© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12867
Echocardiography
Three-Dimensional Echocardiography Enhances Diagnostic Accuracy of Supramitral Ring Pei-Ni Jone, MD,* Carlie Bremen, R.D.C.S.,* Michael DiMaria, MD,* David N. Campbell, MD,† James Jaggers, MD,† Adel Younoszai, MD,* and D. Dunbar Ivy, MD* *Pediatric Cardiology, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado; and †Pediatric Cardiovascular Surgery, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado
Real time three-dimensional echocardiography (RT3DE) allows for accurate morphological characterization of congenital heart disease and complements two-dimensional (2D) echocardiography. Accurate evaluation of the mitral valve and supramitral ring prior to surgery aids the surgeon in successful resection of the ring and repair of associated mitral valve abnormalities. We report a case of congenital mitral valve stenosis from a supramitral ring in a young child using real time three-dimensional transesophageal echocardiography. (Echocardiography 2015;32:1048–1050) Key words: congenital heart disease, mitral stenosis, three-dimensional transesophageal echocardiography
Real time three-dimensional echocardiography (RT3DE) allows for accurate morphological characterization of congenital heart disease and complements two-dimensional (2D) echocardiography.1,2 For example, RT3DE provides precise anatomical analysis of the mitral valve apparatus in children with mitral valve abnormalities.3 Accurate evaluation of the mitral valve and supramitral ring prior to surgery aids the surgeon in successful resection of the ring and repair of associated mitral valve abnormalities. We report a case of congenital mitral valve stenosis from a supramitral ring in a young child using real time three-dimensional transesophageal echocardiography (RT3DTEE). Case Presentation: A 6-year-old male with history of coarctation repair in infancy and bicuspid aortic valve presented to cardiology clinic with symptoms of easy fatigability and lethargy. A 2D echocardiogram was performed which showed an abnormal mitral valve with abnormal chordal attachments and moderate stenosis (peak inflow gradient of 23 mmHg and mean gradient of 13 mmHg) with mild insufficiency (Fig. 1 and movie clip S1). There was moderate Address for correspondence and reprint requests: Pei-Ni Jone, M.D., 13123 East 16th Avenue, B100, Aurora, Colorado 80045. Fax: 720-777-7290; E-mail: [email protected]
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left atrial enlargement and mildly elevated RV pressure. The bicuspid aortic valve had no stenosis and mild aortic insufficiency. There was no gradient at the repaired coarctation site. The patient was sent to cardiac catheterization for a possible ballooning of this mitral valve stenosis and underwent RT3DTEE evaluation with live 3D, 3D zoom, and 3D full volume acquisition of the mitral valve under anesthesia before the procedure. RT3DTEE detected a supramitral ring (Fig. 2 and movie clips S2, S3) and a parachute-like subvalvar apparatus, with the majority of chordal attachments to the posteromedial papillary muscle. The cardiac catheterization was canceled and the patient was scheduled for surgery. The supramitral ring (Fig. 3) was also confirmed in surgery and this ring was successfully resected. No intervention to the mitral valve or subvalvar apparatus was necessary as there was excellent excursion of valve leaflet post resection with a residual mean gradient of 5 mmHg (Fig. 4 and movie clip S4). Discussion: This case highlights the diagnostic accuracy of supramitral ring using RT3DTEE in a pediatric patient and importance in the successful management of the patient obviating the need for cardiac catheterization. The ring can be visualized from the left atrium in an en face view and allows improved visualization of the
Three-Dimensional Echocardiography of Supramitral Ring
Figure 2. A. En face view of the supramitral ring from the left atrium B. Apical four-chamber 3D image of the supramitral ring above the mitral valve leaflets. LA = left atrium; LV = left ventricle; RV = right ventricle.
Figure 1. A. 2D echocardiography of mitral valve stenosis with a probable supramitral ring. B. color Doppler showed that color aliasing starts above the mitral valve. C. The mitral gradient by Doppler. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.
complete separation of the supramitral ring from the mitral valve leaflets in real time (Fig. 2 and movie clip S3). This provides the surgeon with excellent images in order to plan and execute the proper surgical procedure. Supramitral ring has been reported to be visualized in 73% of the patients using 2D echocardiography.4,5 With the addition of RT3DTEE
Figure 3. Surgical left atrial view of supramitral ring.
to 2D echocardiography, the diagnosis of supramitral ring as the cause of congenital mitral valve stenosis can be made accurately and provides better anatomic analysis of mitral valve apparatus. 1049
Jone, et al.
