Left Ventricular Non-Compaction Cardiomyopathy: Case Report and Review of Literature

Non-compaction cardiomyopathy (NCC) is characterized by trabeculations in either one or both ventricles. Clinical presentation is highly variable: dyspnea, palpitation, thromboembolic events, arrhythmia, or sudden cardiac death. There are currently no universally-accepted criteria for classifying and diagnosing left ventricular non-compaction (LVNC) cardiomyopathy. Transthoracic echocardiography (TTE) is the diagnostic exam of choice. The diagnosis is often missed or delayed because of a lack of knowledge about this uncommon disease. Progression of LVNC is highly variable and prognosis is very difficult to predict. We report a case of a 50-year-old female patient with a history of total thyroidectomy under hormonal supplementation who consults for dyspnea and paroxysmal palpitations revealing an isolated LVNC. This case emphasizes the importance of imaging techniques, which are, TTE and cardiac magnetic resonance imaging (MRI) in early diagnosis, management, and follow-up.

A Young Healthy Woman with Supraventricular Tachycardia Reveals an Underlying Left Ventricular Non-compaction Cardiomyopathy: A Rare Case Report

LVNC (left ventricular non-compaction) is a rare congenital cardiomyopathy with a reported incidence of 0.05% in adults. It can occur in isolation or affect both ventricles. It’s characterized by prominent LV trabeculae and deep intertrabecular recesses which are filled with blood from the ventricular cavity without evidence of communication to the epicardial coronary artery system. Frequent premature supra ventricular tachycardia as unique finding in LVNC cardiomyopathy is rare manifestation of this disease. We report a case of a frequent persistent supraventricular tachycardia as first manifestation of a patient with LVNC cardiomyopathy in a young healthy woman who despite radio frequency ablation therapy of the supraventricular tachycardia remains symptomatic. The patient was later placed on medical therapy based on a non-cardio selective beta-blocker with a good clinical outcome without recurrent of supra-ventricular arrythmias.

Research progress on genetic pathogenesis of non-compaction of the ventricular myocardium in children / 中国小儿急救医学

Non-compaction of the ventricular myocardium(NVM), also known as cavernous cardiomyopathy, is dominated by left ventricular non-compaction(LVNC)in most patients.The incidence of NVM in childhood cardiomyopathy is second only to dilated cardiomyopathy and hypertrophic cardiomyopathy, and the pathological changes are prominent trabeculae and deep intertrabecular clefts.Most pediatric patients have serious complications, such as heart failure, serious arrhythmias, and thrombus.Therefore, it is particularly important to study the pathogenesis of LVNC in children.NVM may be caused by the cessation of myocardial compaction in early embryonic development, so the genetic pathogenesis of NVM is particularly important.This review aimed to elucidate the genetic pathogenesis of LVNC in children, and provide more ideas for the pathogenesis and genetic therapy of LVNC in the future.

Research progress on genetic pathogenesis of non-compaction of the ventricular myocardium in children / 中国小儿急救医学

Non-compaction of the ventricular myocardium(NVM), also known as cavernous cardiomyopathy, is dominated by left ventricular non-compaction(LVNC)in most patients.The incidence of NVM in childhood cardiomyopathy is second only to dilated cardiomyopathy and hypertrophic cardiomyopathy, and the pathological changes are prominent trabeculae and deep intertrabecular clefts.Most pediatric patients have serious complications, such as heart failure, serious arrhythmias, and thrombus.Therefore, it is particularly important to study the pathogenesis of LVNC in children.NVM may be caused by the cessation of myocardial compaction in early embryonic development, so the genetic pathogenesis of NVM is particularly important.This review aimed to elucidate the genetic pathogenesis of LVNC in children, and provide more ideas for the pathogenesis and genetic therapy of LVNC in the future.

Non-compaction of ventricular myocardium in children / 中华实用儿科临床杂志

Non-compaction of ventricular myocardium (NVM),a rare congenital and inherited cardiomyopathy,is characterized by prominent trabeculations and deep intertrabecular recesses in communication with the ventricular cavity.NVM has diverse clinical presentations without specificity,of which the major characteristics are cardiac insufficiency,arrhythmias and thrombosis.Echocardiography and cardiac magnetic resonance imaging are necessary technologies to diagnose NVM.Symptomatic therapy is the only choice for most patients.Furthermore,if medical treatment fails,the heart transplantation could be performed.As a whole,NVM has a high mortality and entirely different prognosis,however,the prognosis of asymptomatic patients is relatively good.

