodynamics, as described in the article. The most prognostically dangerous are multiple abnormal chords of left ventricle, which can be a sign of serious hereditary disease - a left ventricular non-compaction.

Surgery for Congenital Tricuspid Valve Cleft: Tricuspid Valve Repair with Neochordae and Annuloplasty.

BACKGROUND AND AIM OF THE STUDY: Congenital abnormalities of the tricuspid valve (TV), including dysplasia, straddling, and those associated with other congenital heart disease, are rare causes of tricuspid regurgitation (TR). In congenital TV anomalies there can be varying levels of abnormalities of leaflet and subvalvular structures. Herein is reported a case of TV cleft with absent chordae, and a technique of TV repair. METHODS: A 14-year-old boy was found to have severe TR due to dysplasia of the anterior TV leaflet. Intraoperatively he was noted to have dysplasia of the TV with a cleft in the anterior leaflet of the TV and an absence of chordae supporting the anterior two-thirds of the anterior leaflet. The anterior papillary muscle was hypoplastic, with chordae to the posterior leaflet and small chordae partly to the anterior leaflet. The cleft was repaired and a neochordae placed onto the anterior leaflet with attachment to the papillary muscle, followed by an annuloplasty. RESULTS: Intraoperative and postoperative echocardiographic assessment showed good mobility of the anterior tricuspid leaflet at six months and two-year follow up. CONCLUSION: Chordal replacement is a useful technique for repairing congenital dysplastic TV with absent chordae. The same technique for mitral valve repair with neochordae can be applied to chordal anomalies of the TV, with excellent outcome.

An uncommon case of isolated parachute-like asymmetric mitral valve in an adult.

A 31-year-old asymptomatic male was referred to hospital for an examination of right bundle brunch block. Both, transthoracic and transesophageal echocardiography revealed normal left ventricular function, and two different-sized papillary muscles; the anterolateral muscle was more pronounced, with almost major chordae tendineae inserted into this dominant muscle, whereas the immature, flat posteromedial papillary muscle had very short chordae tendineae and was located higher in the left ventricle, inserted directly into the mitral annulus. The mitral valve orifice was eccentrically located at the lateral side, but no significant mitral stenosis or regurgitation was observed. No other congenital heart anomalies were identified. Thus, the final diagnosis was isolated parachute-like asymmetric mitral valve (PLAMV), without any other congenital heart anomalies. The patient was followed up closely with periodic echocardiographic examinations. Parachute mitral valve is a rare congenital cardiac defect characterized by focalized attachment of the chordae tendineae of both leaflets to a single papillary muscle. In contrast to true parachute mitral valve, PLAMV has two separate papillary muscles, one of which is more pronounced and into which all chordae are inserted. PLAMV was highly associated with other congenital heart anomalies, and the involved dominant muscle was most frequently a posteromedial papillary muscle. Isolated PLAMV in an adult is even more rare, while the presence of an immature posteromedial papillary muscle--as in the present case--is extremely rare.

Aberrantly inserted chordae tendineae without significant mitral regurgitation.

Aberrantly inserted chordae tendineae in the left atrial side are a rare find. We report here the case of a young patient with aberrantly inserted chordae tendineae not causing significant mitral regurgitation. Because the patient remained asymptomatic and the anomalous chord of the left atrium could be considered within normal human anatomic variation, the authors decided to only monitor the patient's condition for the time being.

Anomalous mitral arcade variant with accessory mitral leaflet and chordae presenting for the first time with acute decompensated heart failure in an adult.

The anomalous mitral arcade is a rare congenital malformation of the mitral valve and its tensor apparatus. It is characterized by enlarged papillary muscles connected to each other and to the free edge of the anterior mitral leaflet by a bridge of fibrous tissue. We report a rare variant of anomalous mitral arcade that was associated with accessory mitral leaflet in subaortic area and accessory chordae. Our patient was asymptomatic till the age of 18 years, when he presented for the first time in acute decompensated heart failure secondary to severe mitral regurgitation and left ventricular dysfunction. The patient had rapid deterioration with fatal outcome.

A case of severe mitral regurgitation due to an unusually long aberrant chorda tendinea straddling the anterior mitral leaflet.