References 1. Simpson JM, Miller O: Three-dimensional echocardiography in congenital heart disease. Arch Cardiovasc Dis 2011;104:45–56. 2. Hage FG, Raslan S, Dean P, et al: Real time three-dimensional transthoracic echocardiography in congenital heart disease. Echocardiography 2012;29:220–231. 3. Takahashi K, Mackie AS, Rebeyka IM, et al: Two-dimensional versus transthoracic real-time three-dimensional echocardiography in the evaluation of the mechanisms and sites of atrioventricular valve regurgitation in a congenital heart disease population. J Am Soc Echocardiogr 2010;23:726–734. 4. Collison SP, Kaushal SK, Dagar KS, et al: Supramitral ring: Good prognosis in a subset of patients with congenital mitral stenosis. Ann Thorac Surg 2006;81:997–1001. 5. Toscano A, Pasquini L, Iacobelli R, et al: Congenital supravalvar mitral ring: An underestimated anomaly. J Thorac Cardiovasc Surg 2009;137:538–542.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie S1. Two-dimensional transesophageal apical four-chamber view demonstrating probable supramitral ring and stenotic mitral valve with tethering of the mitral valve leaflets. Movie S2. Three-dimensional echocardiographic enface view of the left atrium in 3D zoom demonstrating the supramitral ring. Movie S3. Three-dimensional echocardiographic four-chamber view demonstrating the supramitral ring sitting just above the mitral valve leaflets. Movie S4. Three-dimensional echocardiographic four-chamber view postsurgical resection of the supramitral ring and free movement of the mitral valve leaflets. Figure 4. A. An en face view of the mitral valve after resection of the supramitral ring. B. There is good excursion of the mitral valve leaflets. LA = left atrium; LV = left ventricle; RA = right atrium.
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Echocardiography
Three-Dimensional Echocardiography Enhances Diagnostic Accuracy of Supramitral Ring Pei-Ni Jone, MD,* Carlie Bremen, R.D.C.S.,* Michael DiMaria, MD,* David N. Campbell, MD,† James Jaggers, MD,† Adel Younoszai, MD,* and D. Dunbar Ivy, MD* *Pediatric Cardiology, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado; and †Pediatric Cardiovascular Surgery, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado
Real time three-dimensional echocardiography (RT3DE) allows for accurate morphological characterization of congenital heart disease and complements two-dimensional (2D) echocardiography. Accurate evaluation of the mitral valve and supramitral ring prior to surgery aids the surgeon in successful resection of the ring and repair of associated mitral valve abnormalities. We report a case of congenital mitral valve stenosis from a supramitral ring in a young child using real time three-dimensional transesophageal echocardiography. (Echocardiography 2015;32:1048–1050) Key words: congenital heart disease, mitral stenosis, three-dimensional transesophageal echocardiography
Real time three-dimensional echocardiography (RT3DE) allows for accurate morphological characterization of congenital heart disease and complements two-dimensional (2D) echocardiography.1,2 For example, RT3DE provides precise anatomical analysis of the mitral valve apparatus in children with mitral valve abnormalities.3 Accurate evaluation of the mitral valve and supramitral ring prior to surgery aids the surgeon in successful resection of the ring and repair of associated mitral valve abnormalities. We report a case of congenital mitral valve stenosis from a supramitral ring in a young child using real time three-dimensional transesophageal echocardiography (RT3DTEE). Case Presentation: A 6-year-old male with history of coarctation repair in infancy and bicuspid aortic valve presented to cardiology clinic with symptoms of easy fatigability and lethargy. A 2D echocardiogram was performed which showed an abnormal mitral valve with abnormal chordal attachments and moderate stenosis (peak inflow gradient of 23 mmHg and mean gradient of 13 mmHg) with mild insufficiency (Fig. 1 and movie clip S1). There was moderate Address for correspondence and reprint requests: Pei-Ni Jone, M.D., 13123 East 16th Avenue, B100, Aurora, Colorado 80045. Fax: 720-777-7290; E-mail: [email protected]
1048