Non-compaction of ventricular myocardium in children / 中华实用儿科临床杂志

Non-compaction of ventricular myocardium (NVM), a rare congenital and inherited cardiomyopathy, is characterized by prominent trabeculations and deep intertrabecular recesses in communication with the ventricular cavity.NVM has diverse clinical presentations without specificity, of which the major characteristics are cardiac insufficiency, arrhythmias and thrombosis.Echocardiography and cardiac magnetic resonance imaging are necessary technologies to diagnose NVM.Symptomatic therapy is the only choice for most patients.Furthermore, if medical treatment fails, the heart transplantation could be performed.As a whole, NVM has a high mortality and entirely different prognosis, however, the prognosis of asymptomatic patients is relatively good.

Miocardiopatía no compactada / Non-compacted cardiomyopathy / Cardiomiopatia não compactada

Resumen: La miocardiopatía no compactada es un trastorno caracterizado por una morfología anómala del miocardio, con una organización en dos capas diferenciadas: una capa fina epicárdica compactada y bien desarrollada, y otra capa endocárdica gruesa de aspecto esponjoso, con trabéculas y recesos intertrabeculares prominentes. En algunos casos se asocia a dilatación ventricular y disfunción sistólica, lo que conduce a insuficiencia cardíaca, arritmias cardíacas y complicaciones tromboembólicas. No está totalmente claro si se trata de una miocardiopatía definida como tal o si es un rasgo fenotípico compartido por muchas otras patologías subyacentes. Para su diagnóstico, la ecocardiografía representa la primera herramienta a emplear, utilizando varios criterios definidos hasta la fecha, aunque con escasa correlación entre ellos. La cardio-resonancia magnética (Cardio-RM), con criterios diagnósticos bien establecidos, trata de superar las limitaciones de la ecocardiografía. El tratamiento se basa en el manejo de las complicaciones según las guías de práctica general. Se han definido recomendaciones en cuanto al despistaje de la misma y el pronóstico es muy variable, si bien el conocimiento que tenemos de la patología hasta la fecha es aún muy limitado.

Summary: Non-compaction cardiomyopathy is a disorder characterized by an anomalous morphology of the myocardium, with an organization in two differentiated layers: a thin and well developed epicardial layer, and another thick endocardial layer of spongy appearance, with prominent trabeculae and intertrabecular recesses. In some cases, it is associated with ventricular dilatation and systolic dysfunction, which leads to heart failure, cardiac arrhythmias and thromboembolic complications. It is not entirely clear whether it is a cardiomyopathy per se or it is really a phenotypic trait shared by many other underlying pathologies. For its diagnosis, echocardiography represents the first tool to be used, with several diagnostic criteria defined to date, although they present little correlation. To help these limitations, cardiac magnetic resonance is also used, which has established criteria too. The treatment is based on the management of complications according to general practice guidelines. Recommendations have been defined regarding the screening of the pathology and the prognosis is very variable, even though the knowledge we have of the pathology to date is still very limited.

Resumo: A cardiomiopatia não compactada é um distúrbio caracterizado por morfologia anômala do miocárdio, com organização em duas camadas diferenciadas: camada fina epicárdica bem desenvolvida e compacta, e outra camada endocárdica espessa de aspecto esponjoso, com trabéculas e recessos intertrabeculares proeminentes. Em alguns casos está associada à dilatação ventricular e disfunção sistólica, o que leva à insuficiência cardíaca, arritmias cardíacas e complicações tromboembólicas. Não está completamente claro se é uma cardiomiopatia definida como tal ou se é um traço fenotípico compartilhado por muitas outras patologias subjacentes. Para o seu diagnóstico, a ecocardiografia representa a primeira ferramenta a ser utilizada, com vários critérios diagnósticos definidos até o momento, embora com pouca correlação entre eles. Ressonância Cardio-Magnética, com critérios diagnósticos bem estabelecidos, tenta superar as limitações da ecocardiografia. O tratamento baseia-se no manejo das complicações de acordo com as diretrizes da prática geral. Definimos recomendações quanto à triagem das mesmas e o prognóstico é muito variável, embora o conhecimento que temos da patologia até o momento ainda seja muito limitado.

