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The association of ventricular noncompaction and apical long tunnel type ventricular septal defect causes electrocardiographic abnormality
International Journal of Cardiology 187 (2015) 673–675
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
The association of ventricular noncompaction and apical long tunnel type ventricular septal defect causes electrocardiographic abnormality Hamidullah Haqmal a, Cengiz Ozturk a,⁎, Mustafa Aparci b, Sabahattin Sari c a b c
Gulhane Military Medical Academy, School of Medicine, Department of Cardiology, Etlik, Ankara, Turkey Kasimpasa Military Hospital, Department of Cardiology, Istanbul, Turkey Gulhane Military Medical Academy, School of Medicine, Department of Radiology, Etlik, Ankara, Turkey
a r t i c l e
i n f o
Article history: Received 24 March 2015 Accepted 27 March 2015 Available online 28 March 2015 Keywords: Ventricular septal defect Ventricular noncompaction
Dear Editor, Ventricular septal defect (VSD) is a common congenital heart deformity occurring as a single malformation or as part of multiple malformations. The defects are usually located in the perimembranous septum in approximately 80% of cases but in rare cases (10–20%), they are located in the apical area of the trabecular septum. Noncompaction cardiomyopathy (LVNC) is a rare genetic cardiomyopathy characterized by a thin, compacted epicardial layer and an extensive noncompacted endocardial layer. Left ventricular (LV) noncompaction is a rare cardiomyopathy caused by a reduced left ventricular function. Morphologically, there is prominent trabeculation with deep intertrabecular recesses and a thin compacted myocardial layer comprising less than 50% of the LV wall thickness. Echocardiography is usually the first-choice to diagnose these pathologies. There are some reports about the association of ventricular noncompaction and other congenital heart defects. These are atrial septal defect, ventricular septal defect, hypertrophic cardiomyopathy, congenital
⁎ Corresponding author at: Department of Cardiology, Gulhane School of Medicine, Tevfik Saglam St., 06018 Etlik, Ankara, Turkey. E-mail address: [email protected] (C. Ozturk).
http://dx.doi.org/10.1016/j.ijcard.2015.03.396 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
aplasia of the right pulmonary veins, Ebstein's anomaly and diverse cardiac anomalies [1–8]. Some author thesis abnormalities are both genetically determined and familial diseases that possess variable but overlapping genetic defects. The association of ventricular noncompaction and apical long tunnel type ventricular septal defect is rare. We report a case with the association of ventricular noncompaction and apical long tunnel type ventricular septal defect and discuss the role of multimodal imaging in the diagnosis and assessment of the disease. A 44-year old male patient was admitted to our outpatient clinic because of chest pain related exercise in the last six months. No previous history of any cardiac symptom is present. His medical and family history was unremarkable. 2/6 systolic murmur in apical murmur was auscultated on physical examination. Systemic blood pressure was 125/75 mm Hg and pulse rate was 67 beats per minute. The electrocardiogram showed normal sinus rhythm and diffuse T wave inversion in chest leads (Fig. 1). Transthoracic echocardiography showed normal LV ejection fraction (60%) and increased trabeculation in the inferior, lateral and apical portions of the LV wall and also showed a mild aortic regurgitation and there was a left to right shunt and defect of about 15 mm length and 5 mm wide VSD in the inferolateral and apical portions of the left ventricle (Fig. 2, Videos 1–3). The left atrium (LA) and LV size, shape & volume were normal and QP/QS = 1.5, pulmonary artery systolic pressure was 30 mm Hg. To clarify this pathology, we performed computed tomography (CT) and it demonstrated the association of ventricular noncompaction and apical long tunnel type ventricular septal (Fig. 3). After that, left ventriculography was performed and left to right shunt flow was seen on left anterior oblique position (Video 4). Any intervention was not considered and decided for follow-up medically. We report here a case of the association of ventricular noncompaction and apical long tunnel type ventricular septal cause electrocardiographic abnormality detected by multimodality imaging. Transthoracic echocardiography is an excellent tool to scan and diagnose LVNC and related abnormalities. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2015.03.396. Conflict of interest There is no conflict of interest.
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H. Haqmal et al. / International Journal of Cardiology 187 (2015) 673–675
Fig. 1. Color flow pulse wave Doppler jet of shunt at the apical portion.
Fig. 2. CT view of the ventricular noncompaction and apical long tunnel type ventricular septal defect.
H. Haqmal et al. / International Journal of Cardiology 187 (2015) 673–675
675
Fig. 3. CT angiographic window of the ventricular noncompaction and apical long tunnel type ventricular septal.
