© 2013, Wiley Periodicals, Inc. DOI: 10.1111/echo.12478
Echocardiography
Multimodality Imaging of Holmes Heart with Parachute Mitral Valve Rishi Sethi, M.D., D.M., F.A.C.C., Rajiv Bharat Kharwar, M.D., Akhil Sharma, M.D., Vikas Kumar, M.D., and Varun Shankar Narain, M.D., D.M. Department of Cardiology, King George’s Medical University, Lucknow, India
(Echocardiography 2014;31:E132–E135) Key words: Holmes heart, parachute mitral valve, three-dimensional echocardiography, computed tomography
Figure 1. Two-dimensional transthoracic echocardiography with color Doppler. Apical four-chamber view A, B. showing presence of double inlet left ventricular type of single ventricle. Apical long-axis and papillary muscle level parasternal short-axis view showing parachute type mitral valve with a single papillary muscle (indicated by arrow in D, E.) resulting into severe mitral stenosis C. and moderate to severe mitral regurgitation F. LA = left atrium; RA = right atrium; SV = single ventricle.
Case Report: A 10-year-old boy was admitted to our center with complaints of dyspnea on exertion of New Address for correspondence and reprint requests: Rajiv Bharat Kharwar, M.D., Department of Cardiology, King George’s Medical University, Chowk, Lucknow, pincode – 226003 Uttar Pradesh, India. Fax: 05222258948; E-mail: [email protected]
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York Heart Association functional class II and easy fatigueability. On general examination pulse was 90/min, blood pressure was 100/70 mmHg, normal jugular venous pressure and there was central cyanosis with clubbing. Cardiac auscultation revealed a mid-diastolic murmur and a pansystolic murmur at the apex. Evaluation by two-dimensional (2D) transthoracic echocardiography (TTE)
Multimodality Imaging
Figure 2. Two-dimensional transthoracic echocardiography with color Doppler. Subcostal views showing the rudimentary right ventricle connected to the left ventricle via a nonstenotic bulboventricular foramen. The aorta is arising from the left ventricle C, D. and the pulmonary artery from the right ventricle A, B. Ao = aorta; LA = left atrium; PA = pulmonary artery; SV = single ventricle.
(GE Vivid 7 Dimension, GE Healthcare, Horten, Norway) with color Doppler (Figs. 1,2 and movie clips S1–S3) showed the presence of double inlet single ventricle of left ventricular type, parachute type of mitral valve with a single papillary muscle leading to severe mitral valve stenosis and mitral regurgitation. There was a rudimentary right ventricle situated anteriorly and to the left of left ventricle that was connected to the left ventricle via a bulboventricular foramen. The great arteries were normally related, concordant to the ventricles, and there was no stenosis of either the bulboventricular foramen, the pulmonary valve or the
aortic valve. Three-dimensional (3D) TTE with color Doppler (Fig. 3, movie clips S4–S6) clearly delineated the anatomy of the parachute mitral valve as well as the relationship of the great arteries with their respective ventricles. Multidetector computed tomography (MDCT) (Brilliance CT64; Philips Medical Systems, Cleveland, OH, USA) with volume rendering (Fig. 4) confirmed the finding of 2D and 3DTTE and the diagnosis of Holmes heart with parachute mitral valve was confirmed. Patient was started on decongestive therapy and was referred for possible surgical correction of this rare congenital cardiac defect. E133
Sethi, et al.
Figure 3. Three-dimensional transthoracic echocardiography with color Doppler. The stenotic parachute mitral valve as well as the single point of attachment of the subvalvular apparatus to a basally shifted single papillary muscle is clearly delineated A, B. Three-dimensional color flow imaging C, D. showing the flow across the stenotic mitral valve. The relationship of the rudimentary right ventricle to the left ventricle as well as the great arterial relationship with their respective ventricles is clearly outlined E, F. Ao = aorta; LA = left atrium; PA = pulmonary artery; RA = right ventricle; SV = single ventricle.
Figure 4. Multidetector computed tomography. Multiplanar reconstruction showing the relationship of the rudimentary right ventricle to the left ventricle as well as the great arterial relationship with their respective ventricles A, B., double inlet single ventricle of left ventricular type D. along with single papillary muscle (arrow in E, F. Three-dimensional CT reconstruction is shown in C. Ao = aorta; LA = left atrium; PA = pulmonary artery; RA = right ventricle; SV = single ventricle.
