Miocardio no compacto ¿Puede no ser una enfermedad? Razonamiento crítico sobre 140 miocardios no compactos evaluados con resonancia magnética cardiovascular / Non-compacted myocardium: Should it not be considered a disease? Critical thinking on 140 patients with non-compacted myocardium evaluated by cardiovascular magnetic resonance

RESUMEN Para que una alteración física sea considerada una enfermedad, debe cumplir con los criterios definidos por la OMS. El miocardio no compacto (MNC) es una hipertrabeculación ventricular no bien definida, de la que se duda si es una miocardiopatía en sí misma, o solo una variación de la normalidad. Nosotros analizamos 161 resonancias magnéticas cardíacas realizadas a 140 pacientes con MNC y sus relaciones con otras patologías, exponiéndolos a los criterios de "enfermedad" definidos por la OMS. Tras un análisis crítico, consideramos que no debería ser considerada una miocardiopatía en sí misma, sino una adaptación miocárdica ante condiciones adversas.

ABSTRACT For a physical alteration to be considered a disease, it must meet the criteria defined by the WHO. Non-compact myocardium (NCM) is a non-well-defined ventricular hypertrabeculation, where there are doubts whether it is a cardiomyopathy itself or just a variation of normality. We analyzed 161 studies in 140 patients with NCM and their relationships with other pathologies, exposing them to the "disease" criteria defined by the WHO. After a critical analysis, we consider that it should not be considered a cardiomyopathy in itself, but rather a myocardial adaptation to adverse conditions.

Left Ventricular Noncompaction Is More Prevalent in Ventricular Septal Defect Than Other Congenital Heart Defects: A Morphological Study.

Left ventricular noncompaction (LVNC) is a condition characterized by prominent ventricular trabeculae and deep intertrabecular recesses and has been described as a possible substrate for arrhythmias, thromboembolism, and heart failure. Herein, we explored the prevalence of LVNC morphology among hearts with congenital heart defects (CHD). We examined 259 postnatal hearts with one of the following CHD: isolated ventricular septal defect (VSD); isolated atrial septal defect (ASD); atrioventricular septal defect (AVSD); transposition of the great arteries (TGA); isomerism of the atrial appendages (ISOM); Ebstein's malformation (EB); Tetralogy of Fallot (TF). Eleven hearts from children who died of non-cardiovascular causes were used as controls. The thickness of the compacted and non-compacted left ventricular myocardial wall was determined and the specimens classified as presenting or not LVNC morphology according to three criteria, as proposed by Chin, Jenni, and Petersen. Normal hearts did not present LVNC, but the CHD group presented different percentages of LVNC in at least one diagnostic criterium. The prevalence of LVNC was respectively, according to Chin's, Jenni´s and Petersen´s methods: for VSD-54.2%, 35.4%, and 12.5%; ASD-8.3%, 8.3%, and 8.3%; AVSD-2.9%, 2.9%, and 0.0%; TGA-22.6%, 17%, and 5.7%; ISOM-7.1%, 7.1%, and 7.1%; EB-28.6%, 9.5%, and 0.0%; TF-5.9%. 2.9%, and 2.9%. VSD hearts showed a significantly greater risk of presenting LVNC when compared to controls (Chin and Jenni criteria). No other CHD presented similar risk. Current results show some agreement with previous studies, such as LVNC morphology being more prevalent in VSDs. Nonetheless, this is a morphological study and cannot be correlated with symptoms or severity of the CHD.

Fetal noncompaction cardiomyopathy and histologic diagnosis of spongy myocardium: case report and review of the literature

Abstract Noncompaction cardiomyopathy (NCCM) and left ventricular noncompaction (LVNC), in their isolated form, are rare cardiomyopathies. They are characterized by a thickened myocardium due to the presence of deep trabeculae recesses, and to thick trabeculae. This condition is associated with a variable clinical phenotype including heart failure, thromboembolism, and sudden death. We report a case of LVNC at 26 weeks and 4 days of gestation revised on the basis of what is currently reported in the literature. A review of the literature was performed to better describe this rare condition. Left ventricular noncompaction is a rare fetal condition and it should be suspected in case of cardiomyopathy.

Ventrículo izquierdo no compacto: panorámica y arritmogenia / Left ventricular non-compaction cardiomyopathy: outlook and cardiac arrhythmias

Las miocardiopatías constituyen un grupo importante y heterogéneo de enfermedades del miocardio asociadas a disfunción mecánica, eléctrica, o ambas. El ventrículo izquierdo no compacto es una miocardiopatía familiar de etiología incierta de la que se desconocen sus exactas incidencia y prevalencia. Se caracteriza por un aumento en la masa trabecular del VI en contraste con una fina capa epicárdica compacta que puede visualizarse con técnicas de imagen que confirman el diagnóstico. En este artículo se describen la clasificación de MOGE(S) para las miocardiopatías, los trastornos electrocardiográficos que pueden encontrarse en pacientes con ventrículo izquierdo no compacto, el papel de la estimulación eléctrica programada del corazón y otros aspectos de interés de esta enfermedad. Además, se presentan algunos trastornos electrocardiográficos demostrativos (criterios de Stollberger y Jenni) encontrados en pacientes afectados

