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Cerebral infarction and right ventricular noncompaction
International Journal of Cardiology 148 (2011) e45 – e46 www.elsevier.com/locate/ijcard
Letter to the Editor
Cerebral infarction and right ventricular noncompaction Ze-Zhou Song ⁎ Department of Ultrasound, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, P.R. China Received 5 February 2009; accepted 27 February 2009 Available online 25 March 2009
Abstract A 26-year-old man admitted with dysphasia and bilateral headache for three days. He had no known risk factors for stroke. Transthoracic two-dimensional echocardiography showed prominent trabeculations, with deep intertrabecular recesses in apex of the right ventricle. The multi-detector-row spiral cerebral computerized tomography revealed a little infarction in bilateral temporal and frontal lobe. The main mechanisms of neurovascular complications in NVM may be related to development of thrombi in the intertrabecular space. Extensively trabeculated ventricle, depressed systolic function, and/or the development of atrial fibrillation are the causes for thrombus formation7. Embolic stroke has been only rarely described in association with NVM9. However, whether NVM is a risk factor for stroke is controversially discussed. Prevention of embolic complications is an important management issue, and several authors have recommended long-term prophylactic anticoagulation for patients in NVM with atrial fibrillation and severe systolic dysfunction. The field of neurovascular complications, and specially cerebral infarction, in NVM of right ventricular remains not yet fully understood. Keywords: Cerebral infarction; Right ventricular noncompaction
1. Introduction Noncompaction of the ventricular myocardium (NVM) is a relatively new clinicopathologic entity, which is characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses communicating with the ventricular cavity. NVM is thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. Results of recent studies confirmed the hypothesis that NVM is part of a more widespread cardiomyopathy, involving both the morphologically normal and dysmorphic segments. It may not be associated with any factor that could explain the arrest of the development of the myocardial structure (isolated HVM/NVM) [1], and it has been also reported of the possibility that HVM/NVM could present as an acquired disease [2–6]. In the majority of the cases NVM, is associated with other cardiac or extra-cardiac disease [7,8]. The most
⁎ #79 Qingchun Road, Hangzhou, Zhejiang Province, P.R. China. Fax: +86 571 8723 6628. E-mail address: [email protected]. 0167-5273/$ - see front matter doi:10.1016/j.ijcard.2009.02.033
frequent extra-cardiac abnormalities in NVM patients are neuromuscular disorders [9]. Central nervous system affection has also been reported in single cases, usually manifesting as stroke from cardiac embolism, assumed to originate from the inter-trabecular recesses [10]. Few reports are about the association of cerebral infarction and NVM of the right ventricle. Therefore, we present a case of NVM of the right ventricle in an adult man associated with cerebral infarction, which was confirmed by transthoracic echocardiography and cerebral computerized tomography.
2. Case report The patient was a 26-year-old man admitted with dysphasia and bilateral headache for three days. In past, he was never found to have any heart diseases and relevant history of familial heart diseases. He was never found to have diabetes mellitus and denied relevant history of smoking. He had no known risk factors for stroke. On examination, his blood pressure was 105/70 mmHg; pulse 62 beat per minute. The electrocardiogram revealed
e46
Z.-Z. Song / International Journal of Cardiology 148 (2011) e45–e46
Fig. 1. Right ventricular long axis view at next to apex level showing the prominent trabeculations and deep intertrabecular recesses of the right ventricular wall by transthoracic two-dimensional echocardiography.