Prevalence, Characteristics, and Clinical Significance of Concomitant Cardiomyopathies in Subjects with Bicuspid Aortic Valves

PURPOSE: The present study aimed to investigate the prevalence, characteristics, and clinical significance of concomitant specific cardiomyopathies in subjects with bicuspid aortic valves (BAVs). MATERIALS AND METHODS: A total of 1186 adults with BAV (850 males, mean age 56±14 years) at a single tertiary center were comprehensively reviewed. Left ventricular non-compaction, hypertrophic cardiomyopathy, and idiopathic dilated cardiomyopathy were confirmed when patients fulfilled current clinical and echocardiographic criteria. Clinical and echocardiographic characteristics, including comorbidities, heart failure presentation, BAV morphology, function, and aorta phenotypes, in BAV subjects with or without specific cardiomyopathies were compared. RESULTS: Overall, 67 subjects (5.6%) had concomitant cardiomyopathies: 40 (3.4%) patients with left ventricular non-compaction, 17 (1.4%) with hypertrophic cardiomyopathy, and 10 (0.8%) with dilated cardiomyopathy. BAV subjects with hypertrophic cardiomyopathy had higher prevalences of diabetes mellitus and heart failure with preserved ejection fraction, and tended to have type 0 phenotype, while BAV subjects with dilated cardiomyopathy showed higher prevalences of chronic kidney disease and heart failure with reduced ejection fraction. BAV subjects with left ventricular non-compaction were significantly younger and predominantly male, and had greater BAV dysfunction and a higher prevalence of normal aorta shape. In multiple regression analysis, cardiomyopathy was independently associated with heart failure (odds ratio 2.795, 95% confidential interval 1.603–4.873, p<0.001) after controlling for confounding factors. CONCLUSION: Concomitant cardiomyopathies were observed in 5.6% of subjects with BAV. A few different clinical and echocardiographic characteristics were found. The presence of cardiomyopathy was independently associated with heart failure.

Left Ventricular Non-Compaction in an adult with Patent Ductus Arteriosus

Introduction@#Left ventricular non-compaction (LVNC) is a rare form of cardiomyopathy that may occur in isolation or with an associated cardiac anomaly. It presents with a wide array of manifestations, prompting early recognition to be imperative to prevent progression of symptoms.@*Case presentation@#We report a case of a 46-year-old male complaining of palpitations for 10 years who survived sudden cardiac arrest on the same year as symptom onset. Consult was advised but was not done until he had heart failure symptoms. Carvedilol, furosemide and digoxin were given. Initially, some improvement was noted but he later developed dyspnea on exertion prompting consult at our institution. Pertinent physical examination findings include a dynamic precordium, apex beat at sixth left intercostal space-anterior axillary line (LICS AAL), right ventricular heave, distinct heart sounds, normal rate, irregularly irregular rhythm, a grade 4/6 continuous murmur heard best at the left upper sternal border, suggestive of patent ductus arteriosus (PDA), and a grade 3/6 holosystolic murmur at the apex radiating to the axilla, suggestive of mitral regurgitation. Transthoracic echocardiography confirmed presence of a PDA (0.8cm) with left to right shunt and Qp/Qs of 2.7:1. Incidental finding of LVNC was noted characterized by prominent ventricular trabeculations and deep intertrabecular recesses. Optimal medical treatment for heart failure was given with symptomatic relief. Surgical closure of the PDA was contemplated after hemodynamic studies can confirm the absence of irreversible pulmonary hypertension.@*Discussion@#Patients with LVNC may be asymptomatic or may present with heart failure, sudden cardiac death or arrhythmias. The diagnosis of LVNC poses a diagnostic challenge. Echocardiography is a cost-effective diagnostic tool that will allow early diagnosis. Cardiac magnetic resonance (CMR) imaging is an alternative diagnostic modality. Once the diagnosis has been confirmed, prompt initiation of guideline-directed medical treatment for heart failure may prevent progression of disease.@*Conclusion@#Left ventricular non-compaction may occur in isolation or in association with other congenital heart diseases such as patent ductus arteriosus. Closure of a PDA is indicated in the presence of a significant shunt and with confirmation of acute reversibility in the presence of pulmonary hypertension to prevent the possibility of decompensation in a patient with heart failure.