Cardiomyopathies are an important and diverse group of myocardial diseases associated with mechanical, electrical, or both dysfunctions. The left ventricular (LV) non-compaction is a familial cardiomyopathy of uncertain etiology, whose exact incidence and prevalence are unknown. It is characterized by an increase in the trabecular mass of the LV in contrast to a thin compact epicardial layer that can be visualized with imaging techniques that confirm the diagnosis. In this article is described the classification of MOGE (S) for cardiomyopathies, electrocardiographic disorders that can be found in patients with left ventricular non-compaction, the role of programmed electrical stimulation of the heart and other aspects of interest of this disease. In addition, some demonstrative electrocardiographic disorders (Stollberger and Jenni criteria) found in affected patients are presented

Ventrículo izquierdo no compacto / Left ventricular noncompaction

El ventrículo izquierdo no compacto (VINC) o "esponjoso", es una cardiomiopatía recientemente descrita y reconocida con implicaciones pronósticas importantes. Se caracteriza por la presencia de un miocardio ancho con nichos intertrabeculares profundos sobre el lado endocavitario, llevando a hipertrabeculación. Aunque la etiología es desconocida, se ha descrito una importante agregación familiar y una superposición de esta patología con otras cardiopatías de carácter genético como la cardiomiopatía dilatada (CMD) y la cardiomiopatía hipertrófica (CMH). Desde el punto de vista clínico se presenta como un síndrome de falla cardiaca, aunque puede debutar con muerte súbita, arritmias o embolismo sistémico. En la actualidad la herramienta diagnóstica de mayor utilidad es la ecocardiografía, por sus menores costos, reproducibilidad y disponibilidad en forma extensa en los servicios médicos. Sin embargo, se ha demostrado una mayor certeza diagnóstica con la resonancia magnética cardiaca (RMC). El tratamiento de estos pacientes no difiere del recomendado para pacientes con síndrome de falla cardiaca de otras etiologías. (Acta Med Colomb 2011; 36: 187-195).

Left ventricular noncompaction (LVNC), or "spongy" cardiomyopathy, is a recently described and recognized finding with important prognostic implications. It is characterized by the presence of ventricular trabeculations and deep intertrabecular recesses on the endocavitary side, leading to hypertrabeculation. Although the etiology is unknown, a significant family aggregation of this disease as well as overlap with other genetic cardiomyopathies such as dilated cardiomyopathy and hypertrophic cardiomyopathy have been described. From the clinical point of view, LVNC often presents as heart failure, but may present with sudden death, arrhythmias and systemic embolism. At the present time the most useful diagnostic tool is transthoracic echocardiography because of its lower cost, reproducibility and availability, though cardiac magnetic resonance (CMR) imaging has shown greater diagnostic accuracy. Treatment of these patients is no different than that recommended for patients with heart failure syndromes of other etiologies. (Acta Med Colomb 2011; 36: 187-195).

Miocardiopatía espongiforme en una mujer anciana. Descripción ecocardiográfica / Spongy cardiomyopathy in an elderly woman, echocardiographic description

La miocardiopatía espongiforme, también conocida como ausencia aislada de compactación del ventrículo izquierdo, es una anomalía congénita recientemente descrita y que resulta de la interrupción en la morfogénesis embrionaria del miocardio ventricular izquierdo el cual permanece engrosado por grandes trabéculas y sinusoides y que suele expresarse clínicamente con manifestaciones de insuficiencia cardíaca, arritmias y eventos embólicos. Esta enfermedad suele detectarse en la infancia o en la adolescencia, menos frecuentemente en la vida adulta y muy excepcionalmente en la senectud. Presentamos aquí el caso de una mujer de 75 años de edad que corresponde a uno de los casos de miocardiopatía espongiforme de mayor edad entre los que se han publicado; sus manifestaciones clínicas y ecocardiográficas son típicas de esta variedad de miocaridopatía. El caso se comenta a la luz de los hechos más relevantes que existen en la escasa literatura publicada sobre esta interesante enfermedad.

Isolated left ventricular noncompaction, also known as spongy myocardium or spongy cardiomyopathy, is a recently described congenital disease caused by an arrest in the left ventricular myocardial embriogenesis that makes the ventricular wall to persist thickened with multiple trabecular formations and deep sinusoidal recesses. It is clinically characterized by heart failure, cardiac arrhythmia and systemic embolic events. Most of the affected subjects are detected during childhood or adolescence, others in the adult life but very few elderly patients have been reported in the worldwide medical literature. We here report the case of a 75-year-old woman that is one of the oldest patients ever reported, whose clinical picture and echocardiographic findings are typical of this modality of cardiomyopathy. We do comments on this case in regard to the most relevant facts that appear in the limited medical literature about this interesting disease. (Arch Cardiol Mex 2005; 75: 184-187.