sinus regularity, multiple extrasystolic ventricular beats, but without any evidence of serious ventricular arrhythmias. Chest X-ray revealed the heart to be mildly enlarged and 0.61 in cardiothoracic ratio, but there was no pulmonary congestion. Transthoracic two-dimensional echocardiography showed prominent trabeculations, with deep intertrabecular recesses in apex of the right ventricle (Fig. 1). The right ventricular and atrium was mildly dilated, apex of right ventricular were hypokinetic with others eukinesia walls and right ventricular function was mildly impaired with an right ventricular area changes fraction of 42%. The left ventricular was not markedly dilated and its function was normal with a left ventricular ejection fraction of 63%. Color and continued wave Doppler echocardiography revealed the color flow of deep intertrabecular recesses to right ventricular chamber. The multi-detector-row spiral cerebral computerized tomography revealed a little infarction in bilateral temporal and frontal lobe. 3. Discussion The main mechanisms of neurovascular complications in NVM may be related to development of thrombi in the intertrabecular space. Extensively trabeculated ventricle, depressed systolic function, and/ or the development of atrial fibrillation are the causes for thrombus formation [7]. Embolic stroke has been only rarely described in association with NVM [9]. However, whether NVM is a risk factor for stroke is controversially discussed [8,11]. Some previous studies and case reports suggested that stroke is a complication of NVM [7,8,11]. However, the majority of these patients had an increased risk of stroke due to other cardiovascular or coagulation abnormalities, such as atrial fibrillation [7,8,11]. On the contrary, a study on 62 NVMpatients found no increased risk for thrombo-embolism in NVM-patients as compared with 62 controls matched for
sex, age and systolic function [11]. The authors concluded that it could not be decided if NVM carries and increased risk for cardiac embolism or if additional cardiovascular abnormalities, associated with NVM in a considerable number of cases, are responsible for the embolic events. The observed association of decreased ventricular function with stroke in NVM patients suggested that dilated, poorly contracting ventricles promote thrombus formation within the inter-trabecular recesses, whereas this pathomechanism is not effective in NVM with good systolic function. Prevention of embolic complications is an important management issue, and several authors have recommended long-term prophylactic anticoagulation for patients in NVM with atrial fibrillation and severe systolic dysfunction. The field of neurovascular complications, and specially cerebral infarction, in NVM of right ventricular remains not yet fully understood.
Acknowledgements The author of this manuscript has certified that he complies with the Principles of Ethical Publishing in the International Journal of Cardiology [12].
References [1] Ritter M, Oechslin E, Sutsch G, Attenhofer C, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 1997;72:26–31. [2] Song ZZ, Ma J. A rare combination of left ventricular noncompaction and a right coronary artery-to-right ventricle fistula: echocardiographic features. J Ultrasound Med 2007;26:547–50. [3] Stollberger C, Winkler-Dworak M, Blazek G, Finsterer J. Agedependency of cardiac and neuromuscular findings in left ventricular noncompaction. Int J Cardiol 2006;111:131–5. [4] Chiribiri A, Leuzzi S, Salvetti I, Patané S, Bonamini R, Trevi GP, Gaita F, Cesarani F. Isolated noncompaction of the right ventricular myocardium in the adulthood? Int J Cardiol 2009;134:e17–9. [5] Finsterer J, Stfllberger C, Wegmann R, Janssen LAJ. Acquired left ventricular hypertrabeculation/noncompaction in myotonic dystrophy type 1. Int J Cardiol 2009;137:310–3. [6] Song ZZ. A combination of right ventricular hypertrabeculation/ noncompaction and Ebstein's anomaly. Int J Cardiol 2010;143:e30–3. [7] Ying ZQ, Xu G, Chen S, Ma J, You XD. Cerebral infarction in an adult patient with right ventricular hypertrabeculation/noncompaction. Int J Cardiol 2008;127:e150–1. [8] Finsterer J, Stfllberger C, Molzer G. Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy. Int J Cardiol 2008;130:344–8. [9] Finsterer J, Stollberger C, Blazek G. Neuromuscular implications in left ventricular hypertrabeculation/noncompaction. Int J Cardiol 2006;110:288–300. [10] Stollberger C, Winkler-Dworak M, Blazek G, Finsterer J. Cardiologic and neurologic findings in left ventricular hypertrabeculation/noncompaction relating to echocardiographic indication. Int J Cardiol 2007;119:28–32. [11] Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology 2005;103:68–72. [12] Coats AJ. Ethical authorship and publishing. Int J Cardiol 2009;131:149–50.
