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.
Similar content being viewed by others
References
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
Finsterer J (2009) Cardiogenetics, neurogenetics, and pathogenetics of left ventricular hypertrabeculation/noncompaction. Pediatr Cardiol 30:659–681
Breckenridge RA, Anderson RH, Elliott PM (2007) Isolated left ventricular non-compaction: the case for abnormal myocardial development. Cardiol Young 17:124–129
Dusek J, Ostádal B, Duskova M (1975) Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 99:312–317
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
Finsterer J, Stöllberger C, Wegmann R, Jarius C, Janssen B (2001) Left ventricular hypertrabeculation in myotonic dystrophy type 1. Herz 26:287–290
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
Anderson RH (2008) Ventricular non-compaction—a frequently ignored finding? Eur Heart J 29:10–11
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
Stöllberger C, Finsterer J, Blazek G, Bittner RE (1996) Left ventricular non-compaction in a patient with Becker’s muscular dystrophy. Heart 76:380
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
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
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
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
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
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
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
Stöllberger C, Finsterer J (2004) Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr 17:91–100
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
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
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
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
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
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
Saeed S, Vegsundvåg J, Lode I (2009) Noncompaction of the left ventricular myocardium. Tidsskr Nor Laegeforen 129:1104–1107
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
Panduranga P, Mukhaini MK (2009) Left-ventricular non-compaction with coronary artery disease. Int J Cardiol (in press)
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
Burke A, Mont E, Kutys R, Virmani R (2005) Left ventricular noncompaction: a pathological study of 14 cases. Hum Pathol 36:403–411
Buss SJ, Mereles D, Katus HA, Hardt SE (2009) Left ventricular non-compaction cardiomyopathy mimicking an infiltrative cardiac disease. Int J Cardiol (in press)
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
Bladt O, Vanhoenacker R, Bevernage C, Leyman P (2008) Isolated noncompaction of ventricular myocardium. Diagnosis with multidetector computed tomography. JBR-BTR 91:153–154
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
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
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
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
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
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
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
Martín M, Santamarta E, Saiz A, Morís C (2009) Late gadolinium enhancement in non-compaction cardiomyopathy. Rev Esp Cardiol 62:822–823
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
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
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
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
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
Captur G, Nihoyannopoulos P (2009) Left ventricular non-compaction: genetic heterogeneity, diagnosis and clinical course. Int J Cardiol (in press)
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
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
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
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)
Kelley-Hedgepeth A, Towbin JA, Maron MS (2009) Images in cardiovascular medicine. Overlapping phenotypes: left ventricular noncompaction and hypertrophic cardiomyopathy. Circulation 119
Suvarna JC, Deshmukh CT, Hajela SA (2009) Left ventricular noncompaction: a cardiomyopathy often mistaken. Indian J Med Sci 63:303–307
El-Menyar AA, Gendi SM, Numan MT (2007) Noncompaction cardiomyopathy in the state of Qatar. Saudi Med J 28:429–434
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
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
Taylor GP (1992) Cardiovascular system. In: Dimmick JE, Kalousek DK (eds) Development pathology of embryo and fetus. Lippincott, Philadelphia
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
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
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
Finsterer J, Stöllberger C, Schubert B (2004) Acquired left ventricular hypertrabeculation/noncompaction in mitochondriopathy. Cardiology 102:228–230
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
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)
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
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
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
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
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)
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
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
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
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
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
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
Pascal C, Lefèvre M; Société Française de Cardiologie (2005) Non-compaction of the myocardium in childhood. Arch Mal Coeur Vaiss 98443–98448
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
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
Derval N, Jais P, O’Neill MD, Haissaguerre M (2009) Apparent idiopathic ventricular tachycardia associated with isolated ventricular noncompaction. Heart Rhythm 6:385–388
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
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
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
Magno P, Freitas A, Gil VM (2007) Left ventricular noncompaction: case report and literature review. Rev Port Cardiol 26:1381–1391
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)
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
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
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
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
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
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
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
Richards A, Mao CY, Dobson NR (2009) Left ventricular noncompaction: a rare cause of hydrops fetalis. Pediatr Cardiol 30:985–988
Karabulut A, Erden I, Erden E, Cakmak M (2009) Myocardial noncompaction recognized following a transient ischemic attack. Turk Kardiyol Dern Ars 37:205–208
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
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
Oduncu V, Akgun T, Erkol A, Mutlu B (2008) Biventricular noncompaction presenting with stroke. Eur J Cardiothorac Surg 33:737
Sahin S, Karsidag S (2009) An unusual cause of cardioembolic stroke: isolated left ventricular noncompaction. Neurologist 15:51
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
Stöllberger C, Finsterer J (2005) Left ventricular hypertrabeculation/noncompaction and stroke or embolism. Cardiology 103:68–72
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
Everett ME, Kirkpatrick JN, Lang RM (2005) Noncompaction of the myocardium complicated by coronary artery embolism. J Am Soc Echocardiogr 18:194–196
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
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
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
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
Borges AC, Kivelitz D, Baumann G (2003) Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion. Heart 89:e21
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
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
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)
Stöllberger C, Ormin V, Finsterer J (2009) Fatal outcome of aortic valve endocarditis in left ventricular hypertrabeculation/noncompaction. Int J Cardiol (in press)
Finsterer J, Stöllberger C, Feichtinger H (2008) Noncompaction and endocarditis in suspected mitochondrial disorder. Int J Cardiol 123:e45–e47
Jenni R, Oechslin EN, van der Loo B (2007) Isolated ventricular non-compaction of the myocardium in adults. Heart 93:11–15
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
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
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
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
Stöllberger C, Blazek G, Bucher E, Finsterer J (2008) Cardiac resynchronization therapy in left ventricular hypertrabeculation/non-ompaction and myopathy. Europace 10:59–62
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
Tigen K, Karaahmet T, Gurel E, Cevik C, Basaran Y (2008) Biventricular noncompaction: a case report. Echocardiography 25:993–996
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
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
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
Punn R, Silverman NH (2010) Cardiac segmental analysis in left ventricular noncompaction: experience in a pediatric population. J Am Soc Echocardiogr 23:46–53
Koh C, Lee PW, Yung TC, Lun KS, Cheung YF (2009) Left ventricular noncompaction in children. Congenit Heart Dis 4:288–294
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
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
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
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
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
Celiker A, Ozkutlu S, Dilber E, Karagöz T (2005) Rhythm abnormalities in children with isolated ventricular noncompaction. Pacing Clin Electrophysiol 28:1198–1202
Alehan D (2004) Clinical features of isolated left ventricular noncompaction in children. Int J Cardiol 97:233–237
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
Hofer M, Stöllberger C, Finsterer J (2007) Acquired noncompaction associated with myopathy. Int J Cardiol 121:296–297
Finsterer J, Stöllberger C, Gaismayer K, Janssen B (2006) Acquired noncompaction in Duchenne muscular dystrophy. Int J Cardiol 106:420–421
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
Hussein A, Schmaltz AA, Trowitzsch E (1999) [Isolated abnormality ("noncompaction") of the myocardium in 3 children]. Klin Padiatr 211:175–178
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
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
Neudorf UE, Hussein A, Trowitzsch E, Schmaltz AA (2001) Clinical features of isolated noncompaction of the myocardium in children. Cardiol Young 11:439–442
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
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
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
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
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
Acknowledgments
We want to thank Prof. Stöllberger for providing the echocardiographic and autopsy figures.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
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
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10741-010-9175-5