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.

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.

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.

Hypertrophic angulation deformity of the basal interventricular septum combined with abnormality of the papillary muscle and chordae tendineae.

A Chinese woman was admitted to our hospital because of syncope. Transthoracic echocardiography revealed a hypertrophic basal interventricular septum of 15 mm with a sharp angle protruding into the left ventricular outflow tract. Moreover, an anomalous anterolateral papillary muscle (maximum width of 11 mm) was inserted into the left ventricular outflow tract, with short chordae tendineae connecting both basal interventricular septum and anterior leaflet of the mitral valve. All of these abnormalities resulted in a left ventricular outflow gradient of 136 mmHg. Surgical septal myectomy of the sharp angle combined with partial papillary muscle resection and removal of the abnormal chordae tendineae was selected to relieve the left ventricular outflow obstruction. This was a rare combination of deformity of the angulation of the focal basal interventricular septum and abnormalities of the papillary muscle and chordae tendineae, which led to left ventricular outflow obstruction.

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.

Atypical Chordae Tendineae of the Canine (Canis familiaris) Right Atrioventricular Valve.

The canine right atrioventricular valve cusps are anchored to papillary muscles by chordae tendineae. During ventricular systole, these tendineae keep the cusps from being pushed into the atrium. While this is the general description for chordae tendineae, several researchers have briefly documented chordae tendineae in animal and human hearts that do not attach to papillary muscles. In the 39 canine hearts examined, atypical chordae tendineae were observed in two hearts. In both dogs, a single stranded chordae tendineae extended from the free edge of the parietal cusp of the right atrioventricular valve to the ventricular free wall. While the discovery of these atypical tendineae provides additional information on canine cardiac anatomy, their presence may also be clinically significant. A review of the veterinary and biomedical literature showed entanglement in normal chordae tendineae can be a complication during cardiac catheterization or pacemaker lead placement. Given this issue with normal chordae tendineae, it seems logical to propose that these atypical tendineae could also cause catheter or pacemaker lead entanglement and therefore warrant further study and documentation.