J Echocardiogr (2015) 13:79–80 DOI 10.1007/s12574-015-0247-3
IMAGES IN CARDIOVASCULAR ULTRASOUND
Left ventricular outflow obstruction secondary to accessory mitral valve tissue in a patient with hypertrophic cardiomyopathy Christodoulos E. Papadopoulos1,4 • Efstathios Pagourelias1 • Alexandros Kallifatidis2 Dimitrios Zioutas3 • Vassilios Vassilikos1
•
Received: 12 February 2015 / Revised: 8 April 2015 / Accepted: 11 May 2015 / Published online: 21 May 2015 Ó Japanese Society of Echocardiography 2015
A 62-year-old woman with a diagnosis of hypertrophic obstructive cardiomyopathy was referred to our department with symptoms of worsening heart failure and paroxysmal atrial fibrillation. Clinical examination demonstrated a harsh 4/6 systolic ejection murmur that typically increased in intensity following a Valsalva maneuver. Transthoracic echocardiogram (TTE) and transesophageal echocardiogram (TOE) revealed typical features of hypertrophic cardiomyopathy with an asymmetric septal hypertrophy (28 mm). A gradient of 90 mmHg following a Valsalva maneuver was documented, with no clear evidence of systolic anterior motion of the mitral valve. Left ventricular ejection fraction was moderately depressed and was estimated to be around 40 %.
Electronic supplementary material The online version of this article (doi:10.1007/s12574-015-0247-3) contains supplementary material, which is available to authorized users. & Christodoulos E. Papadopoulos [email protected] 1
3rd Cardiology Department, Hippokrateio University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
2
Radiology Department, Agios Loukas Hospital, Thessaloniki, Greece
3
Cardiology Department, Agios Loukas Hospital, Thessaloniki, Greece
4
11 Grigoriou E Str, 55236 Thessaloniki, Greece
Interestingly, during TTE, an excess of the mitral valve projecting in the left ventricular outflow tract (LVOT) in systole was suspected and was clearly visualized with TOE. This accessory mitral valve tissue was situated at the entrance of the LVOT (Fig. 1a, b green arrows), originating from the anterior mitral valve leaflet (A2 scallop), attaching to the neighboring interventricular septum. This excessive tissue was responsible for localized acceleration of flow in systole, reflecting the presence of a flow gradient, and was associated with moderate mitral valve regurgitation secondary to A2 scallop retraction during systole (Fig. 1c) (see the supplementary videos). Magnetic resonance imaging (MRI) confirmed the presence of the accessory mitral valve tissue (Fig. 1e, white arrow) and documented left ventricular septal hypertrophy with excessive late gadolinium enhancement (Fig. 1f, red arrow), supportive of hypertrophic cardiomyopathy. Left heart catheterization established the presence of a left ventricular intracavitary pressure gradient of 28 mmHg (Fig. 1d), with no evidence of transaortic valvular gradient. Differential diagnosis with elongated chordate tendineae was questioned, but, usually, this condition is not associated with a left ventricular intracavitary gradient. The LVOT gradient of 90 mmHg was finally attributed to concomitant fixed and dynamic obstruction secondary to septal hypertrophy following a Valsalva maneuver. The patient was scheduled for cardiothoracic assessment but died suddenly. Hypertrophic cardiomyopathy may rarely coexist with different forms of fixed LVOT obstruction [1–3]. This case illustrates the variable and sometimes coexisting pathologies in LVOT obstruction needing multimodality imaging for early diagnosis and appropriate clinical decisionmaking.
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J Echocardiogr (2015) 13:79–80
Fig. 1 Multimodality imaging in a patient with hypertrophic cardiomyopathy. Transesophageal echocardiogram (TOE) images, magnetic resonance imaging (MRI), and left heart catheterization tracings
documenting the presence of fixed left ventricular outflow tract (LVOT) obstruction secondary to an accessory mitral valve tissue
Conflict of interest C. E. Papadopoulos, E. Pagourelias, A. Kallifatidis, D. Zioutas, and V. Vassilikos declare that they have no conflict of interest.
hypertrophic cardiomyopathy. J Am Soc Echocardiogr. 2011;24: 592.e5–6. 2. Kim MS, Klein AJ, Groves BM, et al. Left ventricular outflow tract obstruction in the presence of asymmetric septal hypertrophy and accessory mitral valve tissue treated with alcohol septal ablation. Eur J Echocardiogr. 2008;9:720–4. 3. Ahn KT, Lee YD, Choi UL, et al. Flail subaortic membrane mimicking left ventricular outflow tract obstruction in hypertrophic cardiomyopathy. J Cardiovasc Ultrasound. 2013;21:90–3.
Human rights statement All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1964 and later revisions. Informed consent Informed consent was obtained from all patients for being included in the study.