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Multimodality Imaging
Univentricular heart is a type of congenital heart defect that is characterized by presence of a single ventricle or 2 ventricular chambers in which one of the ventricle is hypoplastic. Other defects that are associated with single ventricle are mitral or tricuspid atresia, subaortic stenosis, coarctation of aorta, and pulmonic stenosis or atresia.1A parachute mitral valve is a rare congenital defect in which shortened chordae tendinae converge and insert into a single papillary muscle.2 Although the diagnosis is mainly done by 2D echocardiography, there have been reports of computed tomography and magnetic resonance imaging for evaluation of this entity. Our case is the first case to demonstrate the usefulness of 3DTTE and MDCT in the evaluation of Holmes heart with parachute mitral valve. References 1. Fulton DR, Freed MD. The pathology, pathophysiology, recognition, and treatment of congenital heart disease. In Hurst’s The Heart. 11th ed. New York: The McGraw-Hill Companies, 2004, pp. 1840–1842. 2. Schaverien MV, Freedom RM, McCrindle BW: Independent factors associated with outcomes of parachute mitral valve in 84 patients. Circulation 2004;109:2309–2313.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Two-dimensional transthoracic echocardiography with color Doppler. Apical four-chamber view showing double inlet single ventricle (LV type) with an intact inter atrial septum. The mitral valve is stenotic as well as regurgitant due to presence of parachute mitral valve along with presence of accessory mitral valve tissue. LA = left atrium; RA = right atrium; SV = single ventricle.
Movie clip S2. Two-dimensional transthoracic echocardiography with color Doppler. Subcostal view showing the bifurcating pulmonary artery arising from the rudimentary right ventricle which is situated left and anterior to the single ventricle (LV type). LA = left atrium; PA = pulmonary artery; RV = right ventricle; SV = single ventricle. Movie clip S3. Two-dimensional transthoracic echocardiography with color Doppler. Subcostal view showing the aorta arising from the single ventricle (LV type) which is connected to the rudimentary right ventricle via a bulboventricular foramen. Ao = aorta; LA = left atrium; RV = right ventricle; SV = single ventricle. Movie clip S4. Three-dimensional transthoracic echocardiography with volume rendering. The parachute nature of the mitral valve with attachment to a single basally shifted papillary muscle is clearly demonstrated. Also note the presence of mobile accessory mitral valve tissue. LA = left atrium; LV = left ventricle. Movie clip S5. Three-dimensional transthoracic echocardiography. The bifurcating (green arrows) main pulmonary artery arising from the rudimentary right ventricle is clearly delineated. MPA = main pulmonary artery; RV = right ventricle; SV = single ventricle. Movie clip S6. Three-dimensional transthoracic echocardiography. The origin of the aorta from the LV type single ventricle is clearly delineated. Also note the small bulboventricular foramen via which the rudimentary right ventricle is connected to the left ventricle. Ao = aorta; MPA = main pulmonary artery; SV = single ventricle.
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Echocardiography
Multimodality Imaging of Holmes Heart with Parachute Mitral Valve Rishi Sethi, M.D., D.M., F.A.C.C., Rajiv Bharat Kharwar, M.D., Akhil Sharma, M.D., Vikas Kumar, M.D., and Varun Shankar Narain, M.D., D.M. Department of Cardiology, King George’s Medical University, Lucknow, India
(Echocardiography 2014;31:E132–E135) Key words: Holmes heart, parachute mitral valve, three-dimensional echocardiography, computed tomography
Figure 1. Two-dimensional transthoracic echocardiography with color Doppler. Apical four-chamber view A, B. showing presence of double inlet left ventricular type of single ventricle. Apical long-axis and papillary muscle level parasternal short-axis view showing parachute type mitral valve with a single papillary muscle (indicated by arrow in D, E.) resulting into severe mitral stenosis C. and moderate to severe mitral regurgitation F. LA = left atrium; RA = right atrium; SV = single ventricle.
Case Report: A 10-year-old boy was admitted to our center with complaints of dyspnea on exertion of New Address for correspondence and reprint requests: Rajiv Bharat Kharwar, M.D., Department of Cardiology, King George’s Medical University, Chowk, Lucknow, pincode – 226003 Uttar Pradesh, India. Fax: 05222258948; E-mail: [email protected]
E132
York Heart Association functional class II and easy fatigueability. On general examination pulse was 90/min, blood pressure was 100/70 mmHg, normal jugular venous pressure and there was central cyanosis with clubbing. Cardiac auscultation revealed a mid-diastolic murmur and a pansystolic murmur at the apex. Evaluation by two-dimensional (2D) transthoracic echocardiography (TTE)
Multimodality Imaging
Figure 2. Two-dimensional transthoracic echocardiography with color Doppler. Subcostal views showing the rudimentary right ventricle connected to the left ventricle via a nonstenotic bulboventricular foramen. The aorta is arising from the left ventricle C, D. and the pulmonary artery from the right ventricle A, B. Ao = aorta; LA = left atrium; PA = pulmonary artery; SV = single ventricle.