Terapia de resincronización cardíaca: un paciente con extrema disfunción ventricular y múltiples comorbilidades / Cardiac resynchronization therapy: a patient with extreme ventricular dysfunction and multiple comorbidities

Resumen Se presenta el caso de un varón de 56 años con cardiomiopatía no compactada y muy severa disfunción ventricular, con trastorno de conducción intraventricular y QRS ancho. Adolecía de comorbilidades incluyendo nefropatía diabética termi nal (en diálisis) y nunca pudo recibir terapia médica óptima por hipotensión e hiperkalemia. Con indicación IIa para terapia de resincronización cardíaca se le implantó resincronizador con desfibrilador, con respuesta aguda clínica y ecocardiográ fica excelente. Falleció a los tres meses del implante por asistolia que no pudo ser rescatada por el dispositivo. Es nuestra opinión que la presencia de comorbilidades graves y la imposibilidad de dar terapia médica óptima son una limitante para el éxito a mediano y largo plazo de la terapia de resincronización.

Abstract We present the case of a 56-year-old man with non-compaction cardiomyopathy and very severe ventricular dysfunction, with intraventricular conduction disorder and wide QRS. He suffered from comorbidities including terminal diabetic nephropathy (on dialysis) and was never able to receive optimal medical therapy for hypotension and hyperkalemia. With indication IIa for cardiac resynchronization therapy, a resynchronizer with a defibrillator was implanted, with an acute clinical and excellent echocardiographic response. He died three months after the implant due to asystole that could not be rescued by the device. It is our opinion that the presence of severe comorbidities and the impossibility of giving optimal medical therapy are a limitation for the medium and long-term success of the resynchronization therapy.

A Rare Case of Non-compaction Cardiomyopathy in an Indo-Caribbean Patient / Un raro caso de cardiomiopatía no compactada en un paciente indo-caribeño

ABSTRACT Non-compaction cardiomyopathy is a rare form of cardiac disease that presents with symptoms of congestive heart failure, ventricular arrhythmias or thromboembolism. Within the Caribbean setting, there are limited data regarding its existence. We present a case of non-compaction cardiomyopathy, diagnosed by transthoracic echocardiogram and cardiac magnetic resonance imaging, with the use of established criteria. It is interesting to highlight such a condition within the small Caribbean population, especially given its link to heritability and the ease with which it can be mistaken for a dilated cardiomyopathy.

RESUMEN La miocardiopatía no compactada o espongiforme es una forma rara de enfermedad cardíaca que se presenta con síntomas de insuficiencia cardíaca congestiva, arritmias ventriculares o tromboembolismos. En el entorno caribeño, hay datos limitados sobre su existencia. Presentamos un caso de cardiomiopatía no compactada, diagnosticada mediante ecocardiograma transtorácico y resonancia magnética cardíaca, con el uso de criterios establecidos. Es interesante destacar tal condición dentro de la pequeña población caribeña, especialmente dada su relación con la heredabilidad y la facilidad con la que se le puede confundir con una miocardiopatía dilatada.

A Rare Case of Left Ventricular Noncompaction in LEOPARD Syndrome

No abstract available.

Supplementary Diagnostic Landmarks of Left Ventricular Non-Compaction on Magnetic Resonance Imaging

