Abstract
Obstruction of left ventricular inflow includes pulmonary vein stenosis, cor triatriatum and supravalvular and valvular mitral stenosis. Morphologic diagnosis of these anomalies is greatly enhanced by colour Doppler examination. PW and CW Doppler in pulmonary vein stenosis and in cor triatriatum reveal a continuous systolic-diastolic flow profile. Documentation of flow velocities in patients with significant supravalvular mitral ring stenosis and mitral valvular stenosis requires application of CW Doppler. Description of severity of mitral valve stenosis to a large extent relies on determination of the mean gradient. PW and CW Doppler evaluations of possible pulmonary and tricuspid regurgitation are important tools in the detection of secondary pulmonary hypertension. Congenital mitral regurgitation is encountered in patients with mitral valve prolapse, mitral valve cleft or dysplastic mitral valve. Functional mitral regurgitation is found in children with dilated cardiomyopathy, myocarditis or ischaemic alterations of the papillary muscles and in patients with severe aortic stenosis or anomalous origin of coronary arteries from the pulmonary artery. Colour Doppler helps to visualize the origin and size of the regurgitant jet. However assessment of the severity of mitral regurgitation requires the inclusion of clinical data, 2D echo and PW and CW Doppler.
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References
Bernhardt C, Seiler T et al (2003) Asymptomatisches Cor triatriatum bei einer 2¼ Jahre alten Patientin. Z Kardiol 92(9):758–762
Brotherton H, Philip RK (2008) Anomalous left coronary artery from pulmonary artery (ALCAPA) in infants: a 5-year review in a defined birth cohort. Eur J Pediatr 167(1):43–46
Brown DW (2009) Pulmonary venous anomalies. In: Lai WW, Mertens LL, Cohen MS, Geva T (eds) Echocardiography in pediatric and congenital heart disease. Blackwell Publishing Ltd, Chichester
Chu E, Cheitlin MD (1993) Diagnostic considerations in patients with suspected coronary artery anomalies. Am Heart J 126(6):1427–1438
Coelho CC, Aiello VD et al (2010) Chronic rheumatic heart disease. In: Anderson RH, Baker EJ, Penny DJ et al (eds) Pediatric cardiology. Livingstone/Elsevier, Philadelphia/Churchill
Courand PY, Bozio A et al (2013) Focus on echocardiographic and Doppler analysis of coronary artery abnormal origin from the pulmonary trunk with mild myocardial dysfunction. Echocardiography 30(7):829–836
Dahle G, Fiane AE et al (2007) ALCAPA, a possible reason for mitral insufficiency and heart failure in young patients. Scand Cardiovasc J 41(1):51–58
Daubeney PE, Blackstone EH et al (1999) Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children. Cardiol Young 9(4):402–410
DeGroff CG (2002) Doppler echocardiography. Pediatr Cardiol 23(3):307–333
Feigenbaum H, Armstrong WF et al (2005) Feigenbaum’s echocardiography. Baltimore, New York, London, Lippincott Williams & Wilkins, Philadelphia
Freed LA, Levy D et al (1999) Prevalence and clinical outcome of mitral-valve prolapse. N Engl J Med 341(1):1–7
Gheissari A, Malm JR et al (1992) Cor triatriatum sinistrum: one institution’s 28-year experience. Pediatr Cardiol 13(2):85–88
Holt DB, Moller JH et al (2007) Primary pulmonary vein stenosis. Am J Cardiol 99(4):568–572
Kim YH, Pak HN et al (2011) Congenital pulmonary vein stenosis in an adult patient treated with transcatheter balloon angioplasty. Eur J Echocardiogr 12(3)
Lancellotti P, Moura L et al (2010) European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 2: mitral and tricuspid regurgitation (native valve disease). Eur J Echocardiogr 11(4):307–332
Levine RA, Stathogiannis E et al (1988) Reconsideration of echocardiographic standards for mitral valve prolapse: lack of association between leaflet displacement isolated to the apical four chamber view and independent echocardiographic evidence of abnormality. J Am Coll Cardiol 11(5):1010–1019
Levine RA, Handschumacher MD et al (1989) Three-dimensional echocardiographic reconstruction of the mitral valve, with implications for the diagnosis of mitral valve prolapse. Circulation 80(3):589–598
Mizushige K, Shiota T et al (1995) Effects of pulmonary venous flow direction on mitral regurgitation jet area as imaged by color Doppler flow mapping. An in vitro study. Circulation 91(6):1834–1839
Ohta N, Sakamoto K et al (2001) Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects. Jpn J Thorac Cardiovasc Surg 49(11):656–659
Rim Y, McPherson DD et al (2015) Effect of congenital anomalies of the papillary muscles on mitral valve function. J Med Biol Eng 35(1):104–112
Schaverien MV, Freedom RM et al (2004) Independent factors associated with outcomes of parachute mitral valve in 84 patients. Circulation 109(19):2309–2313
Seale AN, Webber SA et al (2009) Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study. Heart 95(23):1944–1949