Letter to the Editor
Cerebral infarction and right ventricular noncompaction Ze-Zhou Song ⁎ Department of Ultrasound, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, P.R. China Received 5 February 2009; accepted 27 February 2009 Available online 25 March 2009
Abstract A 26-year-old man admitted with dysphasia and bilateral headache for three days. He had no known risk factors for stroke. Transthoracic two-dimensional echocardiography showed prominent trabeculations, with deep intertrabecular recesses in apex of the right ventricle. The multi-detector-row spiral cerebral computerized tomography revealed a little infarction in bilateral temporal and frontal lobe. The main mechanisms of neurovascular complications in NVM may be related to development of thrombi in the intertrabecular space. Extensively trabeculated ventricle, depressed systolic function, and/or the development of atrial fibrillation are the causes for thrombus formation7. Embolic stroke has been only rarely described in association with NVM9. However, whether NVM is a risk factor for stroke is controversially discussed. Prevention of embolic complications is an important management issue, and several authors have recommended long-term prophylactic anticoagulation for patients in NVM with atrial fibrillation and severe systolic dysfunction. The field of neurovascular complications, and specially cerebral infarction, in NVM of right ventricular remains not yet fully understood. Keywords: Cerebral infarction; Right ventricular noncompaction
1. Introduction Noncompaction of the ventricular myocardium (NVM) is a relatively new clinicopathologic entity, which is characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses communicating with the ventricular cavity. NVM is thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. Results of recent studies confirmed the hypothesis that NVM is part of a more widespread cardiomyopathy, involving both the morphologically normal and dysmorphic segments. It may not be associated with any factor that could explain the arrest of the development of the myocardial structure (isolated HVM/NVM) [1], and it has been also reported of the possibility that HVM/NVM could present as an acquired disease [2–6]. In the majority of the cases NVM, is associated with other cardiac or extra-cardiac disease [7,8]. The most
⁎ #79 Qingchun Road, Hangzhou, Zhejiang Province, P.R. China. Fax: +86 571 8723 6628. E-mail address: [email protected]. 0167-5273/$ - see front matter doi:10.1016/j.ijcard.2009.02.033
frequent extra-cardiac abnormalities in NVM patients are neuromuscular disorders [9]. Central nervous system affection has also been reported in single cases, usually manifesting as stroke from cardiac embolism, assumed to originate from the inter-trabecular recesses [10]. Few reports are about the association of cerebral infarction and NVM of the right ventricle. Therefore, we present a case of NVM of the right ventricle in an adult man associated with cerebral infarction, which was confirmed by transthoracic echocardiography and cerebral computerized tomography.
2. Case report The patient was a 26-year-old man admitted with dysphasia and bilateral headache for three days. In past, he was never found to have any heart diseases and relevant history of familial heart diseases. He was never found to have diabetes mellitus and denied relevant history of smoking. He had no known risk factors for stroke. On examination, his blood pressure was 105/70 mmHg; pulse 62 beat per minute. The electrocardiogram revealed
e46
Z.-Z. Song / International Journal of Cardiology 148 (2011) e45–e46
Fig. 1. Right ventricular long axis view at next to apex level showing the prominent trabeculations and deep intertrabecular recesses of the right ventricular wall by transthoracic two-dimensional echocardiography.
