Case Study
Aortic valve replacement in a patient with left ventricular noncompaction
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(9) 1065–1067 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314532106 aan.sagepub.com
Tomoaki Hirose1, Tetsuji Kawata2, Yoshihiro Hamada2, Nobuoki Tabayashi1, Takehisa Abe1 and Shigeki Taniguchi1
Abstract Left ventricular noncompaction is a rare cardiac pathology that results from an arrest in endomyocardial development in early embryogenesis. With great advances in imaging modalities, this pathological entity has been noted not only in the pediatric population but also in adults. Herein we report the case of a 62-year-old woman who successfully underwent aortic valve replacement for aortic regurgitation complicated by left ventricular noncompaction.
Keywords Adult, Aortic valve insufficiency, Heart valve prosthesis implantation, Isolated noncompaction of the ventricular myocardium, Heart defects, congenital
Introduction Left ventricular noncompaction (LVNC) is a rare cardiac disorder resulting from an arrest in normal endomyocardial embryogenesis. The prognosis of LVNC is dismal, resulting in progressive heart failure. However, some cases of LVNC in adults have been reported recently. We describe a case of aortic regurgitation complicated by LVNC, for which aortic valve replacement was performed.
Case report A 62-year-old woman came to our hospital because of dyspnea on exertion and a syncopal attack. She had no family history of cardiovascular disease. She had 24-h electrocardiographic monitoring that revealed nonsustained ventricular tachycardia, and she received amiodarone. Transthoracic echocardiography demonstrated severe regurgitation of a tricuspid aortic valve, mild mitral regurgitation, a left ventricular (LV) end-diastolic diameter of 70 mm, and LV ejection fraction of 44%. The LV wall was unusually shown with two layers: a thin compacted external (epicardial) layer; and a much thicker, non-compacted internal (endocardial) layer consisting of a trabecular meshwork with deep endocardial spaces (Figure 1). To confirm the diagnosis, we used multi-detector computed tomography which
demonstrated abnormal LV myocardium with a 2-layer structure, and no thrombi in the LV cavity. The abnormal findings were most prominent at the apical segment of the LV, and the lateral segment was involved more progressively than the septal segment. The ratio of the non-compacted layer to the compacted myocardium was 2.67 (Figure 2). The right ventricular myocardium looked normal. No significant lesions were noted on coronary arteriography. After establishing conventional medication to prevent heart failure, including an angiotensin II receptor blocker and a beta blocker, the patient underwent aortic valve replacement with a 23-mm Mosaic Ultra bioprosthetic valve (Medtronic, Minneapolis, MN, USA) in an intraannular position. The aortic crossclamp and cardiopulmonary bypass times were 92 and 121 min, respectively. She was easily weaned from cardiopulmonary bypass 1 Department of Thoracic and Cardiovascular Surgery, Nara Medical University School of Medicine, Nara, Japan 2 Department of Cardiovascular Surgery, Kouseikai Takai Hospital, Nara, Japan
Corresponding author: Tomoaki Hirose, MD, Department of Thoracic and Cardiovascular Surgery, Nara Medical University School of Medicine, 840 Shijo-cho, Kashihara, Nara, 634-8522 Japan. Email: [email protected]
Downloaded from aan.sagepub.com at WASHINGTON UNIV SCHL OF MED on November 14, 2015
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and recovered uneventfully. Electrocardiographic monitoring after the operation did not show any nonsustained ventricular tachycardia. She was discharged without any complications on postoperative day 14. The latest follow-up transthoracic echocardiography 2 years after the operation showed trivial mitral regurgitation and improvements in LV ejection fraction to 59% and LV end-diastolic diameter to 46 mm. The ratio of the non-compacted layer to the compacted myocardium was 2.8 (Figure 3).
Discussion
This condition is a rare congenital disorder resulting from an arrest in endomyocardial development in early embryogenesis.1 Although patients with a severe form of this pathological entity combined with other intracardiac anomalies are likely to die at an early stage of life, some surgical cases of adult patients with LVNC suffering from an acquired heart disease such as ischemic or valvular heart disease have recently been reported in the English literatures. To our knowledge, 5 cases of aortic valve replacement for aortic valve diseases in patients with LVNC have been reported; there were no complications or mortality in these reports.2–4
LVNC was categorized as an unclassified cardiomyopathy by the World Health Organization in 1996.
