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Left ventricular non-compaction and its cardiac and neurologic implications

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Abstract

Left ventricular non-compaction, also known as left ventricular hypertrabeculation (LVHT), is a morphological abnormality of the left ventricular myocardium, characterised by a meshwork of myocardial strings, interlacing, and orderless in arrangement. LVHT is most frequently located in the apex and the lateral wall and may occur with or without other congenital or acquired cardiac abnormalities. LVHT is believed to be congenital in the majority of the cases but may develop during life in single cases (acquired LVHT). Congenital LVHT is believed to result from defective late-stage embryonic development of the myocardial architecture. The pathogenesis of acquired LVHT remains speculative. LVHT is most frequently found on transthoracic echocardiography and cardiac MRI but may be visualised also with other imaging techniques. In the majority of the cases, LVHT is associated with hereditary cardiac, neuromuscular, non-cardiac/non-muscle disease, or chromosomal aberrations. In the majority of the cases, LVHT is complicated by ventricular arrhythmias, systolic dysfunction, cardiac embolism, or sudden cardiac death. LVHT per se does not require a specific treatment. Only in case of complications, such as ventricular arrhythmias, cardioembolism, or systolic dysfunction, adequate therapy is indicated. Though initially assessed as poor, the prognosis of LVHT has meanwhile improved, most likely due to the increased awareness for the abnormality and the timely administration of adequate therapy.

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References

  1. Engberding R, Yelbuz TM, Breithardt G (2007) Isolated noncompaction of the left ventricular myocardium—a review of the literature two decades after the initial case description. Clin Res Cardiol 96:481–488

    Article  PubMed  CAS  Google Scholar 

  2. Finsterer J (2009) Cardiogenetics, neurogenetics, and pathogenetics of left ventricular hypertrabeculation/noncompaction. Pediatr Cardiol 30:659–681

    Article  PubMed  Google Scholar 

  3. Breckenridge RA, Anderson RH, Elliott PM (2007) Isolated left ventricular non-compaction: the case for abnormal myocardial development. Cardiol Young 17:124–129

    Article  PubMed  Google Scholar 

  4. Dusek J, Ostádal B, Duskova M (1975) Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 99:312–317

    PubMed  CAS  Google Scholar 

  5. Engberding R, Bender F (1984) Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids. Am J Cardiol 53:1733–1734

    Article  PubMed  CAS  Google Scholar 

  6. Finsterer J, Stöllberger C, Wegmann R, Jarius C, Janssen B (2001) Left ventricular hypertrabeculation in myotonic dystrophy type 1. Herz 26:287–290

    Article  PubMed  CAS  Google Scholar 

  7. Bellet S, Gouley BA (1932) Congenital heart disease with multiple cardiac anomalies: report of a case showing aortic atresia, fibrous scar in myocardium and embryonal sinusoidal remains. Am J Med Sci 183:458–465

    Article  Google Scholar 

  8. Anderson RH (2008) Ventricular non-compaction—a frequently ignored finding? Eur Heart J 29:10–11

    Article  PubMed  Google Scholar 

  9. Pfammatter JP, Paul T, Flik J, Drescher J, Kallfelz HC (1995) Q-fever associated myocarditis in a 14-year-old boy. Z Kardiol 84:947–950

    PubMed  CAS  Google Scholar 

  10. Stöllberger C, Finsterer J, Blazek G, Bittner RE (1996) Left ventricular non-compaction in a patient with Becker’s muscular dystrophy. Heart 76:380

    Article  PubMed  Google Scholar 

  11. Ritter M, Oechslin E, Sütsch G, Attenhofer C, Schneider J, Jenni R (1997) Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 72:26–31

    Article  PubMed  CAS  Google Scholar 

  12. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 82:507–513

    PubMed  CAS  Google Scholar 

  13. Tavli V, Kayhan B, Okur FF, Kozan M, Kirman M, Bakiler AR, Tekdoğan M (2001) Noncompaction of the right ventricle following Senning repair. Turk J Pediatr 43:261–263

    PubMed  CAS  Google Scholar 

  14. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R (2000) Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 36:493–500

    Article  PubMed  CAS  Google Scholar 

  15. Stöllberger C, Blazek G, Winkler-Dworak M, Finsterer J (2008) Sex differences in left ventricular noncompaction in patients with and without neuromuscular disorders. Rev Esp Cardiol 61:130–136

    PubMed  Google Scholar 

  16. Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, Craigen WJ, Wu J, El Said H, Bezold LI, Clunie S, Fernbach S, Bowles NE, Towbin JA (2003) Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 108:2672–2678

    Article  PubMed  Google Scholar 

  17. Ali SK, Godman MJ (2004) The variable clinical presentation of, and outcome for, noncompaction of the ventricular myocardium in infants and children, an under-diagnosed cardiomyopathy. Cardiol Young 14:409–416