left atrial enlargement and mildly elevated RV pressure. The bicuspid aortic valve had no stenosis and mild aortic insufficiency. There was no gradient at the repaired coarctation site. The patient was sent to cardiac catheterization for a possible ballooning of this mitral valve stenosis and underwent RT3DTEE evaluation with live 3D, 3D zoom, and 3D full volume acquisition of the mitral valve under anesthesia before the procedure. RT3DTEE detected a supramitral ring (Fig. 2 and movie clips S2, S3) and a parachute-like subvalvar apparatus, with the majority of chordal attachments to the posteromedial papillary muscle. The cardiac catheterization was canceled and the patient was scheduled for surgery. The supramitral ring (Fig. 3) was also confirmed in surgery and this ring was successfully resected. No intervention to the mitral valve or subvalvar apparatus was necessary as there was excellent excursion of valve leaflet post resection with a residual mean gradient of 5 mmHg (Fig. 4 and movie clip S4). Discussion: This case highlights the diagnostic accuracy of supramitral ring using RT3DTEE in a pediatric patient and importance in the successful management of the patient obviating the need for cardiac catheterization. The ring can be visualized from the left atrium in an en face view and allows improved visualization of the
Three-Dimensional Echocardiography of Supramitral Ring
Figure 2. A. En face view of the supramitral ring from the left atrium B. Apical four-chamber 3D image of the supramitral ring above the mitral valve leaflets. LA = left atrium; LV = left ventricle; RV = right ventricle.
Figure 1. A. 2D echocardiography of mitral valve stenosis with a probable supramitral ring. B. color Doppler showed that color aliasing starts above the mitral valve. C. The mitral gradient by Doppler. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.
complete separation of the supramitral ring from the mitral valve leaflets in real time (Fig. 2 and movie clip S3). This provides the surgeon with excellent images in order to plan and execute the proper surgical procedure. Supramitral ring has been reported to be visualized in 73% of the patients using 2D echocardiography.4,5 With the addition of RT3DTEE
Figure 3. Surgical left atrial view of supramitral ring.
to 2D echocardiography, the diagnosis of supramitral ring as the cause of congenital mitral valve stenosis can be made accurately and provides better anatomic analysis of mitral valve apparatus. 1049
Jone, et al.
References 1. Simpson JM, Miller O: Three-dimensional echocardiography in congenital heart disease. Arch Cardiovasc Dis 2011;104:45–56. 2. Hage FG, Raslan S, Dean P, et al: Real time three-dimensional transthoracic echocardiography in congenital heart disease. Echocardiography 2012;29:220–231. 3. Takahashi K, Mackie AS, Rebeyka IM, et al: Two-dimensional versus transthoracic real-time three-dimensional echocardiography in the evaluation of the mechanisms and sites of atrioventricular valve regurgitation in a congenital heart disease population. J Am Soc Echocardiogr 2010;23:726–734. 4. Collison SP, Kaushal SK, Dagar KS, et al: Supramitral ring: Good prognosis in a subset of patients with congenital mitral stenosis. Ann Thorac Surg 2006;81:997–1001. 5. Toscano A, Pasquini L, Iacobelli R, et al: Congenital supravalvar mitral ring: An underestimated anomaly. J Thorac Cardiovasc Surg 2009;137:538–542.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie S1. Two-dimensional transesophageal apical four-chamber view demonstrating probable supramitral ring and stenotic mitral valve with tethering of the mitral valve leaflets. Movie S2. Three-dimensional echocardiographic enface view of the left atrium in 3D zoom demonstrating the supramitral ring. Movie S3. Three-dimensional echocardiographic four-chamber view demonstrating the supramitral ring sitting just above the mitral valve leaflets. Movie S4. Three-dimensional echocardiographic four-chamber view postsurgical resection of the supramitral ring and free movement of the mitral valve leaflets. Figure 4. A. An en face view of the mitral valve after resection of the supramitral ring. B. There is good excursion of the mitral valve leaflets. LA = left atrium; LV = left ventricle; RA = right atrium.
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