Figure 1. Transthoracic echocardiography. The left ventricular wall with a two-layered structure is composed of a thin compacted external (epicardial) layer and a much thicker, non-compacted internal (endocardial) layer consisting of a trabecular meshwork with deep endocardial spaces (arrows).
Figure 3. Transthoracic echocardiography 2 years after the operation. The left ventricular wall with a 2-layer structure is composed of a compacted external layer and a non-compacted internal layer in the apical and lateral segments. The ratio of the non-compacted internal layer to the compacted external myocardium is 2.8. C: compacted myocardium; NC: non-compacted myocardium.
Figure 2. (a,b) Multi-detector computed tomography of the heart. The abnormal findings in the left ventricle were most prominent in the apical and lateral segments. The ratio of the non-compacted internal layer to the compacted external myocardium is 2.67. C: compacted myocardium; NC: non-compacted myocardium.
Downloaded from aan.sagepub.com at WASHINGTON UNIV SCHL OF MED on November 14, 2015
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Sengupta and colleagues5 reported that LVNC was associated with aortic regurgitation in 6.3% and aortic stenosis in 9.4%. While the diagnosis is usually made by echocardiography, great advances in imaging modalities, including multi-detector computed tomography and magnetic resonance imaging, contribute to identifying LVNC. We used multi-detector computed tomography in addition to echocardiography and definitely confirmed the characteristic findings of numerous excessively prominent trabeculations and deep intertrabecular recesses within the ventricle, and communication of the intertrabecular spaces with the ventricular cavity. In our patient, the ratio of the non-compacted endocardial layer to the compacted myocardium was 2.67, which met the diagnostic criteria for LVNC.6 While the apical and lateral segments of the LV were affected by the pathology, the other segments were spared. Oechslin and colleagues,7 in an echocardiographic study, reported that the apical and mid-ventricular segments of both the inferior and lateral walls were affected in more than 80% of their patients. In examining the distribution of noncompacted myocardium in the LV, computed tomography or magnetic resonance imaging may be superior to echocardiography.8 However, there are no accepted computed tomography criteria for the diagnosis of LVNC. LV dilatation and dysfunction in the absence of thrombi in the LV cavity, unremarkable findings on coronary arteriography, and the disappearance of ventricular tachycardia after antiarrhythmic medication prompted us to perform aortic valve replacement in the hope that the procedure would reverse myocardial impairment due in part to aortic regurgitation. As a result of the surgery, an improvement in cardiac function was provided. We think that even a patient with the complication of LVNC should not be denied a surgical intervention for cardiac abnormalities, although long-term followup is needed. An increasing number of cases of LVNC have been reported recently, as imaging modalities
develop, and other intracardiac pathologies with indications for surgery should be aggressively repaired. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Chin TK, Perloff JK, Williams RG, Jue K and Mohrmann R. Isolated noncompaction of left ventricular myocardium: a study of eight cases. Circulation 1990; 82: 507–513. 2. Wilbring M, Kappert U, Scho¨n S, et al. Aortic valve replacement in noncompaction cardiomyopathy at twoyear follow-up. J Card Surg 2009; 24: 684–686. 3. Barbukhatty KO, Boldyrev SY, Rossokha OA, Kosmacheva ED and Porhanov VA. A rare case of coronary artery bypass grafting in a patient with left ventricular noncompaction. Ann Thorac Surg 2010; 90: 2047–2049. 4. Tanaka H, Kimura T, Miyamoto S. Aortic valve replacement for aortic regurgitation with rare left ventricular noncompaction. Ann Thorac Cardiovasc Surg 2012; Nov 30 [Epub ahead of print]. 5. Sengupta PP, Mohan JC, Mehta V, et al. Comparison of echocardiographic features of noncompaction of the left ventricle in adults versus idiopathic dilated cardiomyopathy in adults. Am J Cardiol 2004; 94: 389–391. 6. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C and Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001; 86: 666–671. 7. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA and Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000; 36: 493–500. 8. Hamamichi Y, Ichida F, Hashimoto I, et al. Isolated noncompaction of the ventricular myocardium: ultrafast computed tomography and magnetic resonance imaging. Int J Cardiovasc imaging 2001; 17: 305–314.