    Article  PubMed  Google Scholar 

  18. Stöllberger C, Finsterer J (2004) Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr 17:91–100

    Article  PubMed  Google Scholar 

  19. He T, Zeng HS, Le WB, Li XH, Lu ZY (2007) Clinical characterization and outcome of patients with noncompaction of ventricular myocardium]. Zhonghua Xin Xue Guan Bing Za Zhi 35:548–551

    PubMed  Google Scholar 

  20. Kohli SK, Pantazis AA, Shah JS, Adeyemi B, Jackson G, McKenna WJ, Sharma S, Elliott PM (2008) Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur Heart J 29:89–95

    Article  PubMed  Google Scholar 

  21. Nugent AW, Daubeney PE, Chondros P, Carlin JB, Cheung M, Wilkinson LC, Davis AM, Kahler SG, Chow CW, Wilkinson JL, Weintraub RG (2003) National Australian childhood cardiomyopathy study. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 348:1639–1646

    Article  PubMed  Google Scholar 

  22. Boyd MT, Seward JB, Tajik AJ, Edwards WD (1987) Frequency and location of prominent left ventricular trabeculations at autopsy in 474 normal human hearts: implications for evaluation of mural thrombi by two-dimensional echocardiography. J Am Coll Cardiol 9:323–326

    Article  PubMed  CAS  Google Scholar 

  23. Fernández-Golfín C, Pachón M, Corros C, Bustos A, Cabeza B, Ferreirós J, de Isla LP, Macaya C, Zamorano J (2009) Left ventricular trabeculae: quantification in different cardiac diseases and impact on left ventricular morphological and functional parameters assessed with cardiac magnetic resonance. J Cardiovasc Med (Hagerstown) 10:827–833

    Article  Google Scholar 

  24. Cruden NL, Denvir MA (2008) Isolated left ventricular non-compaction as an unusual cause of heart failure: a case report. J Med Case Rep 2:269

    Article  Google Scholar 

  25. Saeed S, Vegsundvåg J, Lode I (2009) Noncompaction of the left ventricular myocardium. Tidsskr Nor Laegeforen 129:1104–1107

    Article  PubMed  Google Scholar 

  26. Zimpfer A, Miny P, Dombrowski U, Tolnay M, Meyer P, Bruder E (2007) Upper limb amelia, facial clefts, holoprosencephaly, and interrupted aortic arch. Fetal Pediatr Pathol 26:169–176

    Article  PubMed  Google Scholar 

  27. Panduranga P, Mukhaini MK (2009) Left-ventricular non-compaction with coronary artery disease. Int J Cardiol (in press)

  28. Monserrat L, Barriales-Villa R, Hermida-Prieto M (2008) Apical hypertrophic cardiomyopathy and left ventricular non-compaction: two faces of the same disease. Heart 94:1253

    Article  PubMed  CAS  Google Scholar 

  29. Burke A, Mont E, Kutys R, Virmani R (2005) Left ventricular noncompaction: a pathological study of 14 cases. Hum Pathol 36:403–411

    Article  PubMed  Google Scholar 

  30. Buss SJ, Mereles D, Katus HA, Hardt SE (2009) Left ventricular non-compaction cardiomyopathy mimicking an infiltrative cardiac disease. Int J Cardiol (in press)

  31. Fazio G, Novo G, Casalicchio C, Di Gesaro G, Sutera L, Grassedonio E, Cracolici E, Pitruzzella V, Incalcaterra E, Pipitone S, Midiri M, Novo S (2009) Left ventricular non-compaction cardiomyopathy in children: is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction? Int J Cardiol 132:278–280

    Article  PubMed  Google Scholar 

  32. Bladt O, Vanhoenacker R, Bevernage C, Leyman P (2008) Isolated noncompaction of ventricular myocardium. Diagnosis with multidetector computed tomography. JBR-BTR 91:153–154

    PubMed  CAS  Google Scholar 

  33. Nemes A, Caliskan K, Geleijnse ML, Soliman OI, Vletter WB, ten Cate FJ (2009) Reduced regional systolic function is not confined to the noncompacted segments in noncompaction cardiomyopathy. Int J Cardiol 134:366–370

    Article  PubMed  Google Scholar 

  34. van Dalen BM, Caliskan K, Soliman OI, Nemes A, Vletter WB, Ten Cate FJ, Geleijnse ML (2008) Left ventricular solid body rotation in non-compaction cardiomyopathy: a potential new objective and quantitative functional diagnostic criterion? Eur J Heart Fail 10:1088–1093

    Article  PubMed  Google Scholar 

  35. Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi T, Hamada H, Hirose O, Isobe T, Yamada K, Kurotobi S, Mito H, Miyake T, Murakami Y, Nishi T, Shinohara M, Seguchi M, Tashiro S, Tomimatsu H (1999) Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol 34:233–240

