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.

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.

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.

Anatomy of the mitral subvalvular apparatus.

OBJECTIVES: To characterize morphologic variations in the papillary muscles and tendinous cords of the left ventricle and ventricular zones of the mitral valve leaflets. METHODS: A total of 100 autopsied human hearts from healthy donors with classical mitral valve type were investigated. RESULTS: In 1 heart, only 1 group of papillary muscles was found, and in the remaining 99%, we could distinguish 2 groups of muscles: Superolateral (SLPM) and inferoseptal papillary muscle (ISPM) groups. The SLPM group had 1 papillary muscle (75.8%), 2 in 20.2%, and >3 in 4.0%. In the ISPM group, the muscle percentages were 38.4%, 36.4%, and 25.2%, respectively. The apex of at least 1 papillary muscle was situated higher than the plane of the opened anterior leaflet (AML) in 47.5% and 50.5% for the SLPM and ISPM groups, respectively. The number of strut cords arising from the SLPM group was 0 (2.0%), 1 (50.5%), 2 (33.3%), 3 (12.1%), and 4 (2.0%), and from the ISPM group was 0 (6.1%), 1 (52.5%), 2 (35.4%), or 3 (6.1%). Cords to left ventricular outflow tract were present in 14 specimens. Muscular cords were found in eight hearts. In all hearts specimens AML had rough and clear zones. The classical zones (rough, clear, and basal) in the posterior mitral leaflet were observed in 38.4%. CONCLUSIONS: There is a high variability in the papillary muscles and tendinous cords in the mitral valve complex. Proper nomenclature, simple classification, and the most common variants for papillary muscle groups and tendinous cords were presented.

Edge-to-edge repair for mitral regurgitation associated with isolated double-orifice mitral valve.

We report the case of a patient with severe mitral regurgitation who was diagnosed with double-orifice mitral valve by preoperative transthoracic and transoesophageal echocardiography. During surgery, it was revealed that the mitral valve was divided into 2 orifices, anterolateral and posteromedial, by a fibrous bridging tissue that was supported by the chordae tendineae originating from an accessory middle papillary muscle. The posterior scallop of the anterolateral orifice was prolapsed due to chordal elongation. Six interrupted sutures were made between the anterior leaflet and the posterior leaflet at the prolapsed site. Additional interrupted sutures were made at the sites of 2 clefts, and a ring annuloplasty was added. Residual mitral regurgitation was trivial, and the mean postoperative pressure gradient through each orifice was approximately 6 mmHg. To the best of our knowledge, this is the first case report of an edge-to-edge mitral repair for mitral regurgitation associated with a double-orifice mitral valve.

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.

Double accessory left atrial chordae tendineae resulting in mitral regurgitation.

The presence of accessory left atrial chordae tendineae inserting into the mitral valve leaflet is extremely rare. Two long and thin accessory chordae tendineae, one arising from the left atrial dome and the other from the inferior interatrial septum, were incidentally identified during corrective surgery for severe mitral regurgitation from A3 prolapse. Triangular resection of the A3 portion of the anterior mitral valve leaflet including the double accessory chordae tendineae and primary repair followed by posterior ring annuloplasty was successfully performed.

Characteristics and treatment strategies of mitral regurgitation associated with undifferentiated papillary muscle.

PURPOSE: In this report we review our experience of operations on mitral regurgitation associated with abnormal papillary muscles/chordae tendineae of the mitral valves and discussed the clinical characteristics, operative findings, and treatment strategies. METHODS: Undifferentiated papillary muscle was defined as a hypoplastic chordae tendineae with anomalous formation of papillary muscles attached to the mitral valves directly. Consecutive 87 patients undergoing surgery for mitral regurgitation at our institution were reviewed and 6 of them had undifferentiated papillary muscle. RESULTS: The underlying mechanism of regurgitation was prolapse at the center of the anterior leaflet in 3 cases and tethering, a wide area of myxomatous degeneration, and annular dilatation in one case, respectively. Five patients underwent mitral valve plasty and 1 patient received replacement. Anomalous formation of chordae tendineae was corrected by resection and suture with transplantation at the tip of the leaflet to which abnormal chordae were attached in 2 cases, while resection and suture with chordal shortening was performed in 1 case, and chordal reconstruction using artificial chordae was employed in 2 cases. There was no operative death, and postoperative echocardiography showed no residual regurgitation in any of the cases. CONCLUSIONS: Mitral regurgitation associated with undifferentiated papillary muscle resulted from prolapse or tethering and impaired flexibility of leaflets. It was possible to successfully treat the patients by mitral valve plasty unless complex congenital cardiac malformation coexisted. Detailed examinations of attached papillary muscle by echocardiography and intraoperative inspection are necessary and surgical techniques should be selected appropriately in each case.

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.

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.

Congenital anomalous chordae tendinae of the mitral valve: an unusual presentation of mitral insufficiency in children.

Anomalous chordae tendinae (CT) originating from mitral valve leaflet is a rare congenital mitral valve anomaly. Our case report is unique as this anomaly is extremely rare in this pediatric age group. The anomalous CT extended from anterior mitral leaflet to the atrial septum (AS). Surgical repair in the form of anomalous CT excision, anterior leaflet chordoplasty, and posterior mitral annuloplasty was successfully performed. Congenital mitral valve (MV) leaflet or chordae anomalies are rare. In anomalous CT from MV leaflet to the AS, the surgical experience is extremely limited and only reported in adults and adolescents. We describe an unusual presentation of severe mitral insufficiency (MR) associated with anomalous CT from the anterior mitral valve leaflet (AMVL) to the AS that prompted successful repair during childhood.