References [1] Z.Z. Song, A combination of right ventricular hypertrabeculation/noncompaction and atrial septal defect, Int. J. Cardiol. 148 (2) (Apr 14 2011) e25–e27. [2] L. Yuan, M. Xie, T.O. Cheng, X. Wang, F. Zhu, X. Kong, D. Ghoorah, Left ventricular noncompaction associated with hypertrophic cardiomyopathy: echocardiographic diagnosis and genetic analysis of a new pedigree in China, Int. J. Cardiol. 174 (2) (Jun 15 2014) 249–259. [3] G. De Pasquale Meyer, O. Kretschmar, E.R. Valsangiacomo Buechel, C. Kellenberger, U. Bauersfeld, C.H. Attenhofer Jost, Rare combination of congenital aplasia of the right pulmonary veins, left ventricular noncompaction, partial membranous obstruction of left-sided pulmonary veins and secundum atrial septal defect, Int. J. Cardiol. 152 (3) (Nov 3 2011) e49–e51. [4] K. Hirono, Y. Hata, K. Ibuki, N. Yoshimura, Familial Ebstein's anomaly, left ventricular noncompaction, and ventricular septal defect associated with an MYH7 mutation, J. Thorac. Cardiovasc. Surg. 148 (5) (Nov 2014) e223–e226. [5] M.W. Wessels, J.C. Herkert, I.M. Frohn-Mulder, M. Dalinghaus, A. van den Wijngaard, R.R. de Krijger, M. Michels, I.F. de Coo, Y.M. Hoedemaekers, D. Dooijes, Compound
heterozygous or homozygous truncating MYBPC3 mutations cause lethal cardiomyopathy with features of noncompaction and septal defects, Eur. J. Hum. Genet. (Oct 22 2014)http://dx.doi.org/10.1038/ejhg.2014.211. [6] H. Ashraf, L. Pradhan, E.I. Chang, R. Terada, N.J. Ryan, L.E. Briggs, R. Chowdhury, M.A. Zárate, Y. Sugi, H.J. Nam, D.W. Benson, R.H. Anderson, H. Kasahara, A mouse model of human congenital heart disease: high incidence of diverse cardiac anomalies and ventricular noncompaction produced by heterozygous Nkx2-5 homeodomain missense mutation, Circ. Cardiovasc. Genet. 7 (4) (Aug 2014) 423–433. [7] Y. Sasaki, T. Ozawa, H. Matsuura, T. Saji, T. Fujii, Y. Watanabe, N. Shiono, Y. Takanashi, N. Koyama, Ventricular septal defect repair in an infant with severe pulmonary hypertension and preoperatively diagnosed left ventricular noncompaction, J. Thorac. Cardiovasc. Surg. 139 (3) (Mar 2010) e31–e33. [8] M. Emini, H. Selmani, G. Bajraktari, Late detection of noncompaction of the myocardium in an adult with complete interventricular septal defect, Turk Kardiyol. Dern. Ars. 37 (6) (Sep 2009) 407–409.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
The association of ventricular noncompaction and apical long tunnel type ventricular septal defect causes electrocardiographic abnormality Hamidullah Haqmal a, Cengiz Ozturk a,⁎, Mustafa Aparci b, Sabahattin Sari c a b c
Gulhane Military Medical Academy, School of Medicine, Department of Cardiology, Etlik, Ankara, Turkey Kasimpasa Military Hospital, Department of Cardiology, Istanbul, Turkey Gulhane Military Medical Academy, School of Medicine, Department of Radiology, Etlik, Ankara, Turkey
a r t i c l e
i n f o
Article history: Received 24 March 2015 Accepted 27 March 2015 Available online 28 March 2015 Keywords: Ventricular septal defect Ventricular noncompaction
Dear Editor, Ventricular septal defect (VSD) is a common congenital heart deformity occurring as a single malformation or as part of multiple malformations. The defects are usually located in the perimembranous septum in approximately 80% of cases but in rare cases (10–20%), they are located in the apical area of the trabecular septum. Noncompaction cardiomyopathy (LVNC) is a rare genetic cardiomyopathy characterized by a thin, compacted epicardial layer and an extensive noncompacted endocardial layer. Left ventricular (LV) noncompaction is a rare cardiomyopathy caused by a reduced left ventricular function. Morphologically, there is prominent trabeculation with deep intertrabecular recesses and a thin compacted myocardial layer comprising less than 50% of the LV wall thickness. Echocardiography is usually the first-choice to diagnose these pathologies. There are some reports about the association of ventricular noncompaction and other congenital heart defects. These are atrial septal defect, ventricular septal defect, hypertrophic cardiomyopathy, congenital
⁎ Corresponding author at: Department of Cardiology, Gulhane School of Medicine, Tevfik Saglam St., 06018 Etlik, Ankara, Turkey. E-mail address: [email protected] (C. Ozturk).