    Article  PubMed  CAS  Google Scholar 

  36. Belanger AR, Miller MA, Donthireddi UR, Najovits AJ, Goldman ME (2008) New classification scheme of left ventricular noncompaction and correlation with ventricular performance. Am J Cardiol 102:92–96

    Article  PubMed  Google Scholar 

  37. Stanton C, Bruce C, Connolly H, Brady P, Syed I, Hodge D, Asirvatham S, Friedman P (2009) Isolated left ventricular noncompaction syndrome. Am J Cardiol 104:1135–1138

    Article  PubMed  Google Scholar 

  38. Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, Watkins H, Neubauer S (2005) Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 46:101–105

    Article  PubMed  Google Scholar 

  39. Junqueira FP, Fernandes FD, Coutinho AC, De Pontes PV, Domingues RC (2009) Case report. Isolated left ventricular myocardium non-compaction: MR imaging findings from three cases. Br J Radiol 82:e37–e41

    Article  PubMed  CAS  Google Scholar 

  40. Martín M, Santamarta E, Saiz A, Morís C (2009) Late gadolinium enhancement in non-compaction cardiomyopathy. Rev Esp Cardiol 62:822–823

    Article  PubMed  Google Scholar 

  41. Kurita T, Onishi K, Ogawa T, Kasai A, Sakuma H, Ito M (2008) A case of clover-shaped left ventricular non-compaction. Eur Heart J 29:569

    Article  PubMed  Google Scholar 

  42. Calvillo P, Martí-Bonmatí L, Chaustre F, Roldán I, Mora V, Peláez A, Cogollos J, Ballestín J (2009) MRI quantification of myocardial function, perfusion, and enhancement in patients with left-ventricular noncompaction. Radiologia 51:45–56

    Article  PubMed  CAS  Google Scholar 

  43. Ivan D, Flamm SD, Abrams J, Kindo M, Heck K, Frazier OH (2005) Isolated ventricular non-compaction in adults with idiopathic cardiomyopathy: cardiac magnetic resonance and pathologic characterization of the anomaly. J Heart Lung Transplant 24:781–786

    Article  PubMed  Google Scholar 

  44. Amir O, Delgado RM 3rd, Kar B, Smart FW (2009) The value of cardiac magnetic resonance imaging in the diagnosis of isolated non-compaction of the left ventricle. Isr Med Assoc J 11:313–314

    PubMed  Google Scholar 

  45. Shang JF, Chen D, Zhang HX, Fang W, Wu Y, Xiao L, Lian GL (2009) Clinicopathologic analyses of non-compaction of ventricular myocardium. Zhonghua Bing Li Xue Za Zhi 38:333–336

    PubMed  CAS  Google Scholar 

  46. Captur G, Nihoyannopoulos P (2009) Left ventricular non-compaction: genetic heterogeneity, diagnosis and clinical course. Int J Cardiol (in press)

  47. Barbosa ND, Azeka E, Aiello VD, Viana F, Jatene M, Tanamati C, Marcial MB (2008) Isolated left ventricular noncompaction: unusual cause of decompensated heart failure and indication of heart transplantation in the early infancy—case report and literature review. Clinics (Sao Paulo) 63:136–139

    Google Scholar 

  48. Ozkutlu S, Bostan O, Karagöz T, Deren O, Tekinalp G (2007) Prenatal diagnosis of isolated non-compaction of the ventricular myocardium: study of six cases. Pediatr Int 49:172–176

    Article  PubMed  Google Scholar 

  49. Stöllberger C, Preiser J, Finsterer J (2004) Histological detection of intramyocardial abscesses in Candida sepsis mimicking left ventricular non-compaction/hyertrabeculation on echocardiography. Mycoses 47:72–75

    Article  PubMed  Google Scholar 

  50. Wlodarska EK, Wozniak O, Konka M, Piotrowska-Kownacka D, Walczak E, Hoffman P (2009) Isolated ventricular noncompaction mimicking arrhythmogenic right ventricular cardiomyopathy—a study of nine patients. Int J Cardiol (in press)

  51. Kelley-Hedgepeth A, Towbin JA, Maron MS (2009) Images in cardiovascular medicine. Overlapping phenotypes: left ventricular noncompaction and hypertrophic cardiomyopathy. Circulation 119

  52. Suvarna JC, Deshmukh CT, Hajela SA (2009) Left ventricular noncompaction: a cardiomyopathy often mistaken. Indian J Med Sci 63:303–307

    Article  PubMed  Google Scholar 

  53. El-Menyar AA, Gendi SM, Numan MT (2007) Noncompaction cardiomyopathy in the state of Qatar. Saudi Med J 28:429–434

    PubMed  Google Scholar 

  54. Gimeno JR, Lacunza J, García-Alberola A, Cerdán MC, Oliva MJ, García-Molina E, López-Ruiz M, Castro F, González-Carrillo J, de la Morena G, Valdés M (2009) Penetrance and risk profile in inherited cardiac diseases studied in a dedicated screening clinic. Am J Cardiol 104:406–410