PURPOSE: Diagnostic criteria for left ventricular non-compaction (LVNC) are still a matter of dispute. The aim of our present study was to test the diagnostic value of two novel diagnostic cardiac magnetic resonance (CMR) parameters: proof of non-compact (NC) myocardium blood flow using T2 sequences and changes in geometry of the left ventricle. MATERIALS AND METHODS: The study included cases with LVNC and controls, from a data base formed in a period of 3.5 years (n=1890 exams), in which CMR protocol included T2 sequences. Measurement of perpendicular maximal and minimal end diastolic dimensions in the region with NC myocardium from short axis plane was recorded, and calculated as a ratio (MaxMinEDDR), while flow through trabecula was proven by intracavital T2-weighted hyperintensity (ICT2HI). LVNC diagnosis met the following three criteria: thickening of compact (C) layer, NC:C>2.3:1 and NC>20%LV. RESULTS: The study included 200 patients; 71 with LVNC (35.5%; i.e., 3.76% of CMRs) and 129 (64.5%) controls. MaxMinEDDR in patients with LVNC was significantly different from that in controls (1.17±0.08 vs. 1.06±0.04, respectively; p 1.10 had sensitivity of 91.6% [95% confidence intervals (CI) 82.5–96.8], specificity of 85.3% (95% CI 78.0–90.0), and area under curve (AUC) 0.919 (95% CI 0.872–0.953; p < 0.001) for LVNC. Existence of ICT2HI had sensitivity of 100.0% (95% CI 94.9–100.0), specificity of 91.5% (95% CI 85.3–95.7), and AUC 0.957 (95% CI 0.919–0.981; p < 0.001) for LVNC. CONCLUSION: Two additional diagnostic parameters for LVNC were identified in this study. ICT2HI and geometric eccentricity of the ventricle both had relatively high sensitivity and specificity for diagnosing LVNC.

Cardiomyopathies with Mixed and Inapparent Morphological Features in Cardiac Troponin I3 Mutation

The fact that different types of cardiomyopathies can be manifested by the same sarcomere protein gene mutation in a single family is well known. However, mixed features of different types of cardiomyopathies in a single patient have not been well appreciated. We identified a novel mutation in cardiac troponin I3 (Arg186Gly) in the present case, and two of the family members showed mixed morphologic features of hypertrophic cardiomyopathy and left ventricular non-compaction. Moreover, both the features of cardiomyopathies were not apparent for each type of cardiomyopathy. In the patient's family, four other members had unexpected deaths before the age of 30.

Alcohol exposure during pregnancy causes non-compaction cardiomyopathy in offspring mice / 第三军医大学学报

Objective To investigate the relationship of alcohol exposure during pregnancy and non-compaction cardiomyopathy (NCC) in offspring mice.Methods Pregnant mice of ED3.5-ED18.5 were given 56％ alcohol by gavage at a dose of 5 mL/kg.The ED19.5 mice were sacrificed,and the heart of the fetal mice was harvested.Transmission electron microscopy (filaments,mitochondria and sarcoplasmic reticulum) and HE staining were used to verify the changes of structure and ultrastructure of the obtained myocardial tissues.Echocardiography was used to evaluate the cardiac function and ventricular myometrium of the offspring mice after growing up.Results Alcohol exposure during pregnancy caused the disorganized and dissolved myofilaments in the fetal mice.Some offspring mice (31.25％,5/16) had NCC.The ratio of non-compacted myocardium to compact myocardium at the end of systole (N/C) was 2.49 ± 0.6 in the offspring mice of the alcohol exposure group,significantly higher than that in the control offspring mice (0.62 ± 0.23,t =10.397,P =0.000).The volume of heart was decreased in the offspring mice of the alcohol exposure group while the left ventricule was enlarged.Echocardiography showed cardiac dysfunction and thickened ventricular septal/left ventricular posterior walls in the grown-up mice of the exposure group.Conclusion Large dose of alcohol exposure during pregnancy cause trabeculations and non-compaction in ventricular myocardium,and it might be one of causers for NCC in the offspring.

MRI characteristics of primary dilated cardiomyopathy and isolated left ventricular non-compaction / 中国医学影像技术

Objective To explore the MRI characterisitics of primary dilated cardiomyopathy and isolated left ventricular non-compaction.Methods The patients who were diagnosed as primary dilated cardiomyopathy (n=18) and isolated left ventricular non-compaction (n=10) were enrolled,and the MRI was performed.The thickness of non-compaction myocardium (NC),compaction myocardium (C) in end-diastole,the feature of movement of myocardium,the number of non-compaction segment,the fraction shortening of non-compaction and the distribution were compared.Results Totally 823 segments were analyzed in primary dilated cardiomyopathy,in which 124 segments were non-compaction myocardium;397 segments were analyzed in isolated left ventricular non-compaction,in which 115 segments were non-compaction myocardium.The NC,NC/C,NC/(NC+C),and the fraction shortening of the isolated left ventricular non-compaction patients were higher than those of primary dilated cardiomyopathy patients (all P＜0.05).The features of distribution showed that the apical segment was mostly involved,and the basal segment was less involved or hardly involved.The anterior and lateral segments were more involved in the free wall,the septal was less involved.Conclusion The MRI characteristics of primary dilated cardiomyopathy and isolated left ventricular non-compaction are different,especially in the distribution,non compacted segments,NC and NC/C,which is important for diagnosis and differential diagnosis of the two diseases.