References 1. Musumeci B, Spirito P, Parodi MI, et al. Congenital accessory mitral valve tissue anomaly in a patient with genetically confirmed
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IMAGES IN CARDIOVASCULAR ULTRASOUND
Left ventricular outflow obstruction secondary to accessory mitral valve tissue in a patient with hypertrophic cardiomyopathy Christodoulos E. Papadopoulos1,4 • Efstathios Pagourelias1 • Alexandros Kallifatidis2 Dimitrios Zioutas3 • Vassilios Vassilikos1
•
Received: 12 February 2015 / Revised: 8 April 2015 / Accepted: 11 May 2015 / Published online: 21 May 2015 Ó Japanese Society of Echocardiography 2015
A 62-year-old woman with a diagnosis of hypertrophic obstructive cardiomyopathy was referred to our department with symptoms of worsening heart failure and paroxysmal atrial fibrillation. Clinical examination demonstrated a harsh 4/6 systolic ejection murmur that typically increased in intensity following a Valsalva maneuver. Transthoracic echocardiogram (TTE) and transesophageal echocardiogram (TOE) revealed typical features of hypertrophic cardiomyopathy with an asymmetric septal hypertrophy (28 mm). A gradient of 90 mmHg following a Valsalva maneuver was documented, with no clear evidence of systolic anterior motion of the mitral valve. Left ventricular ejection fraction was moderately depressed and was estimated to be around 40 %.
Electronic supplementary material The online version of this article (doi:10.1007/s12574-015-0247-3) contains supplementary material, which is available to authorized users. & Christodoulos E. Papadopoulos [email protected] 1
3rd Cardiology Department, Hippokrateio University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
2
Radiology Department, Agios Loukas Hospital, Thessaloniki, Greece
3
Cardiology Department, Agios Loukas Hospital, Thessaloniki, Greece
4
11 Grigoriou E Str, 55236 Thessaloniki, Greece
Interestingly, during TTE, an excess of the mitral valve projecting in the left ventricular outflow tract (LVOT) in systole was suspected and was clearly visualized with TOE. This accessory mitral valve tissue was situated at the entrance of the LVOT (Fig. 1a, b green arrows), originating from the anterior mitral valve leaflet (A2 scallop), attaching to the neighboring interventricular septum. This excessive tissue was responsible for localized acceleration of flow in systole, reflecting the presence of a flow gradient, and was associated with moderate mitral valve regurgitation secondary to A2 scallop retraction during systole (Fig. 1c) (see the supplementary videos). Magnetic resonance imaging (MRI) confirmed the presence of the accessory mitral valve tissue (Fig. 1e, white arrow) and documented left ventricular septal hypertrophy with excessive late gadolinium enhancement (Fig. 1f, red arrow), supportive of hypertrophic cardiomyopathy. Left heart catheterization established the presence of a left ventricular intracavitary pressure gradient of 28 mmHg (Fig. 1d), with no evidence of transaortic valvular gradient. Differential diagnosis with elongated chordate tendineae was questioned, but, usually, this condition is not associated with a left ventricular intracavitary gradient. The LVOT gradient of 90 mmHg was finally attributed to concomitant fixed and dynamic obstruction secondary to septal hypertrophy following a Valsalva maneuver. The patient was scheduled for cardiothoracic assessment but died suddenly. Hypertrophic cardiomyopathy may rarely coexist with different forms of fixed LVOT obstruction [1–3]. This case illustrates the variable and sometimes coexisting pathologies in LVOT obstruction needing multimodality imaging for early diagnosis and appropriate clinical decisionmaking.
123
80
J Echocardiogr (2015) 13:79–80
Fig. 1 Multimodality imaging in a patient with hypertrophic cardiomyopathy. Transesophageal echocardiogram (TOE) images, magnetic resonance imaging (MRI), and left heart catheterization tracings
documenting the presence of fixed left ventricular outflow tract (LVOT) obstruction secondary to an accessory mitral valve tissue
Conflict of interest C. E. Papadopoulos, E. Pagourelias, A. Kallifatidis, D. Zioutas, and V. Vassilikos declare that they have no conflict of interest.
hypertrophic cardiomyopathy. J Am Soc Echocardiogr. 2011;24: 592.e5–6. 2. Kim MS, Klein AJ, Groves BM, et al. Left ventricular outflow tract obstruction in the presence of asymmetric septal hypertrophy and accessory mitral valve tissue treated with alcohol septal ablation. Eur J Echocardiogr. 2008;9:720–4. 3. Ahn KT, Lee YD, Choi UL, et al. Flail subaortic membrane mimicking left ventricular outflow tract obstruction in hypertrophic cardiomyopathy. J Cardiovasc Ultrasound. 2013;21:90–3.
Human rights statement All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1964 and later revisions. Informed consent Informed consent was obtained from all patients for being included in the study.
References 1. Musumeci B, Spirito P, Parodi MI, et al. Congenital accessory mitral valve tissue anomaly in a patient with genetically confirmed
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