Congenital Mitral Valve Diseases

  • Teerapat YingchoncharoenEmail author


A 66-year-old woman has a history of coronary artery bypass grafting in 1987, when she had left main stenosis and underwent bypass with anastomosis of the left internal mammary artery to the left anterior descending artery and a saphenous vein graft to the obtuse marginal branch. The patient now returns with symptoms of congestive heart failure. She is found to have an atrioventricular canal defect including mitral valve regurgitation with mitral valve cleft, primum atrial septal defect, and severe tricuspid regurgitation. She also has a left-sided superior vena cava, into which she has an anomalous left upper pulmonary vein. She has recently noticed a significant decline and can now walk only half a block at a slow pace before having to stop. She was referred to our institute for surgical correction of her complex congenital heart disease (Figs. 9.1, 9.2, 9.3, 9.4, 9.5, 9.6, 9.7, 9.8, 9.9, and 9.10).


Congenital AV canal defects Cleft mitral valve Parachute mitral valve Cor triatriatum 

Supplementary material

Video 9.1

Transthoracic echocardiography (TTE), parasternal long-axis view, showing dilatation of the coronary sinus in the setting of persistent left superior vena cava and mitral regurgitation (AVI 1923 kb)

Video 9.2

TTE, parasternal short-axis view, showing atrial septal defect with left-to-right shunt (AVI 2013 kb)

Video 9.3

Three-dimensional (3D) TTE short-axis view at the aortic valve level showing the bicuspid aortic valve. The aortic valve orifice appears to have a “fish-mouth” appearance (AVI 1832 kb)

Video 9.4

TTE X-plane imaging showing two orthogonal views showing an anterior mitral valve cleft (AVI 3804 kb)

Video 9.5

TTE parasternal short-axis view of the mitral valve with color Doppler imaging showing anterior mitral valve cleft with corresponding mitral regurgitation (AVI 1818 kb)

Video 9.6

TTE four-chamber view showing dilated right-sided cardiac chambers with a ventricular upper septal aneurysm (AVI 5815 kb)

Video 9.7

TTE four-chamber view with color Doppler imaging showed atrial septal defect with left-to-right shunt at the level close to the cardiac crux, consistent with primum atrial septal defect. Also seen are mitral and tricuspid regurgitation (AVI 2320 kb)

Video 9.8

Transesophageal echocardiography (TEE) four-chamber view showed primum atrial septal defect (AVI 6583 kb)

Video 9.9

TEE short axis view at aortic valve level showed bicuspid aortic valve (AVI 6459 kb)

Video 9.10

3D TEE of mitral valve (surgical view) showed anterior mitral valve cleft (AVI 218 kb)

Video 9.11

TTE parasternal long-axis view showed an elongated anterior mitral leaflet with parachute mitral valve with a unifocal attachment of the mitral valve chordae to a single papillary muscle (AVI 7949 kb)

Video 9.12

TTE parasternal long-axis view with color Doppler imaging showed turbulent flow across the mitral valve during diastole (AVI 3947 kb)

Video 9.13

TTE apical four-chamber view showed mitral valve opening during diastole. The valve opening was restricted because of abnormal chordal attachment, giving the appearance of the parachute mitral valve (AVI 6701 kb)

Video 9.14

TTE apical four-chamber view with color Doppler flow showed diastolic flow acceleration across the mitral valve, consistent with mitral stenosis, as well as mild mitral regurgitation (AVI 1969 kb)

310450_1_En_9_MOESM15_ESM.avi (954 kb)
Video 9.15 TTE parasternal long-axis view showing a thin membrane separating the left atrium into two chambers. The proximal or superior chamber drains the pulmonary venous blood, and the distal inferior chamber (or true atrium) is in contact with the atrioventricular valve (AVI 954 kb)
Video 9.16

TTE apical four-chamber view showing a thin membrane separating the left atrium into two chambers, a finding consistent with cor triatriatum sinister (AVI 1141 kb)

Video 9.17

TTE apical two-chamber view showed that the left atrial appendage and the foramen ovale are distal to the cor triatriatum membrane. This feature is important in differentiating cor triatriatum from a supramitral valve ring, which tends to attach to the base of the mitral valve, past the left atrial appendage and the foramen ovale (AVI 1095 kb)

Video 9.18

TTE subcostal view zoomed at the interatrial septum, demonstrating atrial septal defect with left-to-right shunt (AVI 1582 kb)

Video 9.19

TTE apical four-chamber view with color Doppler flow imaging showed nonturbulent flow across the cor triatriatum membrane. This finding was confirmed by pulsed-wave Doppler and cardiac cathetherization showing a nonobstructive cor triatriatum membrane (AVI 1502 kb)

Video 9.20

TEE short-axis view zoomed at the interatrial septum confirmed the presence of the cor triatriatum membrane. The color Doppler flow imaging showed nonturbulent flow across the membrane (AVI 2049 kb)


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Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  1. 1.Department of Cardiovascular MedicineCleveland ClinicClevelandUSA

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