Clinical manifestations of non-compaction of ventricular myocardium in children / 中华实用儿科临床杂志

Objective To study the clinical characteristics and curing methods of non-compaction of ventricular myocardium(NVM) in children with a view to improve its diagnosis and treatment.Methods From Jan.2009 to Dec.2012,the clinical data,treatment and follow-up of 22 patients with NVM in Beijing Children's Hospital Affiliated to Capital Medical University were analyzed.Results The 22 patients included 13 boys and 9 girls aged from 4 months to 13 years and 3 months with an average age of 3 years and 7 months.Five of them had a family history of cardiac diease.Twenty patients suffered from ventricular dysfunction,with 2 cases at level Ⅰ,5 cases at level Ⅱ,7 cases at level Ⅲ and 6 cases at level Ⅳ.Asymptomatic heart murmur was heard in 6 patients and creatine kinase car-diac isoenzymes raise was found in 6 patients.X-ray indicated that heart shadow enlargement and electrocardiographic abnormalities in all 22 patients.Echocardiography features of all cases indicated left ventricular enlargement,and 20 cases of the children had ventricular systolic dysfunction.Left ventricle was detected in 21 patients,while double ventricle dysfunction detected in 1 patient.Thrombus was found in none of the patients.Magnetic resonance imaging was performed in 8 cases of the patients and CT was performed in 14 cases,all of them showed abnormalities.Anti-heart failure medications were administered to 20 patients and aspirin to all of the children.The patients were followed up for 0.5 to 3.0years.Two patients died from heart failure during the follow-up.In 2 cases whose cardiac ejection fraction was more than 60％ had little change of the internal diameter of left ventricle and cardiac function.In 3 cases,the left ventricular diameter narrowed by 3 to 5 millimeter and ejection fraction increased by 5％ to 10％ over the previous.In 5 cases,the left ventricular diameter expanded by 5 to 7 millimeter and ejection fraction decreased by 3％ to 5％.In 10 cases,the left ventricular diameter and heart function had no significant change.Conclusions The main clinical manifestions of NVM in children are cardiac dysfunction,and the prognosis is usually poor.Echocardiography is the principal means of diagnosis.Magnetic resonance imaging can contribute to the diagnosis.

Left ventricle non-compaction cardiomyopathy: Different clinical scenarios and magnetic resonance imaging findings / Miocardiopatía no compactada: diferentes escenarios clínicos y hallazgos en cardiorresonancia magnética

Left ventricle non-compaction cardiomyopathy is currently considered as a well-defined individual entity. However, it includes a broad spectrum of clinical, radiological and pathophysiological findings. In this review we describe 3 different scenarios of this entity: an isolated case with severe left ventricle dysfunction, an "associated" case in a patient with previous atrial septum defect and pulmonary stenosis and finally, as a finding in a patient with a transient cerebrovascular ischemic attack. In the 2 last cases, both asymptomatic, morphological criteria of left ventricle non-compaction were found but, ventricular function was normal and cardiac-MRI showed no late gadolinium hyperenhancement. Periodical follow-up and familial screening were recommended. Natural history and prognosis factors of this disease are still not well known. Further and longer series of patients with this diagnosis are needed to completely define radiological criteria, clinical presentation and evolution.

La miocardiopatía no compactada está considerada actualmente como una entidad independiente y bien definida. Sin embargo, presenta un espectro amplio de hallazgos clínicos, radiológicos y fisiopatológicos. En la presente revisión describimos 3 escenarios clínicos diferentes de dicha entidad: un caso con disfunción ventricular severa, un caso como entidad «asociada¼ a una cardiopatía congènita en un pacientes con un defecto del septo interauricular previo y estenosis pulmonar, y finalmente, como un hallazgo casual en un paciente con un accidente cerebrovascular transitorio. En estos 2 últimos casos se encontraron criterios morfológicos de miocardiopatía no compactada con función ventricular normal y sin presencia de realce tardío de gadolinio en el estudio de cardio-RM. En todos ellos se recomendó estudio familiar. La historia natural y el pronóstico de esta anatomía patológica no son todavía del todo conocidos. Series mayores y seguimiento más largos son necesarios para definir completamente los criterios radiológicos, la presentación clínica y la evolución de esta fascinante entidad.