    Article  PubMed  Google Scholar 

  55. Ogawa K, Nakamura Y, Terano K, Ando T, Hishitani T, Hoshino K (2009) Isolated non-compaction of the ventricular myocardium associated with long QT syndrome. Circ J 73:2169–2172

    Article  PubMed  Google Scholar 

  56. Taylor GP (1992) Cardiovascular system. In: Dimmick JE, Kalousek DK (eds) Development pathology of embryo and fetus. Lippincott, Philadelphia

    Google Scholar 

  57. Chen Q, Chen H, Zheng D, Kuang C, Fang H, Zou B, Zhu W, Bu G, Jin T, Wang Z, Zhang X, Chen J, Field LJ, Rubart M, Shou W, Chen Y (2009) Smad7 is required for the development and function of the heart. J Biol Chem 2(284):292–300

    Google Scholar 

  58. Bleyl SB, Mumford BR, Brown-Harrison MC, Pagotto LT, Carey JC, Pysher TJ, Ward K, Chin TK (1997) Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals. Am J Med Genet 72:257–265

    Article  PubMed  CAS  Google Scholar 

  59. Gewillig M, Brown SC, De Catte L, Debeer A, Eyskens B, Cossey V, Van Schoubroeck D, Van Hole C, Devlieger R (2009) Premature foetal closure of the arterial duct: clinical presentations and outcome. Eur Heart J 30:1530–1536

    Article  PubMed  Google Scholar 

  60. Finsterer J, Stöllberger C, Schubert B (2004) Acquired left ventricular hypertrabeculation/noncompaction in mitochondriopathy. Cardiology 102:228–230

    Article  PubMed  Google Scholar 

  61. Stöllberger C, Finsterer J (2007) Disappearance of left ventricular hypertrabeculation/noncompaction after biventricular pacing in a patient with neuromuscular disorder. J Card Fail 13:211–214

    Article  PubMed  Google Scholar 

  62. Eurlings LW, Pinto YM, Dennert RM, Bekkers SC (2009) Author reply: consider potential pitfalls, myopathy and chromosomal abnormalities when diagnosing left ventricular hypertrabeculation/noncompaction. Int J Cardiol (in press)

  63. Matsumoto N, Sato Y, Kunimasa T, Matsuo S, Kato M, Yoda S, Suzuki Y, Tani S, Takahashi M, Saito S (2006) Noncompaction of the ventricular myocardium mimicking ischemic cardiomyopathy. Ann Nucl Med 20:639–641

    Article  PubMed  Google Scholar 

  64. Stöllberger C, Finsterer J, Blazek G (2002) Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 90:899–902

    Article  PubMed  Google Scholar 

  65. Cremer K, Lüdecke HJ, Ruhr F, Wieczorek D (2008) Left-ventricular non-compaction (LVNC): a clinical feature more often observed in terminal deletion 1p36 than previously expected. Eur J Med Genet 51:685–688

    Article  PubMed  Google Scholar 

  66. Markovic NS, Dimkovic N, Damjanovic T, Loncar G, Dimkovic S (2008) Isolated ventricular noncompaction in patients with chronic renal failure. Clin Nephrol 70:72–76

    PubMed  CAS  Google Scholar 

  67. Stöllberger C, Blazek G, Wegner C, Winkler-Dworak M, Finsterer J (2010) Neuromuscular and cardiac comorbidity determine survival in 140 patients with left ventricular hypertrabeculation/noncompaction. Int J Cardiol (in press)

  68. Yousef ZR, Foley PW, Khadjooi K, Chalil S, Sandman H, Mohammed NU, Leyva F (2009) Left ventricular non-compaction: clinical features and cardiovascular magnetic resonance imaging. BMC Cardiovasc Disord 9:37.ef09

    Article  Google Scholar 

  69. Stöllberger C, Winkler-Dworak M, Blazek G, Finsterer J (2004) Left ventricular hypertrabeculation/noncompaction with and without neuromuscular disorders. Int J Cardiol 97:89–92

    Article  PubMed  Google Scholar 

  70. Kovacevic-Preradovic T, Jenni R, Oechslin EN, Noll G, Seifert B, Attenhofer Jost CH (2009) Isolated left ventricular noncompaction as a cause for heart failure and heart transplantation: a single center experience. Cardiology 112:158–164

    Article  PubMed  CAS  Google Scholar 

  71. Andrews RE, Fenton MJ, Ridout DA, Burch M, British Congenital Cardiac Association (2008) New-onset heart failure due to heart muscle disease in childhood: a prospective study in the United kingdom and Ireland. Circulation 117:79–84

    Article  PubMed  Google Scholar 

  72. Steffel J, Kobza R, Namdar M, Wolber T, Brunckhorst C, Lüscher TF, Jenni R, Duru F (2009) Electrophysiological findings in patients with isolated left ventricular non-compaction. Europace 11:1193–1200