http://dx.doi.org/10.1016/j.ijcard.2015.03.396 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
aplasia of the right pulmonary veins, Ebstein's anomaly and diverse cardiac anomalies [1–8]. Some author thesis abnormalities are both genetically determined and familial diseases that possess variable but overlapping genetic defects. The association of ventricular noncompaction and apical long tunnel type ventricular septal defect is rare. We report a case with the association of ventricular noncompaction and apical long tunnel type ventricular septal defect and discuss the role of multimodal imaging in the diagnosis and assessment of the disease. A 44-year old male patient was admitted to our outpatient clinic because of chest pain related exercise in the last six months. No previous history of any cardiac symptom is present. His medical and family history was unremarkable. 2/6 systolic murmur in apical murmur was auscultated on physical examination. Systemic blood pressure was 125/75 mm Hg and pulse rate was 67 beats per minute. The electrocardiogram showed normal sinus rhythm and diffuse T wave inversion in chest leads (Fig. 1). Transthoracic echocardiography showed normal LV ejection fraction (60%) and increased trabeculation in the inferior, lateral and apical portions of the LV wall and also showed a mild aortic regurgitation and there was a left to right shunt and defect of about 15 mm length and 5 mm wide VSD in the inferolateral and apical portions of the left ventricle (Fig. 2, Videos 1–3). The left atrium (LA) and LV size, shape & volume were normal and QP/QS = 1.5, pulmonary artery systolic pressure was 30 mm Hg. To clarify this pathology, we performed computed tomography (CT) and it demonstrated the association of ventricular noncompaction and apical long tunnel type ventricular septal (Fig. 3). After that, left ventriculography was performed and left to right shunt flow was seen on left anterior oblique position (Video 4). Any intervention was not considered and decided for follow-up medically. We report here a case of the association of ventricular noncompaction and apical long tunnel type ventricular septal cause electrocardiographic abnormality detected by multimodality imaging. Transthoracic echocardiography is an excellent tool to scan and diagnose LVNC and related abnormalities. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2015.03.396. Conflict of interest There is no conflict of interest.
674
H. Haqmal et al. / International Journal of Cardiology 187 (2015) 673–675
Fig. 1. Color flow pulse wave Doppler jet of shunt at the apical portion.
Fig. 2. CT view of the ventricular noncompaction and apical long tunnel type ventricular septal defect.
H. Haqmal et al. / International Journal of Cardiology 187 (2015) 673–675
675
Fig. 3. CT angiographic window of the ventricular noncompaction and apical long tunnel type ventricular septal.
References [1] Z.Z. Song, A combination of right ventricular hypertrabeculation/noncompaction and atrial septal defect, Int. J. Cardiol. 148 (2) (Apr 14 2011) e25–e27. [2] L. Yuan, M. Xie, T.O. Cheng, X. Wang, F. Zhu, X. Kong, D. Ghoorah, Left ventricular noncompaction associated with hypertrophic cardiomyopathy: echocardiographic diagnosis and genetic analysis of a new pedigree in China, Int. J. Cardiol. 174 (2) (Jun 15 2014) 249–259. [3] G. De Pasquale Meyer, O. Kretschmar, E.R. Valsangiacomo Buechel, C. Kellenberger, U. Bauersfeld, C.H. Attenhofer Jost, Rare combination of congenital aplasia of the right pulmonary veins, left ventricular noncompaction, partial membranous obstruction of left-sided pulmonary veins and secundum atrial septal defect, Int. J. Cardiol. 152 (3) (Nov 3 2011) e49–e51. [4] K. Hirono, Y. Hata, K. Ibuki, N. Yoshimura, Familial Ebstein's anomaly, left ventricular noncompaction, and ventricular septal defect associated with an MYH7 mutation, J. Thorac. Cardiovasc. Surg. 148 (5) (Nov 2014) e223–e226. [5] M.W. Wessels, J.C. Herkert, I.M. Frohn-Mulder, M. Dalinghaus, A. van den Wijngaard, R.R. de Krijger, M. Michels, I.F. de Coo, Y.M. Hoedemaekers, D. Dooijes, Compound
heterozygous or homozygous truncating MYBPC3 mutations cause lethal cardiomyopathy with features of noncompaction and septal defects, Eur. J. Hum. Genet. (Oct 22 2014)http://dx.doi.org/10.1038/ejhg.2014.211. [6] H. Ashraf, L. Pradhan, E.I. Chang, R. Terada, N.J. Ryan, L.E. Briggs, R. Chowdhury, M.A. Zárate, Y. Sugi, H.J. Nam, D.W. Benson, R.H. Anderson, H. Kasahara, A mouse model of human congenital heart disease: high incidence of diverse cardiac anomalies and ventricular noncompaction produced by heterozygous Nkx2-5 homeodomain missense mutation, Circ. Cardiovasc. Genet. 7 (4) (Aug 2014) 423–433. [7] Y. Sasaki, T. Ozawa, H. Matsuura, T. Saji, T. Fujii, Y. Watanabe, N. Shiono, Y. Takanashi, N. Koyama, Ventricular septal defect repair in an infant with severe pulmonary hypertension and preoperatively diagnosed left ventricular noncompaction, J. Thorac. Cardiovasc. Surg. 139 (3) (Mar 2010) e31–e33. [8] M. Emini, H. Selmani, G. Bajraktari, Late detection of noncompaction of the myocardium in an adult with complete interventricular septal defect, Turk Kardiyol. Dern. Ars. 37 (6) (Sep 2009) 407–409.