Seale AN, Uemura H et al (2013) Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction. J Thorac Cardiovasc Surg 145(5):1255–1262
Shone JD, Sellers RD et al (1963) The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 11:714–725
Smallhorn JF, Anderson RH (2010) Anomalies of the morphologically mitral valve. In: Anderson RH, Baker EJ, Penny DJ et al (eds) Pediatric cardiology. Livingstone/Elsevier, Philadelphia/Churchill
Smallhorn JF, de Leval M et al (1982) Isolated anterior mitral cleft. Two dimensional echocardiographic assessment and differentiation from “clefts” associated with atrioventricular septal defect. Br Heart J 48(2):109–116
Snider RA, Serwer GA et al (1997) Echocardiography in pediatric heart disease. Mosby, St. Louis
Sulafa AK, Tamimi O et al (2005) Echocardiographic differentiation of atrioventricular septal defects from inlet ventricular septal defects and mitral valve clefts. Am J Cardiol 95(5):607–610
Tamura M, Menahem S et al (2000) Clinical features and management of isolated cleft mitral valve in childhood. J Am Coll Cardiol 35(3):764–770
Trowitzsch E, Bano-Rodrigo A et al (1985) Two-dimensional echocardiographic findings in double orifice mitral valve. J Am Coll Cardiol 6(2):383–387
Wu YT, Chang AC et al (1993) Semiquantitative assessment of mitral regurgitation by Doppler color flow imaging in patients aged < 20 years. Am J Cardiol 71(8):727–732
Yaroglu Kazanci S, Emani S et al (2012) Outcome after repair of cor triatriatum. Am J Cardiol 109(3):412–416
Zalzstein E, Hamilton R et al (2004) Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol 93(8):1067–1069
Zhu D, Bryant R et al (2009) Isolated cleft of the mitral valve: clinical spectrum and course. Tex Heart Inst J 36(6):553–556
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18.1 Electronic Supplementary Material
Video 18.1
High left parasternal short-axis view in a newborn displaying the left atrium and the pulmonary veins. This plane is obtained by some caudal tilt of the transducer from the parasternal short-axis view displaying the pulmonary bifurcation (AVI 2287 kb)
Video 18.2
Apical four-chamber view in a 3-year-old child revealing the membrane of cor triatriatum separating the left atrium with some distance to the mitral valve (same patient as in Videos 18.3, 18.4, 18.5, 18.6 and 18.7) (AVI 6485 kb)
Video 18.3
Colour Doppler in the apical four-chamber view (same patient as in Videos 18.2, 18.4, 18.5, 18.6 and 18.7) shows significant acceleration of flow with aliasing due to the restrictive membrane (AVI 1922 kb)
Video 18.4
The obstructing membrane with its restrictive opening is also displayed in this oblique apical five-chamber view (same patient as in Videos 18.2, 18.3, 18.5, 18.6 and 18.7) (AVI 5841 kb)
Video 18.5
Colour Doppler in the oblique apical five-chamber view confirms acceleration of flow and turbulence across the restrictive membrane (same patient as in Videos 18.2, 18.3, 18.4, 18.6 and 18.7) (AVI 1501 kb)
Video 18.6
The parasternal long-axis view shows the obstruction membrane in the left atrium behind the aortic root (same patient as in Videos 18.2, 18.3, 18.4, 18.5 and 18.7)(AVI 5192 kb)
Video 18.7
Colour Doppler in the parasternal long-axis view reveals significant acceleration of flow across the obstructing membrane in cor triatriatum despite a rather unfavourable insonation angle for Doppler interrogation (AVI 737 kb)
Video 18.8
Supravalvular mitral ring is displayed in the apical four-chamber view of an adolescent, with a membrane that is difficult to distinguish from the mitral valve leaflets (AVI 8661 kb)
Video 18.9
The membrane of the supravalvular mitral ring can be distinguished from the mitral valve leaflets in this apical two-chamber view (same patient as in Videos 18.8, 18.10 and 18.11)(AVI 14066 kb)
Video 18.10
Colour Doppler in the apical two-chamber view shows acceleration of flow starting at the level of the supravalvular mitral ring proximal to the level of the mitral valve while there is good separation of the mitral valve leaflets (same patient as in Videos 18.8, 18.9 and 18.11) (AVI 1432 kb)
Video 18.11
The membrane of the supravalvular mitral ring is displayed in the parasternal long-axis view in close proximity to the mitral valve leaflets in contrast to cor triatriatum, which presents with the obstructing membrane located more proximal in the left atrium (same patient as in Videos 18.8, 18.9 and 18.10) (AVI 14781 kb)
Video 18.12
The apical four-chamber view in an infant displays mitral stenosis due to hypoplastic mitral valve annulus (WMV 1079 kb)
Video 18.13
Colour Doppler in the apical four-chamber view reveals significant acceleration of flow due to the restrictive mitral valve annulus (same patient as in Video 18.12) (WMV 1250 kb)
Video 18.14