Miocárdio não-compactado como diagnóstico diferencial de cardiomiopatia periparto: A propósito de um caso

A cardiomiopatia periparto (CMPP) é uma das causas comuns de cardiomiopatia secundária de etiologia desconhecida. Caracteriza-se pela presença de insuficiência cardíaca congestiva (ICC) materna, no último mês de gestação ou até cinco meses pós-parto, com disfunção ventricular sistólica esquerda, na ausência de outras causas de insuficiência cardíaca e em mulheres previamente saudáveis. A terapêutica médica consiste no bloqueio neuro-hormonal, suporte inotrópico, redução da pré e pós-carga cardíaca e anticoagulação. O transplante cardíaco está reservado aos casos graves e refratários à terapêutica medicamentosa. O prognóstico é variável: aproximadamente 50-60% das pacientes recuperam completamente a função cardíaca, na maioria das vezes dentro dos primeiros seis meses. Relata-se um caso de mulher negra, 37 anos, multípara, pré-natal sem intercorrências, sem comorbidades prévias ou uso de drogas, que desenvolveu um quadro de ICC, de instalação súbita, com disfunção ventricular comprovada ao ecocardiograma 15 dias após parto normal. Houve suspeição clínica de miocárdio não-compactado (MNC) que levou a dúvida quanto ao diagnóstico de CMPP, posto ser esta um diagnóstico de exclusão. A presença de MNC foi afastada pela ressonância nuclear magnética cardíaca.

La miocardiopatía periparto (MCPP) es una causa frecuente de miocardiopatía secundaria de etiología desconocida. Se caracteriza por la presencia de insuficiencia cardíaca congestiva (ICC) en la madre en el último mes del embarazo o hasta cinco meses después del parto, con disfunción sistólica del ventrículo izquierdo en ausencia de otras causas de insuficiencia cardíaca en mujeres previamente sanas. El tratamiento médico consiste en el bloqueo neurohormonal, soporte inotrópico, la reducción de la pre y post-carga cardíaca y anticoagulación. El trasplante cardíaco está reservado para casos graves y refractarios al tratamiento farmacológico. El pronóstico es variable: aproximadamente el 50-60% de las pacientes recuperan completamente la función cardíaca, en la mayoría de los casos en los primeros seis meses. Se presenta el caso clínico de una mujer de raza negra de 37 años, multípara, prenatal sin complicaciones, sin comorbilidades previas o uso de drogas, que desarrollo ICC, de aparición súbita, con disfunción ventricular demostrada en el ecocardiograma 15 días después de un parto normal. No había sospecha clínica de miocardiopatía no compactada (MNC) que dio lugar a duda sobre el diagnóstico de MCPP, ya que éste es un diagnóstico de exclusión. La presencia de MNC fue descartada por resonancia magnética nuclear cardíaca.

Peripartum cardiomyopathy (PPCM) is a common cause of secondary cardiomyopathy of unknown etiology. It is characterized by the presence of congestive heart failure (CHF) in the mother in the last month of pregnancy or until five months after birth with left ventricular systolic dysfunction in the absence of other causes of heart failure in previously healthy women. Medical treatment consists of neurohormonal blockade, inotropic support, reduced pre-and post-cardiac load and anticoagulation. Heart transplantation is reserved for severe cases refractory to medical therapy. The prognosis is variable: approximately 50-60% of patients recover full cardiac function, in most cases in the first six months. We report the case of a black woman of 37 years, multiparous, prenatal without complications, comorbidities or previous drug use that developed CHF, sudden onset, demonstrated ventricular dysfunction on echocardiography 15 days after childbirth normal. There was no clinical suspicion of non-compaction cardiomyopathy (NCC) which gave rise to doubt about the diagnosis of PPCM, since this is a diagnosis of exclusion. The presence of NCC was ruled out by cardiac magnetic resonance imaging.