sinus regularity, multiple extrasystolic ventricular beats, but without any evidence of serious ventricular arrhythmias. Chest X-ray revealed the heart to be mildly enlarged and 0.61 in cardiothoracic ratio, but there was no pulmonary congestion. Transthoracic two-dimensional echocardiography showed prominent trabeculations, with deep intertrabecular recesses in apex of the right ventricle (Fig. 1). The right ventricular and atrium was mildly dilated, apex of right ventricular were hypokinetic with others eukinesia walls and right ventricular function was mildly impaired with an right ventricular area changes fraction of 42%. The left ventricular was not markedly dilated and its function was normal with a left ventricular ejection fraction of 63%. Color and continued wave Doppler echocardiography revealed the color flow of deep intertrabecular recesses to right ventricular chamber. The multi-detector-row spiral cerebral computerized tomography revealed a little infarction in bilateral temporal and frontal lobe. 3. Discussion The main mechanisms of neurovascular complications in NVM may be related to development of thrombi in the intertrabecular space. Extensively trabeculated ventricle, depressed systolic function, and/ or the development of atrial fibrillation are the causes for thrombus formation [7]. Embolic stroke has been only rarely described in association with NVM [9]. However, whether NVM is a risk factor for stroke is controversially discussed [8,11]. Some previous studies and case reports suggested that stroke is a complication of NVM [7,8,11]. However, the majority of these patients had an increased risk of stroke due to other cardiovascular or coagulation abnormalities, such as atrial fibrillation [7,8,11]. On the contrary, a study on 62 NVMpatients found no increased risk for thrombo-embolism in NVM-patients as compared with 62 controls matched for
sex, age and systolic function [11]. The authors concluded that it could not be decided if NVM carries and increased risk for cardiac embolism or if additional cardiovascular abnormalities, associated with NVM in a considerable number of cases, are responsible for the embolic events. The observed association of decreased ventricular function with stroke in NVM patients suggested that dilated, poorly contracting ventricles promote thrombus formation within the inter-trabecular recesses, whereas this pathomechanism is not effective in NVM with good systolic function. Prevention of embolic complications is an important management issue, and several authors have recommended long-term prophylactic anticoagulation for patients in NVM with atrial fibrillation and severe systolic dysfunction. The field of neurovascular complications, and specially cerebral infarction, in NVM of right ventricular remains not yet fully understood.
Acknowledgements The author of this manuscript has certified that he complies with the Principles of Ethical Publishing in the International Journal of Cardiology [12].
References [1] Ritter M, Oechslin E, Sutsch G, Attenhofer C, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 1997;72:26–31. [2] Song ZZ, Ma J. A rare combination of left ventricular noncompaction and a right coronary artery-to-right ventricle fistula: echocardiographic features. J Ultrasound Med 2007;26:547–50. [3] Stollberger C, Winkler-Dworak M, Blazek G, Finsterer J. Agedependency of cardiac and neuromuscular findings in left ventricular noncompaction. Int J Cardiol 2006;111:131–5. [4] Chiribiri A, Leuzzi S, Salvetti I, Patané S, Bonamini R, Trevi GP, Gaita F, Cesarani F. Isolated noncompaction of the right ventricular myocardium in the adulthood? Int J Cardiol 2009;134:e17–9. [5] Finsterer J, Stfllberger C, Wegmann R, Janssen LAJ. Acquired left ventricular hypertrabeculation/noncompaction in myotonic dystrophy type 1. Int J Cardiol 2009;137:310–3. [6] Song ZZ. A combination of right ventricular hypertrabeculation/ noncompaction and Ebstein's anomaly. Int J Cardiol 2010;143:e30–3. [7] Ying ZQ, Xu G, Chen S, Ma J, You XD. Cerebral infarction in an adult patient with right ventricular hypertrabeculation/noncompaction. Int J Cardiol 2008;127:e150–1. [8] Finsterer J, Stfllberger C, Molzer G. Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy. Int J Cardiol 2008;130:344–8. [9] Finsterer J, Stollberger C, Blazek G. Neuromuscular implications in left ventricular hypertrabeculation/noncompaction. Int J Cardiol 2006;110:288–300. [10] Stollberger C, Winkler-Dworak M, Blazek G, Finsterer J. Cardiologic and neurologic findings in left ventricular hypertrabeculation/noncompaction relating to echocardiographic indication. Int J Cardiol 2007;119:28–32. [11] Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology 2005;103:68–72. [12] Coats AJ. Ethical authorship and publishing. Int J Cardiol 2009;131:149–50.