Downloaded from aan.sagepub.com at WASHINGTON UNIV SCHL OF MED on November 14, 2015
Aortic valve replacement in a patient with left ventricular noncompaction
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(9) 1065–1067 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314532106 aan.sagepub.com
Tomoaki Hirose1, Tetsuji Kawata2, Yoshihiro Hamada2, Nobuoki Tabayashi1, Takehisa Abe1 and Shigeki Taniguchi1
Abstract Left ventricular noncompaction is a rare cardiac pathology that results from an arrest in endomyocardial development in early embryogenesis. With great advances in imaging modalities, this pathological entity has been noted not only in the pediatric population but also in adults. Herein we report the case of a 62-year-old woman who successfully underwent aortic valve replacement for aortic regurgitation complicated by left ventricular noncompaction.
Keywords Adult, Aortic valve insufficiency, Heart valve prosthesis implantation, Isolated noncompaction of the ventricular myocardium, Heart defects, congenital
Introduction Left ventricular noncompaction (LVNC) is a rare cardiac disorder resulting from an arrest in normal endomyocardial embryogenesis. The prognosis of LVNC is dismal, resulting in progressive heart failure. However, some cases of LVNC in adults have been reported recently. We describe a case of aortic regurgitation complicated by LVNC, for which aortic valve replacement was performed.
Case report A 62-year-old woman came to our hospital because of dyspnea on exertion and a syncopal attack. She had no family history of cardiovascular disease. She had 24-h electrocardiographic monitoring that revealed nonsustained ventricular tachycardia, and she received amiodarone. Transthoracic echocardiography demonstrated severe regurgitation of a tricuspid aortic valve, mild mitral regurgitation, a left ventricular (LV) end-diastolic diameter of 70 mm, and LV ejection fraction of 44%. The LV wall was unusually shown with two layers: a thin compacted external (epicardial) layer; and a much thicker, non-compacted internal (endocardial) layer consisting of a trabecular meshwork with deep endocardial spaces (Figure 1). To confirm the diagnosis, we used multi-detector computed tomography which
demonstrated abnormal LV myocardium with a 2-layer structure, and no thrombi in the LV cavity. The abnormal findings were most prominent at the apical segment of the LV, and the lateral segment was involved more progressively than the septal segment. The ratio of the non-compacted layer to the compacted myocardium was 2.67 (Figure 2). The right ventricular myocardium looked normal. No significant lesions were noted on coronary arteriography. After establishing conventional medication to prevent heart failure, including an angiotensin II receptor blocker and a beta blocker, the patient underwent aortic valve replacement with a 23-mm Mosaic Ultra bioprosthetic valve (Medtronic, Minneapolis, MN, USA) in an intraannular position. The aortic crossclamp and cardiopulmonary bypass times were 92 and 121 min, respectively. She was easily weaned from cardiopulmonary bypass 1 Department of Thoracic and Cardiovascular Surgery, Nara Medical University School of Medicine, Nara, Japan 2 Department of Cardiovascular Surgery, Kouseikai Takai Hospital, Nara, Japan
Corresponding author: Tomoaki Hirose, MD, Department of Thoracic and Cardiovascular Surgery, Nara Medical University School of Medicine, 840 Shijo-cho, Kashihara, Nara, 634-8522 Japan. Email: [email protected]
Downloaded from aan.sagepub.com at WASHINGTON UNIV SCHL OF MED on November 14, 2015
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and recovered uneventfully. Electrocardiographic monitoring after the operation did not show any nonsustained ventricular tachycardia. She was discharged without any complications on postoperative day 14. The latest follow-up transthoracic echocardiography 2 years after the operation showed trivial mitral regurgitation and improvements in LV ejection fraction to 59% and LV end-diastolic diameter to 46 mm. The ratio of the non-compacted layer to the compacted myocardium was 2.8 (Figure 3).
Discussion
This condition is a rare congenital disorder resulting from an arrest in endomyocardial development in early embryogenesis.1 Although patients with a severe form of this pathological entity combined with other intracardiac anomalies are likely to die at an early stage of life, some surgical cases of adult patients with LVNC suffering from an acquired heart disease such as ischemic or valvular heart disease have recently been reported in the English literatures. To our knowledge, 5 cases of aortic valve replacement for aortic valve diseases in patients with LVNC have been reported; there were no complications or mortality in these reports.2–4
LVNC was categorized as an unclassified cardiomyopathy by the World Health Organization in 1996.