We present a case of a 27-year-old female with severe mitral regurgitation caused by a single long aberrant chorda tendinea. This chorda extended from the base of the right coronary cusp of the aortic valve, through the A2 scallop of the mitral valve, and attached to the dome of the left atrium. Initial transthoracic echocardiogram (TTE) demonstrated a mildly redundant anterior mitral leaflet with thickened leaflet tip and moderate eccentric, posteriorly directed mitral regurgitation. Repeat TTE revealed a chord-like structure attached to the midportion of the anterior mitral leaflet and extending to the left ventricular outflow tract. Transesophageal echocardiography (TEE) suggested two aberrant chordae tendineae tethering the A2 scallop on both the left atrial and left ventricular side. Patient underwent surgical resection of the aberrant chorda. During the excision of the chorda the structural integrity of the A2 scallop was compromised, necessitating mitral valve repair with excellent results.

Mitral insufficiency caused by left atrial chordae.

We report an unusual case of moderate mitral regurgitation caused by abnormal insertion of chordae tendinea to the interatrial septum and tethering the middle scallop of the anterior mitral leaflet. This is an extremely rare congenital abnormality causing mitral regurgitation.

Anomalous chord of the left atrium without involvement of the mitral valve.

An anomalous chord from the left side of the atrial septum to the left atrial free wall was incidentally noted on transthoracic echocardiography in a 14-year-old boy with vasovagal syncope. Previously reported cases of anomalous chords in the left atrium were associated with the mitral valve leaflets in all but two cases. This is the first reported case of an echocardiographic diagnosis of anomalous left atrial chord without insertion of the chord into the mitral valve.

Comprehensive left ventricular outflow tract management beyond septal reduction to relieve obstruction.

The surgical management of patients with hypertrophic obstructive cardiomyopathy can be extremely challenging. Relieving the left ventricular outflow tract obstruction in these patients is often achieved by performing a septal myectomy. However, in many instances, septal reduction alone is not enough to relieve the obstruction. Interventions on the sub-valvular apparatus, including the anomalous chordae tendineae and the abnormal papillary muscles, are often required. In this review, we summarize the embryology and the pathophysiology of the different elements that may contribute to the left ventricular outflow tract obstruction in the setting of hypertrophic obstructive cardiomyopathy. In addition, we highlight the different surgical procedures that a surgeon may adopt to relieve the left ventricular outflow tract obstruction, beyond the septal myectomy.

Parachute mitral valve associated with reticular chordae tendineae in an adult: case report.

BACKGROUND: Parachute mitral valve with reticular chordae tendineae is an extremely rare anomaly. CASE PRESENTATION: We present a case of parachute mitral valve associated with distinctive reticular chordae tendineae in an adult. It was diagnosed from the echocardiogram. The patient was referred for surgery. Valve analysis showed thickened mitral valve leaflets and commissures. The chordae tendinae were lengthy and thick. All the chordae tendinae merged into a solitary papillary muscle. A distinctive reticular fibrous tissue was found on mitral valve apparatus as the chordae tendinae intermixed each other. The only functional communication between the left atrium and the left ventricle was through the reticular spaces. This anomaly was considered to be unrepairable and was replaced with a mechanical valve. CONCLUSIONS: An extremely rare and unique case of parachute mitral valve associated with reticular chordae tendineae was reported. Mitral valve replacement is a reasonable choice in patients with parachute mitral valve with reticular chordae tendineae.

Repair of congenitally absent chordae in a tricuspid valve leaflet with hypoplastic papillary muscle using artificial chordae.

Isolated congenital tricuspid regurgitation without downward displacement of the leaflet is uncommon in adults, and repair of such valves often requires a procedure that is more complex than simple tricuspid annuloplasty. We describe a technique of tricuspid valvoplasty using the neo-papillary loop technique to reconstruct multiple artificial chords for isolated congenital tricuspid regurgitation associated with a dysplastic anterior leaflet with agenesis of the chordae tendineae and a hypoplastic anterior papillary muscle. This technique provides a simple and valuable option for the repair of dysplastic valves lacking chordae, even those with a hypoplastic papillary muscle.