Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician.

Noncompaction of the left ventricular myocardium is a morphogenetic abnormality involving loss of compaction of the myocardial fiber meshwork during intrauterine life. It is an extremely rare condition, accounting for only 0.05% of the cases evaluated in databanks. It has been described in both genders, in many ethnic groups and at different ages. Recently published studies of case series have shown a high mortality rate among these patients during follow-up of up to 48 months. Many cases have so far been misdiagnosed due to poor knowledge of the findings relating to this syndrome. There needs to be an attempt at early and accurate diagnosis, because of the need to investigate the patients family upon such diagnosis, and today this can be achieved using echocardiographic criteria.

[1]  F Bender,et al.  Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids. , 1984, The American journal of cardiology.

[2]  J. Perloff,et al.  Isolated noncompaction of left ventricular myocardium. A study of eight cases. , 1990, Circulation.

[3]  J. Carey,et al.  Neonatal, lethal noncompaction of the left ventricular myocardium is allelic with Barth syndrome. , 1997, American journal of human genetics.

[4]  J. Carey,et al.  Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals. , 1997, American journal of medical genetics.

[5]  G. F. Manzillo,et al.  Isolated noncompaction of the ventricular myocardium. A study in an adult male and literature review. , 2000, Italian heart journal : official journal of the Italian Federation of Cardiology.

[6]  P. Kaufmann,et al.  Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. , 2000, Journal of the American College of Cardiology.

[7]  C. Buonanno,et al.  Isolated noncompaction of the myocardium: an exceedingly rare cardiomyopathy. A case report. , 2000, Italian heart journal : official journal of the Italian Federation of Cardiology.

[8]  J. Elias,et al.  Isolated noncompaction of the myocardium. , 2000, Arquivos brasileiros de cardiologia.

[9]  D. M. Rubio,et al.  Forma aislada de miocardiopatía espongiforme , 2002 .

[10]  J. Finsterer,et al.  Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. , 2002, The American journal of cardiology.

[11]  C. Jaffe,et al.  Isolated Noncompaction of the Ventricular Myocardium: Clinical and Molecular Aspects of a Rare Cardiomyopathy , 2002, Laboratory Investigation.

[12]  J. R. Siles Rubio,et al.  [Isolated form of spongy myocardiopathy]. , 2002, Revista espanola de cardiologia.

[13]  S. Pipitone,et al.  [Isolated non-compaction of left ventricle in childhood: clinical experience with 5 cases]. , 2002, Italian heart journal. Supplement : official journal of the Italian Federation of Cardiology.

[14]  P. Toutouzas,et al.  Isolated Left Ventricular Noncompaction: An Unclassified Cardiomyopathy with Severe Prognosis in Adults , 2002, Cardiology.

[15]  C. Ayabakan,et al.  Noncompaction of ventricular myocardium: a study of twelve patients. , 2002, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[16]  M. Buchalter,et al.  Abnormal myocardial strain rate in noncompaction of the left ventricle. , 2003, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[17]  M. Godman,et al.  Noncompaction of the ventricular myocardium associated with mitral regurgitation and preserved ventricular systolic function. , 2004, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[18]  A. Takashima,et al.  [Isolated left ventricular noncompaction in the elderly: a case report]. , 2004, Journal of cardiology.

[19]  Claudia Stöllberger,et al.  Left ventricular hypertrabeculation/noncompaction. , 2004, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[20]  I. Hashimoto,et al.  Isolated noncompaction of the ventricular myocardium: Ultrafast computed tomography and magnetic resonance imaging , 2001, The International Journal of Cardiovascular Imaging.

[21]  J. Finsterer,et al.  Cardiologic and neurologic findings in left ventricular hypertrabeculation/non‐compaction related to wall thickness, size and systolic function , 2005, European journal of heart failure.

[22]  K. Connelly,et al.  An unusual cause of heart failure identified by echocardiography in an octogenarian , 2004, European journal of heart failure.