Figure 1. Transthoracic echocardiography. The left ventricular wall with a two-layered structure is composed of a thin compacted external (epicardial) layer and a much thicker, non-compacted internal (endocardial) layer consisting of a trabecular meshwork with deep endocardial spaces (arrows).
Figure 3. Transthoracic echocardiography 2 years after the operation. The left ventricular wall with a 2-layer structure is composed of a compacted external layer and a non-compacted internal layer in the apical and lateral segments. The ratio of the non-compacted internal layer to the compacted external myocardium is 2.8. C: compacted myocardium; NC: non-compacted myocardium.
Figure 2. (a,b) Multi-detector computed tomography of the heart. The abnormal findings in the left ventricle were most prominent in the apical and lateral segments. The ratio of the non-compacted internal layer to the compacted external myocardium is 2.67. C: compacted myocardium; NC: non-compacted myocardium.
Downloaded from aan.sagepub.com at WASHINGTON UNIV SCHL OF MED on November 14, 2015
Hirose et al.
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Sengupta and colleagues5 reported that LVNC was associated with aortic regurgitation in 6.3% and aortic stenosis in 9.4%. While the diagnosis is usually made by echocardiography, great advances in imaging modalities, including multi-detector computed tomography and magnetic resonance imaging, contribute to identifying LVNC. We used multi-detector computed tomography in addition to echocardiography and definitely confirmed the characteristic findings of numerous excessively prominent trabeculations and deep intertrabecular recesses within the ventricle, and communication of the intertrabecular spaces with the ventricular cavity. In our patient, the ratio of the non-compacted endocardial layer to the compacted myocardium was 2.67, which met the diagnostic criteria for LVNC.6 While the apical and lateral segments of the LV were affected by the pathology, the other segments were spared. Oechslin and colleagues,7 in an echocardiographic study, reported that the apical and mid-ventricular segments of both the inferior and lateral walls were affected in more than 80% of their patients. In examining the distribution of noncompacted myocardium in the LV, computed tomography or magnetic resonance imaging may be superior to echocardiography.8 However, there are no accepted computed tomography criteria for the diagnosis of LVNC. LV dilatation and dysfunction in the absence of thrombi in the LV cavity, unremarkable findings on coronary arteriography, and the disappearance of ventricular tachycardia after antiarrhythmic medication prompted us to perform aortic valve replacement in the hope that the procedure would reverse myocardial impairment due in part to aortic regurgitation. As a result of the surgery, an improvement in cardiac function was provided. We think that even a patient with the complication of LVNC should not be denied a surgical intervention for cardiac abnormalities, although long-term followup is needed. An increasing number of cases of LVNC have been reported recently, as imaging modalities
develop, and other intracardiac pathologies with indications for surgery should be aggressively repaired. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Chin TK, Perloff JK, Williams RG, Jue K and Mohrmann R. Isolated noncompaction of left ventricular myocardium: a study of eight cases. Circulation 1990; 82: 507–513. 2. Wilbring M, Kappert U, Scho¨n S, et al. Aortic valve replacement in noncompaction cardiomyopathy at twoyear follow-up. J Card Surg 2009; 24: 684–686. 3. Barbukhatty KO, Boldyrev SY, Rossokha OA, Kosmacheva ED and Porhanov VA. A rare case of coronary artery bypass grafting in a patient with left ventricular noncompaction. Ann Thorac Surg 2010; 90: 2047–2049. 4. Tanaka H, Kimura T, Miyamoto S. Aortic valve replacement for aortic regurgitation with rare left ventricular noncompaction. Ann Thorac Cardiovasc Surg 2012; Nov 30 [Epub ahead of print]. 5. Sengupta PP, Mohan JC, Mehta V, et al. Comparison of echocardiographic features of noncompaction of the left ventricle in adults versus idiopathic dilated cardiomyopathy in adults. Am J Cardiol 2004; 94: 389–391. 6. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C and Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001; 86: 666–671. 7. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA and Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000; 36: 493–500. 8. Hamamichi Y, Ichida F, Hashimoto I, et al. Isolated noncompaction of the ventricular myocardium: ultrafast computed tomography and magnetic resonance imaging. Int J Cardiovasc imaging 2001; 17: 305–314.
Downloaded from aan.sagepub.com at WASHINGTON UNIV SCHL OF MED on November 14, 2015