    Article  PubMed  Google Scholar 

  73. Zaragoza MV, Arbustini E, Narula J (2007) Noncompaction of the left ventricle primary cardiomyopathy with an elusive genetic etiology. Curr Opin Pediatr 19:619–627

    Article  PubMed  Google Scholar 

  74. Pascal C, Lefèvre M; Société Française de Cardiologie (2005) Non-compaction of the myocardium in childhood. Arch Mal Coeur Vaiss 98443–98448

  75. Steffel J, Kobza R, Oechslin E, Jenni R, Duru F (2009) Electrocardiographic characteristics at initial diagnosis in patients with isolated left ventricular noncompaction. Am J Cardiol 104:984–989

    Article  PubMed  Google Scholar 

  76. Coppola G, Guttilla D, Corrado E, Falletta C, Marrone G, Farulla RA, Ciaramitaro G, Assennato P, Novo S (2009) ICD implantation in noncompaction of the left ventricular myocardium: a case report. Pacing Clin Electrophysiol 32:1092–1095

    Article  PubMed  Google Scholar 

  77. Derval N, Jais P, O’Neill MD, Haissaguerre M (2009) Apparent idiopathic ventricular tachycardia associated with isolated ventricular noncompaction. Heart Rhythm 6:385–388

    Article  PubMed  Google Scholar 

  78. Paparella G, Capulzini L, de Asmundis C, Francesconi A, Sarkozy A, Chierchia G, Brugada P (2009) Electro-anatomical mapping in a patient with isolated left ventricular non-compaction and left ventricular tachycardia. Europace 11:1227–1229

    Article  PubMed  Google Scholar 

  79. Biagini E, Ragni L, Ferlito M, Pasquale F, Lofiego C, Leone O, Rocchi G, Perugini E, Zagnoni S, Branzi A, Picchio FM, Rapezzi C (2006) Different types of cardiomyopathy associated with isolated ventricular noncompaction. Am J Cardiol 98:821–824

    Article  PubMed  Google Scholar 

  80. Cavusoglu Y, Tunerir B, Birdane A, Timuralp B, Ata N, Gorenek B, Unalir A, Aslan R (2005) Transesophageal echocardiographic diagnosis of ventricular noncompaction associated with an atrial septal aneurysm in a patient with dilated cardiomyopathy of unknown etiology. Can J Cardiol 21:705–707

    PubMed  Google Scholar 

  81. Magno P, Freitas A, Gil VM (2007) Left ventricular noncompaction: case report and literature review. Rev Port Cardiol 26:1381–1391

    PubMed  Google Scholar 

  82. Drago F, Stefano Silvetti M, Annichiarico M, Michielon G, Brancaccio G, Zanoni S, Valsecchi S (2009) Biventricular pacing in an infant with noncompaction of the ventricular myocardium, congenital AV block, and prolonged QT interval. J Interv Card Electrophysiol (in press)

  83. Yildiz A, Ozeke O, Akyol S, Demirbag R, Yilmaz R, Gur M (2009) Biventricular myocardial noncompaction presenting with complete atrioventricular block. Int J Cardiol 132:e34–e36

    Article  PubMed  Google Scholar 

  84. Karamitsos TD, Bull S, Spyrou N, Neubauer S, Selvanayagam JB (2008) Tako-tsubo cardiomyopathy presenting with features of left ventricular non-compaction. Int J Cardiol 128:e34–e36

    Article  PubMed  Google Scholar 

  85. Nil M, Mori K, Yuasa Y, Ichida F (2003) Isolated noncompaction of myocardium associated with calcification in the interventricular septum. Pediatr Cardiol 24:591–594

    Article  PubMed  CAS  Google Scholar 

  86. Nemes A, Anwar AM, Caliskan K, Soliman OI, van Dalen BM, Geleijnse ML, ten Cate FJ (2008) Evaluation of left atrial systolic function in noncompaction cardiomyopathy by real-time three-dimensional echocardiography. Int J Cardiovasc Imaging 24:237–242

    Article  PubMed  Google Scholar 

  87. McMahon CJ, Murchan H, Prendiville T, Burch M (2007) Long-term support with milrinone prior to cardiac transplantation in a neonate with left ventricular noncompaction cardiomyopathy. Pediatr Cardiol 28:317–318

    Article  PubMed  CAS  Google Scholar 

  88. Saito K, Ibuki K, Yoshimura N, Hirono K, Watanabe S, Watanabe K, Uese K, Yasukouchi S, Ichida F, Miyawaki T (2009) Successful cardiac resynchronization therapy in a 3-year-old girl with isolated left ventricular non-compaction and narrow QRS complex. Circ J 73:2173–2177

    Article  PubMed  Google Scholar 

  89. Tufekcioglu O, Aras D, Yildiz A, Topaloglu S, Maden O (2008) Myocardial contraction properties along the long and short axes of the left ventricle in isolated left ventricular non-compaction: pulsed tissue Doppler echocardiography. Eur J Echocardiogr 9:344–350