The parasternal long-axis view in a 2-year-old girl shows mitral stenosis due to thickening of the mitral valve leaflets and restricted mobility of the valve leaflets. In addition there is aortic valvular stenosis (same patient as in Videos 18.15, 18.16 and 18.17) (AVI 5167 kb)
Video 18.15
Colour Doppler in the parasternal long-axis view shows acceleration of flow across the stenotic mitral and aortic valve (same patient as in Videos 18.14, 18.16 and 18.17) (AVI 1756 kb)
Video 18.16
The apical four-chamber view shows an adequate-size mitral valve annulus but thickening of the mitral valve leaflets and shortening of the chordae tendineae resulting in restriction of mitral valve opening (same patient as in Videos 18.14, 18.15 and 18.17) (AVI 7561 kb)
Video 18.17
Colour Doppler in the apical four-chamber view reveals acceleration of flow which is located distal to the mitral valve annulus within the left ventricle (same patient as in Videos 18.14, 18.1 5 and 18.15) (AVI 5783 kb)
Video 18.18
The parasternal short-axis view in a child with parachute mitral valve shows a rudimentary anterolateral papillary muscle, while the valve apparatus is orientated exclusively towards the dominant posterolateral papillary muscle, which is not visualized in this plane (AVI 10533 kb)
Video 18.19
The parasternal long-axis view in a 14-year-old girl shows systolic prolapse of the anterior mitral valve leaflet. Thickening of the anterior mitral valve leaflet is apparent during diastole (WMV 1985 kb)
Video 18.20
Colour Doppler in the parasternal long-axis view (same patient as in Video 18.19) reveals mild mitral regurgitation directed towards the posterior wall of the left atrium (AVI 3568 kb)
Video 18.21
The parasternal short-axis view at the level of the mitral valve in a child with large VSD shows an isolated cleft in the anterior mitral valve leaflet (AVI 9460 kb)
Video 18.22
Colour Doppler in the parasternal short-axis view reveals mitral regurgitation originating from the cleft (same patient as in Video 18.21) (AVI 2433 kb)
Video 18.23
The parasternal short-axis view at the level of the mitral valve in a 10-year-old patient shows double-orifice mitral valve with two separate openings (AVI 11263 kb)
Video 18.24
Severe dilatation of the left ventricle and severely reduced function are demonstrated in the apical four-chamber view of a 3-month-old infant. The fibrosis of the chordae tendineae and papillary muscles of the mitral valve is almost pathognomonic for the underlying diagnosis of anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome) (AVI 33652 kb)
Video 18.25
Mitral regurgitation resulting from the fibrosis of the papillary muscles and dilatation of the left ventricle is demonstrated by colour Doppler interrogation in the apical four-chamber view (same patient as in Videos 18.24, 18.26 and 18.27) (AVI 7381 kb)
Video 18.26
The parasternal short-axis view at the level of the aortic root underlines the difficulty of visualization of the left coronary artery. At the end of the clip, the left coronary artery can be identified originating from the pulmonary artery (same patient as in Videos (AVI 11752 kb)
Video 18.27
The oblique high left parasternal short-axis view displays the pulmonary valve in cross section (same patient as in Videos 18.24, 18.25 and 18.26). The left coronary artery is shown with its abnormal origin from the pulmonary artery. Colour Doppler reveals retrograde flow both in the left circumflex and in the left anterior descending branch directed towards the pulmonary artery (AVI 7127 kb)
Video 18.28
Colour Doppler in the apical four-chamber view of an 8-week-old infant (A) shows significant turbulence and acceleration and aliasing of flow due to stenosis of a left pulmonary vein (same patient as in Videos 18.29 and 18.30) (AVI 2951 kb)
Video 18.29
Acceleration of flow in the moderately stenotic pulmonary vein (same patient as in Videos 18.28 and 18.30) is also visualized in this oblique parasternal short-axis view (AVI 1669 kb)
Video 18.30
Acceleration of flow in the common left pulmonary vein is confirmed by colour Doppler in the posterior plane of the subcostal coronal view (same patient as in Videos 18.28 and 18.29) and becomes even more evident in comparison to normal venous flow in the right upper pulmonary vein (AVI 2333 kb)
Video 18.31
Colour Doppler in the apical four-chamber view of a 14-year-old, who suffered from several episodes of rheumatic fever, shows severe mitral regurgitation with a broad-based jet reaching the posterior wall of the left atrium. Due to chronic mitral regurgitation, the left atrium is massively dilated (AVI 2094 kb)
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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Anomalies of Left Ventricular Inflow and Mitral Valve. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_18
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DOI: https://doi.org/10.1007/978-3-319-42919-9_18
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