Evaluación de la miocardiopatía no compactada con resonancia magnética cardíaca en pacientes con función sistólica del ventrículo izquierdo conservada y disminuida / Evaluation of non-compaction cardiomyopathy with cardiac magnetic resonance imaging in patients with preserved and decreased left ventricular systolic function

Objective: Non-compaction cardiomyopathy (NCC) is a genetic disorder characterized by the presence of an extensive trabecular layer of myocardium with intertrabecular recesses in communication with the ventricular cavity. The objective of this study is to evaluate the clinical and morphological characteristics of patients with NCC with and without systolic dysfunction assessed by cardiac magnetic resonance (CMR) imaging, as well as to discuss the value and limitations of echocardiography and CMR imaging in its diagnosis. Twenty patients diagnosed with NCC were retrospectively included. Studies were performed on a 1.5 Tesla resonator. The end-diastolic volume (LVEDV) and end-systolic volumen (LVESV), end-diastolic diameter (LVEDD) and end-systolic diameter (LVESD), left ventricular ejection fraction (LVEF), cardiac mass and LV trabeculations were determined. A 17-segment myocardium segmentation model was applied. Student s T test was used to compare variables between groups. Significance level was set at p <0.05. Correlation between 2 continuous variables was calculated using the linear regression model. The statistical software package used for data analysis was StatsDirect (Version 2.6.5,. Altrincham, UK. Results: The average thickness of NC myocardial segments and normal myocardium was 13.1 +/- 3.3 mm and 3.6 +/- 0.6 mm, respectively. The average number of myocardial segments with NC was 8.2 +/- 1.2. The more affected segments were the apex of the heart and the lateral segments at the apical and mid-ventricular level. LVEDD, LVEDV, global LV mass, compacted and trabeculated, were significantly increased in the group of patients with ventricular dysfunction. LVEF had a negative linear correlation with trabecular myocardal mass (MM)/ m2 (R = 0.67, p = 0.001), LVEDV /m2 (R = 0.77, p <0.001) and LVEDD/m2 (R = 0.7, p <0.001). Similarly, a negative linear correlation between LVTM/m² and LVEDV/m² (R = 0.76, p < 0.001) was observed. Conclusion: In our population...

Objetivos: La miocardiopatía no compactada (MNC) es un desorden genético caracterizado por la presencia de una extensa capa de miocardio trabeculado con recesos intertrabeculares comunicados con la cavidad ventricular. El objetivo del trabajo es evaluar las características clínicas y morfológicas de los pacientes con MNC con y sin disfunción sistólica evaluados por resonancia magnética cardíaca (RMC), y poner en discusión los alcances y las limitaciones de la ecocardiografía y la RMC en su diagnóstico. Material y métodos: Se incluyeron en forma retrospectiva 20 pacientes con diagnóstico de MNC. Los estudios se realizaron en un resonador de 1.5 Tesla. Se determinaron: volumen de fin de diástole (VFDVI) y sístole, diámetro de fin de diástole (DFDVI) y sístole, fracción de eyección (FEVI), masa cardíaca y trabeculaciones del VI. La distribución del miocardio NC se llevó a cabo con el modelo de 17 segmentos miocárdicos. Se empleó el test de Student para comparar las variables entre ambos grupos. El nivel de significancia fue establecido en p < 0,05. La correlación entre 2 variables continuas se calculó usando el modelo de regresión lineal. Los análisis se realizaron utilizando el software de estadística StatsDirect (versión 2.6.5, Altrincham, UK). Resultados: El espesor medio del miocardio NC y el miocardio normal fue 13.1 +/- 3.3 mm y 3.6 +/- 0.6 mm respectivamente. El número medio de segmentos con miocardio NC fue 8.2 +/- 1.2, siendo los más afectados el ápex y los segmentos laterales a nivel apical y medioventricular. El DFDVI, el VFDVI, la masa global, compactada y trabeculada del VI estuvieron incrementados en forma significativa en el grupo de pacientes con disfunción ventricular. La FEVI tuvo una correlación lineal negativa con masa de miocardio trabeculado del VI (MTVI)/m2 (R = 0.67, p = 0.001), el VFDVI/m2 (R = 0.77, p < 0.001) y el DFDVI/m2 (R = 0.7, p < 0.001). Asimismo, hubo una correlación lineal negativa entre la MTVI/m2 y el VFDVI/m2...