    Article  PubMed  Google Scholar 

  90. Richards A, Mao CY, Dobson NR (2009) Left ventricular noncompaction: a rare cause of hydrops fetalis. Pediatr Cardiol 30:985–988

    Article  PubMed  Google Scholar 

  91. Karabulut A, Erden I, Erden E, Cakmak M (2009) Myocardial noncompaction recognized following a transient ischemic attack. Turk Kardiyol Dern Ars 37:205–208

    PubMed  Google Scholar 

  92. Fazio G, Corrado G, Zachara E, Rapezzi C, Sulafa AK, Sutera L, Stollberger C, Sormani L, Finsterer J, Benatar A, Di Gesaro G, Visconti C, D’angelo L, Novo G, Mongiovi M, Cavusoglu Y, Baumhakel M, Drago F, Indovina G, Ferrara F, Carerj S, Pipitone S, Novo S (2008) Anticoagulant drugs in noncompaction: a mandatory therapy? J Cardiovasc Med (Hagerstown) 9:1095–1097

    Article  Google Scholar 

  93. Finsterer J, Stollberger C, Mölzer G, Winkler-Dworak M, Blazek G (2008) Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy. Int J Cardiol 130:344–348

    Article  PubMed  Google Scholar 

  94. Oduncu V, Akgun T, Erkol A, Mutlu B (2008) Biventricular noncompaction presenting with stroke. Eur J Cardiothorac Surg 33:737

    Article  PubMed  Google Scholar 

  95. Sahin S, Karsidag S (2009) An unusual cause of cardioembolic stroke: isolated left ventricular noncompaction. Neurologist 15:51

    Article  PubMed  Google Scholar 

  96. Sir JJ, Lee B, Nah JC, Kim YL, Cho WH, Choi SK (2008) Serious cardioembolic stroke resulting from an overlooked left ventricular noncompaction. South Med J 101:969–970

    PubMed  Google Scholar 

  97. Stöllberger C, Finsterer J (2005) Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology 103:68–72

    Article  PubMed  Google Scholar 

  98. Blessing E, Rottbauer W, Mereles D, Hosch W, Benz A, Friess H, Autschbach F, Müller M, Stremmel W, Katus H (2005) Isolated left ventricular noncompaction of the myocardium as a cause of embolic superior mesenteric artery occlusion. J Am Soc Echocardiogr 18:693

    PubMed  Google Scholar 

  99. Everett ME, Kirkpatrick JN, Lang RM (2005) Noncompaction of the myocardium complicated by coronary artery embolism. J Am Soc Echocardiogr 18:194–196

    Article  PubMed  Google Scholar 

  100. Horisberger T, Lorenzana D, Noethiger CB, Jenni R (2007) Isolated noncompaction of the ventricular myocardium in a patient presenting with subarachnoid hemorrhage. Int J Cardiol 116:e98–e100

    Article  PubMed  Google Scholar 

  101. Dabarian AL, Mady C, Rochitte CE, Shiozaki AA, Lemos PA, Salemi VM (2008) An unusual case of angina pectoris: a patient with isolated non compaction of the left ventricular myocardium. Eur J Echocardiogr 9:728–730

    Article  PubMed  Google Scholar 

  102. Hamamichi Y, Ichida F, Hashimoto I, Uese KH, Miyawaki T, Tsukano S, Ono Y, Echigo S, Kamiya T (2001) Isolated noncompaction of the ventricular myocardium: ultrafast computed tomography and magnetic resonance imaging. Int J Cardiovasc Imaging 17:305–314

    Article  PubMed  CAS  Google Scholar 

  103. Toyono M, Kondo C, Nakajima Y, Nakazawa M, Momma K, Kusakabe K (2001) Effects of carvedilol on left ventricular function, mass, and scintigraphic findings in isolated left ventricular non-compaction. Heart 86:e4

    Article  PubMed  CAS  Google Scholar 

  104. Borges AC, Kivelitz D, Baumann G (2003) Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion. Heart 89:e21

    Article  PubMed  CAS  Google Scholar 

  105. Jenni R, Wyss CA, Oechslin EN, Kaufmann PA (2002) Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol 39:450–454

    Article  PubMed  Google Scholar 

  106. Yavuzgil O, Gürgün C, Soydas Cinar C, Yüksel A (2006) Anterior myocardial infarction in an adult patient with left ventricular hypertrabeculation/noncompaction. Int J Cardiol 106:394–395

    Article  PubMed  Google Scholar 

  107. D’Angeli I, Sürder D, Pedrazzini GB, Moccetti T (2009) Type B aortic dissection in a patient with unknown left ventricular non-compaction cardiomyopathy: cardiovascular magnetic resonance diagnosis. Int J Cardiol (in press)