(GE Vivid 7 Dimension, GE Healthcare, Horten, Norway) with color Doppler (Figs. 1,2 and movie clips S1–S3) showed the presence of double inlet single ventricle of left ventricular type, parachute type of mitral valve with a single papillary muscle leading to severe mitral valve stenosis and mitral regurgitation. There was a rudimentary right ventricle situated anteriorly and to the left of left ventricle that was connected to the left ventricle via a bulboventricular foramen. The great arteries were normally related, concordant to the ventricles, and there was no stenosis of either the bulboventricular foramen, the pulmonary valve or the
aortic valve. Three-dimensional (3D) TTE with color Doppler (Fig. 3, movie clips S4–S6) clearly delineated the anatomy of the parachute mitral valve as well as the relationship of the great arteries with their respective ventricles. Multidetector computed tomography (MDCT) (Brilliance CT64; Philips Medical Systems, Cleveland, OH, USA) with volume rendering (Fig. 4) confirmed the finding of 2D and 3DTTE and the diagnosis of Holmes heart with parachute mitral valve was confirmed. Patient was started on decongestive therapy and was referred for possible surgical correction of this rare congenital cardiac defect. E133
Sethi, et al.
Figure 3. Three-dimensional transthoracic echocardiography with color Doppler. The stenotic parachute mitral valve as well as the single point of attachment of the subvalvular apparatus to a basally shifted single papillary muscle is clearly delineated A, B. Three-dimensional color flow imaging C, D. showing the flow across the stenotic mitral valve. The relationship of the rudimentary right ventricle to the left ventricle as well as the great arterial relationship with their respective ventricles is clearly outlined E, F. Ao = aorta; LA = left atrium; PA = pulmonary artery; RA = right ventricle; SV = single ventricle.
Figure 4. Multidetector computed tomography. Multiplanar reconstruction showing the relationship of the rudimentary right ventricle to the left ventricle as well as the great arterial relationship with their respective ventricles A, B., double inlet single ventricle of left ventricular type D. along with single papillary muscle (arrow in E, F. Three-dimensional CT reconstruction is shown in C. Ao = aorta; LA = left atrium; PA = pulmonary artery; RA = right ventricle; SV = single ventricle.
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Multimodality Imaging
Univentricular heart is a type of congenital heart defect that is characterized by presence of a single ventricle or 2 ventricular chambers in which one of the ventricle is hypoplastic. Other defects that are associated with single ventricle are mitral or tricuspid atresia, subaortic stenosis, coarctation of aorta, and pulmonic stenosis or atresia.1A parachute mitral valve is a rare congenital defect in which shortened chordae tendinae converge and insert into a single papillary muscle.2 Although the diagnosis is mainly done by 2D echocardiography, there have been reports of computed tomography and magnetic resonance imaging for evaluation of this entity. Our case is the first case to demonstrate the usefulness of 3DTTE and MDCT in the evaluation of Holmes heart with parachute mitral valve. References 1. Fulton DR, Freed MD. The pathology, pathophysiology, recognition, and treatment of congenital heart disease. In Hurst’s The Heart. 11th ed. New York: The McGraw-Hill Companies, 2004, pp. 1840–1842. 2. Schaverien MV, Freedom RM, McCrindle BW: Independent factors associated with outcomes of parachute mitral valve in 84 patients. Circulation 2004;109:2309–2313.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Two-dimensional transthoracic echocardiography with color Doppler. Apical four-chamber view showing double inlet single ventricle (LV type) with an intact inter atrial septum. The mitral valve is stenotic as well as regurgitant due to presence of parachute mitral valve along with presence of accessory mitral valve tissue. LA = left atrium; RA = right atrium; SV = single ventricle.
Movie clip S2. Two-dimensional transthoracic echocardiography with color Doppler. Subcostal view showing the bifurcating pulmonary artery arising from the rudimentary right ventricle which is situated left and anterior to the single ventricle (LV type). LA = left atrium; PA = pulmonary artery; RV = right ventricle; SV = single ventricle. Movie clip S3. Two-dimensional transthoracic echocardiography with color Doppler. Subcostal view showing the aorta arising from the single ventricle (LV type) which is connected to the rudimentary right ventricle via a bulboventricular foramen. Ao = aorta; LA = left atrium; RV = right ventricle; SV = single ventricle. Movie clip S4. Three-dimensional transthoracic echocardiography with volume rendering. The parachute nature of the mitral valve with attachment to a single basally shifted papillary muscle is clearly demonstrated. Also note the presence of mobile accessory mitral valve tissue. LA = left atrium; LV = left ventricle. Movie clip S5. Three-dimensional transthoracic echocardiography. The bifurcating (green arrows) main pulmonary artery arising from the rudimentary right ventricle is clearly delineated. MPA = main pulmonary artery; RV = right ventricle; SV = single ventricle. Movie clip S6. Three-dimensional transthoracic echocardiography. The origin of the aorta from the LV type single ventricle is clearly delineated. Also note the small bulboventricular foramen via which the rudimentary right ventricle is connected to the left ventricle. Ao = aorta; MPA = main pulmonary artery; SV = single ventricle.
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