  108. Stöllberger C, Ormin V, Finsterer J (2009) Fatal outcome of aortic valve endocarditis in left ventricular hypertrabeculation/noncompaction. Int J Cardiol (in press)

  109. Finsterer J, Stöllberger C, Feichtinger H (2008) Noncompaction and endocarditis in suspected mitochondrial disorder. Int J Cardiol 123:e45–e47

    Article  PubMed  Google Scholar 

  110. Jenni R, Oechslin EN, van der Loo B (2007) Isolated ventricular non-compaction of the myocardium in adults. Heart 93:11–15

    Article  PubMed  CAS  Google Scholar 

  111. Kobza R, Jenni R, Erne P, Oechslin E, Duru F (2008) Implantable cardioverter-defibrillators in patients with left ventricular noncompaction. Pacing Clin Electrophysiol 31:461–467

    Article  PubMed  Google Scholar 

  112. Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M (2006) American College of Cardiology/American Heart Association Task Force; European Society of Cardiology Committee for Practice Guidelines; European Heart Rhythm Association; Heart Rhythm Society. ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation 114:e385–e484

    Google Scholar 

  113. Garnier A, Girod G (2009) Cardiac re-synchronization therapy in a patient with isolated ventricular non-compaction: a case report. Eur J Echocardiogr 10:713–715

    Article  PubMed  Google Scholar 

  114. Oginosawa Y, Nogami A, Soejima K, Aonuma K, Kubota S, Sato T, Sugiyasu A, Yoshida K, Kowase S, Sakamaki M, Kurosaki K, Kato K (2008) Effect of cardiac resynchronization therapy in isolated ventricular noncompaction in adults: follow-up of four cases. J Cardiovasc Electrophysiol 19:935–938

    Article  PubMed  Google Scholar 

  115. Stöllberger C, Blazek G, Bucher E, Finsterer J (2008) Cardiac resynchronization therapy in left ventricular hypertrabeculation/non-ompaction and myopathy. Europace 10:59–62

    Article  PubMed  Google Scholar 

  116. Fan KY, Chan CW, Cheng LC, Ko RL, Lam YM, Jim MH, Au TW, Ho CK, Wang EP, Chau EM, Chow WH (2009) Isolated left ventricular non-compaction: an unusual indication for heart transplantation. Hong Kong Med J 15:378–380

    PubMed  Google Scholar 

  117. Tigen K, Karaahmet T, Gurel E, Cevik C, Basaran Y (2008) Biventricular noncompaction: a case report. Echocardiography 25:993–996

    Article  PubMed  Google Scholar 

  118. Aras D, Tufekcioglu O, Topaloglu S, Ergun K, Ozeke O, Yildiz A, Korkmaz S (2006) Preserved systolic function with isolated left ventricular noncompaction in an elderly patient. Eur J Echocardiogr 7:71–74

    Article  PubMed  Google Scholar 

  119. Stöllberger C, Winkler-Dworak M, Blazek G, Finsterer J (2007) Prognosis of left ventricular hypertrabeculation/noncompaction is dependent on cardiac and neuromuscular comorbidity. Int J Cardiol 121:189–193

    Article  PubMed  Google Scholar 

  120. Murphy RT, Thaman R, Blanes JG, Ward D, Sevdalis E, Papra E, Kiotsekolglou A, Tome MT, Pellerin D, McKenna WJ, Elliott PM (2005) Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J 26:187–192

    Article  PubMed  Google Scholar 

  121. Punn R, Silverman NH (2010) Cardiac segmental analysis in left ventricular noncompaction: experience in a pediatric population. J Am Soc Echocardiogr 23:46–53

    Article  PubMed  Google Scholar 

  122. Koh C, Lee PW, Yung TC, Lun KS, Cheung YF (2009) Left ventricular noncompaction in children. Congenit Heart Dis 4:288–294

    Article  PubMed  Google Scholar 

  123. Lofiego C, Biagini E, Ferlito M, Pasquale F, Rocchi G, Perugini E, Leone O, Bracchetti G, Caliskan K, Branzi A, ten Cate FJ, Rapezzi C (2006) Paradoxical contributions of non-compacted and compacted segments to global left ventricular dysfunction in isolated left ventricular noncompaction. Am J Cardiol 97:738–741

    Article  PubMed  Google Scholar 

  124. Tsai SF, Ebenroth ES, Hurwitz RA, Cordes TM, Schamberger MS, Batra AS (2009) Is left ventricular noncompaction in children truly an isolated lesion? Pediatr Cardiol 30:597–602

    Article  PubMed  Google Scholar 

  125. Fazio G, Pipitone S, Iacona MA, Marchì S, Mongiovì M, Zito R, Sutera L, Novo S (2007) The noncompaction of the left ventricular myocardium: our paediatric experience. J Cardiovasc Med (Hagerstown) 8:904–908

    Article  Google Scholar 

  126. Marín Rodríguez C, Ossaba Vélez S, Maroto Álvaro E, Sánchez Alegre ML (2010) Lack of MR late-enhancement in left ventricular non-compaction in infants and young children. Radiologia 52:138–143

    Article  PubMed  Google Scholar 

  127. Eitel I, Fuernau G, Walther C, Razek V, Kivelitz D, Schuler G, Thiele H (2008) Delayed enhancement magnetic resonance imaging in isolated noncompaction of ventricular myocardium. Clin Res Cardiol 97:277–279

    Article  PubMed  Google Scholar 

  128. Celiker A, Ozkutlu S, Dilber E, Karagöz T (2005) Rhythm abnormalities in children with isolated ventricular noncompaction. Pacing Clin Electrophysiol 28:1198–1202

    Article  PubMed  Google Scholar 

  129. Alehan D (2004) Clinical features of isolated left ventricular noncompaction in children. Int J Cardiol 97:233–237

    Article  PubMed  Google Scholar 

  130. Klaassen S, Probst S, Oechslin E, Gerull B, Krings G, Schuler P, Greutmann M, Hürlimann D, Yegitbasi M, Pons L, Gramlich M, Drenckhahn JD, Heuser A, Berger F, Jenni R, Thierfelder L (2008) Mutations in sarcomere protein genes in left ventricular noncompaction. Circulation 117:2893–2901

    Article  CAS  Google Scholar 

  131. Hofer M, Stöllberger C, Finsterer J (2007) Acquired noncompaction associated with myopathy. Int J Cardiol 121:296–297

    Article  PubMed  Google Scholar 

  132. Finsterer J, Stöllberger C, Gaismayer K, Janssen B (2006) Acquired noncompaction in Duchenne muscular dystrophy. Int J Cardiol 106:420–421

    Article  PubMed  Google Scholar 

  133. Khalid SK, Omran AS, Najm H, Godman MJ (2004) Noncompaction of the ventricular myocardium associated with mitral regurgitation and preserved ventricular systolic function. J Am Soc Echocardiogr 17:87–90

    Article  Google Scholar 

  134. Hussein A, Schmaltz AA, Trowitzsch E (1999) [Isolated abnormality ("noncompaction") of the myocardium in 3 children]. Klin Padiatr 211:175–178

    Article  PubMed  CAS  Google Scholar 

  135. Pauli RM, Scheib-Wixted S, Cripe L, Izumo S, Sekhon GS (1999) Ventricular noncompaction and distal chromosome 5q deletion. Am J Med Genet 85:419–423

    Article  PubMed  CAS  Google Scholar 

  136. Steiner I, Hrubecký J, Pleskot J, Kokstejn Z (1996) Persistence of spongy myocardium with embryonic blood supply in an adult. Cardiovasc Pathol 5:47–53

    Article  Google Scholar 

  137. Neudorf UE, Hussein A, Trowitzsch E, Schmaltz AA (2001) Clinical features of isolated noncompaction of the myocardium in children. Cardiol Young 11:439–442

    Article  PubMed  CAS  Google Scholar 

  138. Grillo R, Pipitone S, Mongiovì M, Cipolla T, Giudice G, Gagliano S, Sperandeo V (2002) [Isolated non-compaction of left ventricle in childhood: clinical experience with 5 cases]. Ital Heart J Suppl 3:858–863

    PubMed  Google Scholar 

  139. Ozkutlu S, Ayabakan C, Celiker A, Elshershari H (2002) Noncompaction of ventricular myocardium: a study of twelve patients. J Am Soc Echocardiogr 15:1523–1528

    Article  PubMed  Google Scholar 

  140. Wald R, Veldtman G, Golding F, Kirsh J, McCrindle B, Benson L (2004) Determinants of outcome in isolated ventricular noncompaction in childhood. Am J Cardiol 94:1581–1584

    Article  PubMed  Google Scholar 

  141. Kawasaki T, Azuma A, Taniguchi T, Asada S, Kamitani T, Kawasaki S, Matsubara H, Sugihara H (2005) Heart rate variability in adult patients with isolated left ventricular noncompaction. Int J Cardiol 99:147–150

    Article  PubMed  Google Scholar 

  142. Lofiego C, Biagini E, Ferlito M, Pasquale F, Rocchi G, Perugini E, Leone O, Bracchetti G, Caliskan K, Branzi A, ten Cate FJ, Rapezzi C (2006) Paradoxical contributions of non-compacted and compacted segments to global left ventricular dysfunction in isolated left ventricular noncompaction. Am J Cardiol 97:738–741

    Article  PubMed  Google Scholar 

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Acknowledgments

We want to thank Prof. Stöllberger for providing the echocardiographic and autopsy figures.

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Correspondence to Josef Finsterer.

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Finsterer, J. Left ventricular non-compaction and its cardiac and neurologic implications. Heart Fail Rev 15, 589–603 (2010). https://doi.org/10.1007/s10741-010-9175-5

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